Waterskier’s Hirayama syndrome
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Hirayama disease is a segmental inferior cervical myelopathy. Presentation is usually in young males with unilateral or asymmetrical upper limb weakness and atrophy. The clinical features can be similar to the initial presentation of amyotrophic lateral sclerosis, but Hirayama syndrome has a benign course and is typically limited to distal muscles of the upper limbs.
The typical teenage onset of the syndrome corresponds with juvenile growth spurt. The disproportionate growth of the vertebral column compared to the spinal cord resulting in the relative shortening of the dorsal roots and the loss of attachment between the posterior dural sac and the subjacent lamina are considered to be contributing factors .
Several reports document antecedent events including repetitive movements, competitive sport or strenuous physical work. Jeannet et al.  documented the case of a 10-year-old girl who had a nocturnal rhythmic movement disorder, leading to prolonged repetitive neck...
KeywordsAmyotrophic Lateral Sclerosis Multifocal Motor Neuropathy Motor Unit Activation Potential Neck Flexion Sensory Nerve Action Potential
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