Acute headache followed by focal neuropsychological impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
Occasionally, patients with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) present atypical features such as confusion, coma, or nonconvulsive status epilepticus. Acute focal neuropsychological syndrome revealing the disease has been poorly documented. We report the atypical presentation of two patients in whom CADASIL was revealed by an episode of headache followed by focal neuropsychological impairment.
Ducray
François
F
Department of Neurology B, Cerebrovascular Unit, Université Lyon 1, Lyon, France. francois.ducray@chu-lyon.fr
Ritzenthaler
Thomas
T
Cho
Tae Hee
TH
Bruyas
Amandine
A
Cotton
François
F
Cartalat-Carel
Stéphanie
S
Honnorat
Jérôme
J
Nighoghossian
Norbert
N
D000208:Acute Disease; D000328:Adult; D046589:CADASIL / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000523:psychology; D004252:DNA Mutational Analysis; D038524:Diffusion Magnetic Resonance Imaging; D006261:Headache / Q000209:etiology* / Q000235:genetics / Q000523:psychology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000209:etiology* / Q000235:genetics / Q000523:psychology; D009483:Neuropsychological Tests; D000071656:Receptor, Notch3; D051880:Receptors, Notch / Q000235:genetics
C499374:NOTCH3 protein, human; D000071656:Receptor, Notch3; D051880:Receptors, Notch