Гиперкинезы

Гиперкинезы
(греч. hyper- + kinēsis движение)
непроизвольные движения, вызванные сокращением мышц лица, туловища, конечностей, реже гортани, мягкого неба, языка, наружных мышц глаз. Развиваются при инфекционных поражениях ц.н.с. (эпидемический энцефалит, клещевой энцефалит и др.), сосудистых, демиелинизирующих заболеваниях головного мозга, черепно-мозговой травме, опухолях мозга, интоксикациях. Ряд Г. является признаком наследственных дегенеративных заболеваний нервной системы, передающихся по аутосомно-доминантному или аутосомно-рецессивному типу.
Гиперкинез является следствием поражения экстрапирамидной системы (Экстрапирамидная система), а также мозжечка, таламуса, коры больших полушарий головного мозга и их сложных связей. В механизме развития Г. ведущую роль играют нарушения метаболизма, прежде всего дофамина, норадреналина, гамма-аминомасляной кислоты, глутаминовой кислоты, серотонина, ацетилхолина, вещества Р и мет-энкефалина. Установлено также определенное значение изменений церебральных дофаминовых рецепторов.
Классификация Г. основывается на локализации патологического процесса в головном мозге и клинических особенностях. Выделяют Г. преимущественно стволового уровня (паркинсоническое дрожание, статическое дрожание, симптоматическая миоклония, миоритмии, спастическая кривошея), преимущественно подкоркового происхождения (хореический Г., атетозные Г., гемибаллизм, торсионная дистония), Г. в сочетании с эпилепсией преимущественно подкорково-коркового происхождения (миоклонус-эпилепсия, миоклоническая асинергия Ханта) и корковые Г. (джексоновская и кожевниковская эпилепсия, гемитония). Дифференциально-диагностическая характеристика основных гиперкинезов приведена в таблице.
Таблица
Дифференциально-диагностическая характеристика основных гиперкинезов
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| Название                   | Этиология                  | Локализация        | Нейрохимические            | Возраст            | Течение                               | Топография           | Характеристика     | Ритмичность  | Стабильность          | Другие особенности | Тонус мышц      |
| гиперкинеза               |                                   | патологического   | нарушения                      | начала             |                                            | гиперкинеза           | движений               | гиперкинеза    | гиперкинеза            |                                 |                         |
|                                  |                                   | процесса             |                                        | заболевания     |                                            |                              |                              |                       |                                |                                 |                         |
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| 1                               | 2                                | 3                          | 4                                     | 5                      | 6                                         | 7                           | 8                            | 9                    | 10                           | 11                            | 12                     |
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| Атетоз двойной         | Наследование по        | Хвостатое ядро,  | Не установлены              | Первые годы    | Прогрессирующее в            | Преобладает в      | Медленные            | Неритмичен    | Наблюдается в       | Отсутствуют            | Мышечная        |
|                                  | аутосомно-                 | скорлупа (утрата  |                                        | жизни               | течение 5—10 лет, затем     | мышцах кистей,     | червеобразные,     |                       | покое, исчезает во  |                                 | дистония          |
|                                  | рецессивному типу,    | нейронов с           |                                        |                         | стабильное                          | лица, шеи,             | вычурные, с           |                       | сне, усиливаясь      |                                 |                         |
|                                  | возможно,                  | образованием      |                                        |                         |                                            | туловища              | тоническим            |                       | при волнении, во     |                                 |                         |
|                                  | энцефалит,                | глиальных            |                                        |                         |                                            | (симметрично).      | напряжением         |                       | время                     |                                 |                         |
|                                  | асфиксия, родовая     | рубцов — status   |                                        |                         |                                            | Участвуют             | пальцев, их            |                       | произвольных         |                                 |                         |
|                                  | травма, желтуха         | mamioratus),        |                                        |                         |                                            | мышцы-                 | застыванием в       |                       | движений                |                                 |                         |
|                                  | новорожденных          | черная                 |                                        |                         |                                            | антагонисты          | неестественном     |                       |                                |                                 |                         |
|                                  |                                   | субстанция,         |                                        |                         |                                            |                              | положении, чаще   |                       |                                |                                 |                         |
|                                  |                                   | таламус               |                                        |                         |                                            |                              | в состоянии           |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | переразгибания      |                       |                                |                                 |                         |
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| Атетоз                       | Сосудистые,              | Хвостатое ядро,  | Не установлены              | В любом           | Зависит от течения              | Преобладает в      | Медленные            | Неритмичен    | Наблюдается в       | Нередко сочетается | Мышечная        |
| симптоматический     | опухолевые,               | скорлупа, черная |                                        | возрасте          | основного заболевания        | мышцах рук           | червеобразные,     |                       | покое, исчезает во  | с гемипарезом,        | дистония          |
|                                  | инфекционные и         | субстанция,         |                                        |                         |                                            | (особенно кисти),   | вычурные, с           |                       | сне, усиливается    | пирамидными           |                         |
|                                  | другие заболевания;   | таламус               |                                        |                         |                                            | реже                     | тоническим            |                       | при волнении, во     | симптомами,            |                         |
|                                  | травмы головного       |                            |                                        |                         |                                            | наблюдается в      | напряжением         |                       | время                     | чувствительными     |                         |
|                                  | мозга                         |                            |                                        |                         |                                            | ногах и мышцах     | пальцев, их            |                       | произвольных         | нарушениями на       |                         |
|                                  |                                   |                            |                                        |                         |                                            | лица. Иногда         | застыванием в       |                       | движений                | противоположной     |                         |
|                                  |                                   |                            |                                        |                         |                                            | распространяется  | неестественных    |                       |                                | очагу стороне          |                         |
|                                  |                                   |                            |                                        |                         |                                            | по гемитипу;          | позах, обычно в     |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | участвуют             | состоянии              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 | переразгибания      |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | антагонисты          |                              |                       |                                |                                 |                         |
