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- W4249967318 abstract "Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease caused by mutations of the <i>Notch3</i> 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." @default.
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- W4249967318 date "2009-02-02" @default.
- W4249967318 modified "2023-09-23" @default.
- W4249967318 title "Non-convulsive status epilepticus causing focal neurological deficits in CADASIL" @default.
- W4249967318 doi "https://doi.org/10.1136/bcr.08.2008.0713" @default.
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