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"code": "G24.4",
"name": "Идиопатическая рото-лицевая дистония",
"icd_name": "Идиопатическая рото-лицевая дистония",
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"lead": "дистония пароксизмальная, охватывающая мышцы, расположенные вокруг рта и мышцы нижней челюсти",
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"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Дистонии могут носить врожденный или приобретенный характер, рото-лицевая дистония чаще относится ко второй группе. В некоторых в случаях его формирование ассоциируют с мутацией в локусе DYT1 на 9-й хромосоме. Также определенную роль могут играть гены DYT 4, DYT 6, DYT 7, которые способны передаваться как по аутосомно-доминантному, так и по аутосомно-рецессивному типу. Приобретенную форму заболевания обуславливают следующие факторы:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Сосудистые нарушения. Развитие рото-лицевой дистонии может быть связано с поражением экстрапирамидной системы на фоне внутричерепных кровоизлияний, артериовенозных мальформаций, аневризм мозговых артерий, инсультов.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Травмы головного мозга. Повреждение стрио-паллидарной системы и формирование ОМД вызывают тяжелые перинатальные и черепно-мозговые травмы, неправильное протезирование. В части случаев провоцирующим фактором у предрасположенных пациентов может быть стоматологическое лечение.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Влияние медикаментов. Появление симптомов патологии часто ассоциировано с длительным приемом фармацевтических средств, в том числе – антагонистов дофамина, некоторых нейролептиков, блокаторов кальциевых каналов, противоэпилептических препаратов.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Неопластические процессы. Насильственные движения могут быть признаком формирования злокачественных или доброкачественных опухолей головного мозга, энцефалита при паранеопластическом синдроме.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Токсины. Становление стойких гиперкинезов провоцирует хроническая интоксикация производственными или бытовыми химикатами, в частности – метанолом, дисульфирамом, цианидами, сероуглеродом, марганцем, кобальтом.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Инфекционные заболевания. Рото-лицевая дистония может развиваться на фоне тяжелого течения вирусных энцефалитов, подострого склерозирующего панэнцефалита, поражения тканей ЦНС при ВИЧ-инфекции.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
"pathogenesis": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Составляющие патогенеза заболевания досконально не изучены. Считается, что в основе лежит нейродинамический дефект на уровне базальных ганглиев – полосатого и субталамического ядер, черной субстанции, ножкомостового ядра покрышки. Он характеризуется повышением активности холинергических и дофаминергических систем. Также при этой патологии вероятно наличие нарушения суставно-мышечного чувства, кинестезии на фоне дисфункции проприорецепторов (нервно-мышечных, нервно-сухожильных волокон) и сенсомоторной дезинтеграции, обусловленной нарушением проводящих путей между двигательными зонами прецентральной извилины коры головного мозга, структурами экстрапирамидной системы и мозжечком. В результате формируются спонтанные сокращения жевательной, подбородочной мускулатуры, мышц языка, подкожной мышцы шеи, круговой мышцы рта.</span></p>",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">План обследования пациента включает:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Опрос. Врач-невролог детализирует жалобы больного, выясняет возможное наличие подобных расстройств у ближайших кровных родственников. Специалист уточняет потенциальную связь между развитием имеющихся симптомов и приемом медикаментов, ранее перенесенными заболеваниями или травмами ЦНС.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Физикальное обследование. При определении неврологического статуса оценивают функцию V и VII пар черепно-мозговых нервов, устанавливают характер насильственных движений и их изменения в ответ на использование корригирующих приемов, речевую активность.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Генетический анализ. Поиск мутаций проводится только после постановки предварительного диагноза ОМД в нетипичных ситуациях, например – при дебюте заболевания в возрасте до 30 лет или манифестации с дистонического гиперкинеза конечности.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Нейровизуализация. Церебральная МРТ показана при наличии анамнестических сведений, указывающих на вторичные формы патологии. КТ головного мозга информативна при подозрении на формировании кальцинатов и скоплений железа в ЦНС.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">На ранних этапах лечения используются медикаментозные средства различных групп. Основной целью терапии является восстановление нейротрансмиттерного баланса в подкорковых ганглиях. При неэффективности фармакотерапевтических препаратов применяется ботулинотерапия, проводятся оперативные вмешательства. В целом программа лечения может состоять из следующих пунктов:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Фармакотерапия. В качестве базовых назначают препараты из групп миорелаксантов центрального действия, антиконвульсантов или транквилизаторов. Наблюдается характерный для этого заболевания парадокс – отмечается эффективность медикаментов, которые могут его провоцировать (нейролептиков и вальпроатов). Из-за этой особенности средства из перечисленных групп постепенно заменяются атипичными аналогами. Симптоматически применяются НПВС.