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Hypotonia with decreased or normal dynamic tone is generalized, proximal more than distal, and particularly involves the face and neck muscles. Dysphagia and respiratory compromise are less common. Extraocular movements are usually normal. The disease is always progressive but an apparent initial improvement in muscle strength sometimes occurs during the neonatal period. Some patients with congenital muscular dystrophy have associated brain anomalies. Fukuyama congenital muscular dystrophy is associated with cerebral and cerebellar gyral abnormalities (migrational errors) and occasionally white matter hypodensity on CT. Congenital muscular dystrophy with white matter hypodensity on CT, but without gyral abnormalities, have been reported. Walker-Warburg syndrome consists of congenital muscular dystrophy with cerebral and cerebellar gyral abnormalities, white matter hypodensity on CT, and ocular abnormalities (congenital glaucoma, retinal and optic nerve hypoplasia, and cataracts). The absence of brain malformation on CT or MRI is highly indicative of normal intellect. Treatment of all congenital muscular dystrophies is supportive.

Polymyositis
Polymyositis produces marked hypotonia with decreased dynamic tone (especially in neck muscles), weak cry, and poor sucking and feeding ability. Serum creatine phosphokinase is very elevated. Electromyography shows fibrillations and brief and small motor unit potentials. Muscle biopsy shows muscle destruction with lymphocytic infiltration. Steroid treatment is beneficial.

 

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McMenamin, 1982 Egger, 1983 Aihara, 1992 Heckman, 1985 Volpe, 1995a