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| Гемибаллизм             | Сосудистые               | Субталамическое | Не установлены              | Средний,          | Развивается остро или        | Наблюдается в      | Быстрые                | Неритмичен    | Наблюдается в       | Отсутствуют            | Мышечная        |
|                                  | заболевания (в           | ядро Льюиса и     |                                        | пожилой           | подостро                             | мышцах половины | вращательные,      |                       | покое и при             |                                 | дистония          |
|                                  | большинстве              | его связи с          |                                        |                         |                                            | тела,                     | бросковые             |                       | движениях,             |                                 |                         |
|                                  | случаев) и опухоли     | бледным шаром   |                                        |                         |                                            | преимущественно  | движения большой |                      | усиливается при     |                                 |                         |
|                                  | головного мозга          |                            |                                        |                         |                                            | проксимальных     | амплитуды и          |                       | эмоциональных       |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | отделов рук и ног  | силы, возможны     |                       | нагрузках                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | травмы при ударе  |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | о препятствие или  |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | разрыв мышцы       |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | при резком             |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | движении               |                       |                                |                                 |                         |
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| Гемитония                 | Сосудистые               | Кора больших      | Не установлены              | Средний и        | Появляется через                | Мышцы                 | Тонические            | Неритмичен    | Наблюдается в       | Отсутствуют            | Спастический   |
| Бехтерева                 | поражения головного  | полушарий,          |                                        | пожилой           | несколько дней или             | конечностей, лица | судороги в             |                       | покое, ослабевает   |                                 | тонус,               |
|                                  | мозга                         | пирамидный путь |                                        | возраст            | месяцев после развития      | (по гемитипу);        | паретичных            |                       | при отвлечении       |                                 | дистония          |
|                                  |                                   |                            |                                        |                         | гемипареза, течение            | участвуют             | конечностях           |                       | внимания, легком    |                                 |                         |
|                                  |                                   |                            |                                        |                         | прогрессирующее или         | мышцы-                 |                              |                       | массаже                 |                                 |                         |
|                                  |                                   |                            |                                        |                         | стабильное                          | синергисты            |                              |                       | конечностей,           |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              |                              |                       | усиливается при     |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              |                              |                       | эмоциональном       |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              |                              |                       | напряжении,            |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              |                              |                       | активных                 |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              |                              |                       | движениях              |                                 |                         |
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| Дрожание                  | Демиелинизирующие, | Зубчатое ядро и  | Не установлены              | В любом           | Зависит от течения              | Конечности (в        | Толчкообразные     | Неритмичен    | Появляется только  | Зависят от               | Низкий,             |
| интенционное            | дегенеративные,        | верхние ножки     |                                        | возрасте          | основного заболевания        | большей степени   | движения,              |                       | при движении,         | основного                | дистония или    |
|                                  | сосудистые,               | мозжечка             |                                        |                         |                                            | дистальные их      | амплитуда             |                       | преимущественно   | заболевания             | повышение по   |
|                                  | воспалительные         |                            |                                        |                         |                                            | отделы), голова,    | которых                 |                       | в конце движения    |                                 | спастическому  |
|                                  | заболевания               |                            |                                        |                         |                                            | туловище,             | возрастает по        |                       |                                |                                 | типу                 |
|                                  | головного мозга          |                            |                                        |                         |                                            | участвуют             | мере приближения |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 | к цели данного       |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | антагонисты          | произвольного       |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | действия               |                       |                                |                                 |                         |
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| Дрожание                  | Возможны                  | Черная                | Нарушение равновесия   | В любом           | Медленно                            | Преобладает         | Простые                | 4—6 (3—7)     | Наблюдается в       | Сочетание                | Повышен по      |
| паркинсоническое     | наследственные         | субстанция          | в системах дофамин —  | возрасте,          | прогрессирующее                | тремор                  | ритмичные             | колебаний в    | покое, сохраняется | дрожания с              | пластическому  |
|                                  | причины (болезнь       | (поражение          | ацетилхолин (дефицит    | чаще в             |                                            | дистальных           | колебательные       | 1 с, частота    | при движениях,       | гипобрадикинезией   | типу (феномен  |
|                                  | Паркинсона), связь с  | содержащих        | дофамина) и серотонин   | среднем и        |                                            | отделов                 | движения или        | тремора         | исчезает во сне      | и ригидностью         | «зубчатого        |
|                                  | инфекционными         | меланин               | — гистамин (снижение    | пожилом           |                                            | конечностей,         | более сложные      | изменяется    |                                | мышц: флексорная   | колеса»)           |
|                                  | (энцефалит),              | нейронов             | уровня серотонина)         |                         |                                            | может отмечаться | («счет монет»,       | мало              |                                | поза туловища,        |                         |
|                                  | сосудистыми,             | компактной          |                                        |                         |                                            | тремор головы,      | «скатывание          |                       |                                | маскообразное лицо |                         |
|                                  | токсическими,            | зоны), голубое     |                                        |                         |                                            | нижней челюсти,   | пилюль»);              |                       |                                |                                 |                         |
|                                  | травматическими       | пятно, бледный    |                                        |                         |                                            | языка, участвуют  | амплитуда             |                       |                                |                                 |                         |
