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KMID : 0361419940180020427
Journal of Korean Academy of Rehabilitation Medicine
1994 Volume.18 No. 2 p.427 ~ p.432
Scapuloperoneal Syndrome-A Case Report-





Abstract
Scapuloperonal syndrome is a rare disease entity characterized by weakness and atrophy of proximal shoulder girdle and distal anterolateral leg muscles. The disease is inherited by a variable mode. Electromyography and muscle biopsy show
denervation and
dystrophic changes.
We report one case of scapuloperoneal syndrome with clinical, electrophysiological, and muscle biopsy findings. Twenty two year-old male patient ws admitted because of progressive gait disturbace. Weakness and atrophy of scapuloperoneal area
muscles
were noted without sonsory ross. The Gowers sign was positive and pes cavus deformity was developed due to achilles tightness on both sides. The deep tendon reflexes were hypoactive and pathologic reflexes was absent. The muscles enzyme study and
nerve
conduction study was normal. The electromyographic study revealed combined peripheral motor neuropathy (axonal type) and myopathy. The muscle biopsy study revealed myopathic predominant findings. This case was considered as mixed pattern
scapuloperoneal
syndrome of sporadic form.
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