title: "CADASIL coma": an underdiagnosed acute encephalopathy.
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.
authors.affiliation: Clinical Neuroscience, St George's Hospital Medical School, London, UK. schon@globalnet.co.uk
authors.forename: F
authors.initials: F
authors.lastname: Schon
authors.forename: R J
authors.initials: RJ
authors.lastname: Martin
authors.forename: M
authors.initials: M
authors.lastname: Prevett
authors.forename: C
authors.initials: C
authors.lastname: Clough
authors.forename: T P
authors.initials: TP
authors.lastname: Enevoldson
authors.forename: H S
authors.initials: HS
authors.lastname: Markus
mesh_terms: D000208:Acute Disease; D000328:Adult; D001921:Brain / Q000473:pathology; D002869:Chromosome Aberrations; D003128:Coma / Q000209:etiology* / Q000235:genetics; D015161:Dementia, Multi-Infarct / Q000175:diagnosis* / Q000235:genetics; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D005799:Genes, Dominant / Q000235:genetics; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020325:Migraine with Aura / Q000175:diagnosis / Q000235:genetics; D009460:Neurologic Examination