A clinical, electrophysiological, morphological and immunological study of chronic sensory neuropathy with ataxia and paraesthesia.
We have observed 9 patients (8 men and 1 woman), 58 to 77 years of age with neuropathy with only sensory symptoms and insidious onset. Five of them (4 men and 1 woman) aged 65 to 77 years, had normal serum electrophoretic profiles, while the others (all men), 58 to 74 years, had IgM monoclonal gammopathy of undetermined significance (MGUS). Clinical data were consistent with a sensory neuropathy affecting predominantly the kinesthetic sense (position and vibration sensation). The electrophysiological data indicated predominant sensory axonal neuropathy. Morphological data confirmed the primary axonal damage. Western immunoblot showed that the IgG from a patient without MGUS reacted with a 55 kD protein of dorsal root ganglion homogenate. Three of four patients with IgM MGUS were serum reactive against chondroitin sulfate C (ChS-C) in double immunodiffusion. After absorption with ChS-C the monoclonal peak completely disappeared from two patients and was decreased in the third patient. Our data indicate that immunological abnormalities are part of the pathogenesis for a subgroup of chronic neuropathy with only sensory symptoms.
Mamoli
A
A
Neurology Division, Riuniti Hospital, Bergamo, Italy.
Nemni
R
R
Camerlingo
M
M
Quattrini
A
A
Castro
L
L
Lorenzetti
I
I
Canal
N
N
D000368:Aged; D001259:Ataxia / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D001706:Biopsy; D004576:Electromyography; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D009132:Muscles / Q000294:innervation; D009413:Nerve Fibers, Myelinated / Q000473:pathology / Q000502:physiology; D009422:Nervous System Diseases / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D009460:Neurologic Examination; D010292:Paresthesia / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D010543:Peroneal Nerve / Q000503:physiopathology; D012677:Sensation / Q000502:physiology*; D013497:Sural Nerve / Q000473:pathology / Q000503:physiopathology; D009435:Synaptic Transmission / Q000502:physiology
D007075:Immunoglobulin M