|                                  | поражениями              | шар,                    |                                        |                         |                                            | мышцы-                 | мелкая, средняя    |                       |                                |                                 |                         |
|                                  | головного мозга,         | ретикулярная       |                                        |                         |                                            | антагонисты          | или крупная,          |                       |                                |                                 |                         |
|                                  | выделяют также         | формация            |                                        |                         |                                            |                              | может изменяться  |                      |                                |                                 |                         |
|                                  | лекарственный           |                            |                                        |                         |                                            |                              | под влиянием        |                       |                                |                                 |                         |
|                                  | паркинсонизм             |                            |                                        |                         |                                            |                              | экзо- и                   |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | эндогенных            |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | факторов               |                       |                                |                                 |                         |
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| Дрожание                  | Наследование по        | Базальные           | Снижение                        | Чаще в             | Медленно                            | Тремор                  | Качательные          | 4—12             | В начале                 | Других изменений в  | Не изменен      |
| эссенциальное          | аутосомно-                 | ганглии,               | катехоламинергической,  | возрасте 20—   | прогрессирующее,               | преобладает в       | движения головы   | колебаний в    | заболевания            | неврологическом      | или мышечная  |
| (наследствен-            | доминантному типу с  | мозжечок,           | главным образом            | 30 лет              | возможны длительные         | верхней половине  | («да-да», «нет-       | 1 с, частота    | наблюдается           | статусе не               | дистония          |
| ное, семейное,          | различной                  | ретикулярная       | норадреналинергической |                         | ремиссии                             | туловища,             | нет»), сгибание —  | непостоянна,  | только при              | наблюдается            |                         |
| идиопатическое)        | экспрессивностью      | формация            | активности                      |                         |                                            | вовлекаются         | разгибание кистей  | с возрастом   | движении, затем и  |                                 |                         |
|                                  | мутантного гена         |                            |                                        |                         |                                            | голова, руки,         | рук, пальцев          | частота          | в покое,                  |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | почти не                |                              | уменьшается, | уменьшается во      |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | наблюдается в      |                              | амплитуда      | сне, при приеме      |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | ногах; участвуют   |                              | возрастает     | алкоголя, β-             |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 |                              |                       | блокаторов              |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | антагонисты          |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Интенционная            | Не уточнена               | Моторные зоны    | Не установлены              | Детский,           | Прогрессирующее или         | Возникает             | Тонические,           | Неритмичен    | Приступ может        | Потери сознания не  | Не изменен       |
| судорога Рюльфа     |                                   | коры больших      |                                        | молодой           | стабильное                          | внезапно,              | тонико-                  |                       | прекратиться при    | происходит, после   |                         |
|                                  |                                   | полушарий           |                                        |                         |                                            | приступообраэно   | клонические           |                       | болевом или            | приступа нет            |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            | в любой мышце     | судороги, могут     |                       | другом                    | парезов                    |                         |
|                                  |                                   |                            |                                        |                         |                                            | конечности или      | перейти в общий    |                       | раздражении           | конечностей             |                         |
|                                  |                                   |                            |                                        |                         |                                            | лица, особенно      | судорожный           |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | при неожиданном  | припадок,              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | движении,             | появляется после  |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | генерализуется,    | внезапных,            |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | распространяясь   | требующих            |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | на всю                  | быстрого темпа и   |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | одноименную        | автоматизма          |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | половину тела       | движений               |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Кривошея                  | Инфекционные,          | Ножки мозга,       | Не установлены              | Чаще в             | Прогрессирующее до          | Спазм                   | Стереотипные        | Неритмичен    | Наблюдается в       | Фиксация поворота  | Повышен в        |
| спастическая            | сосудистые                | покрышка             |                                        | возрасте 20—   | стойкой фиксации головы,   | локализован в       | тонические или      |                       | покое, усиливается | или наклона головы, | вовлеченных     |
|                                  | заболевания,              | ствола,                |                                        | 40 лет              | затем стабильное,               | грудино-                | тонико-                  |                       | при движении          | изменения статики   | мышцах            |
|                                  | интоксикации,             | ретикулярная       |                                        |                         | отдельных случаях              | ключично-             | клонические           |                       | (ходьбе)                  | (перераспределение |                         |
|                                  | травмы. Как               | формация            |                                        |                         | переходит в торсионную      | сосцевидной,        | судороги с             |                       |                                | тонуса мышц            |                         |
|                                  | рефлекторный            |                            |                                        |                         | дистонию                            | трапециевидной    | вращением или      |                       |                                | туловища); на          |                         |
|                                  | симптом                     |                            |                                        |                         |                                            | мышцах, задних    | наклоном головы в |                       |                                | ранних стадиях        |                         |
|                                  | наблюдается также    |                            |                                        |                         |                                            | мышцах шеи;         | сторону                 |                       |                                | прикосновение руки  |                         |
|                                  | при остеохондрозе,    |                            |                                        |                         |                                            | участвуют             |                              |                       |                                | к подбородку или     |                         |
|                                  | шейном радикулите,   |                            |                                        |                         |                                            | мышцы-                 |                              |                       |                                | щеке на стороне      |                         |
