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

Autor: Martinez ARM; Department of Neurology - Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., de Lima FD; Department of Neurology - Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Martins MP; Department of Neurology - Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Pereira IE; Department of Internal Medicine - Gastroenterology Division (Gastrocentro), University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Miotto N; Department of Internal Medicine - Infectious Diseases Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Mazo DFC; Department of Internal Medicine - Gastroenterology Division (Gastrocentro), University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Vigani AG; Department of Internal Medicine - Infectious Diseases Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., da Costa LBE; Department of Pathology - Hepatic Disorders Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Stucchi RSB; Department of Internal Medicine - Infectious Diseases Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Almeida JRS; Department of Internal Medicine - Gastroenterology Division (Gastrocentro), University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., Nucci A; Department of Neurology - Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil., França MC Jr; Department of Neurology - Neuromuscular Division, University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil.
Jazyk: angličtina
Zdroj: European journal of neurology [Eur J Neurol] 2020 Oct; Vol. 27 (10), pp. 2072-2078. Date of Electronic Publication: 2020 Jun 25.
DOI: 10.1111/ene.14355
Abstrakt: 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.
(© 2020 European Academy of Neurology.)
Databáze: MEDLINE
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