Neuropediatrics 2023; 54(01): 064-067
DOI: 10.1055/a-1896-6687
Short Communication

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

Perrine Marion
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
,
Aliénor De Chalus
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
,
Laetitia Giorgi
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
,
Céline Bellesme
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
,
Pascale Crétien
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
,
Hélène Maurey
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
2   Pediatric Neurologic Department, National Referral Center for Rare Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
,
1   Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
2   Pediatric Neurologic Department, National Referral Center for Rare Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
3   Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bicêtre, France
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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.

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.




Publikationsverlauf

Eingereicht: 19. Dezember 2021

Angenommen: 06. Juli 2022

Accepted Manuscript online:
11. Juli 2022

Artikel online veröffentlicht:
21. Dezember 2022

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