Dados do Trabalho

Título

HIRAYAMA DISEASE. CASE REPORT.

Descrição sucinta do(s) objetivo(s)

To describe the case of a young patient with localized atrophy of the lower cervical spinal cord and myelopathy, secondary to displacement of the posterior thecal sac visualized in a dynamic sequence imaging.

História clínica

An 18-year-old male, with no relevant personal or family history.
He came to the traumatology unit due to gradual loss of muscle mass and weakness in both hands.
Physical examination revealed marked tenar and hypothenar atrophy in both hands, fifth finger in claw position and the rest of the fingers in semi-claw position. Muscle strength 4/5. Symmetrical reflexes. No damage to cranial nerves, pyramidal signs, sensory disturbances.
Electromyography indicates active and chronic denervation in myotomes (C6-C8-D1), bilateral, suggestive of anterior horn damage.
A magnetic resonance imaging was performed in neutral position as well as in bending, showing a thinned spinal cord with signs of myelopathy from C5 to C7. In bending there is an anterior displacement of the posterior theca from C4 to D1 with expansion of the dorsal epidural space.

Discussão e diagnóstico

Hirayama disease is a rare type of cervical myelopathy related to neck bending characterized by progressive muscle weakness and atrophy of the distal upper extremities that is most commonly seen in young men.
It is believed to be secondary to abnormal anterior displacement of the posterior dura mater with secondary compression of the lower cervical spinal cord and chronic anterior horn injury.
A dynamic magnetic resonance imaging is a diagnostic tool that visualizes the forward displacement of the posterior dural sac during neck bending, causing anteroposterior flattening and asymmetric atrophy of the lower cervical spinal cord.

Conclusões

The visualization of localized atrophy in the lower cervical spinal cord, associated or not with myelopathy, without obvious compressive cause, in young patients leads to the suspicion of this diagnosis, which may increase the detection rate. An additional magnetic resonance imaging study in neck bending should be scheduled.

Palavras Chave

HIRAYAMA DISEASE; ATROPHY CERVICAL SPINAL CORD

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