A 71-year-old male with 4 decades of symptoms referable to both central and peripheral nervous system.

May 2005. Combined demyelination of the central and peripheral nervous system is an uncommon disorder and has been referred to by many appellations. We present the case of a 71-year-old man with a progressive nervous system disorder beginning in his 30s. The diagnosis in life was unclear, but had over the years been variously considered to be Guillain-Barrè Syndrome (GBS), Friedreich ataxia, or multiple sclerosis (MS). At autopsy old CNS demyelination consistent with MS was found as well as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with onion bulb formation in hypertrophic nerves. Microscopic examination showed some onion bulb formation in the CNS as well as in peripheral nerves. The nosology of these disorders is discussed and relevant animal models briefly reviewed.