Neuropediatrics 2021; 52(05): 406-409
DOI: 10.1055/s-0040-1722677
Short Communication

Childhood-Onset Multifocal Motor Neuropathy with IgM Antibodies to Gangliosides GM1: A Case Report with Poor Outcome

Stefano Pro
1   Neurophysiology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Fabiana Ursitti
2   Child Neurology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Gian Luca Pruneddu
3   Paediatric Neurorehabilitation Units Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Matteo Di Capua
1   Neurophysiology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
,
Massimiliano Valeriani
2   Child Neurology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
› Author Affiliations

Abstract

Background Multifocal motor neuropathy (MMN) is a slowly progressive motor neuropathy characterized by asymmetric muscle weakness without sensory involvement. Typically, MMN respond completely to treatment with intravenous immunoglobulin (IVIg). MMN is even rarer in the pediatric population, where only five patients have been reported up to now.

Case Report We discuss the 3-year follow-up of a 13-year-old girl with MMN who was positive for IgM antibodies to gangliosides GM1. She was diagnosed with MMN in accordance with the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. Serological studies revealed that she tested positive for IgM antibodies to GM1. She underwent intravenous methylprednisolone followed by an oral prednisone taper, intravenous immunoglobulin (IVIg), plasma exchange followed by IVIG and prednisone and Rituximab. No improvement was referred. At the present, she shows flaccid tetraplegia, facial diplegia, and bulbar cranial nerve palsy.

Conclusion Although childhood onset MMN is rare, most patients reported in literature respond to IVIg treatment. In a few cases, however, IVIg can be ineffective. In our patient, IVIg as well as treatment with prednisolone, plasma exchange and rituximab have failed.



Publication History

Received: 18 June 2020

Accepted: 23 October 2020

Article published online:
28 January 2021

© 2021. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Vlam L, van der Pol WL, Cats EA. et al. Multifocal motor neuropathy: diagnosis, pathogenesis and treatment strategies. Nat Rev Neurol 2011; 8 (01) 48-58
  • 2 Van Asseldonk JT, Franssen H, Van den Berg-Vos RM, Wokke JH, Van den Berg LH. Multifocal motor neuropathy. Lancet Neurol 2005; 4 (05) 309-319
  • 3 Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J Peripher Nerv Syst 2010; 15 (04) 295-301
  • 4 Ramdas S, Prasad M, Spillane K, Kirkpatrick M. Acute motor neuropathy with pure distal involvement--a case report of multifocal motor neuropathy. Eur J Paediatr Neurol 2013; 17 (04) 415-418
  • 5 Edelman F, Naddaf E, Waclawik AJ. Upper extremity multifocal neuropathy in a 10-year-old boy associated with NS6S disaccharide antibodies. J Child Neurol 2015; 30 (07) 945-948
  • 6 Ishigaki H, Hiraide T, Miyagi Y. et al. Childhood-onset multifocal motor neuropathy with immunoglobulin M antibodies to gangliosides GM1 and GM2: a case report and review of the literature. Pediatr Neurol 2016; 62: 51-57
  • 7 Moroni I, Bugiani M, Ciano C, Bono R, Pareyson D. Childhood-onset multifocal motor neuropathy with conduction blocks. Neurology 2006; 66 (06) 922-924
  • 8 Maeda H, Ishii R, Kusunoki S, Chiyonobu T. Childhood-onset multifocal motor neuropathy with IgM antibodies to GM2 and GalNac-GD1a. Brain Dev 2020; 42 (01) 88-92
  • 9 Léger JM, Viala K, Cancalon F. et al. Intravenous immunoglobulin as short- and long-term therapy of multifocal motor neuropathy: a retrospective study of response to IVIg and of its predictive criteria in 40 patients. J Neurol Neurosurg Psychiatry 2008; 79 (01) 93-96
  • 10 Terenghi F, Cappellari A, Bersano A, Carpo M, Barbieri S, Nobile-Orazio E. How long is IVIg effective in multifocal motor neuropathy?. Neurology 2004; 62 (04) 666-668
  • 11 Van Asseldonk JT, Van den Berg LH, Kalmijn S. et al. Axon loss is an important determinant of weakness in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 2006; 77 (06) 743-747