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

European Journal of Neurology Jul 01, 2020

Martinez ARM, de Lima FD, Martins MP, et al. - To clarify if sensory neuronopathy (SN) is an autoimmune hepatitis (AIH)‐specific manifestation or associated with chronic liver damage, researchers undertook this study enrolling 70 consecutive AIH patients. These patients were compared with 52 consecutive patients experiencing chronic active hepatitis B, in terms of characteristics. The groups demonstrated similar frequencies of carpal tunnel syndrome, radiculopathy and polyneuropathy. Only AIH patients had SN; the overall prevalence of AIH‐associated SN was estimated to be 7% with an average profile of a woman in her 40s with asymmetric start of sensory deficits that chronically progressed to disabling proprioceptive ataxia related to marked dysautonomia. In 3/5 (60%) of the patients having AIH‐associated SN, anti‐fibroblast growth factor receptor type 3 antibodies were detected. Overall, findings revealed sensory neuronopathy, but not other peripheral nervous system diseases, as a specific AIH neurological manifestation, which is commonly disabling and non-responsive to immunosuppression in contrast to hepatocellular injury.

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