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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

Sensory neuronopathy is a specific and disabling neurological manifestation of autoimmune hepatitis

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Author(s):
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Martinez, A. R. M. [1] ; de Lima, F. D. [1] ; Martins, M. P. [1] ; Pereira, I. E. [2] ; Miotto, N. [3] ; Mazo, D. F. C. [2] ; Vigani, A. G. [3] ; da Costa, L. B. E. [4] ; Stucchi, R. S. B. [3] ; Almeida, J. R. S. [2] ; Nucci, A. [1] ; Franca Jr, M. C.
Total Authors: 12
Affiliation:
[1] Univ Estadual Campinas, UNICAMP, Neuromuscular Div, Dept Neurol, Campinas, SP - Brazil
[2] Univ Estadual Campinas, UNICAMP, Dept Internal Med, Gastroenterol Div, Gastroctr, Campinas, SP - Brazil
[3] Univ Estadual Campinas, UNICAMP, Dept Internal Med, Infect Dis Div, Campinas, SP - Brazil
[4] Univ Estadual Campinas, UNICAMP, Dept Pathol, Hepat Disorders Div, Campinas, SP - Brazil
Total Affiliations: 4
Document type: Journal article
Source: EUROPEAN JOURNAL OF NEUROLOGY; v. 27, n. 10 JUN 2020.
Web of Science Citations: 0
Abstract

Background and purpose Neurological manifestations have been identified in the context of autoimmune hepatitis (AIH). Previous case reports highlighted the association between AIH and sensory neuronopathy (SN). Despite that, little is known about the frequency of AIH-related SN and its clinical/neurophysiological profile. Moreover, it is not clear whether SN is an AIH-specific manifestation or related to chronic liver damage. Methods Seventy consecutive AIH patients were enrolled and their characteristics were compared with 52 consecutive patients with chronic active hepatitis B. All subjects underwent clinical and neurophysiological evaluation. Further comparisons were performed between AIH SN and AIH non-SN patients. Results Mean ages and male:female proportions in the AIH and chronic active hepatitis B groups were 42.2 +/- 16.3/51.7 +/- 13.6 years and 14:56/29:23, respectively. The frequencies of carpal tunnel syndrome, radiculopathy and polyneuropathy were similar between groups. In contrast, SN was identified only in AIH patients (5/70 vs. 0/52,P = 0.04); the overall prevalence of AIH-related SN was 7% with an average profile of a woman in her 40s with asymmetric onset of sensory deficits that chronically evolved to disabling proprioceptive ataxia associated with marked dysautonomia. Neurological disability and hepatocellular damage did not follow in parallel. Anti-fibroblast growth factor receptor type 3 antibodies were found in 3/5 (60%) of the patients with AIH-related SN. Clinical or demographic predictors of SN in the context of AIH could not be identified. Conclusion Sensory neuronopathy, but not other peripheral nervous system diseases, is a specific AIH neurological manifestation. It is often disabling and, in contrast to hepatocellular injury, does not respond to immunosuppression. (AU)

FAPESP's process: 13/01766-7 - Sensory neuronopathies: investigation of new diagnostic methods, mechanisms of the disease and therapeutic strategies
Grantee:Marcondes Cavalcante Franca Junior
Support Opportunities: Research Grants - Young Investigators Grants
FAPESP's process: 13/26410-0 - Clinical, immunological and neurophysiological characterization of sensory neuronopathies
Grantee:Alberto Rolim Muro Martinez
Support Opportunities: Scholarships in Brazil - Doctorate (Direct)