Faculty Bibliography

Some have suggested that nonfamilial motor neuron disease (MND) may be autoimmune, and the neurological disorder may benefit from immunotherapy. There have been reports of over 30 cases of lymphoproliferative disease (lymphoma, multiple myeloma, Waldenstrom's macroglobulinemia) with MND, and these patients might he offered immunosuppressive therapy. Bone marrow examination might increase the sensitivity of the diagnostic workup for lymphoma and other lymphoproliferative disorders. We examined the bone marrow in our first evaluation of 161 patients with MND seen at Columbia-Presbyterian Medical Center during 1991-1994. Four of 161 patients (2.5%) had lymphoproliferative disease in the marrow; only 1 of these had a monoclonal paraprotein. Routine bone marrow examination of patients with MND increases the diagnostic yield of lymphoproliferative diseases. The frequency of these bone marrow abnormalities in comparison with a group of age-matched control subjects should be studied further

Progress has been made in the diagnosis of neuromuscular disease over the past several decades in electrophysiology, muscle and nerve immunocytochemistry, molecular genetics and immunology. This is the first in a series of articles on the diagnosis and therapy of the neuromuscular diseases. $$:

We performed quantitative immunohistochemical studies of sural nerve biopsy specimens from 20 patients to determine whether endoneurial and epineurial lymphocytic infiltration occurs in diabetic nerves. The diabetic nerves contained a mean of 129 CD3+ cells per tissue section compared to 19 cells in patients with chronic neuropathy matched for the histologic severity of disease, and 0-5 cells in normal control nerves. The T-cell infiltrates in the diabetic nerves were predominantly of the CD8+ cell type. Activated endoneurial lymphocytes expressed immunoreactive cytokines and major histocompatibility class II antigens. Microvasculitis was found in 12 (60%) patients. Infiltrative T cells may contribute to the pathogenesis of diabetic neuropathy through a variety of effector mechanisms