Early and Aggressive Treatment May Modify Anti-Hu Associated Encephalitis Prognosis

Abstract

Anti-Hu encephalitis is a paraneoplastic syndrome in adults. In children, rare cases of anti-Hu encephalitis were reported mostly without underlying tumors and clinical outcome are usually severe. Here, we describe a 4-year-old girl who developed cerebellar syndrome with abnormal behavior. The brain magnetic resonance imaging showed several T2/fluid-attenuated inversion recovery bilateral brain lesions and autoimmune assessment showed positive anti-Hu antibodies. Computed tomography scan revealed ganglioneuroblastoma which was surgically removed 3 months after onset. Aggressive immunotherapy including dexamethasone, rituximab, and intravenous immunoglobulins were used and a marked neurological improvement soon after 9 months of onset was observed with the child being able to go back to school. The short delay between diagnosis and start of aggressive immunotherapy demonstrate the paramount importance of early diagnosis and early specific therapy after onset of symptoms.

Keywords

anti-Hu antibodies - limbic encephalitis - paraneoplastic syndrome - anti-ANNA-1

Ethical Approval

Both patients are part of the KidbioSEP cohort and parents have given written consent to participate.

Authors' Contributions

All authors contributed equally and agreed to the published version of the manuscript.

Publication History

Received: 19 December 2021

Accepted: 06 July 2022

Accepted Manuscript online:
11 July 2022

Article published online:
21 December 2022

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