Incomplete Brown-SÉquard syndrome caused by cervical kyphosis secondary to neurofibromatosis: report of a case

Abstract

The case of a 17-year-old boy who suffered from incomplete Brown-SÉquard syndrome caused by dystrophic cervical kyphosis secondary to neurofibromatosis is presented. He became aware of weakness of his left upper and lower extremities and was unable to walk within 1 month of his first feeling numbness in the right upper and lower extremities. Thereafter his condition deteriorated rapidly. On radiological investigation the entire dural sac was shifted anteriorly and the spinal cord was flattened anteroposteriorly and asymmetrically at the angular kyphosis level, as in Hirayama's disease. Despite morphological similarities to Hirayama's disease, this patient presented with quite different clinical features: incomplete Brown-SÉquard syndrome and rapidly progressive symptoms. It is suspected that the myelopathy in this case can be attributed to compression of the spinal cord between the vertebral body and the posterior dural wall on only one side. He was successfully treated with posterior interspinous wiring combined with anterior decompression and fusion. The neurological deficit fully disappeared, and complete bony union was obtained 1 year after the operation.