title: [Late-onset leukoencephalopathy without hypertension in a case of young-adult-onset alopecia and spondylosis: a variant of CARASIL?].
abstract: We report herein a 61-year-old man with diffuse leukoencephalopathy, subcortical infarcts and cervical and lumbar spondylosis. Medical history included baldness and lumbar spondylosis at young-adult onset. His parents were consanguineous (cousin). He had been experiencing severe lumbago since 20-years-old, with hair loss starting around the same time. He noticed dysarthria and gait disturbance at 59-years-old. He was admitted to our hospital at 61-years-old with aggravation of gait disturbance. On admission, no abnormalities were evident on physical examination except for diffuse baldness. Neurological findings included mild dementia, bilateral hyperreflexia, paraparesis, right Babinski's sign, and pseudobulbar palsy. Blood pressure was normal. T2-weighted imaging of the brain revealed diffuse high-intensity in the periventricular white matter and subcortical infarcts in the brainstem and bilateral basal ganglia. Marked lumbar deformations were observed on spinal MRI. Clinical features in this case met the criteria for cerebral autosomal recessive arteriopathy with subcortical infarctions and leukoencephalopathy (CARASIL), apart from late onset of cerebral infarction.
authors.affiliation: Department of Neurology, Saitama Medical University.
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Nakazato
authors.forename: Aya
authors.initials: A
authors.lastname: Ohkuma
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Mizoi
authors.forename: Naotoshi
authors.initials: N
authors.lastname: Tamura
authors.forename: Kunio
authors.initials: K
authors.lastname: Shimazu
mesh_terms: D017668:Age of Onset; D000505:Alopecia; D001921:Brain / Q000473:pathology; D002539:Cerebral Arterial Diseases / Q000175:diagnosis; D015140:Dementia, Vascular / Q000175:diagnosis; D006801:Humans; D006973:Hypertension; D017116:Low Back Pain; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013128:Spinal Osteophytosis / Q000175:diagnosis; D013131:Spine / Q000473:pathology; D013577:Syndrome