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

Perrine Marion; Aliénor De Chalus; Laetitia Giorgi; Céline Bellesme; Pascale Crétien; Hélène Maurey; Kumaran Deiva

doi:10.1055/a-1896-6687

Neuropediatrics, Table of Contents

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

Authors

Perrine Marion

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
Aliénor De Chalus

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
Laetitia Giorgi

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
Céline Bellesme

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
Pascale Crétien

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France
Hélène Maurey

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France

²Pediatric Neurologic Department, National Referral Center for Rare Brain and Spinal Diseases, Le Kremlin-Bicêtre, France
Kumaran Deiva

¹Pediatric Neurology Department, Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Le Kremlin Bicêtre, France

²Pediatric Neurologic Department, National Referral Center for Rare Brain and Spinal Diseases, Le Kremlin-Bicêtre, France

³Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bicêtre, France

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

Full Text

References

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