Open AccessNon-convulsive status epilepticus causing focal neurological deficits in CADASIL
Author(s) -
Philipp O. Valko,
Massimiliano M. Siccoli,
A. Schiller,
Heinz Gregor Wieser,
Hans H. Jung
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.08.2008.0713
Subject(s) - cadasil , leukoencephalopathy , medicine , migraine with aura , migraine , status epilepticus , stroke (engine) , aura , white matter , pathology , pediatrics , epilepsy , magnetic resonance imaging , disease , anesthesia , radiology , psychiatry , mechanical engineering , engineering
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease caused by mutations of the Notch3 gene. Clinical manifestations include migraine with or without aura, psychiatric disorders, recurrent ischaemic strokes and cognitive decline. Brain MRI shows confluent hyperintense signal alterations involving characteristically the anterior part of the temporal lobes and widespread areas of the deep and periventricular white matter. Focal or generalised seizures represent a rare neurological manifestation in CADASIL with a frequency of 6-10% in two large series. Status epilepticus, however, has not been reported so far. Herein we describe a patient with CADASIL with an acute focal neurological deficit following a prolonged migraine attack. The symptoms were first interpreted as an ischaemic stroke but subsequently diagnosed to be due to a non-convulsive status epilepticus.