Takahito Fujimori1, Akiko Tamura2, Toshitada Miwa1, Motoki Iwasaki3, Takenori Oda4. 1. Departments of Orthopedic Surgery, Sumitomo Hospital, Osaka, Japan. 2. Departments of Neurology, Sumitomo Hospital, Osaka, Japan. 3. Department of Orthopedic Surgery, Osaka Rosai Hospital, Osaka, Japan. 4. Department of Orthopedic Surgery, Osaka Minami Medical center, Osaka, Japan.
Abstract
INTRODUCTION: 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. CASE PRESENTATION: 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. However, her spastic gait and muscle atrophy remained. DISCUSSION: Most cases of cervical flexion myelopathy as represented by Hirayama disease have a self-limiting benign prognosis. However, some cases can develop advanced myelopathy with long tract signs. Long-term follow-up is recommended for these cases because they may require early surgical treatment.
INTRODUCTION: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. CASE PRESENTATION: 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. However, her spastic gait and muscle atrophy remained. DISCUSSION: Most cases of cervical flexion myelopathy as represented by Hirayama disease have a self-limiting benign prognosis. However, some cases can develop advanced myelopathy with long tract signs. Long-term follow-up is recommended for these cases because they may require early surgical treatment.