Late-onset leukoencephalopathy without hypertension in a case of young-adult-onset alopecia and spondylosis: a variant of CARASIL?

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  • 高齢で高血圧をともなわずに白質脳症を発症した若年性禿頭・変形性脊椎症の１例：ＣＡＲＡＳＩＬの不全型か？
  • 症例報告 高齢で高血圧をともなわずに白質脳症を発症した若年性禿頭・変形性脊椎症の1例--CARASILの不全型か?
  • ショウレイ ホウコク コウレイ デ コウケツアツ オ トモナワズ ニ ハクシツ ノウショウ オ ハッショウシタ ジャクネンセイ ハゲアタマ ヘンケイセイ セキツイショウ ノ 1レイ CARASIL ノ フゼンガタ カ

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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.<br> 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.<br>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 48 (6), 406-409, 2008

    Societas Neurologica Japonica

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