|                                  | миозите шейных         |                            |                                        |                         |                                            | синергисты; чаще  |                              |                       |                                | гиперкинеза             |                         |
|                                  | мышц и др.                 |                            |                                        |                         |                                            | односторонняя      |                              |                       |                                | уменьшает его         |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Лицевой гемиспазм   | Воспалительное или   | Лицевой нерв       | Не установлены              | Средний,          | Прогрессирующее, затем    | В начале               | Стереотипные        | Неритмичен    | В спокойной            | Отсутствуют            | Не изменен       |
|                                  | компрессионное         | (ядро, корешок),   |                                        | пожилой           | стабильное, возможно         | заболевания          | движения               |                       | обстановке              |                                 |                         |
|                                  | поражение лицевого   | базальные           |                                        |                         | постепенное развитие          | легкие спазмы       | преимущественно  |                       | гемиспазм               |                                 |                         |
|                                  | нерва                         | ганглии (?)           |                                        |                         | двустороннего гемиспазма  | мышц век,             | тонического, реже  |                       | развивается            |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | круговой мышцы    | клонического,        |                       | несколько раз в       |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | глаза, затем          | тикозного               |                       | день, приступы       |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | спазм                    | характера              |                       | провоцируются       |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | распространяется  |                              |                       | движениями            |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | на всю половину   |                              |                       | мимическихмышц,   |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | лица, вовлекается |                               |                       | эмоциями,               |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | только                   |                              |                       | продолжительность |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мимическая           |                              |                       | 2—3 мин                 |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мускулатура          |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Лицевой параспазм   | Сосудистые,              | Базальные           | Не установлены              | В любом           | Прогрессирующее или         | Симметрично в      | Тонические или      | Неритмичен    | Наблюдается в       | Отсутствуют            | Не изменен       |
|                                  | инфекционные            | ганглии,               |                                        | возрасте          | стабильное                          | обеих половинах   | тонико-                  |                       | покое,                     |                                 |                         |
|                                  | заболевания, травмы  | мозговой ствол    |                                        |                         |                                            | лица, вначале по   | клонические           |                       | провоцируется        |                                 |                         |
|                                  | головного мозга          |                            |                                        |                         |                                            | типу                      | судороги с             |                       | эмоциональным      |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | блефароспазма,    | преобладанием      |                       | напряжением,         |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | затем                    | тонического           |                       | исчезает во сне      |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | вовлекается          | компонента            |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мимическая           |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мускулатура,         |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | иногда мышцы       |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | глотки, языка,        |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | плечевого пояса    |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Миоклоническая        | Наследование по        | Красное ядро,      | Не установлены              | Средний,          | Медленно                            | Миоклонические    | Распространенный | Неритмичен    | Наблюдается в       | Возможны                | Снижен или      |
| асинергия Ханта        | аутосомно-                 | зубчатое ядро      |                                        | пожилой           | прогрессирующее                | судороги               | миоклонический     |                       | покое и при             | кифосколиоз,           | мышечная        |
|                                  | рецессивному типу     | мозжечка,            |                                        |                         |                                            | различных мышц;  | гиперкинез,            |                       | движениях,             | снижение                 | дистония          |
|                                  |                                   | нижняя олива,      |                                        |                         |                                            | мышц-синергистов | атаксия,                 |                       | усиливается при     | интеллекта,              |                         |
|                                  |                                   | кора мозжечка,    |                                        |                         |                                            | и мышц-                | мозжечковая          |                       | эмоциональных       | гипотрофия мышц    |                         |
|                                  |                                   | ретикулярная       |                                        |                         |                                            | антагонистов         | асинергия, общие  |                       | нагрузках                |                                 |                         |
|                                  |                                   | формация, кора   |                                        |                         |                                            |                              | эпилептические     |                       |                                |                                 |                         |
|                                  |                                   | больших              |                                        |                         |                                            |                              | припадки               |                       |                                |                                 |                         |
|                                  |                                   | полушарий           |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Миоклонии                | Инфекционные,          | Красное ядро,      | Не установлены              | В любом           | Зависит от течения              | Отдельные            | Быстрые, резкие,   | Неритмичен    | Наблюдается в       | Зависят от               | Не изменен       |
| симптоматические     | сосудистые                | зубчатое ядро      |                                        | возрасте          | основного заболевания        | группы мышц или  | кратковременные   |                       | покое, несколько     | основного                | или мышечная  |
|                                  | заболевания, травмы  | мозжечка,            |                                        |                         |                                            | мышцы-                 | сокращения           |                       | усиливается при     | заболевания             | дистония          |
|                                  | и опухоли головного   | нижняя олива       |                                        |                         |                                            | синергисты            | мышц, обычно        |                       | движениях,             |                                 |                         |
|                                  | мозга, уремия            |                            |                                        |                         |                                            |                              | отмечается            |                       | исчезает во сне      |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | вздрагивание;        |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | двигательный        |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | эффект                 |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | наблюдается при   |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | вовлечении мышц- |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | синергистов           |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Миоклонус-               | Наследование по        | Красное ядро,      | Не установлены              | Чаще в             | Прогрессирующее, имеет 3  | Мышцы-                | Миоклонический    | Неритмичен    | Наблюдается в       | Снижение                 | Дистония; в      |
