PT - JOURNAL ARTICLE AU - F Schon AU - R J Martin AU - M Prevett AU - C Clough AU - T P Enevoldson AU - H S Markus TI - “CADASIL coma”: an underdiagnosed acute encephalopathy AID - 10.1136/jnnp.74.2.249 DP - 2003 Feb 01 TA - Journal of Neurology, Neurosurgery & Psychiatry PG - 249--252 VI - 74 IP - 2 4099 - http://jnnp.bmj.com/content/74/2/249.short 4100 - http://jnnp.bmj.com/content/74/2/249.full SO - J Neurol Neurosurg Psychiatry2003 Feb 01; 74 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.