Международный обучающий курс по эпилепсии. Клинические формы и классификация эпилепсии /International Educations Course of Epilepsy. The clinical forms of epilepsy and their classification /

Содержание

2017 Revised Classification of Seizures

Международный обучающий курс по эпилепсии. Клинические формы и классификация эпилепсии /International Educations Course of Epilepsy. The clinical forms of epilepsy and their classification /

The International League Against Epilepsy (ILAE) is the world's main scientific body devoted to the study of epilepsy, and it has recently revised its classification of seizures. The changes will help make diagnosing and classifying seizures more accurate and easier.

In this article, you’ll find the new general outline of basic seizure classification. The Types of Seizures section has been updated to reflect the new classification system.

Background

People with epilepsy have recurring seizures that often occur spontaneously and without warning. The official definition of a seizure is “a transient occurrence of signs and/or symptoms due to an abnormal excessive or synchronous neuronal activity in the brain.”

  • This means that during a seizure, large numbers of brain cells are activated abnormally at the same time. It is an “electrical storm” in the brain.
  • The nature of the seizures depends on many factors, such as the person’s age, the sleep-wake cycle, prior injuries to the brain, genetic tendencies, medications, which circuits in the brain are involved, and many others.

Separating seizures into different types helps guide further testing, treatment, and prognosis or outlook.

Using a common language for seizure classification also makes it easier to communicate among clinicians caring for people with epilepsy and doing research on epilepsy.

The classification also provides common words for people with epilepsy and the general public to describe their seizures.

History of Seizure Classification

  • For decades, the most common words to describe seizures were grand mal and petit mal. Although the medical meaning of these terms was fairly precise, some people often used them loosely when referring to any big or little seizure.
  • For over 35 years, the terms partial and generalized seizures were used to describe types of seizures. This system divided seizures into partial (seizures starting in one area or side of the brain) and generalized (seizures starting in both sides of the brain at the same time).
  • Partial seizures were then defined by whether a person was aware or conscious during the seizure.
    • Simple partial seizures: Person is aware of what happens during the event.
    • Complex partial seizures: Person has some impaired awareness during the seizure. They may be confused, partially aware, or not aware of anything during a seizure.
  • The old classifications worked for many years but did not capture many types of seizures. This new version will hopefully be more complete.

The New Basic Classification

The basic classification is a simple version of the major categories of seizures. The new basic seizure classification is 3 key features.

  1. Where seizures begin in the brain
  2. Level of awareness during a seizure
  3. Other features of seizures

Defining Where Seizures Begin

The first step is to separate seizures by how they begin in the brain. The type of seizure onset is important because it affects choice of seizure medication, possibilities for epilepsy surgery, outlook, and possible causes.

  • Focal seizures: Previously called partial seizures, these start in an area or network of cells on one side of the brain.
  • Generalized seizures: Previously called primary generalized, these engage or involve networks on both sides of the brain at the onset.
  • Unknown onset: If the onset of a seizure is not known, the seizure falls into the unknown onset category. Later on, the seizure type can be changed if the beginning of a person’s seizures becomes clear.
  • Focal to bilateral seizure: A seizure that starts in one side or part of the brain and spreads to both sides has been called a secondary generalized seizures. Now the term generalized refers only to the start of a seizure. The new term for secondary generalized seizure would be a focal to bilateral seizure.

Describing Awareness

Whether a person is aware during a seizure is of practical importance because it is one of the main factors affecting a person’s safety during a seizure. Awareness is used instead of consciousness, because it is simpler to evaluate.

  • Focal aware: If awareness remains intact, even if the person is unable to talk or respond during a seizure, the seizure would be called a focal aware seizure. This replaces the term simple partial.
  • Focal impaired awareness: If awareness is impaired or affected at any time during a seizure, even if a person has a vague idea of what happened, the seizure would be called focal impaired awareness. This replaces the term complex partial seizure.
  • Awareness unknown: Sometimes it’s not possible to know if a person is aware or not, for example if a person lives alone or has seizures only at night. In this situation, the awareness term may not be used or it would be described as awareness unknown.
  • Generalized seizures: These are all presumed to affect a person’s awareness or consciousness in some way. Thus no special terms are needed to describe awareness in generalized seizures.

