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A 22-year-old woman presented with leg muscle weakness, back pain, and urinary disturbance for 4 years. Symptoms were aggravated by walking. She reported having been involved in a traffic accident 1 year ago. Neurological examination showed muscle weakness of the lower legs. The deeper tendon reflex was hyperactive, and Babinski’s sign was positive. Ocular movement was ataxic and symmetric. Atrophy of the proximal limb muscles was present. There was urinary disturbance with marked distension of the bladder and involuntary micturition. Sensation was preserved in the lower limbs. Facial expression was normal. Her muscle weakness slightly improved after a night’s sleep. An electromyogram (EMG) showed slight denervation in the proximal limb muscles. Muscle biopsy showed frequent rimmed vacuoles containing Hirano bodies in the cytoplasm. The acid alpha-glucosidase assay revealed a remarkable increase of the acid alpha-glucosidase activity in the cerebrospinal fluid, the first case reported with Hirano bodies in the spinal cord. A diagnosis of familial thoracic myelopathy with Hirano bodies was made. She had neither consanguinity nor a family history of neuromuscular diseases, and no mutations in the genes responsible for familial vacuolar myopathy were found.#!/bin/sh
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