Clinical Manifestations of Amyotrophic Lateral Sclerosis (Continued)

Juvenile monomelic amyotrophy (JMA) is characterized by insidious onset of unilateral or asymmetric muscular atrophy and weakness of the hand and forearm in the absence of sensory or pyramidal signs. JMA predominantly affects young men between 15 and 25 years of age. The manifestations are often unilateral, although bilateral, asymmetric involvement may occur. The clinical course is initially progressive for a few years, followed by spontaneous stabilization. Although disputed, some specialists believe that the pathophysiology of JMA may be related to abnormal movement of the lower cervical spinal cord during neck flexion, which somehow damages the anterior horn cells of the lower cervical spinal cord, leading to a focal motor neuronopathy manifesting as weakness and atrophy of lower cervical segment–innervated muscles. Treatment options for JMA may include patient education to avoid neck flexion, wearing a soft cervical collar, and surgical decompression. The majority of patients show spontaneous arrest of progression within 3 to 5 years. However, hand weakness and atrophy are permanent.

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