Spinal and cranial hypertrophic neuropathy in multiple sclerosis

• Published in: Muscle & nerve 2005-06, Vol.31 (6), p.772-779
• Main Authors: Quan, Dianna, Pelak, Victoria, Tanabe, Jody, Durairaj, Vikram, Kleinschmidt-Demasters, B.K.
• Format: Article
• Language: English
• Subjects: Adult; Axons - pathology; Biological and medical sciences; Brain - pathology; Brain - physiopathology; central demyelination; chronic inflammatory demyelinating polyneuropathy; Cranial Nerve Diseases - drug therapy; Cranial Nerve Diseases - etiology; Cranial Nerve Diseases - pathology; Cranial Nerves - pathology; Cranial Nerves - physiopathology; Cranial nerves. Spinal roots. Peripheral nerves. Autonomic nervous system. Gustation. Olfaction; Female; Graves Disease - immunology; Humans; Hypertrophy - etiology; Hypertrophy - pathology; Hypertrophy - physiopathology; Immunosuppressive Agents - therapeutic use; Magnetic Resonance Imaging; Male; Medical sciences; multiple sclerosis; Multiple Sclerosis - complications; Multiple Sclerosis - drug therapy; Multiple Sclerosis - pathology; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Myelin Sheath - immunology; Myelin Sheath - pathology; Nerve Fibers, Myelinated - immunology; Nerve Fibers, Myelinated - pathology; Nervous system (semeiology, syndromes); Neurology; onion bulb; peripheral demyelination; Peripheral Nervous System Diseases - drug therapy; Peripheral Nervous System Diseases - etiology; Peripheral Nervous System Diseases - pathology; polyradiculoneuropathy; Spinal Nerve Roots - pathology; Spinal Nerve Roots - physiopathology; Sural Nerve - pathology; Sural Nerve - physiopathology; Treatment Failure
• ISSN: 0148-639X; 1097-4598
• DOI: 10.1002/mus.20312

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. Muscle Nerve, 2005