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“CADASIL coma”: an underdiagnosed acute encephalopathy
  1. F Schon1,
  2. R J Martin1,
  3. M Prevett2,
  4. C Clough3,
  5. T P Enevoldson4,
  6. H S Markus1
  1. 1Clinical Neuroscience, St George’s Hospital Medical School, London, UK
  2. 2Southampton General Hospital, Southampton, UK
  3. 3King’s College Hospital, London
  4. 4Walton Centre for Neurology and Neurosurgery, Liverpool, UK
  1. Correspondence to:
    Fred Schon, Department of Neurology, Atkinson Morley’s Hospital, London SW20 ONE, UK;
    schon{at}globalnet.co.uk

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.

  • encephalopathy
  • CADASIL syndrome
  • dementia

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