Acute transverse myelitis following an opsoclonus-myoclonus syndrome: An unusual presentation. (September 2018)

• Record Type: Journal Article
• Authors: Simon, Thomas; Cheuret, Emmanuel; Fiedler, Léa; Mengelle, Catherine; Baudou, Eloïse; Deiva, Kumaran
• Is Part Of: European journal of paediatric neurology. Volume 22:Number 5(2018:Sep.)
• Page Start: 878
• Page End: 881
• Publication Date: 2018-09
• Subjects: HHV-6 -- Opsoclonus-myoclonus syndrome -- Transverse myelitis; OMS opsoclonus myoclonus syndrome -- HHV-6 Herpes human virus 6; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2018.05.002
• Languages: English
• ISSNs: 1090-3798

Abstract

Abstract: Opso-myoclonus syndrome (OMS) is a very rare and severe condition. Ataxia, opsoclonus, myoclonus and/or behavioral and sleeping disturbances define that autoimmune disorder syndrome which is paraneoplastic or triggered by an infection. Here, we report a 3 year-old immunocompetent boy who developed an atypical OMS which was later complicated by an acute transverse myelitis. Screening for neuroblastoma was negative and extensive infectious screening revealed an active HHV-6 infection confirmed by blood and cerebrospinal fluid PCR. A parainfectious disease was suggested and immunosuppressive treatment was initiated. After 2 years of follow-up, the patient has a left leg paresia needing a splint and is otherwise normal. Transverse myelitis can be associated with parainfectious OMS and earlier immunosuppressive treatment in these cases may be useful especially in young and immunocompetent children. Highlights: Opsoclonus myoclonus syndrome is rare and devastating disease. It is usually associated to paraneoplastic or para or postinfection conditions. Here, we describe an atypical OMS syndrome in a child associated with transverse myelitis following an HHV-6 infection.