Describing Motor and Other Symptoms in Focal Seizures

Many other symptoms may occur during a seizure. In this basic system, seizure behaviors are separated into groups that involve movement.

  • Focal motor seizure: This means that some type of movement occurs during the event. For example twitching, jerking, or stiffening movements of a body part or automatisms (automatic movements such as licking lips, rubbing hands, walking, or running).
  • Focal non-motor seizure: This type of seizure has other symptoms that occur first, such as changes in sensation, emotions, thinking, or experiences.
  • It is also possible for a focal aware or impaired awareness seizure to be sub-classified as motor or non-motor onset.
  • Auras: The term aura, which describes symptoms a person may feel in the beginning of a seizure, is not in the new classification. Yet people may continue to use this term. It’s important to know that in most cases, these early symptoms may be the start of a seizure.

Describing Generalized Onset Seizures

Seizures that start in both sides of the brain, called generalized onset, can be motor or non-motor.

  • Generalized motor seizure: The generalized tonic-clonic seizure term is still used to describe seizures with stiffening (tonic) and jerking (clonic). This loosely corresponds to “grand mal.” Other forms of generalized motor seizures may happen. Many of these terms have not changed, and a few new terms have been added. (see image below)
  • Generalized non-motor seizure: These are primarily absence seizures, and the term corresponds to the old term “petit mal.” These seizures involve brief changes in awareness, staring, and some may have automatic or repeated movements lipsmacking.

Describing Unknown Onset Seizures

When the beginning of a seizure is not known, this classification still gives a way to describe whether the features are motor or non-motor.

The New Expanded Classification

The expanded classification keeps the framework of the basic classification, but adds more seizure types as subheadings. In the following image, the types of features under motor and non-motor seizures are listed for all types: focal, generalized, and unknown onset.

General Comments

Classification of a seizure type is only part of the seizure description. The work to update the seizure classification has been done by a large group of dedicated people in epilepsy over a number of years. This new sysyem will move us forward, making it easier to describe seizures and using a common language to talk about them.

A few other points:

  • The new classification is designed to have some flexibility. Use of other descriptive terms or even free text is encouraged.
  • Most seizures can be classified by signs and symptoms that happen during a seizure. However, other information is useful when available. For example, phone videos, EEG (electroencephalogram), MRI (magnetic resonance imaging), and other brain imaging, blood tests, or gene tests may be helpful. For practical purposes, long descriptive terms are probably not useful for day-to-day life.
  • This new seizure classification does not change the definition of epilepsy or epilepsy syndromes. The ILAE also has produced a new classification of the epilepsies, which we look forward to learning more about. The epilepsy classification includes the whole clinical picture, with information on seizure types, causes, EEG pattern, brain imaging, genetics, and epilepsy syndromes, such as Lennox-Gastaut syndrome and juvenile myoclonic epilepsy.

While the ILAE 2017 seizure classification is exciting, changing terms can be confusing and can take a lot of work. The Epilepsy Foundation is committed to helping educate people about the changes, what it means for them, and how older terminology relates to this new system.

Authored by: Robert S. Fisher MD, PhD, Patricia O. Shafer RN, MN, and Carol D’Souza MA Psych on 12/2016 Reviewed by: Joseph I. Sirven MD on 12/2016

Источник: https://www.epilepsy.com/article/2016/12/2017-revised-classification-seizures

Epilepsy: Classification, Etiology, Diagnosis & Treatment

Международный обучающий курс по эпилепсии. Клинические формы и классификация эпилепсии /International Educations Course of Epilepsy. The clinical forms of epilepsy and their classification /

Epilepsy is characterized by recurrent episodic, paroxysmal, involuntary clinical events associated with the abnormal electrical activity of the neurons. The patient may present with motor, sensory or psychomotor phenomenon, often with alteration in sensorium.

Classification of Epilepsy

Epilepsy is classified by appraisal of

  • Seizure type
  • Etiology
  • Electroencephalographic data

If the underlying etiology is identified it is symptomatic epilepsy otherwise it is called idiopathic. In cryptogenic, a cause is presumed.

A simple modified version of classification proposed by the International League Against Epilepsy (ILAE) is given.

