title: Acute headache followed by focal neuropsychological impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).
abstract: 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.
authors.affiliation: Department of Neurology B, Cerebrovascular Unit, Université Lyon 1, Lyon, France. francois.ducray@chu-lyon.fr
authors.forename: François
authors.initials: F
authors.lastname: Ducray
authors.forename: Thomas
authors.initials: T
authors.lastname: Ritzenthaler
authors.forename: Tae Hee
authors.initials: TH
authors.lastname: Cho
authors.forename: Amandine
authors.initials: A
authors.lastname: Bruyas
authors.forename: François
authors.initials: F
authors.lastname: Cotton
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Cartalat-Carel
authors.forename: Jérôme
authors.initials: J
authors.lastname: Honnorat
authors.forename: Norbert
authors.initials: N
authors.lastname: Nighoghossian
mesh_terms: 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
chemical_list: C499374:NOTCH3 protein, human; D000071656:Receptor, Notch3; D051880:Receptors, Notch