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Ultrastructural and histochemical abnormalities of skeletal muscle in patients with chronic vitamin E deficiency

From the Denver Veterans Administration Medical Center Neurology Service [Dr. Neville and Ms. Wehling) the University of Colorado School of Medicine, Departments of Neurology (Drs. Neville. Ringel, and Guggenheim, and Ms. Starcevich) and Pediatrics (Dr. Guggenheim).

We report muscle biopsy abnormalities in four patients with a chronic cholestatic syndrome, low serum vitamin E levels, absent reflexes, mild limb weakness, ataxia, and sensory loss in arms and legs. Skeletal muscle fibers contained multiple autofluorescent inclusions that show strong acid phosphatase and esterase reactivity. By electronmicroscopy, the inclusions lying between myofibrils were membrane-bound dense bodies having characteristics of both lysosomes and lipopigment material. The material was similar to that observed in vitamin E-deficient animals and probably formed in response to disordered intracellular lipid peroxidation.

Address correspondence and reprint requests to Dr. Neville, Neurology Service, Veterans Administration Medical Center, 1055 Clermont Street, Denver, CO 80220.

This study was supported in part by funding to Dr. Neville from the Veterans Administration and by a grant to Dr. Guggenheim from the Muscular Dystrophy Association of America.

Accepted for publication July 26, 1982

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