What is Hirayama disease?
Hirayama disease (Monomelic Amyotrophy) is a rare, self-limiting lower motor neuron disorder primarily affecting young males, characterized by asymmetric muscle weakness and atrophy in the distal upper extremities (often sparing the brachioradialis muscle). Its cause is attributed to a disproportionate adolescent growth between the cervical spine and the dural sac, leading to dynamic spinal cord compression and ischemia upon neck flexion. Signs often include cold paresis and no sensory loss. Since the condition spontaneously stabilizes, treatment is conservative, mainly involving the use of a cervical collar to restrict neck flexion and prevent further cord compression.
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