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.
"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. "
[Show abstract][Hide abstract] ABSTRACT: Acquired sensory neuronopathies encompass a group of paraneoplastic, dysimmune, toxic or idiopathic disorders characterized by degeneration of peripheral sensory neurons in dorsal root ganglia. As dorsal root ganglia cannot easily be explored, the clinical diagnosis of these disorders may be difficult. The question as to whether there exists a common clinical pattern of sensory neuronopathies, allowing the establishment of validated and easy-to-use diagnostic criteria, has not yet been addressed. In this study, logistic regression was used to construct diagnostic criteria on a retrospective study population of 78 patients with sensory neuronopathies and 56 with other sensory neuropathies. For this, sensory neuronopathy was provisionally considered as unambiguous in 44 patients with paraneoplastic disorder or cisplatin treatment and likely in 34 with a dysimmune or idiopathic setting who may theoretically have another form of neuropathy. To test the homogeneity of the sensory neuronopathy population, likely candidates were compared with unambiguous cases and then the whole population was compared with the other sensory neuropathies population. Criteria accuracy was checked on 37 prospective patients referred for diagnosis of sensory neuropathy. In the study population, sensory neuronopathy showed a common clinical and electrophysiological pattern that was independent of the underlying cause, including unusual forms with only patchy sensory loss, mild electrical motor nerve abnormalities and predominant small fibre or isolated lower limb involvement. Logistic regression allowed the construction of a set of criteria that gave fair results with the following combination: ataxia in the lower or upper limbs + asymmetrical distribution + sensory loss not restricted to the lower limbs + at least one sensory action potential absent or three sensory action potentials <30% of the lower limit of normal in the upper limbs + less than two nerves with abnormal motor nerve conduction study in the lower limbs.
[Show abstract][Hide abstract] ABSTRACT: A case of chronic, sporadic, slowly progressive, purely sensory, ataxic neuropathy is reported. In previously published similar cases only muscle and nerve biopsies have been available for study. In the present case the patient died of an unrelated illness 39 years after onset of the neuropathy. A full neuropathological study was performed. The disease process was limited to the dorsal root ganglia and their central and peripheral processes. Large myelinated fibers were preferentially involved. Involvement of the dorsal root ganglia has also been reported in certain toxic ganglioneuropathies and in the sensory neuropathy associated with carcinoma. The long duration and insidious development set the present case apart from those conditions. An inflammatory component was lacking. Except for a microscopic focus of adenocarcinoma of the prostate no malignancy was present. The etiology of chronic idiopathic ataxic neuropathy is unknown, but it is likely that the dorsal root ganglion is the main target for the disease process in most if not all cases.
[Show abstract][Hide abstract] ABSTRACT: Thirteen patients, 11 women and 2 men, developed sensory and autonomic neuronopathies in association with features of primary Sjögren's syndrome. In 11, Sjögren's syndrome had not been previously diagnosed at the time of neurological presentation. All had prominent loss of kinesthesia and proprioception. Pain and thermal sensibility were less severely affected. Most had evidence of autonomic insufficiency. In some this was severe, with Adie's pupils, fixed tachycardia, and orthostatic hypotension. The course ranged from an abrupt, devastating onset to indolent progression over years. Stabilization or functional improvement occurred in 6 patients, 2 of whom received no drug therapy. Sensory nerve conduction studies and examination of nerve biopsy specimens demonstrated a wide spectrum in the severity of loss of large myelinated fibers. The cutaneous nerves of 6 patients had perivascular mononuclear infiltrates without necrotizing arteritis. Examination of biopsy specimens of dorsal root ganglia in 3 patients revealed lymphocytic (T-cell) infiltration in the dorsal roots and ganglia, with focal clusters around neurons. In the more mildly affected ganglia, individual sensory neurons were undergoing degeneration. In the most advanced case, very few neurons remained. The possibility of Sjögren's syndrome should be considered in patients, especially women, who develop acute, subacute, or chronic sensory and autonomic neuropathies, with ataxia and kinesthetic loss.
Annals of Neurology 03/1990; 27(3):304-15. DOI:10.1002/ana.410270313 · 9.98 Impact Factor
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