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• Vol.45 No.5
• CLINICA NEUROL, 45: 357−361, 2005
• Vol.45 No.5 contents

Case Report

A case of Brown-Vialetto-van Laere (BVVL) syndrome in Japan

Hiroshi Nemoto, M.D., Ph.D., Shingo Konno, M.D., Nobuatsu Nomoto, M.D., Nobuo Wakata, M.D. and Teruyuki Kurihara, M.D.

Division of Neurology, Department of Internal Medicine, Toho University Ohashi Hospital

Here we report a sixty-year-old woman of Brown-Vialetto-van Laere (BVVL) syndrome in Japan. She had sensorineural deafness, weakness and atrophy of her extremities from 15 years of age. Her neurological symptoms slowly progressed. She first visited our hospital in 1993 when she was 49 years old. At that time, she had distal muscle weakness and atrophy of the four extremities and bulbar palsy. Deep tendon reflexes were absent and the plantar toe reflex was flexor. EMG revealed neurogenic changes and the nerve conduction studied were normal. The vital capacity was marked decreased. On August 10, 2003, she was admitted to our hospital because of CO₂ narcosis. She had III, VII, X, XI, XII cranial nerve palsy, distal muscle weakness and atrophy of the four extremities. From her neurological symptoms and signs, we made a diagnosis of BVVL syndrome. MRI revealed no high signal in pyramidal tract by FLAIR image. ABR showed no response, and VEP demonstrated delay of the P₁₀₀. She was intubated, and was attached to a respirator to improve her CO₂ narcosis. After treatment she improved and did not need to be assisted by a respirator during daytime. During night time, she had apnea, and her blood gas showed the retention of CO₂, and she still required the respiratory assistance during her sleep. This is the first report of BVVL syndrome in Japanese literature.

(CLINICA NEUROL, 45: 357−361, 2005)
key words: Brown-Vialetto-van Laere syndrome, sensorineural deafness, ABR, VEP, SMA

(Received: 8-Jul-04)