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

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症例は61歳男性である.両親は従兄妹結婚.20歳頃からびまん性の頭部脱毛,激しい腰痛が出現した.30歳代後半からは腰椎椎間板ヘルニアの診断でしばしば入院し加療された.59歳で構音障害,痙性歩行が出現した.脳MRIで脳幹,基底核に多発性ラクナ梗塞,両側深部白質に脳MRI T<sub>2</sub>強調画像でびまん性高信号をみとめた.本症例の臨床像は白質脳症の発症年齢が高齢であったことを除いて,cerebral autosomal recessive arteriopathy with subcortical infarctions and leukoencephalopathyに合致していた.<br>

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>

収録刊行物

  • 臨床神経学 : CLINICAL NEUROLOGY  

    臨床神経学 : CLINICAL NEUROLOGY 48(6), 406-409, 2008-06-01 

    Societas Neurologica Japonica

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各種コード

  • NII論文ID(NAID)
    10021187750
  • NII書誌ID(NCID)
    AN00253207
  • 本文言語コード
    JPN
  • 資料種別
    SHO
  • ISSN
    0009918X
  • NDL 記事登録ID
    9553121
  • NDL 雑誌分類
    ZS31(科学技術--医学--精神神経科学)
  • NDL 請求記号
    Z19-298
  • データ提供元
    CJP書誌  NDL  J-STAGE 
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