Demyelinating Peripheral Neuropathy in Devic Disease

Abstract

This is a report of a 20‐year‐old man with typical features of Devic disease (neuromyelitis optica) associated with demyelinating peripheral neuropathy. A sural nerve biopsy showed markedly decreased myelinated fibers of a large diameter. Teased fiber preparations showed segmental remyelination in 50% of examined fibers, as well as a few demyelination. Demyelination of bilateral optic nerves, spinal cord, and peripheral nerves at the same time suggests a possibility of common pathogenetic mechanisms in both the central and peripheral nervous systems.

Abnormal Nerve Conduction Study Findings Indicating the Existence of Peripheral Neuropathy in Multiple Sclerosis and Neuromyelitis Optica

“…One (3.6%) of the 28 NMO patients showed peripheral neuropathy at the same time of NMO relapse. This clinical course is similar to those in two previous reports about the characteristics of peripheral neuropathy as a complication of NMO [ 7 , 8 ]. Aimoto et al [ 7 ] reported that demyelination of bilateral optic nerves, spinal cord, and peripheral nerves occurred at the same time in Devic's disease.…”

“…Neuromyelitis optica (NMO) is another inflammatory demyelinating disease of the CNS which is characterized by lesions confined to the optic nerve and spinal cord, especially longitudinally extensive spinal cord lesions [ 4 ], antiaquaporin-4 (AQP-4) autoantibody seropositivity [ 5 ], and astrocytic impairment associated with the loss of AQP-4 in NMO lesions [ 6 ]. There have been limited reports about the characteristics of peripheral neuropathy as a complication of NMO [ 7 , 8 ]. In this paper, we evaluated the electrophysiological changes with nerve conduction studies (NCS) in MS and NMO patients and showed the characteristics and differences between peripheral neuropathy as a complication of MS and NMO.…”

Acute Combined Central and Peripheral Demyelination Showing Anti-Aquaporin 4 Antibody Positivity

“…In the present case, the central and peripheral nervous tissues were involved simultaneously. In 1991, Aimoto et al (7) reported that demyelination of bilateral optic nerves, the spinal cord, and peripheral nerves occurred at the same time in Devic disease. However, an examination of anti-AQP4 antibodies was not performed in these patients.…”

Neuromyelitis Optica

“…However, some overlap may occur, as evidenced by one report of typical NMO with evidence of segmental demyelinating peripheral neuropathy demonstrated by teased fiber preparation of the sural nerve. 121…”