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Ботулинотерапия. В связи с риском формирования дисфагии и дизартрии осуществляется только при выраженных функциональных нарушениях. Место локальной инъекции и доза препаратов на основе ботулотоксина А подбирается в индивидуальном порядке на основании клинических особенностей у конкретного пациента.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Физиотерапия. Эффективна в комплексе с фармакотерапевтическими средствами. Используется лазеротерапия, электросонтерапия, биоптронтерапия, физиотерапевтические ванны. Для нормализации функций височно-нижнечелюстного сочленения рекомендуются окклюзионные каппы.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Нейрохирургические операции. Хирургическое лечение при орофациальной дистонии показано только при резистентности ко всем вышеупомянутым методам, представлено имплантацией электродов в область внутренней части бледного шара с целью глубокой стимуляции ЦНС.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Специфическая профилактика отсутствует. Неспецифические превентивные мероприятия направлены на исключение причин вторичной и медикаментозной форм, подразумевают рациональный прием медикаментов, раннее лечение инфекционных заболеваний и органических патологий головного мозга, минимизацию контакта с нейротоксическими веществами.</span></p>",
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"description": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Блефароспазм — это локальное неврологическое расстройство, при котором человек усиленно моргает и невольно зажмуривает глаза. К стойкому спазму и смыканию век приводят непроизвольные сокращения круговых мышц глаз.</span></p>",
"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Врождённый блефароспазм связан с генетическими нарушениями, которые передаются при определённых типах наследования:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">аутосомно-доминантном — болезнь возникает в каждом поколении независимо от пола;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">аутосомно-рецессивном — болезнь передаётся через поколение, возникает независимо от пола;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">X-сцепленном рецессивном — болезнь передаётся через поколение, возникает только у мальчиков (девочки становятся носителями);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">митохондриальном — болезнь передаётся по материнской линии, возникает у детей независимо от пола.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Приобретённый блефароспазм вызывают другие заболевания и состояния:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">повреждение головного мозга ребёнка в утробе матери, во время родов или в первую неделю жизни;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">нейроинфекции (менингит, энцефалит);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">лекарственное воздействие (леводопа, бромокриптин, агонисты дофамина, нейролептики, метоклопрамид, противосудорожные средства, антидепрессанты, блокаторы кальциевых каналов, СИОЗС и антигистаминные препараты);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">интоксикации (отравление цианидами, метанолом, марганцем, сероуглеродом, кобальтом, дисульфирамом, 3-нитропропионовой кислотой);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">дегенеративные и сосудистые заболевания центральной нервной системы (мультисистемная атрофия, дисциркуляторная энцефалопатия);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">неопластические процессы (опухоль головного мозга, паранеопластический энцефалит);</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">черепно-мозговая травма;</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">функциональные нарушения (психогении).</span></li>\r\n</ul>\r\n<p> </p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">В основе механизма развития лежит гиперреактивность периокулярных мышц, обусловленная краниальной формой мышечной дистонии. Ученые полагают, что при данной патологии поражаются базальные ганглии. При этом нарушается прохождение импульса от ядер к структурам ствола мозга, мозжечка и премоторной коры. В патофизиологическом процессе задействованы дофаминергические и холинергические системы. Нарушение в работе премоторной коры приводит к дисфункции интегративных процессов двигательного контроля, что проявляется недостаточным реципрокным торможением мышц-антагонистов.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При снижении остроты зрения часто выявляется сопутствующее поражение таламуса, приводящее к замедлению прохождения импульса из стриопаллидарного комплекса к коре. Некорректное модулирование моторной программы потенцирует развитие клиники спастического сокращения век. Экспериментальным путем доказано, что важную роль в патогенезе играет нарушение метаболизма глюкозы. Согласно результатам протонной МР-спектроскопии, у пациентов отмечается снижение N-ацетиласпартата, креатинфосфокиназы. Нарушение афферентации – это лишь изменения в первичном звене патогенеза. Прогрессирование патологии связано с развитием вторичных осложнений.