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Homovanillic Acid, Magnetic Resonance, and Sleep Apnea Studies

Junichi Kitamura, MD; Yoko Kubuki, MD; Kazuhito Tsuruta, MD; Teruyuki Kurihara, MD; Shigeru Matsukura, MD

Arch Neurol. 1989;46(4):425-428.

Abstract
• Four male patients and one female patient of a new family with Joseph disease are reported. Their disease was characterized by autosomal dominant inheritance, bulging eyes, rigidity and spasticity of the lower extremities, dystonia, and bradykinesia. Cerebrospinal fluid homovanillic acid level was markedly reduced. Levodopa improved dystonia. Magnetic resonance imaging revealed mild atrophy of the frontal lobe and the cerebellum and marked atrophy of the lenticular nucleus and the brain stem. Polysomnographic studies revealed non-rapid eye movement stage central type sleep apnea syndrome. This is the first report using magnetic resonance imaging and sleep apnea studies of Joseph disease.

Author Affiliations
From the Third Department of Internal Medicine, Miyazaki (Japan) Medical College.

Footnotes
Accepted for publication Oct 13, 1987.

Reprint requests to Department of Neurology, Junwa Memorial Hospital, Komatsu, Miyazaki 880-21, Japan (Dr Kitamura)

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