Cervical Myelopathy secondary to Hirayama Disease in 16-year-old male




Rodgers, Abigail
Tandon, Saloni


0000-0003-2695-6433 (Tandon, Saloni)

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16-year-old male with past medical history of asthma presented to the ED with a chief complaint of months of persistent numbness, tingling and twitching sensations in his left calf and toes. The patient also noted weakness, paresthesia and loss of muscle mass of his left hand and wrist, and inability to straighten his fourth and fifth digit. Physical exam showed weakened hand grip and wrist flexion/extension with left forearm and intrinsic hand muscle atrophy. Patient had a positive Hoffman sign and brisk reflexes with no other neurologic deficits. CK elevated at 296. MRI of the brain showed volume loss with signal abnormality and enhancement in the lower cervical spinal cord (C4-C7). Findings were consistent with a non-acute, non-expansile myelopathy.

Hirayama Syndrome (HS) is a rare condition caused by anterior movement of the posterior dural sac of the cervical spine during neck flexion, resulting in cord compression. Although a self-limiting condition, HS can cause chronic motor disabilities, including weakness of the extremities and loss of fine motor movements: resulting in diffuse muscle atrophy and contractures. Early interventions are key to preventing complications. Cervical collars have shown to be very effective. Nerve conduction studies can show the extent of nerve loss and possibility for reinnervation.

If not treated early, cervical fusion is the only remaining intervention for HS, resulting in severe loss of cervical mobility, leading to functional, social and occupational disability. Education on prognosis and prevention of motor deficits will facilitate informed decision making, expedite diagnosis, and encourage patient compliance. Physical therapy has shown to not only reduce long term complications, but also help patients with residual motor deficits reach functional independence.