Tongue Fasciculations and Electrocardiographic Tremors in Spinal Muscular Atrophy

A 6-year-old girl, born out of a nonconsanguineous union, presented with delayed motor milestones. On examination, she had a normal head size; normal cognitive, social, and language function, tongue fasciculations (Video; available at www.jpeds.com), generalized hypotonia, proximal muscle weakness involving lower limbs more than upper limbs, and areflexia. The examinations of other systems were unremarkable. Her nerve conduction studies and echocardiography were normal. A 12-lead electrocardiogram (ECG) showed muscle fasciculations (Figure).

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