| эпилепсия                 | аутосомно-                 | зубчатое ядро      |                                        | возрасте 10—   | стадии: первая —                | синергисты и         | гиперкинез в          |                       | покое, усиливается | интеллекта, в           | третьей стадии |
| наследственная        | рецессивному типу     | мозжечка,            |                                        | 20 лет              | эпилептическая; вторая —   | мышцы-                 | сочетании с           |                       | при движениях,       | третьей стадии —    | —                     |
| Унферрихта —          |                                   | нижняя олива,      |                                        |                         | эпилептикомиоклоническая; | антагонисты          | общими                 |                       | переохлаждении,    | кахексия                  | нарастающая   |
| Лундборга                 |                                   | ретикулярная       |                                        |                         | третья — терминальная;      |                              | эпилептическими   |                       | эмоциях, исчезает  |                                 | ригидность       |
|                                  |                                   | формация,           |                                        |                         | длительность болезни —     |                              | припадками           |                       | во сне; характерна  |                                 | мышц               |
|                                  |                                   | черная                 |                                        |                         | 10—15 лет, исход                |                              |                              |                       | неодина ковая         |                                 |                         |
|                                  |                                   | субстанция,         |                                        |                         | летальный                           |                              |                              |                       | выраженность в      |                                 |                         |
|                                  |                                   | бледный шар,      |                                        |                         |                                            |                              |                              |                       | «хорошие» и           |                                 |                         |
|                                  |                                   | таламус, кора      |                                        |                         |                                            |                              |                              |                       | «плохие» дни          |                                 |                         |
|                                  |                                   | больших              |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  |                                   | полушарий           |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
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| Миоритмии                | Инфекционные,          | Ядро нижней        | Не установлены              | В любом           | Зависит от основного          | Преимущественно | Локальный             | Ритмичный     | Наблюдается в       | Отсутствуют            | Не изменен       |
|                                  | демиелинизирующие, | оливы, красное    |                                        | возрасте          | заболевания                        | в мышцах мягкого  | миоклонический    |                       | покое и при             |                                 | или мышечная  |
|                                  | сосудистые                | ядро, зубчатое     |                                        |                         |                                            | неба, гортани,       | гиперкинез с          |                       | движениях, иногда  |                                 | дистония          |
|                                  | заболевания, травмы  | ядро мозжечка,    |                                        |                         |                                            | голосовых             | четким                   |                       | сохраняется во       |                                 |                         |
|                                  |                                   | центральный        |                                        |                         |                                            | складок,                | двигательным        |                       | время сна               |                                 |                         |
|                                  |                                   | путь покрышки     |                                        |                         |                                            | диафрагмы,          | эффектом             |                       |                                |                                 |                         |
|                                  |                                   | мозгового ствола |                                        |                         |                                            | иногда                   |                              |                       |                                |                                 |                         |
|                                  |                                   | (при вело-            |                                        |                         |                                            | распространяется  |                              |                       |                                |                                 |                         |
|                                  |                                   | палатинной          |                                        |                         |                                            | с одной группы      |                              |                       |                                |                                 |                         |
|                                  |                                   | миоритмии)          |                                        |                         |                                            | мышц на другую    |                              |                       |                                |                                 |                         |
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| Писчий спазм            | Дегенеративные,        | Базальные           | Не установлены              | В возрасте       | Стационарное                      | Мышцы кисти        | Тоническая            | Неритмичен    | Развивается           | Остальные функции | Не изменен       |
|                                  | инфекционные и         | ганглии                |                                        | 20—40 лет        |                                            |                              | судорога пальцев  |                       | только во время      | руки, как правило,    |                         |
|                                  | другие поражения      |                            |                                        |                         |                                            |                              |                              |                       | письма                    | сохранены               |                         |
|                                  | ц.н.с., неврозы,          |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  | иногда является         |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  | локальной формой     |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  | торсионной дистонии  |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
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| Тик (болезнь Бриссо) | Неврозы.                    | Не уточнена         | Не установлены              | В любом           | Зависит от основного          | Могут                    | Быстрые,               | Неритмичен    | Исчезает во сне      | Отсутствуют            | Не изменен       |
|                                  | Инфекционные,          |                            |                                        | возрасте          | заболевания                        | наблюдаться в      | повторяющиеся     |                       |                                |                                 |                         |
|                                  | токсические и другие  |                            |                                        |                         |                                            | любой части тела, | движения в            |                       |                                |                                 |                         |
