Severe cervical flexion myelopathy with long tract signs: a case report and a review of literature

Spinal Cord Series and Cases
Takahito FujimoriTakenori Oda

Abstract

Hirayama disease, a type of cervical flexion myelopathy, is a rare neurological disease characterized by muscular atrophy of the forearms and hands. Generally, the pathology is limited to the gray matter of the anterior horns in the lower cervical spinal cord. However, in rare cases the damage can spread to the white matter and present as long tract signs. We report on a 30-year-old female whose onset presented as unilateral muscle atrophy of the right hand in her teens. Despite conservative treatment using a cervical collar, she developed prolonged bilateral muscle atrophy, sensory disturbance and spastic gait, along with bladder and rectal disturbances. Her hands were frozen into a 'claw-like' gesture and her intrinsic muscles were highly atrophic. She was unable to unclench her hands. Although the space available for the spinal cord was large, the spinal cord was highly atrophic. She had local kyphosis with a large (61°) flexion range of motion. During flexion the spinal cord was stretched, resulting in contact with the posterior wall of the vertebrae. Posterior fusion surgery was performed to prevent progression of the myelopathy. After surgery, she gained mild improvement in both muscle strength and her hand's movement. Ho...Continue Reading

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Citations

Sep 25, 2017·Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration·Kiran PolavarapuAtchayaram Nalini
Apr 22, 2018·European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society·Takeo Furuya
Apr 8, 2020·Frontiers in Neurology·Annibale AntonioniEnrico Granieri

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