Etiology

  1. Idiopathic or primary epilepsy: the brain is anatomically normal though electric waves on EEG demonstrate abnormality. It may probably be an inherent tendency (genetic factor)

  2. Symptomatic or secondary epilepsy: the periodic burst of impulses is due to irritation of the brain by some other diseases and the fits are simple. It may be due to cerebral tumor inflammatory conditions encephalitis head injury, CVA, uremic condition, and toxic substances.

Generalized seizures

  1. Major epilepsy: grand mal seizures, it refers to major convulsions in which patient may lose consciousness for several minutes. The convulsion proceeds as follows:

  • Aura: aura is a warning signal prior to the onset of seizure e.g. sensation of peculiar taste or smell spots before eyes, dizziness, and feeling of weakness; it serves a useful purpose in known epileptics as the patient seeks safety and privacy before the onset of a seizure.
  • Epileptic cry: the patient loses consciousness with an epileptic cry; it is caused by spasm of thoracic and abdominal muscles expelling air through the glottis.
  • Tonic stage: the patient fails to the ground with all his muscle in the increased tone with rigidity. The skin is pale and cyanotic because respiration ceases, hands are clenched, tongue bit, eyes widely open, pupil dilated and fixed; it lasts for 10-30sec.
  • Clonic stage: it is relaxation period when movement of all body muscles and quick irregular respiration begins. This stage lasts 1-5min. there is frothing at the mouth and incontinence of urine and faces.
  • Coma stage or postictal phase: after the clonic stage. The patient, if undisturbed, passes into ordinary deep sleep for hours and then awakens with a pulsatile headache
  • Petit mal (Absence seizure): it occurs often in children end usually disappears often puberty. It consists of transient loss of consciousness lasting a few seconds. As a rule, such a patient doesn’t fall. The patient demonstrates a brief change such as rolling of the eyes, blinking and slight mouth movement. Patient often describe their attacks as “blackout”
  • Partial seizures (focal/Jacksonian seizures): begins as a localized motor seizure, with convulsions starting in one part of the body for e.g. from the thumb and gradually involving hand and arms, face and other parts of the body. A patient may or may not lose consciousness. The patient of seizure depends upon the area of the brain which is being irritated by the underlying cause.
  • Status epileptics: Status epileptic is when recurrent generalized seizure activity occurs at such frequencies that full consciousness is not regained between seizures. It is accompanied by respiratory distress. It is invariably always fatal. Status epileptic can lead to death from exhaustion.

Emergency management includes constant suction to clear the mouth, padded side rails, 02 inhalation, cardiac stimulants, injection diazepam etc.

Diagnosis and investigation

  • A complete neurological evaluation should follow, including the level of consciousness, reflexes, sensory and motor responses
  • Laboratory studies:
  • Serum electrolytes, magnesium, calcium and fasting blood sugar
  • Toxicology screen: drug overdose may cause seizures
  • Observation of an attack if possible
  • Patient history and description of seizure activity
  • Electroencephalogram (EEG)
  • EEG might be indicated to assess the brain’s electrical activity
  • Radiologic imaging such as CT or MRI may be performed to note any structural abnormality
  • Other investigation may include: serum glucose, calcium, skull X-ray, lumbar puncture

Treatment

A chronic illness epilepsy produces emotional and psychosocial disturbance. A physician must, therefore, give due consideration to them besides medical aspects such as drug therapy, side effects of drugs and identifying etiology.

Medical treatment

  • There is no known cure for idiopathic epilepsy, only prevention for attacks is striven for.
  • A general well-balanced diet is usually recommended variety of vegetables or fresh fruits
  • Drug therapy consists of administration of anticonvulsant drugs which must be continued for at least two years. It may vary except in severe neurodevelopment delay or structural malformation neurologic deficits where a risk of breakthrough and reoccurrence of seizures is higher.
  • The mainstay of treatment of epilepsy is anticonvulsant medications
  • Often, anticonvulsant medication treatment will be lifelong and can have major effects on quality of life
  • The choice among anticonvulsants and their effectiveness differs from epilepsy syndrome. Mechanism, effectiveness for particular epilepsy syndromes, and side effects among the individual anticonvulsant medications
  • Most patients can achieve this balance best with monotherapy, the use of a single anticonvulsant medication

