@article {Schon249, author = {F Schon and R J Martin and M Prevett and C Clough and T P Enevoldson and H S Markus}, title = {{\textquotedblleft}CADASIL coma{\textquotedblright}: an underdiagnosed acute encephalopathy}, volume = {74}, number = {2}, pages = {249--252}, year = {2003}, doi = {10.1136/jnnp.74.2.249}, publisher = {BMJ Publishing Group Ltd}, 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.}, issn = {0022-3050}, URL = {https://jnnp.bmj.com/content/74/2/249}, eprint = {https://jnnp.bmj.com/content/74/2/249.full.pdf}, journal = {Journal of Neurology, Neurosurgery \& Psychiatry} }