|                                  | поражения ц. н. с.      |                            |                                        |                         |                                            | чаще в мышцах     | определенной        |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | лица и                   | части тела,            |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | проксимальных     | напоминают           |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | отделах верхних   | произвольные        |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | конечностей          |                              |                       |                                |                                 |                         |
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| Торсионная               | Наследование по        | Скорлупа,            | Поражение                      | 4—15 лет         | В случаях раннего (до 15     | Наблюдается        | Медленные            | Неритмичен    | Наблюдается в       | Выраженный лордоз | Мышечная        |
| дистония                   | аутосомно-                 | хвостатое ядро,   | специфических               | (рецессивный   | лет) начала (с нижних          | преимущественно  | вращательные,      |                       | покое, усиливается | (при переразгибании | дистония          |
|                                  | рецессивному или      | таламус,              | моноаминосодержащих   | тип                   | конечностей) —                   | в мышцах              | вычурные              |                       | при движении          | позвоночника),         |                         |
|                                  | аутосомно-                 | бледный шар,      | нейрональных систем     | наследования), | прогрессирующее с             | туловища, рук,      | движения (иногда  |                       | (ходьбе), эмоциях   | «верблюжья»           |                         |
|                                  | доминантному типу,   | черная                 |                                        | до 40 лет          | генерализацией, при более  | участвуют             | штопорообразные)  |                      |                                | походка                   |                         |
|                                  | симптоматическая      | субстанция          |                                        | (доминантный   | позднем начале (с мышц     | мышцы-                 | с задержкой и        |                       |                                |                                 |                         |
|                                  | торсионная дистония  |                            |                                        | тип                   | головы и туловища) —         | синергисты и         | фиксацией поз       |                       |                                |                                 |                         |
|                                  | при дегенеративных,  |                            |                                        | наследования)  | более медленное                 | мышцы-                 |                              |                       |                                |                                 |                         |
|                                  | сосудистых,               |                            |                                        |                         |                                            | антагонисты          |                              |                       |                                |                                 |                         |
|                                  | инфекционных            |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  | заболеваниях ц.н.с.,   |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|                                  | интоксикациях            |                            |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Хорея                        | Атеросклероз             | Базальные           | Не установлены              | 60 лет и            | Прогрессирующее               | Мышцы туловища, | Быстрые,               | Неритмичен    | Наблюдается в       | Менее грубое (чем   | Снижен или      |
| атеросклеротическая | сосудов головного      | ганглии,               |                                        | старше             |                                            | конечностей,         | нестереотипные,    |                       | покое, исчезает во  | при хорее                 | мышечная        |
|                                  | мозга                         | внутренняя          |                                        |                         |                                            | лица, участвуют    | размашистые;        |                       | сне, усиливается    | Гевтингтона)            | дистония          |
|                                  |                                   | капсула, кора       |                                        |                         |                                            | мышцы-                 | больные                 |                       | при волнении          | снижение                 |                         |
|                                  |                                   | больших              |                                        |                         |                                            | антагонисты и       | гримасничают,       |                       |                                | интеллекта,              |                         |
|                                  |                                   | полушарий           |                                        |                         |                                            | мышцы-                 | жестикулируют      |                       |                                | признаки                  |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            | синергисты            |                              |                       |                                | атеросклероза          |                         |
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| Хорея Гентингтона    | Наследование по        | Хвостатое ядро,  | Уменьшение уровня        | Чаще в             | Неуклонно                           | Мышцы тела,         | Быстрые,               | Неритмичен    | Наблюдается в       | Сопутствующая       | Снижен или      |
|                                  | аутосомно-                 | скорлупа              | дофамина в хвостатом   | возрасте 30—   | прогрессирующее, средняя | конечностей,         | неожиданные,        |                       | покое, исчезает во  | деменция; при          | мышечная        |
|                                  | доминантному типу с  | (поражение          | ядре, снижение               | 50 лет              | продолжительность жизни   | лица, участвуют    | нестереотипные,    |                       | сне, усиливается    | позднем начале       | дистония          |
|                                  | полной                       | шиповидных        | плотности ГАМК-             |                         | после начала заболевания   | мышцы-                 | размашистые, без  |                       | при волнении,         | деменция                 |                         |
|                                  | пенетрантностью        | нейронов), кора    | эргических рецепторов    |                         | — приблизительно 15 лет;   | антагонисты и       | напряжения;          |                       | движениях              | развивается редко   |                         |
|                                  | патологического гена  | больших              |                                        |                         | при позднем начале (60—    | мышцы-                 | больные                 |                       | (ходьбе), возможно |                                 |                         |
|                                  |                                   | полушарий           |                                        |                         | 80 лет) — медленно             | синергисты            | гримасничают,       |                       | временное              |                                 |                         |
|                                  |                                   | головного мозга   |                                        |                         | прогрессирующее                |                              | жестикулируют,     |                       | произвольное          |                                 |                         |
|                                  |                                   | и мозжечка          |                                        |                         |                                            |                              | «пританцовывают» |                       | подавление             |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            |                              | при ходьбе            |                       | гиперкинезов           |                                 |                         |
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| Хорея                        | Ревматический           | Хвостатое ядро,  | Не установлены              | Чаще в             | Средняя длительность 1—5 | Начинается в        | Быстрые                | Неритмичен    | Наблюдается в       | Другие признаки       | Снижен или      |
| ревматическая          | энцефалит                 | скорлупа, кора     |                                        | возрасте 8—16 | месяцев, возможны             | мышцах лица, рук, | неожиданные,        |                       | покое, исчезает во  | ревматизма              | мышечная        |
|                                  |                                   | больших              |                                        | лет                   | рецидивы                            | затем может          | нестереотипные,    |                       | сне, усиливается    | (кардит, артрит,       | дистония          |
