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Non-convulsive status epilepticus causing focal neurological deficits in CADASIL
  1. Philipp O Valko,
  2. Massimiliano M Siccoli,
  3. Andreas Schiller,
  4. Heinz-Gregor Wieser,
  5. Hans H Jung
  1. Department of Neurology, University Hospital of Zurich, Frauenklinikstrasse 26, CH-8091 Zurich, Switzerland
  1. Dr Hans H Jung, Department of Neurology, University Hospital Zurich, 8091 Zurich, Switzerland; hans.jung{at}usz.ch

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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.1 2 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.

Case report

The 63-year-old right-handed female had a history of severe and longlasting migraine attacks with visual aura since the age of 40 years. The patient’s father died at age 63 years after a history of progressive behavioural changes interpreted as the result of recurrent strokes. At age 59 years, the patient underwent a …

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