Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Cerebral amyloid angiopathy-related leukodystrophy: a case report

Takeshi Iwanaga, M.D.1)3), Nobuyuki Kaneko, M.D.1), Hirotake Nishimura, M.D.2) and Kazumi Kimura, M.D.1)

1)Department of Stroke Medicine, Kawasaki Medical School
2)Department of Pathology, Kawasaki Medical School
3)Department of Stroke Medicine, Okayama Red Cross Hospital

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.
Full Text of this Article in Japanese PDF (675K)

(CLINICA NEUROL, 52: 585|588, 2012)
key words: Amyloid angiopathy, Leukodystrophy, microbleeds

(Received: 22-Aug-11)