</span></p>",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Для верификации диагноза проводится сбор анамнеза, физикальное и специальное офтальмологическое обследование. Изучаются критерии Харрисона (особенности родов и развития, сопутствующая неврологическая симптоматика, результаты лабораторных и инструментальных исследований). В ходе диагностики применяются:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">МРТ головы. Магниторезонансная томография проводится для визуализации структуры мозговых оболочек, интракраниальных сосудов у больных с подозрением на артериовенозные мальформации.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Реоэнцефалография. Диагностика кровенаполнения сосудов головы и шеи информативна для оценки характера венозного оттока. При снижении показателей РЭГ можно предположить, что в основе болезни лежат поражения ишемической природы.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Электроэнцефалография. Регистрация биоэлектрических сигналов, которые исходят от нервных клеток, позволяет оценить степень функциональной активности мозга.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Биомикроскопия глаза. Исследование дает возможность выявить вторичные поражения роговицы глаза, проявляющиеся дегенеративно-дистрофическими изменениями, формированием участков изъязвления на оболочке.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Офтальмоскопия. Применяется для изучения состояния сетчатки и диска зрительного нерва (ДЗН). Выраженный спазм может потенцировать нарушение оттока крови с последующим застоем и образованием мелкоочаговых геморрагий. ДЗН без изменений.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Визометрия. Острота зрения нарушается только при распространении патологического процесса на таламус, что проявляется клиникой корковой слепоты.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Тактика ведения пациента зависит от выраженности симптомов и частоты возникновения приступов. На ранних стадиях возможно применение психотерапии, иглорефлексотерапии. В качестве консервативной терапии используется:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Ботулинический нейротоксин типа А. Препарат оказывает пресинаптическое блокирующее действие на холинергические нервные окончания. Эффект от инъекции сохраняется на протяжении 3-4 месяцев, после чего требуется повторное введение.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Препараты бензодиазепинового ряда. Данная группа лекарственных средств входит в базовую схему лечения. Благодаря взаимодействию бензодиазепинов с ГАМК-рецепторами увеличивается связывание гамма-аминомасляной кислоты с белковыми структурами, что, в свою очередь, снижает возбудимость нейронов. При недостаточной эффективности медикаментов этой группы дополнительно используют нейролептики, антидепрессанты, миорелаксанты или седативные препараты.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Холинолитики. Антихолинергические средства блокируют медиатор ацетилхолин, снижая скорость прохождения нервного импульса. Наряду с купированием спастических сокращений отмечается расширение зрачков и паралич аккомодации.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Увлажняющие средства. Инстилляции препаратов искусственной слезы назначают при развитии ксерофтальмии.</span></li>\r\n</ul>\r\n<p><span style=\"background-color: transparent; font-family: Verdana; font-size: 7pt; white-space: pre-wrap;\">Оперативное лечение показано при стойком блефароспазме и неэффективности медикаментозной терапии. Осуществляется пластика верхних, реже – нижних век. Применение этого метода лечения наиболее целесообразно при синдроме апраксии. При отсутствии эффекта от блефаропластики проводят билатеральное пересечение ветвей лицевого нерва. Ранее в лечении больных с идиопатическим блефароспазмом использовали термолитическое повреждение нервных волокон, иннервирующих веко.</span></p>\r\n<p> </p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Для профилактики следует оптимизировать условия на своём рабочем месте, особенно для людей, работающих с повышенной нагрузкой на зрительный аппарат. Важно соблюдать рациональный режим труда и отдыха, регулярно выполнять дозированные физические нагрузки, избегать стрессовых перегрузок, травм и болезненных манипуляций на голове и шее, включая мануальную терапию.</span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Первое проявление заболевания – маловыраженное подергивание век, которое со временем сменяется непроизвольным зажмуриванием глаз. Развитию клинической картины нередко предшествует учащенное моргание, прищуривание, самопроизвольное опускание века без спастического компонента. Сенсорные нарушения включают дискомфорт, ощущение инородного тела в глазнице, жжение. Лица, страдающие данной патологией, долго не обращаются за помощью к специалисту, связывая данную симптоматику со светобоязнью. Прогрессирование болезни ведет к появлению стабильных тонических или тонико-клонических спазмов. Пациенты предъявляют жалобы на стойкое смыкание век. Самостоятельно разомкнуть глазную щель не удается.