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

Division of Neurology, Aichi Medical University, Japan.
Neurology (Impact Factor: 8.3). 04/1988; 38(3):463-7. DOI: 10.1212/WNL.38.3.463
Source: PubMed

ABSTRACT 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.

  • [Show abstract] [Hide abstract]
    ABSTRACT: There are no validated criteria for the diagnosis of sensory neuronopathy (SNN) yet. In a preliminary monocenter study a set of criteria relying on clinical and electrophysiological data showed good sensitivity and specificity for a diagnosis of probable SNN. The aim of this study was to test these criteria on a French multicenter study. 210 patients with sensory neuropathies from 15 francophone reference centers for neuromuscular diseases were included in the study with an expert diagnosis of non-SNN, SNN or suspected SNN according to the investigations performed in these centers. Diagnosis was obtained independently from the set of criteria to be tested. The expert diagnosis was taken as the reference against which the proposed SNN criteria were tested. The set relied on clinical and electrophysiological data easily obtainable with routine investigations. 9/61 (16.4 %) of non-SNN patients, 23/36 (63.9 %) of suspected SNN, and 102/113 (90.3 %) of SNN patients according to the expert diagnosis were classified as SNN by the criteria. The SNN criteria tested against the expert diagnosis in the SNN and non-SNN groups had 90.3 % (102/113) sensitivity, 85.2 % (52/61) specificity, 91.9 % (102/111) positive predictive value, and 82.5 % (52/63) negative predictive value. Discordance between the expert diagnosis and the SNN criteria occurred in 20 cases. After analysis of these cases, 11 could be reallocated to a correct diagnosis in accordance with the SNN criteria. The proposed criteria may be useful for the diagnosis of probable SNN in patients with sensory neuropathy. They can be reached with simple clinical and paraclinical investigations.
    Journal of Neurology 08/2014; 261(11). DOI:10.1007/s00415-014-7423-7 · 3.84 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sensory neuronopathies (SNNs) encompass paraneoplastic, infectious, dysimmune, toxic, inherited, and idiopathic disorders. Recently described diagnostic criteria allow SNN to be differentiated from other forms of sensory neuropathy, but there is no validated strategy based on routine clinical investigations for the etiological diagnosis of SNN. In a multicenter study, the clinical, biological, and electrophysiological characteristics of 148 patients with SNN were analyzed. Multiple correspondence analysis and logistic regression were used to identify patterns differentiating between forms of SNNs with different etiologies. Models were constructed using a study population of 88 patients and checked using a test population of 60 cases. Four patterns were identified. Pattern A, with an acute or subacute onset in the four limbs or arms, early pain, and frequently affecting males over 60 years of age, identified mainly paraneoplastic, toxic, and infectious SNN. Pattern B identified patients with progressive SNN and was divided into patterns C and D, the former corresponding to patients with inherited or slowly progressive idiopathic SNN with severe ataxia and electrophysiological abnormalities and the latter to patients with idiopathic, dysimmune, and sometimes paraneoplastic SNN with a more rapid course than in pattern C. The diagnostic strategy based on these patterns correctly identified 84/88 and 58/60 patients in the study and test populations, respectively.
    Journal of the Peripheral Nervous System 09/2012; 17(3):331-340. DOI:10.1111/j.1529-8027.2012.00411.x · 2.50 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Des anticorps dirigés contre des antigènes intracellulaires du système nerveux périphérique ont été principalement décrits dans le cadre des syndromes neurologiques paranéoplasiques et incluent les anticorps anti-Hu et anti-CV2/CRMP5. Avec l’anticorps anti-Hu la neuropathie sensitive est la plus fréquente des manifestations cliniques. Elle est fréquemment associée à une atteinte du système nerveux autonome. Avec l’anticorps anti-CV2/CRMP5 la neuropathie est fréquemment sensitivomotrice avec un profil électrophysiologique axonal ou axono-myélinique. Les manifestions cliniques de ces neuropathies sont conformes à la distribution cellulaire dans le système nerveux périphérique d’HuD et de CRMP5. Bien que présents en titre élevé, ces anticorps ne sont probablement pas directement responsables de la neuropathie. Des études anatomopathologies et expérimentales indiquent que les cellules T cytotoxiques sont probablement les principaux effecteurs de la réponse immune. Ces maladies s’opposent donc à celles au cours desquelles des anticorps reconnaissant des antigènes de la surface cellulaire provoquent probablement le processus pathologique. La mort neuronale et la dégénérescence axonale qui résultent de l’activation de l’immunité cellulaire médiée par les cellules T expliquent que le traitement de ces neuropathies reste difficile.
    Revue Neurologique 10/2014; DOI:10.1016/j.neurol.2014.07.002 · 0.60 Impact Factor