[Peripheral neuropathy with monoclonal gammopathy].
The case is reported of a patient with IgM-kappa paraproteinemia and polyneuropathy (PN) with very slow nerve conduction velocities. The association of demyelinating PN with severely decreased nerve conduction velocities and an IgM-kappa paraprotein usually suggests the diagnosis of monoclonal gammopathy of undetermined significance (MGUS), but less often of clinically manifest macroglobulinemia. Demyelinating PN with IgG-lambda- or IgA-lambda paraproteinemia occurs often in the uncommon but readily treatable osteosclerotic myeloma. A monoclonal protein combined with peripheral neuropathy involving predominant pain, temperature and autonomic loss (small fibre neuropathy) is by and large pathognomonic for primary systemic amyloidosis or amyloidosis associated with multiple myeloma or Waldenström's macroglobulinemia. Mild sensorimotor axonal polyneuropathy is an unspecific syndrome found in all gammopathies. It is important to differentiate between paraproteinemia with clinically pure chronic sensory PN and paraneoplastic sensory polyneuropathy. Unlike amyotrophic lateral sclerosis (ALS), paraproteinemias associated with a motor neuron disease-like syndrome sometimes evolve unusually slowly and exhibit neurophysiologically subclinical lesions of sensory nerve fibers. Mononeuropathy or multifocal neuropathy with a monoclonal protein is typical of cryoglobulinemia. Demyelinating PN of osteosclerotic myelomas presenting with one or a small number of bone lesions usually regress after excision or irradiation of the plasma cell proliferations. Immunosuppressants and plasmapheresis in the treatment of the demyelinating IgM-kappa neuropathies, of paraproteinemia with the motor neuron disease-like syndrome, and of mononeuropathy or multifocal neuropathy with cryoglobulinemia, are of doubtful value.(ABSTRACT TRUNCATED AT 250 WORDS)
Meier
T
T
Neurologische Klinik, Universitätsspital, Zürich.
Meyer
M
M
D003711:Demyelinating Diseases / Q000209:etiology / Q000503:physiopathology; D003937:Diagnosis, Differential; D006801:Humans; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D010265:Paraproteinemias / Q000150:complications*; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*
D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains
Periphere Neuropathien mit monoklonaler Gammopathie.