Abstract
A 46-year-old man with a 1-year history of distal paresthesias and mild distal weakness subacutely developed paralysis of the left hand. Electrodiagnostic evaluation revealed a demyelinating peripheral neuropathy that met criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Magnetic resonance imaging of the brain revealed a mass that enhanced with contrast, but revealed focal myelin loss with intense macrophage activity and axonal preservation on biopsy. The mass and hand weakness improved following steroid therapy. The combination of CIDP and central demyelination with mass effect broadens the spectrum of demyelinating disease in association with CIDP.
MeSH terms
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Action Potentials
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Axons / chemistry
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Axons / pathology
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Axons / ultrastructure
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Brain Diseases / complications*
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Brain Diseases / diagnosis*
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Brain Diseases / pathology
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Demyelinating Diseases / complications*
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Demyelinating Diseases / diagnosis*
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Demyelinating Diseases / pathology
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Electrodiagnosis
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Hand Strength
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Humans
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Immunohistochemistry
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Macrophages / chemistry
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Macrophages / pathology
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Macrophages / ultrastructure
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Motor Neurons / physiology
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Muscle Weakness / diagnosis
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Muscle Weakness / drug therapy
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Muscle Weakness / physiopathology
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Myelin Sheath
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Neural Conduction
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Neurofilament Proteins / analysis
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Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / complications*
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Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
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Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology
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Steroids / therapeutic use
Substances
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Neurofilament Proteins
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Steroids