Cerebral amyloid angiopathy-related leukodystrophy: a case report

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  • 副腎皮質ステロイドが奏功した脳アミロイドアンギオパチー関連白質脳症の1例
  • フクジン ヒシツ ステロイド ガ ソウコウ シタ ノウ アミロイドアンギオパチー カンレン ハクシツ ノウショウ ノ 1レイ

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Abstract

An 86-year-old woman was admitted following generalized seizure. Postictally she showed disturbance of consciousness, right hemiparesis, and right spatial neglect. Brain fluid attenuated inversion recovery (FLAIR) imaging demonstrated mainly left-sided, but asymmetrical, subcortical white matter lesions. On the second day, level of consciousness improved, along with right hemiparesis and right spatial neglect. Electroencephalography displayed mainly α waves and sporadic θ waves without sharp waves. One week later, however, the patient again experienced somnolence and right hemiparesis. FLAIR revealed day-by-day enlargement of white matter lesions. The possibilities of hypoglycemia, hyperammonemia, hypothyroidism, Hashimoto encephalopathy, collagen disease, antineutrophil cytoplasmic antibody-related angiitis, and infection were excluded based on the results of blood biochemistry and cerebrospinal fluid. We initially suspected intravascular lymphoma, so random skin biopsy was performed, but the results were negative. We then suspected cerebral amyloid angiopathy because of the presence of dementia and multiple microbleeds on T2* weighted magnetic resonance imaging. Cerebral biopsy revealed amyloid deposition in cortical arterioles and CD3-positive T cells in the perivascular space. Cerebral amyloid angiopathy-related leukodystrophy was therefore diagnosed and immunosuppressive treatment was started. After 14 days of treatment, clinical symptoms and results of FLAIR imaging were significantly improved. When patients display asymmetrical subcortical white matter lesions with microbleeds on T2* weighted imaging, amyloid angiopathy-related inflammation should be considered.<br>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 52 (8), 585-588, 2012

    Societas Neurologica Japonica

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