کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3053322 1186157 2006 9 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other?
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی عصب شناسی
پیش نمایش صفحه اول مقاله
Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other?
چکیده انگلیسی

BackgroundEarly myoclonic encephalopathy (EME) and the Ohtahara syndrome are currently listed as two separate syndromes in the classification of epilepsies. The most prominent differentiating points are the observations that patients with Ohtahara syndrome experience predominantly tonic seizures; their seizures evolve to infantile spasms and the prognosis is often worse than patients with EME.Summary pointsWe performed a literature review of published cases. Although syndromes may have distinct courses, the differentiation early on may be impossible as both myoclonus and tonic seizures may coexist. There is also an overlap in the etiologies. Tonic seizures are considered a manifestation of brainstem dysfunction and it is possible that this is more prominent in Ohtahara syndrome. To date, there are 17 autopsy cases (12 presumed to be Ohtahara cases and 5 EME). Evidence of hindbrain pathology was present in all. Tonic seizures or tonic posturing was a feature of all cases. We suggest that the two syndromes may represent a continuum and that the prominence of tonic seizures in the Ohtahara syndrome may be an indication of brainstem dysfunction which may play an important role in the subsequent transition to infantile spasms.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Epilepsy Research - Volume 70, Supplement, August 2006, Pages 68–76
نویسندگان
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