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

Division of Neurology, Aichi Medical University, Japan.
Neurology (Impact Factor: 8.29). 04/1988; 38(3):463-7. DOI: 10.1212/WNL.38.3.463
Source: PubMed


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.

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    • "Acquired sensory neuronopathies (SNN) or ganglionopathies encompass different disorders characterized by a primary degeneration of sensory neurons in dorsal root ganglia (Kuntzer et al., 2004; Sghirlanzoni et al., 2005). This has been pathologically demonstrated with paraneoplastic SNN (Graus et al., 1990; Dalmau et al., 1991; Wanschitz et al., 1997), HIV infection (Scaravilli et al., 1992; Esiri et al., 1993), Sjö gren's syndrome, unclassified connective diseases and rare idiopathic cases (Okajima et al., 1983; Sobue et al., 1988; Griffin et al., 1990; Hainfellner et al., 1996; Kurokawa et al., 1998; Colli et al., 2008). Interestingly, in all of these circumstances, dorsal root ganglia degeneration was associated with an inflammatory T-cell reaction suggesting that the disorder is mainly driven by a cellmediated immune response. "
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