Conus medulla—cauda compression from nerve root hypertrophy in a child with Dejerine—Sottas syndrome: improvement with laminectomy and duraplasty

Abstract

✓ This 7-year-old boy with Dejerine—Sottas syndrome caused by a mutation in the myelin protein zero gene began to suffer rapid deterioration with increasing leg weakness, loss of the ability to ambulate, and bowel and bladder incontinence. Magnetic resonance imaging of the spine revealed nerve root hypertrophy resulting in compression of the conus medullaris and cauda equina. Decompressive surgery was successful in reversing some of his deficits.