RT Journal Article
SR Electronic
T1 “CADASIL coma”: an underdiagnosed acute encephalopathy
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 249
OP 252
DO 10.1136/jnnp.74.2.249
VO 74
IS 2
A1 Schon, F
A1 Martin, R J
A1 Prevett, M
A1 Clough, C
A1 Enevoldson, T P
A1 Markus, H S
YR 2003
UL http://jnnp.bmj.com/content/74/2/249.abstract
AB 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.