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

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.

[Re-evaluation of clinical and electroencephalographical criteria of brain death determination in the child]. / Reavaliação dos critérios clínicos e eletrencefalográficos de determinação da morte cerebral na criança.

A case is reported in which there was a return of the electroencephalogram after a period of more than 36 hours of electrocerebral silence. Electroencephalographic technique and instrumentation were in accordance with the recommendations of Silverman, Saunders, Schwab and Masland . The possibilities of hypothermia or drug overdosage, known to reversibly depress the electroencephalogram, were excluded in the related case. The return of EEG activity was preceded by improvement in neurological status. It is concluded that criteria accepted for the determination of brain death in adults should not be valid for children.

Reavaliacao dos criterios clinicos e eletrencefalograficos de determinacao da morte cerebral na crianca. / Re-evalution of clinical and electroencephalographical criterion of brain death determination in the child

Descreve-se um caso de recuperacao temporaria de funcoes de SNC apos persistencia de coma profundo com apneia, midriase paralitica bilateral, arreatividade e silencio eletrico cerebral por cerca de 40 horas. O caso serve para dar enfase ao fato de que a determinacao da "morte cerebral" deve-se fundamentar na associacao de criterios e eletrencefalograficos. Por criterios clinicos ou eletrencefalograficos isolados o caso aqui descrito teria, ja nas primeiras 24 horas, cumprido os requisitos de determinacao da morte cerebral e, posivelmente, nao se teria observado o reaparecimento de atividade eletrica cerebral e reatividade clinica. Sugere-se que os criterios clinicos e eletrencefalograficos de determinacao da morte cerebral estabelecidos para o adulto nao sejam necessariamenvalidos para a crianca