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Chronic progressive sensory ataxic neuropathy with polyclonal gammopathy and disseminated focal perivascular cellular infiltrations

From the Division of Neurology (Dr. Sobue), Fourth Department of Internal Medicine, Aichi Medical University; the Department of Neurology (Dr. Yanagi), Nagoya Second Red Cross Hospital; and the Department of Pathology (Dr. Hashizume), Nagoya University School of Medicine, Aichi, Japan.

One autopsied case of chronic progressive sensory-ataxic neuropathy with polyclonal elevation of serum and CSF IgG and IgA and without malignancy is reported. A marked loss of large myelinated fibers was universal in both the central and peripheral rami of primary sensory neurons. Fiber loss showed a multifocal patchy pattern in the proximal nerve trunks. The posterior root ganglion cell bodies were moderately atrophic and loss of large cells was observed. Unmyelinated axons were well preserved. The ventral spinal roots, ventral spinal horn cells, and muscles showed minimal involvement. There were focal perivascular mononuclear inflammatory cells without necrotizing vasculitis around the endoneurial and epineurial vessels. Similar perivascular cellular invasions were observed in the visceral organs, occasionally forming germinal follicle centers. This case suggested that this neuropathy has a unique background with a possible immune-mediated basis.

Address correspondence and reprint requests to Dr. Sobue, Division of Neurology, Fourth Department of Internal Medicine, Aichi Medical University, Yazako, Nagakute-cho, Aichi-gun, 480–11, Aichi, Japan.

Received March 16, 1987. Accepted for publication in final form June 8, 1987.

This article has been cited by other articles:

				H. Takeuchi, K. Misu, N. Hattori, M. Nagamatsu, and G. Sobue Immunoglobulin therapy for idiopathic chronic sensory ataxic neuropathy Neurology, February 22, 2000; 54(4): 1008 - 1010. [Full Text] [PDF]