A Case Report on Juvenile Neuromyelitis Optica: Early Onset, Long Remission Period, and Atypical Treatment Response

Abstract

Neuromyelitis optica (NMO) is a severe inflammatory demyelinating disease of the central nervous system and preferentially targets the optic nerves and spinal cord. NMO is rare in children and clinical course of the disease is highly variable as described in studies. Here, we present a case report of a young girl presenting with a rare course of pediatric NMO with an early disease onset at the age of 12 years, a relapse free interval of 4 years, evidence of NMO immunoglobulin G (IgG) and an unusual response against immunosuppressive therapy. The aim of this report is to highlight the potentially long remission period between relapses complicating proper diagnosis despite well defined diagnostic criteria. In addition, we want to encourage the use of rituximab in pediatric NMO, although larger cohorts are warranted to establish B cell depleting therapies in juvenile NMO.

Keywords

neuromyelitis optica - aquaporin 4 (NMO–IgG) - rituximab

• References

• 1 Wingerchuk DM. Diagnosis and treatment of neuromyelitis optica. Neurologist 2007; 13 (1) 2-11
• 2 Trebst C, Jarius S, Berthele A , et al; Neuromyelitis Optica Study Group (NEMOS). Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS). J Neurol 2014; 261 (1) 1-16
• 3 Bomprezzi R, Postevka E, Campagnolo D, Vollmer TL. A review of cases of neuromyelitis optica. Neurologist 2011; 17 (2) 98-104
• 4 Huppke P, Blüthner M, Bauer O , et al. Neuromyelitis optica and NMO-IgG in European pediatric patients. Neurology 2010; 75 (19) 1740-1744
• 5 Absoud M, Lim MJ, Appleton R , et al. Paediatric neuromyelitis optica: clinical, MRI of the brain and prognostic features. J Neurol Neurosurg Psychiatry 2015; 86 (4) 470-472
• 6 Banwell B, Tenembaum S, Lennon VA , et al. Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Neurology 2008; 70 (5) 344-352
• 7 Sato DK, Callegaro D, Lana-Peixoto MA , et al. Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders. Neurology 2014; 82 (6) 474-481
• 8 Rostasy K, Reindl M. Role of autoantibodies in acquired inflammatory demyelinating diseases of the central nervous system in children. Neuropediatrics 2013; 44 (6) 297-301
• 9 Costanzi C, Matiello M, Lucchinetti CF , et al. Azathioprine: tolerability, efficacy, and predictors of benefit in neuromyelitis optica. Neurology 2011; 77 (7) 659-666
• 10 Tenembaum SN. Treatment of multiple sclerosis and neuromyelitis optica in children and adolescents. Clin Neurol Neurosurg 2013; 115 (Suppl. 01) S21-S29
• 11 Mealy MA, Wingerchuk DM, Palace J, Greenberg BM, Levy M. Comparison of relapse and treatment failure rates among patients with neuromyelitis optica: multicenter study of treatment efficacy. JAMA Neurol 2014; 71 (3) 324-330
• 12 Beres SJ, Graves J, Waubant E. Rituximab use in pediatric central demyelinating disease. Pediatr Neurol 2014; 51 (1) 114-118
• 13 Pellkofer HL, Krumbholz M, Berthele A , et al. Long-term follow-up of patients with neuromyelitis optica after repeated therapy with rituximab. Neurology 2011; 76 (15) 1310-1315
• 14 Dale RC, Brilot F, Duffy LV , et al. Utility and safety of rituximab in pediatric autoimmune and inflammatory CNS disease. Neurology 2014; 83 (2) 142-150
• 15 Kim SH, Kim W, Li XF, Jung IJ, Kim HJ. Repeated treatment with rituximab based on the assessment of peripheral circulating memory B cells in patients with relapsing neuromyelitis optica over 2 years. Arch Neurol 2011; 68 (11) 1412-1420