Histiocytosis-X isolated to the cervical spinal cord

Histiocytosis-X may involve many tissues, including the central nervous system. This report is the first documentation of histiocytosis-X occurring exclusively in the spinal cord. We present a patient with cervical spinal cord disease causing spastic paraplegia, flaccid right arm weakness, and sensory loss. Magnetic resonance imaging of the spinal cord clearly demonstrated the entire lesion. Following surgical decompression and biopsy, the cervical spinal cord was treated with low-dose radiation, which resulted in complete regression of the lesion by radiologic assessment. The child became neurologically normal, and histiocytosis-X has not been detected in other organ systems.