Rinsho Shinkeigaku (Clinical Neurology)

Case Report

A case of chronic progressive neuro-Behcet's disease with cerebellar ataxia and bulbar palsy preceding mucocutaneo-ocular symptoms

Masashi Watanabe, M.D.1)3), Rei Kobayashi, M.D., Ph.D.1), Takakazu Hasegawa, M.D.2), Shunsuke Yokoi, M.D.2), Hisashi Okada, M.D., Ph.D.1) and Satoshi Okuda, M.D., Ph.D.1)

1)Department of Neurology, National Hospital Organization Nagoya Medical Center
2)Department of Rheumatology and Clinical Immunology, National Hospital Organization Nagoya Medical Center
3)Present address: Department of Neurology, Ehime Prefectural Central Hospital

A 77-year-old man with a history of cigarette smoking had suffered from vertigo and depression repeatedly for twelve years. He gradually developed bradykinesia in the past half decade and fell down 3 times in the last half year. On admission, he presented with cerebellar ataxia and bulbar symptoms. Brain MRI showed atrophy in the cerebellum and brainstem. 123I-IMP SPECT showed hypoperfusion bilaterally in the cerebellum. Blood examinations showed various elevated inflammatory values and positive for HLA-B51. Cerebrospinal fluid (CSF) revealed aseptic meningitis and increased IL-6 levels. Therefore, we strongly suspected that he had chronic progressive neuro-Behcet's disease (CPNBD), clinically. Systemic mucocutaneous symptoms appeared 1 month after starting treatments. Pathological findings of his skin biopsy were consistent with Behcet's disease. It should be kept in mind that both positive HLA-B51 and increased CSF IL-6 levels have the possibility of containing important clues in the diagnosis of CPNBD.
Full Text of this Article in Japanese PDF (823K)

(CLINICA NEUROL, 58: 105|110, 2018)
key words: chronic progressive neuro-Behcet's disease, cerebellar ataxia, HLA-B51, cerebrospinal fluid IL-6, 123I-IMP SPECT

(Received: 15-Aug-17)