Abstract
The authors report the clinical and electromyographic (EMG) findings in a series of 13 children with a pseudomyopathic motor deficit in the context of malnutrition (Body Mass Index < or = 3rd percentile) caused by primary protein-calorie deficiency or secondary to chronic disease. The infants (nine cases) manifested a regression or stagnation of motor abilities, with hypotonia and amyotrophy; older children and adolescents (four cases) presented clear amyotrophy with a deficit in muscle strength consisting primarily of proximal muscular weakness. Detection and stimulation-detection EMG demonstrated myogenic signs in at least two muscles in all patients. Myogenic signs were dominant in all proximal muscles. Latencies were normal in all patients. Motor nerve conduction velocities were slowed in three infants and in one adolescent. A temporal dispersion of motor responses was observed in 11 proximal muscles. The muscle biopsy, performed in five cases, revealed an inequality in the calibre of fibres, with atrophy dominating in type II fibres. The authors emphasize the value of EMG in disclosing the myopathic process, thereby contributing to the etiological diagnosis of motor difficulties in children suffering from malnutrition or a chronic disease.
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