Abstract
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.
MeSH terms
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Adult
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Axons / pathology
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Brain / pathology
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Brain / physiopathology
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Cranial Nerve Diseases / drug therapy
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Cranial Nerve Diseases / etiology*
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Cranial Nerve Diseases / pathology*
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Cranial Nerves / pathology
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Cranial Nerves / physiopathology
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Female
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Graves Disease / immunology
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Humans
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Hypertrophy / etiology
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Hypertrophy / pathology
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Hypertrophy / physiopathology
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Immunosuppressive Agents / therapeutic use
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Magnetic Resonance Imaging
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Male
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Multiple Sclerosis / complications*
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Multiple Sclerosis / drug therapy
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Multiple Sclerosis / pathology*
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Myelin Sheath / immunology
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Myelin Sheath / pathology
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Nerve Fibers, Myelinated / immunology
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Nerve Fibers, Myelinated / pathology
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Peripheral Nervous System Diseases / drug therapy
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Peripheral Nervous System Diseases / etiology*
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Peripheral Nervous System Diseases / pathology*
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Spinal Nerve Roots / pathology*
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Spinal Nerve Roots / physiopathology
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Sural Nerve / pathology
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Sural Nerve / physiopathology
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Treatment Failure