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Больные не могут предвидеть, когда возникнет следующий приступ. Средняя длительность спастического сокращения составляет 3-5 минут. В межприступный период общее состояние не нарушено. На начальных стадиях патология поддается волевому контролю. Как правило, поражаются оба глаза, однако степень выраженности симптомов на правом и левом глазу может незначительно различаться. Со временем в процесс вовлекаются мышцы нижней половины лица и шеи. Возникает повышенное слезотечение, сухость глаз. Зрительной дисфункции не наблюдается. Реакция зрачков на свет сохранена.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При тяжелом течении существенно снижается качество жизни больного, иногда – вплоть до невозможности самостоятельного передвижения. Пациенты отмечают, что болезнь значительно затрудняет повседневную активность (чтение книг, шитье, работу за компьютером). Симптомы часто возникают в самый неудобный момент (при вождении автомобиля, переходе дороги), но отсутствуют во время сна. Выраженность клинических проявлений снижается после длительного отдыха и усиливается при попытке фиксации взгляда, в стрессовых ситуациях. Спонтанные ремиссии отсутствуют.</span></p>",
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"lead": "Шейная дистония — это неврологическое нарушение, при котором наблюдается патологическое сокращение шейных мышц. Из-за этого человек постоянно наклоняет голову вперед или вбок.\r\n\r\nПрогрессирование дистонии проходит несколько стадий. Первоначально дистонические движения преходящие и появляются только во время произвольных движений или стресса. В дальнейшем, у пациентов могут возникать дистонические ненормальные позы и движения во время ходьбы и, в конечном счете, даже в состоянии покоя. Дистонические движения могут со временем привести к стойким физическим дефектам, так как возникают укорочения сухожилий.\r\n\r\nПри вторичных дистониях вследствие травмы или инсульта у пациентов отмечаются аномальные движения только с одной стороны тела, которые могут появиться сразу после травмы головного мозга (инсульта) или через некоторое время после. Симптомы обычно не прогрессируют и не охватывают другие части тела.",
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"lead": "наследственное заболевание, характеризующееся дрожанием рук, головы, голосовых мышц, которое усиливается при движениях, попытке сохранить определенную позу и прекращается при засыпании",
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"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">До сих пор ученые не смогли точно определить, что именно провоцирует развитие заболевания. Проблема может появиться у человека в любом возрасте. Согласно данным исследований, если болезнь наблюдалась у обоих родителей, то вероятность, что у ребенка он возникнет, составляет 75 %. Специалисты указывают, что основной причиной появления эссенциального тремора является генетическая предрасположенность. Также заболевание может возникать из-за нарушения взаимодействия следующих структур головного мозга.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 7pt; font-family: Verdana; background-color: transparent; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Основные причины появления эссенциального тремора являются:</span></p>\r\n<p> </p>\r\n<ul style=\"color: #000000; font-family: Verdana, Arial, Helvetica, sans-serif; font-size: 10px; font-style: normal; font-variant-ligatures: normal; font-variant-caps: normal; font-weight: 400; letter-spacing: normal; orphans: 2; text-align: start; text-indent: 0px; text-transform: none; white-space: normal; widows: 2; word-spacing: 0px; -webkit-text-stroke-width: 0px; text-decoration-thickness: initial; text-decoration-style: initial; text-decoration-color: initial;\">\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">генетическая предрасположенность;</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">нарушения взаимодействия структур головного мозга;</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">нарушение строения и функционирования отдельных структур мозга;</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">сбой в работе дофаминергической системы;</span></li>\r\n<li><span style=\"font-size: 7pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">эндокринные заболевания.</span></li>\r\n</ul>",
