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Multicore disease

Report of a case with onset in middle age

Department of Neurology and the Neuromuscular Disease Research Center of the Vanderbilt University School of Medicine, Nashville, Tennessee.

A 46-year-old white man had a 12-year history of progressive, symmetrical proximal weakness. Changes consistent with a myopathy were seen on electromyography, along with normal nerve conduction velocities. Serum creatine phosphokinase was slightly elevated. Muscle biopsies showed type I fiber predominance and focal areas of absent or increased oxidative enzyme activity, with other fibers containing focal areas of absent myofibril-lar ATPase activity. Serial sections showed little correlation between abnormalities demonstrated with oxidative enzymes and ATPase. Ultrastruc-tural changes included focal disruption of myofibrils and Z-band streaming. Similar morphologic changles have previously been described in a congenital, nonprogressive myopathy.

Dr. Bonnette's address is Department of Neurology, Massachusetts General Hospital, Fruit St., Boston, MA 02114.

This study was supported by grants from the USPHS (1 POINS 10175) and from the Muscular Dystrophy Association of America, Inc.

Received for publication February 19, 1974.

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