Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children

Nail Benallegue; Frédérique Beaudonnet; Béatrice Husson; Despina Moshous; Patrick Van Bogaert; Kumaran Deiva

doi:10.1016/j.ejpn.2021.08.002

Neurological involvement in secondary hemophagocytic lymphohistiocytosis in children

Eur J Paediatr Neurol. 2021 Sep:34:110-117. doi: 10.1016/j.ejpn.2021.08.002. Epub 2021 Aug 23.

Authors

Nail Benallegue¹, Frédérique Beaudonnet², Béatrice Husson³, Despina Moshous⁴, Patrick Van Bogaert⁵, Kumaran Deiva⁶

Affiliations

¹ Pediatric Department, Nantes University Hospital, Nantes, France. Electronic address: nail.benallegue@univ-nantes.fr.
² Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Site Bicêtre, Pediatric Neurology Department, National Reference Centre for Rare Inflammatory and Auto-immune Brain and Spinal Diseases, Le Kremlin Bicêtre. Electronic address: fbeaudonnet@ch-lagny77.fr.
³ Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Site Bicêtre, Pediatric Neurology Department, National Reference Centre for Rare Inflammatory and Auto-immune Brain and Spinal Diseases, Le Kremlin Bicêtre. Electronic address: beatrice.husson@aphp.fr.
⁴ Pediatric Immunology, Hematology and Rheumatology Unit, Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Laboratory « Genome Dynamics in the Immune System », Imagine Institute Paris, INSERM UMR1163, University of Paris, Paris, France. Electronic address: despina.moshous@inserm.fr.
⁵ Department of Pediatric Neurology, Angers University Hospital, Angers, France; LARIS Laboratoire Angevin de Recherche en Ingenierie des Systémes, University of Angers, France. Electronic address: Patrick.VanBogaert@chu-angers.fr.
⁶ Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris Saclay, Site Bicêtre, Pediatric Neurology Department, National Reference Centre for Rare Inflammatory and Auto-immune Brain and Spinal Diseases, Le Kremlin Bicêtre; UMR1184 « Immunology of Viral Infections and Autoimmune Diseases », CEA, IDMIT, Faculty of Medicine Paris-Saclay, France. Electronic address: kumaran.deiva@aphp.fr.

PMID: 34482125
DOI: 10.1016/j.ejpn.2021.08.002

Abstract

Objectives: To describe neurological characteristics and CNS involvement on MRI in secondary hemophagocytic lymphohistiocytosis (sHLH) and differentiate it from primary hemophagocytic lymphohistiocytosis (pHLH) and acute disseminated encephalomyelitis (ADEM).

Methods: Nine children with sHLH who had neurological symptoms were retrospectively included. Characteristics of brain MRI were compared to those of 15 children with pHLH and neurological involvement and 44 children with ADEM.

Results: Three children (33%) presented with isolated neurological symptoms. Neurological signs occurred within one month following Epstein-Barr virus primary infection or systemic juvenile arthritis exacerbation in 8 patients (89%). Eight children (89%) had MRI lesions. sHLH MRI lesions were distinct of those of pHLH by morphology and signal with more frequent hyposignal intensities on T1-weighted sequences (p = 0.01) and well-defined and less fuzzy lesions (p = 0.03). All patients survived and one patient presented severe motor and cognitive disability.

Conclusion: Neurological symptoms of sHLH are non-specific and their outcome is favorable in most of the children. MRI at onset may help to differentiate this condition from pHLH.

Keywords: Acute disseminated encephalomyelitis; CNS; Demyelination; Hemophagocytic lymphohistiocytosis; Pediatric.

MeSH terms

Child
Epstein-Barr Virus Infections* / complications
Herpesvirus 4, Human
Humans
Lymphohistiocytosis, Hemophagocytic* / complications
Lymphohistiocytosis, Hemophagocytic* / diagnosis
Magnetic Resonance Imaging
Retrospective Studies