"pathogenesis": "",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Эссенциальный тремор диагностируется только после исключения всех возможных причинных заболеваний и основывается преимущественно на особенностях клинической картины и анамнестических данных. Диагностический процесс проходит в несколько этапов.</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Консультация невролога. Проводится исследование неврологического статуса пациента, анализ его психического состояния, осмотр шеи на предмет изменений со стороны щитовидной железы. Физикальное обследование помогает определить время возникновения и характер дрожательного феномена, последовательность вовлечения в него различных частей тела. Объективно неврологический осмотр выявляет дрожание пальцев и кистей в позе Ромберга, при выполнении пальце-носовой пробы. Дрожание нижних конечностей определяется при выполнении коленно-пяточной пробы. В расслабленном удобном положении тела оно исчезает.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">МРТ головного мозга или КТ. Исследование визуализирует мозговые структуры и различные церебральные поражения (опухоли, дегенеративные изменения, атрофические участки, признаки энцефалита, пр.).</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">УЗ-диагностика. УЗДГ и дуплексное сканирование мозговых сосудов помогают оценить состояние церебрального кровообращения. УЗИ щитовидной железы выполняется по показаниям, может быть дополнено консультацией эндокринолога.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Электронейрография — позволяет исключить полиневропатию.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Лабораторные исследования: биохимический анализ крови, исследование функции щитовидной железы, определение уровня паратгормона.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Консультация генетика и генеалогическое исследование требуется для подтверждения генетического характера заболевания. Наличие в анамнезе родственников с аналогичными симптомами свидетельствует в пользу наследственного тремора.</span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Терапия направлена на уменьшение дрожания и замедление прогрессирования заболевания. Проводится годами. Включает фармакотерапию, расслабляющий массаж, иглоукалывание. Пациентам не рекомендованы повышенные физические и психо-эмоциональные нагрузки. В медикаментозном лечении препаратами выбора являются:</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Бета-блокаторы (пропанолол) — обладают хорошим эффектом. Их назначают длительно при обязательном контроле артериального давления и пульса.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Антиконвульсанты. Ранее успешно применялся примидон, клоназепам. Сейчас перспективными являются противоэпилептические препараты нового поколения, а именно леветирацетам. Предупреждение побочных эффектов (сонливости и астении) достигается постепенным тщательным подбором дозы.</span></li>\r\n<li><span style=\"font-size: 7pt; color: #000000; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Пиридоксин — высокие дозы витамина В6 способствуют существенному замедлению прогрессирования болезни. Препарат вводится внутримышечно. Курс лечения длится 1 месяц, повторяется ежегодно 2-3 раза.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">При дрожании головы возможна терапия путём введения ботулотоксина в кивательные мышцы. Эффект от введения сохраняется до 2,5 мес., затем требуется повторная процедура.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Нейрохирургические методы</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Выраженный эссенциальный тремор, стойкий к консервативным методам терапии, может быть показанием к альтернативным методам лечения, таким как глубокая церебральная стимуляция. Интракраниально имплантируют электроды, соединённые с небольшим аппаратом, который посылает подавляющие тремор импульсы. Недавно группой исследователей был разработан способ аблации определённых участков таламуса проходящим через кости черепа прицельно направленным ультразвуковым излучением. Разработки в этом направлении продолжаются.</span></p>",
"prevention": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">В качестве профилактики прогрессирования тремора рук показан специальный курс упражнений с эспандером, а также занятия, развивающие мелкую моторику рук (вязание, бисероплетение, рисование, и т.д.).</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Заядлым кофеманам рекомендуется максимально снизить или полностью отказаться от кофе, поскольку кофеин возбуждающе действует на нервную систему, что негативно сказывается на течении тремора.</span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Основное клиническое проявление — мелкоразмашистый тремор, частота которого варьирует от 6 до 12 Гц. Наиболее часто дрожанию подвержены кисти. Оно хорошо заметно при вытягивании рук перед собой и удержании их в такой позе (постуральный тремор). Характерен также интенционный тремор - дрожание во время целенаправленных движений. В ряде случаев дрожательный феномен наблюдается в покое. У всех пациентов указанные изменения полностью исчезают в период сна. Усиливать тремор способны физические нагрузки, эмоциональные переживания.</span></p>\r\n<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Эссенциальный тремор может охватывать голову, подбородок, губы, язык. Тремор голосовых связок обуславливает вибрирование голоса, нарушает чёткость речи. Реже отмечается дрожание ног и туловища. Лёгкие изменения особенно не влияют на качество жизни. Умеренный тремор верхних конечностей затрудняет мелкую работу и приводит к изменению почерка: буквы становятся заострёнными, отсутствуют соединения между ними. Выраженное дрожание снижает работоспособность и может стать причиной инвалидности. Заметный окружающим тремор вызывает психологический дискомфорт. Возможно развитие мнительности, депрессивного невроза, ипохондрии.</span></p>",
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