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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) is a rare hereditary disease characterised by recurrent transient ischaemic attacks, strokes, and vascular dementia. Pathological studies reveal multiple small infarcts and diffuse white matter changes as well as vascular alterations most prominent in small arteries.1 The presence of granular osmiophilic material in arterial walls on ultrastructural examination is pathognomonic. Mutations in the notch3 gene located on chromosome 19 are associated with the disease.2 Here we report a patient with an unusual clinical course with recurrent intracerebral haemorrhage.
Case report
A 47 year old woman was admitted after a fall at home, followed by several minutes of unconsciousness. Her past medical history was uneventful. In particular, there was no history of migraine or depression, and she was not taking any drugs. On admission, her husband reported memory deficits for several weeks. Initially, the patient complained about headaches and mild dysarthria. Moderate left sided hemiataxia and pronation of the left arm were present. Blood pressure was raised to 220/120 mm Hg without a known history of arterial hypertension. Neuropsychological deficits were prominent in impaired semantic, visual-spatial, and episodic memory, and in deterioration in …
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Competing interests: none declared
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