Surgical management

  • Surgical removal is the appropriate treatment for an identified lesion, such as a hematoma or brain tumor
  • This treatment, such as hemispherectomy, nontemporal lobe resection, or corpus callosotomy or commissurotomy, may be performed in children with severe, medically intractable seizure disorders

Prognosis

  • Prognosis depends on type and severity of seizure disorder, co-existing mental retardation, organic disorders and the type of medical management
  • Medically treated seizures- spontaneous cessation of seizure may occur. Drug may be gradually discontinued when the patient has been free from attacks for an extended period and the EEG pattern has reverted to normal
  • Non-treated epilepsy- seizures tend to become more numerous

Complication

  • Apnea/hypoventilation
  • Injuries sustained during a seizure

Источник: https://soundhealthsolution.com/epilepsy/

Доклад комиссии ILAE по классификации и терминологии (2001)

Международный обучающий курс по эпилепсии. Клинические формы и классификация эпилепсии /International Educations Course of Epilepsy. The clinical forms of epilepsy and their classification /

  • Тонико – клонические (включая варианты начала с клонической или миоклонической фазы)
  • Клонические (с легким тоническим компонентом или без него)
  • Типичные абсансы
  • Атипичные абсансы
  • Миоклонические абсансы
  • Тонические
  • Эпилептические спазмы
  • Эпилептический миоклонус
  • Миоклонус век (с абсансами или без них)
  • Миоклонически – атонические (миатонические)
  • Негативный миоклонус
  • Атонические
  • Рефлекторные генерализованные
  • Фокальные сенсорные (с простыми симптомами, связанными с раздражением затылочной или теменной доли или со сложными симптомами, связанными с раздражением височно-теменно-затылочной коры)
  • Фокальные моторные: клонические, асимметричные тонические (связанные с раздражением дополнительной моторной зоны), с типичными автоматизмами, с гиперкинетическими  автоматизмами, с фокальным негативным миоклонусом, ингибиторные
  • Геластические
  • Гемиклонические
  • Вторично – генерализованные
  • Рефлекторные фокальные
  • Статус генерализованных тонико – клонических приступов
  • Статус клонических приступов
  • Статус абсансов
  • Статус тонических приступов
  • Статус миоклонических приступов

Фокальный эпилептический статус.

  • Кожевниковская эпилепсия
  • Продолженная аура
  • Статус лимбических приступов (психомоторный статус)
  • Гемиконвульсивный статус с гемипарезом

Провоцируюшие факторы при рефлекторных приступах

  • Зрительные стимулы: мелькающий свет (желательно указать цвет), фотосенситивный паттерн, другие зрительные стимулы
  • Мыслительный процесс
  • Музыка
  • Еда
  • Выполнение движений
  • Соматосенсорные стимулы
  • Проприоцептивные стимулы
  • Чтение
  • Горячая вода
  • Резкий звук (стартл – приступы)

Доклад комиссии ILAE по классификации и терминологии (Epilepsia. – 2001. – V. 42. – N 6. – P. 796-803)

ЭТАПЫ УСТАНОВЛЕНИЯ ДИАГНОЗА ПРИ ЭПИЛЕПСИИ.

  • Описание пароксизмального события (возможно по данным анамнеза)
  • Классификация приступа (анамнез, визуальное наблюдение, ЭЭГ)
  • Диагностика формы эпилепсии (клиника + ЭЭГ + нейровизуализация)
  • Установление этиологии (МРТ, кариотип)
  • Диагностика сопутствующих заболеваний и установление степени инвалидизации

Идиопатические фокальные эпилепсии младенчества и детства

  • Доброкачественные младенческие приступы (несемейные)
  • Доброкачественная эпилепсия детства с центрально – височными спайками (роландическая)
  • Доброкачественная затылочная эпилепсия детства с ранним дебютом (тип Панайотопулоса)
  • Доброкачественная затылочная эпилепсия детства с поздним дебютом (тип Гасто)

Семейные (аутосомно – доминантные) фокальные эпилепсии

  • Доброкачественные семейные приступы новорожденных
  • Доброкачественные семейные приступы младенчества
  • Аутосомно – доминантная ночная лобная эпилепсия
  • Семейная височная эпилепсия
  • Семейная фокальная эпилепсия с вариабельным фокусом *