|                                  |                                   | полушарий           |                                        |                         |                                            | распространиться  | размашистые;       |                       | при волнении,         | васкулит)                 |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            | на мышцы всего    | больные                 |                       | движениях,             |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | тела, часто по       | гримасничают,       |                       | произвольное          |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | гемитипу               | пританцовывают    |                       | подавление             |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | (гемихорея);          | при ходьбе            |                       | невозможно            |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | участвуют             |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | антагонисты и       |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | синергисты            |                              |                       |                                |                                 |                         |
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| Эпилепсия                 | Клещевой энцефалит | Моторные,           | Не установлены              | В любом           | Прогрессирующее, затем    | Преимущественно | Постоянный           | Неритмичен    | Наблюдается в       | Периодически —      | Спастический   |
| Кожевниковская        |                                   | премоторные       |                                        | возрасте          | стабильное                          | в мышцах лица,     | клонический           |                       | покое, во сне не      | большие                   | тонус,               |
|                                  |                                   | зоны коры            |                                        |                         |                                            | руки                      | гиперкинез в          |                       | исчезает                 | судорожные             | дистония          |
|                                  |                                   | больших              |                                        |                         |                                            |                              | определенной        |                       |                                | припадки                  | мышц               |
|                                  |                                   | полушарий           |                                        |                         |                                            |                              | группе мышц          |                       |                                |                                 |                         |
|                                  |                                   | головного мозга   |                                        |                         |                                            |                              |                              |                       |                                |                                 |                         |
|-------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------------|
| Эпилепсия                 | Опухоли,                    | Моторные,           | Не установлены              | В любом           | Зависит от течения              | Судороги               | Клонические          | Неритмичен    | Приступ                  | Как правило, потери | Пирамидный     |
| кортикальная             | сосудистые,               | премоторные       |                                        | возрасте          | основного заболевания        | начинаются в        | судороги                |                       | продолжается в      | сознания не             | тонус,               |
| (джексоновская)        | паразитарные,            | зоны коры            |                                        |                         |                                            | определенной        |                              |                       | течение нескольких | происходит, после   | дистония          |
|                                  | инфекционные            | больших              |                                        |                         |                                            | части тела (чаще   |                              |                       | минут                      | приступа возможен  |                         |
|                                  | заболевания               | полушарий           |                                        |                         |                                            | в руке, лице),        |                              |                       |                                | паралич                   |                         |
|                                  | головного мозга          | головного мозга   |                                        |                         |                                            | затем                    |                              |                       |                                | конечностей, в         |                         |
|                                  |                                   |                            |                                        |                         |                                            | распространяются |                              |                       |                                | которых были           |                         |
|                                  |                                   |                            |                                        |                         |                                            | на мышцы той же  |                               |                       |                                | судороги, в ряде      |                         |
|                                  |                                   |                            |                                        |                         |                                            | стороны, могут      |                              |                       |                                | случаев переходит   |                         |
|                                  |                                   |                            |                                        |                         |                                            | переходить на       |                              |                       |                                | в большой                |                         |
|                                  |                                   |                            |                                        |                         |                                            | другую сторону;    |                              |                       |                                | судорожный             |                         |
|                                  |                                   |                            |                                        |                         |                                            | участвуют             |                              |                       |                                | припадок                  |                         |
|                                  |                                   |                            |                                        |                         |                                            | мышцы-                 |                              |                       |                                |                                 |                         |
|                                  |                                   |                            |                                        |                         |                                            | антагонисты          |                              |                       |                                |                                 |                         |
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Клиническими признаками Г. стволового уровня являются ритмичность, стереотипность и простая структура движений. Для Г. подкоркового происхождения характерны аритмичность, асинхронность, разнообразие двигательных проявлений и их сложная структура, например, атетозный Г. представляет собой медленные, червеобразные, вычурные движения (обычно кистей, пальцев рук и ног) с их тоническим напряжением. Он может быть обусловлен сосудистыми, инфекционными и другими заболеваниями головного мозга (так называемый симптоматический атетоз), а также наследоваться по аутосомно-рецессивному типу с развитием симметричного Г. в детском возрасте (двойной атетоз, болезнь Хаммонда). Гемибаллизм характеризуется быстрыми, бросковыми движениями конечностей большой амплитуды и силы; наблюдается при поражении сосудистого и опухолевого генеза субталамического ядра Льюиса и его связей с бледным шаром.
Лечение Г. должно быть комплексным. Применяют препараты, влияющие на этиологические факторы (вазоактивные, противовоспалительные и другие средства). Патогенетическая и симптоматическая терапия Г. включает назначение холинолитических и дофаминергических средств, миорелаксантов, ганглиоблокирующих, седативных, дегидратирующих препаратов, а также средств, влияющих на метаболизм головного мозга. При наличии показаний проводят нейрохирургические стереотаксические операции с деструкцией ядер таламуса, базальных ганглиев (см. Функциональная нейрохирургия), при спастической кривошее возможно нейрохирургическое вмешательство на шейных корешках и черепном добавочном нерве.
См. также Атетоз двойной, Гентингтона хорея, Дрожание, Миоклония, Миоклонус-эпилепсия, Писчий спазм, Судороги, Тик, Торсионная дистония, Хорея.
Библиогр.: Бархатова В.П. Нейротрансмиттеры и экстрапирамидная патология, М., 1988; Гусев Е.И., Боголепов Н.Н. и Бурд Г.С. Сосудистые заболевания головного мозга, М., 1979; Петелин Л.С. Экстрапирамидные гиперкинезы (современное состояние проблемы), М., 1983.

