Chronic inflammatory demyelinating polyradiculoneuropathy with tumefactive central demyelination.
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.
Krivickas
Lisa S
LS
Department of Physical Medicine and Rehabilitation, Harvard Medical School and Spaulding Rehabilitation Hospital, Boston, Massachusetts 02114, USA. lkrivickas@partners.org
Hochberg
Fred H
FH
Freeman
Stefanie
S
D000200:Action Potentials; D001369:Axons / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure; D001927:Brain Diseases / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D003711:Demyelinating Diseases / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D004568:Electrodiagnosis; D018737:Hand Strength; D006801:Humans; D007150:Immunohistochemistry; D008264:Macrophages / Q000737:chemistry / Q000473:pathology / Q000648:ultrastructure; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology; D018908:Muscle Weakness / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D009186:Myelin Sheath; D009431:Neural Conduction; D016900:Neurofilament Proteins / Q000032:analysis; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use
D016900:Neurofilament Proteins; D013256:Steroids