Spectrum of acquired sensory neuropathy: Clinical, electrodiagnostic, and pathologic studies
Clinical review of 33 patients with acquired predominantly sensory neuropathy defined by electrodiagnostic and clinical findings showed a igh female predominance (76%). Characteristically the neuropathy was asymmetric at onset (73%), evolved in a subacute (45%) or chronic fashion (40%), and started with upper extremity pain (67%). Ataxia (52%) and Adie’s pupils (18%) also seemed characteristic of this neuropathy. In 16 patients (48%) underlying causes such as cancer (12%), connective tissue disorders (9%), drug-induced (9%), and others (18%) were identified, but no etiology was determined in the rest of the patients (52%). Nonspecific serum and CSF immunological abnormalities were common (55%), suggesting underlying immune-mediated disorders in some patients. Electrodiagnostic studies suggested that sensory neuropathy was mainly an axon-loss disorder. The upper extremity preponderance and the asymmetric involvement were confirmed electrodiagnostically in some patients. Nerve biopsies showed scattered acute axonal degeneration and severe loss of myelinated fibers but well-preserved unmyelinated fibers. Corticosteroid or other immunotherapy was not beneficial in most patients treated. In all but two patients, the neuropathy was either progressive or unimproved over an average of 6.2 years of follow-up.
KeywordsSensory Neuropathy Myelinated Fiber Unmyelinated Fiber Sensory Nerve Action Potential Electrodiagnostic Study
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