“CADASIL coma”: an underdiagnosed acute encephalopathy
- 1Clinical Neuroscience, St George’s Hospital Medical School, London, UK
- 2Southampton General Hospital, Southampton, UK
- 3King’s College Hospital, London
- 4Walton Centre for Neurology and Neurosurgery, Liverpool, UK
- Correspondence to: Fred Schon, Department of Neurology, Atkinson Morley’s Hospital, London SW20 ONE, UK; schon{at}globalnet.co.uk
- Received 4 February 2002
- Accepted 24 April 2002
- Revised 19 April 2002
Abstract
The main clinical features of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy) are stroke, dementia, and migraine. A reversible acute encephalopathy was the principal presentation in six of 70 patients in a British prevalence study. The episodes lasted seven to 14 days, presenting with fever, acute confusion, coma, and fits; there was full recovery but in two cases identical episodes recurred some years later. All patients had a previous history of migraine with aura and were originally misdiagnosed as viral encephalitis. CADASIL should be considered in acute unexplained encephalopathies. MRI white matter changes, previous migraine with aura, and a family history of stroke and dementia may be useful pointers to the diagnosis.
Footnotes
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Competing interests: none declared
