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The syndrome of 'pure akinesia' and its relationship to progressive supranuclear palsy

Department of Neurology (Drs. Riley and Leigh), Case Western Reserve University School of Medicine, Cleveland; the Movement Disorders Center (Dr. Riley), Mt. Sinai Medical Center, Cleveland; and the Departments of Optometry (Dr. Fogt) and Neurology (Dr. Leigh), Veterans Affairs Medical Center, Cleveland, OH.

Five patients developed a stereotyped syndrome of progressive akinesia of gait, speech, and handwriting without rigidity, tremor, or dementia. The symptoms did not improve with levodopa. These clinical findings conform to a syndrome described repeatedly in Japan since 1974 as "pure akinesia." Evidence has indicated that pure akinesia often represents a pre-ocular motor, and in some cases an ocular motor-sparing, form of progressive supranuclear palsy (PSP). Although we found disorders of eyelid movements, none of the patients demonstrated a gaze palsy on clinical examination. Four patients underwent eye movement recordings. The two patients with the longest disease duration had slow or small vertical saccades. These findings support the notion that patients with pure akinesia may develop a vertical gaze palsy, similar to that in PSP, late in their course. Our patients show that pure akinesia occurs in North America as well as in Japan. Recognition of the syndrome of pure akinesia may suggest the diagnosis of PSP before the development of abnormalities of ocular movement.

Address correspondence and reprint requests to Dr. David Riley, Movement Disorders Center, Mt. Sinai Medical Center, 1 Mt. Sinai Drive, Cleveland, OH 44106.

Dr. Leigh was supported by USPHS grant EY06717 and NASA grant NAG9-571, the Department of Veterans Affairs, and the Evenor Armington Fund.

Received August 16, 1993. Accepted for publication in final form November 30, 1993.

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