Download PDF
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
,
Kumaran Deiva 
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
› Author Affiliations
› Further Information
    Permissions and Reprints

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

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

  • References:

  • 1 Graus F, Keime-Guibert F, Reñe R. et al. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain 2001; 124 (Pt 6): 1138-1148
    CrossrefPubMedGoogle Scholar
  • 2 Vogrig A, Gigli GL, Segatti S. et al. Epidemiology of paraneoplastic neurological syndromes: a population-based study. J Neurol 2020; 267 (01) 26-35
    CrossrefPubMedGoogle Scholar
  • 3 Senties-Madrid H, Vega-Boada F. Paraneoplastic syndromes associated with anti-Hu antibodies. Isr Med Assoc J 2001; 3 (02) 94-103
    PubMedGoogle Scholar
  • 4 Langer JE, Lopes MBS, Fountain NB. et al. An unusual presentation of anti-Hu-associated paraneoplastic limbic encephalitis. Dev Med Child Neurol 2012; 54 (09) 863-866
    CrossrefPubMedGoogle Scholar
  • 5 Haberlandt E, Bast T, Ebner A. et al. Limbic encephalitis in children and adolescents. Arch Dis Child 2011; 96 (02) 186-191
    CrossrefPubMedGoogle Scholar
  • 6 Silsby M, Clarke CJ, Lee K, Sharpe D. Anti-Hu limbic encephalitis preceding the appearance of mediastinal germinoma by 9 years. Neurol Neuroimmunol Neuroinflamm 2020; 7(03):
    PubMedGoogle Scholar
  • 7 Graus F, Delattre JY, Antoine JC. et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004; 75 (08) 1135-1140
    CrossrefPubMedGoogle Scholar
  • 8 Cellucci T, Van Mater H, Graus F. et al. Clinical approach to the diagnosis of autoimmune encephalitis in the pediatric patient. Neurol Neuroimmunol Neuroinflamm 2020; 7 (02) e663
    CrossrefPubMedGoogle Scholar
  • 9 Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 2000; 123 (Pt 7): 1481-1494
    CrossrefPubMedGoogle Scholar
  • 10 Rossor T, Yeh EA, Khakoo Y. et al; OMS Study Group. Diagnosis and management of opsoclonus-myoclonus-ataxia syndrome in children: an international perspective. Neurol Neuroimmunol Neuroinflamm 2022; 9 (03) e1153
    CrossrefPubMedGoogle Scholar
  • 11 Gable MS, Sheriff H, Dalmau J, Tilley DH, Glaser CA. The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. Clin Infect Dis 2012; 54 (07) 899-904
    CrossrefPubMedGoogle Scholar
  • 12 Montaut S, Mallaret M, Laguna AE. et al. Anti-Hu-associated brainstem encephalitis with ganglioneuroblastoma in a young adult. J Neurol 2014; 261 (09) 1822-1824
    CrossrefPubMedGoogle Scholar
  • 13 Sweeney M, Sweney M, Soldán MMP, Clardy SL. Antineuronal nuclear autoantibody type 1/anti-Hu-associated opsoclonus myoclonus and epilepsia partialis continua: case report and literature review. Pediatr Neurol 2016; 65: 86-89
    CrossrefPubMedGoogle Scholar
  • 14 Honnorat J, Didelot A, Karantoni E. et al. Autoimmune limbic encephalopathy and anti-Hu antibodies in children without cancer. Neurology 2013; 80 (24) 2226-2232
    CrossrefPubMedGoogle Scholar
  • 15 Aravamuthan BR, Sánchez Fernández I, Zurawski J, Olson H, Gorman M, Takeoka M. Pediatric anti-Hu-associated encephalitis with clinical features of Rasmussen encephalitis. Neurol Neuroimmunol Neuroinflamm 2015; 2 (05) e150
    CrossrefPubMedGoogle Scholar
  • 16 Sabanathan S, Abdel-Mannan O, Mankad K. et al. Clinical features, investigations, and outcomes of pediatric limbic encephalitis: a multicenter study. Ann Clin Transl Neurol 2022; 9 (01) 67-78
    CrossrefPubMedGoogle Scholar
  • 17 Keime-Guibert F, Graus F, Fleury A. et al. Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone. J Neurol Neurosurg Psychiatry 2000; 68 (04) 479-482
    CrossrefPubMedGoogle Scholar
  • 18 Posner JB. Anti-Hu autoantibody associated sensory neuropathy/encephalomyelitis: a model of paraneoplastic syndrome. Perspect Biol Med 1995; 38 (02) 167-181
    CrossrefPubMedGoogle Scholar
  • 19 Berzero G, Karantoni E, Dehais C. et al. Early intravenous immunoglobulin treatment in paraneoplastic neurological syndromes with onconeural antibodies. J Neurol Neurosurg Psychiatry 2018; 89 (07) 789-792
    CrossrefPubMedGoogle Scholar
  • 20 de Alarcon PA, Matthay KK, London WB. et al. Intravenous immunoglobulin with prednisone and risk-adapted chemotherapy for children with opsoclonus myoclonus ataxia syndrome associated with neuroblastoma (ANBL00P3): a randomised, open-label, phase 3 trial. Lancet Child Adolesc Health 2018; 2 (01) 25-34
    CrossrefPubMedGoogle Scholar
  • 21 Fisher PG, Wechsler DS, Singer HS. Anti-Hu antibody in a neuroblastoma-associated paraneoplastic syndrome. Pediatr Neurol 1994; 10 (04) 309-312
    CrossrefPubMedGoogle Scholar
  • 22 Morales La Madrid A, Rubin CM, Kohrman M, Pytel P, Cohn SL. Opsoclonus-myoclonus and anti-Hu positive limbic encephalitis in a patient with neuroblastoma. Pediatr Blood Cancer 2012; 58 (03) 472-474
    CrossrefPubMedGoogle Scholar