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Washington University Experience | PERIPHERAL NEUROPATHY | 12 ANTI-MAG NEUROPATHY | 3A0 Case 3 History
Case 3 History ---- In 1977, a 57-year-old man developed recurring and remitting symptoms of polyneuropathy beginning in the legs and then the arms. Family history was negative for neurological disease. Nerve conduction studies showed generalized demyelinating sensory/motor neuropathy with motor conduction velocities of 23 and 18 m/s in the median and ulnar nerves respectively, and unobtalnable sensory responses in the arms and legs. Investigations showed hypothyroidism but replacement therapy did not influence the polyneuropathy. Biopsy of the left sural nerve showed no inflammation, decreased number of fibers and onion bulbs. Records indicate presence of an IgM paraproteinemia which was not investigated further. Symptoms progressed slowly over the years with worsening numbness. increasing weakness of lower extremities and wide-based gait. A short trial of Prednisone treatment did not alter disease severity; however, the patient improved slightly over subsequent years spontaneously. At the age of 69 (1989), examination revealed minimal weakness, postural tremor. generalized areflexia, loss of vibration sense, ataxia of the lower extremities, and unsteady wide-based gait. Sensory responses were not obtainable. Motor conduction velocity was 15 m/s in the median nerve. At the age of 72 years (1993). the patient presented with fatigue. anemia, lymphadenopathy and inability to walk unassisted. Laboratory investigation revealed IgM/kappa paraprotein with a total IgM concentration of 54.5 gms per liter and relative plasma viscosity of 12.2. Lymph node and bone marrow biopsies revealed a diffuse small lymphocytic malignant lymphoma with a monoclonal plasmacytoid B cell population expressing immunoglobulin light chain kappa and immunoglobulin heavy chain IgM. Treatment with Chlorambucil and Prednisone was instituted with apparent remission of the lymphoma. Plasma exchange was performed to treat hyperviscosity syndrome. The patient noted marked subjective improvement. A second nerve biopsy was performed 16 years after the onset of his original symptoms (1993). The patient died in 1996 of disseminated lymphoma. Material Submitted: Semithin sections stained with toluidine blue of left sural nerve (1978) and right sural nerve (1993); and a lantern slide of an electron photo-micrograph. Images are shown subsequently of this patient with paraproteinemia early in her disease (3A1-3) and 16 years later (3B1-4). These images show the progression of axonal loss and an increase in onion-bulbs in these right and left sural nerve biopsies. (plastic sections).
