|
Jeavons syndrome is a type of epilepsy. It is one of the most distinctive reflex syndromes of idiopathic generalized epilepsy characterized by the triad of eyelid myoclonia with and without absences, eye-closure-induced seizures, EEG paroxysms, or both, and photosensitivity. Eyelid myoclonia with or without absences is a form of epileptic seizure manifesting with myoclonic jerks of the eyelids with or without a brief absence. These are mainly precipitated by closing of the eyes and lights.〔Panayiotopoulos CP, Agathonikou A, Koutroumanidis M, Giannakodimos S, Rowlinson S, Carr CP. Eyelid myoclonia with absences: the symptoms. In: Duncan JS, Panayiotopoulos CP, editors. Eyelid myoclonia with absences. London: John Libbey and Company Ltd, 1996:17-26〕〔Capovilla G, Striano P, Gambardella A, Beccaria F, Hirsch E, Casellato S et al. Eyelid fluttering, typical EEG pattern, and impaired intellectual function: a homogeneous epileptic condition among the patients presenting with eyelid myoclonia. Epilepsia 2009; 50(6):1536-1541.〕 Eyelid myoclonia is the defining seizure type of Jeavons syndrome.〔Giannakodimos S, Panayiotopoulos CP. Eyelid myoclonia with absences in adults: a clinical and video-EEG study. Epilepsia 1996;37:36-44.〕〔Panayiotopoulos CP. Reflex seizures and related epileptic syndromes. In: A Clinical Guide to Epileptic Syndromes and Their Treatment. Revised 2nd edition. London: Springer, 2010:497-531.〕〔Covanis A. Jeavons syndrome. In: Panayiotopoulos CP, editor. Atlas of epilepsies. London: Springer; 2010. 1081-1092.〕 == Signs and symptoms == Eyelid myoclonia, not the absences, is the hallmark of Jeavons syndrome. Eyelid myoclonia consists of marked jerking of the eyelids often associated with jerky upwards deviation of the eyeballs and retropulsion of the head (eyelid myoclonia without absences). This may be associated with or followed by mild impairment of consciousness (eyelid myoclonia with absences). The seizures are brief (3–6 s), and occur mainly and immediately after closing of the eyes (eye closure) and consistently many times a day. All patients are photosensitive. Generalised tonic-clonic seizures, either induced by lights or spontaneous, are probably inevitable in the long term and are provoked particularly by precipitating factors (sleep deprivation, alcohol) and inappropriate AED modifications. Myoclonic jerks of the limbs may occur, but are infrequent and random. Eyelid myoclonic status epilepticus, either spontaneous (mainly on awakening) or photically induced, occurs in a fifth of patients.〔Agathonikou A, Panayiotopoulos CP, Giannakodimos S, Koutroumanidis M. Typical absence status in adults: diagnostic and syndromic considerations. Epilepsia 1998; 39(12):1265-1276.〕 It consists of repetitive and discontinuous episodes of eyelid myoclonia with mild absence, rather than continuous non- convulsive absence status epilepticus. Onset is typically in childhood with a peak at age 6–8 years (range 2–14 years). There is a twofold preponderance of girls. Prevalence and incidence is probably low. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Jeavons syndrome」の詳細全文を読む スポンサード リンク
|