"CADASIL coma": an underdiagnosed acute encephalopathy.
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.
Schon
F
F
Clinical Neuroscience, St George's Hospital Medical School, London, UK. schon@globalnet.co.uk
Martin
R J
RJ
Prevett
M
M
Clough
C
C
Enevoldson
T P
TP
Markus
H S
HS
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