Симптоматические (или вероятно симптоматические) фокальные эпилепсии

  • Лимбические эпилепсии
  • Мезиальная височная эпилепсия с гиппокампальным склерозом
  • Мезиальная височная эпилепсия обусловленная специфической этиологией
  • Другие формы определенной локализации и этиологии
  • Неокортикальные эпилепсии
  • Синдром Расмуссена
  • Гемиконвульсивно – гемиплегический синдром
  • Мигрирующие парциальные приступы раннего младенчества *
  •  Другие формы определенной локализации и этиологии 

Идиопатические генерализованные эпилепсии

  • Доброкачественная миоклоническая эпилепсия младенчества
  • Эпилепсия с миоклонически – астатическими приступами
  • Детская абсанс эпилепсия
  • Эпилепсия с миоклоническими абсансами
  • Идиопатические генерализованные эпилепсии с вариабельным фенотипом
  • Юношеская абсанс эпилепсия
  • Юношеская миоклоническая эпилепсия
  • Эпилепсия с изолированными генерализованными тонико – клоническими приступами
  • Генерализованная эпилепсия с фебрильными приступами плюс * 

Рефлекторные эпилепсии

  • Идиопатическая фотосенситивная затылочная эпилепсия
  • Другие формы эпилепсии с приступами, возникающими вследствие зрительной стимуляции
  • Первичная эпилепсия чтения
  • Стартл эпилепсия

Эпилептические энцефалопатии (при которых эпилептиформная активность на ЭЭГ может приводить к прогрессированию неврологических нарушений)

  • Ранняя миоклоническая энцефалопатия
  • Синдром Отахара
  • Синдром Веста
  • Синдром Драве
  • Миоклонический статус при непрогрессирующих энцефалопатиях *
  • Синдром Леннокса – Гасто
  • Синдром Ландау – Клеффнера
  • Эпилепсия с продолженной пик – волновой активностью во время медленного сна

Прогрессирующие миоклонус эпилепсии

Специфические заболевания (типа болезней Лафора, Унферрихта – Лундборга, нейронального цероидного липофусциноза и др.)

Приступы, для обозначения которых дефиниция «эпилепсия» необязательна

  • Доброкачественные приступы новорожденных
  • Фебрильные приступы
  • Рефлекторные приступы
  • Приступы, связанные с отменой алкоголя
  • Приступы, вызванные лекарственными препаратами или другими химическими агентами
  • Приступы, возникающие сразу после или в раннем периоде черепно — мозговой травмы
  • Единичные приступы или единичные серии приступов
  • Редко повторяемые приступы (олигоэпилепсия)

Знаком * помечены синдромы, находящиеся в процессе разработки

ОБСУЖДЕНИЕ (К.Ю. Мухин)

Замена дефиниции «криптогенные формы» на «вероятно симптоматические формы». В определении синдромов замена слова «судороги» на «приступы». Понятие «приступы» значительно шире «судорог», и далеко не все приступы проявляются именно судорогами.

Например, правильнее называть синдром «доброкачественные младенческие приступы», а не «доброкачественные младенческие судороги», так как заболевание необязательно проявляется именно судорожными приступами. Термин «парциальные приступы и парциальные эпилепсии» заменен на «фокальные приступы и фокальные эпилепсии».

Упразднено подразделение фокальных приступов на простые и сложные в зависимости от нарушения уровня сознания. Это связано с тем, что в большинстве случаев врачу не удается детально тестировать сознание пациента во время приступа, в связи с чем оценка уровня сознания всегда очень ориентировочна.

Классификация вызывает много вопросов, в частности, отнесение таких приступов, как атонические, атипичные абсансы и спазмы исключительно к генерализованным. В большинстве случаев эти приступы имеют фокальное начало, и в их основе нередко лежит феномен вторичной билатеральной синхронизации.

В классификацию эпилептических синдромов не вошел синдром псевдоленнокса, встречающийся в педиатрической практике чаще синдрома Леннокса – Гасто. 

Источник: http://epileptologist.ru/ru/archives/1355

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