Cervical cord tethering mimicking focal muscular atrophy

Spinal cord tethering rarely occurs in the cervical region. In adults, it u sually results from previous operations. However, congenital origin is alwa ys diagnosed and treated early in the infant period. We report a 12-year-ol d boy with cervical spinal dysraphism which was erroneously diagnosed as fo cal muscular atrophy, a benign form of motor neuron disease. The patient wa s brought to our hospital because of rapid deterioration of symptoms. Caref ul evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C- 4 to C-6 levels and fusion of the laminae of C-4 and C-5. Spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and t he ventral nerve roots were markedly stretched, especially over the left si de. Surgical intervention was undertaken and the patient's muscle power imp roved after untethering. The purpose of this report is to acquaint the read er with a surgically treatable condition that may appear to be benign focal amyotrophy. Skin lesion at the nuchal area should be carefully looked for.