Spinal and cranial hypertrophic neuropathy in multiple sclerosis.
Two patients with multiple sclerosis developed symptomatic chronic inflammatory demyelinating polyneuropathy with massive spinal or cranial nerve hypertrophy revealed by neuroimaging. Sural nerve biopsy in one showed only moderate demyelination, axonal loss, and onion-bulb formation, illustrating dichotomy between severe proximal and milder distal nerve involvement. Patients with coexistent central and peripheral demyelination usually are symptomatic from dysfunction at one site or the other, but not from both. Our patients showed minimal response to steroids, intravenous immunoglobulin, or azathioprine. These cases suggest that the mechanism of disease in symptomatic central and peripheral demyelination may differ from that of disease in only one region, and that optimal therapy in this situation must be explored further.
Quan
Dianna
D
Department of Neurology, University of Colorado Health Sciences Center, Box F727, 4200 East Ninth Avenue, Denver, Colorado 80262, USA. dianna.quan@uchsc.edu
Pelak
Victoria
V
Tanabe
Jody
J
Durairaj
Vikram
V
Kleinschmidt-Demasters
B K
BK
D000328:Adult; D001369:Axons / Q000473:pathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D003389:Cranial Nerve Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D003391:Cranial Nerves / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006111:Graves Disease / Q000276:immunology; D006801:Humans; D006984:Hypertrophy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D009103:Multiple Sclerosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D009186:Myelin Sheath / Q000276:immunology / Q000473:pathology; D009413:Nerve Fibers, Myelinated / Q000276:immunology / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D013126:Spinal Nerve Roots / Q000473:pathology* / Q000503:physiopathology; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology; D017211:Treatment Failure
D007166:Immunosuppressive Agents