1. Малая медицинская энциклопедия. — М.: Медицинская энциклопедия. 1991—96 гг. 2. Первая медицинская помощь. — М.: Большая Российская Энциклопедия. 1994 г. 3. Энциклопедический словарь медицинских терминов. — М.: Советская энциклопедия. — 1982—1984 гг.

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Полезное


Смотреть что такое "Гиперкинезы" в других словарях:

  • Гиперкинезы — Гиперкинезия …   Википедия

  • ГИПЕРКИНЕЗЫ (от — греч. hyper сверх, чрезмерно и kinesis движение), чрезмерные непроизвольные движения, развивающиеся при болезнях ц. н. с. К Г. относятся, напр., судороги …   Ветеринарный энциклопедический словарь

  • Гиперкинезы — Непроизвольные, насильственные, автоматизированные движения. Они многовариантны по локализации, темпу, амплитуде и состоянию при этом мышечного тонуса, обычно усиливаются при волнении. Во сне Г., как правило, исчезают. Обычно обусловлены… …   Энциклопедический словарь по психологии и педагогике

  • гиперкинезы — (от греч. hypér — сверх, чрезмерно и kinēsis — движение), чрезмерные непроизвольные движения, развивающиеся при болезнях центральной нервной системы. К Г. относятся, например, судороги …   Ветеринарный энциклопедический словарь

  • Миоклония – хореоатетоз — Гиперкинезы конечностей в форме сочетания миоклонии (см.) и хореоатетоза (см.). Описал в 1936 г. бельгийский невропатолог L. van Bogart (род. в 1897 г.) …   Энциклопедический словарь по психологии и педагогике

  • Торсио́нная дистони́я — (лат. torsio, torsionis вращение, скручивание; греч. dys + tomos напряжение; синонимы: торсионный спазм, деформирующая мышечная дистония, прогрессирующий торсионный спазм) хроническое прогрессирующее заболевание, в основе которого лежит поражение …   Медицинская энциклопедия

  • Хорея — I Хорея (chorea; греч. choreia пляска) форма гиперкинеза, проявляющаяся непроизвольными быстрыми, беспорядочными, неритмичными, нестереотипными подергиваниями различных мышечных групп. В процессе изучения гиперкинезов (Гиперкинезы) выделены… …   Медицинская энциклопедия

  • Де́тские параличи́ — непрогрессирующие неврологические расстройства, возникающие в результате повреждения нервной системы на разных этапах развития. Различают детские периферические параличи (см. Родовая травма новорожденных (Родовая травма новорождённых)) и детские… …   Медицинская энциклопедия

  • Энцефалит — Энцефалит …   Википедия

  • Энцефалиты — Энцефалит Вирус энцефалита: внутри вирусные гены, снаружи шипики для сцепления с клетками жертвами. МКБ 10 A83. A …   Википедия

  • Туре́тта синдро́м — (G. G. de la Tourette, франц. неврол., 1857 1904; синоним пароксизмальный генерализованный тик) симптомокомплекс поражения ц.н.с., характеризующийся пароксизмальными (реже постоянными) тикоидными подергиваниями мышц лица, шеи, верхних и нижних… …   Медицинская энциклопедия


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