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NMDA receptor encephalitis : case report A 12-year-old girl developed anti-N-methyl-d-aspartate receptor (NMDAr) encephalitis following treatment with etanercept, tocilizumab and abatacept for juvenile idiopathic arthritis [not all routes stated; dosages and time to reaction onset not stated]. The girl had been receiving IV abatacept every 3 weeks for juvenile idiopathic arthritis. Her previous immune modulating treatment included methotrexate (since the age of 2 years), etanercept (age 5–8 years) and tocilizumab (aged 8–10 years) for juvenile idiopathic arthritis. Flare of juvenile idiopathic arthritis following a period of control led to the discontinuation of methotrexate, etanercept and tocilizumab. Three years following the initiation of abatacept, she presented to hospital with a generalised tonic-clonic seizure on background of preceding headache and fatigue. The seizure self-terminated but disorientation and encephalopathy ensued. An MRI of the brain demonstrated lesions with high signal intensity in the left periventricular and right occipital regions. The clinical picture and radiological imaging suggested a broad differential diagnosis including CNS infection, JC virus associated progressive multifocal leucoencephalomalacia, acute disseminated encephalomyelitis and NMDAr encephalitis. The girl received treatment with ceftriaxone, acyclovir and clarithromycin. Both inflammatory markers and infectious serology [details not stated] were unremarkable. CSF was acellular and JC virus PCR negative. NMDAr encephalitis was initially considered unlikely (pre-existing immunosuppression, abnormal MRI brain and no evidence of ovarian teratoma on abdominal and pelvic MRI). However, she subsequently developed more classical clinical features of NMDAr encephalitis comprising further seizures, significant movement disorder, cognitive dysfunction, sleep and speech dysfunction. Subsequently, anti-NMDAr antibodies in both serum and CSF were found to be positive. As a result, she received treatment with unspecified steroids, plasmapheresis and rituximab. She made a good recovery following the treatment and was discharged home after a 10-week admission. Close R, et al. Growing up on biologics: A case report of development of nmdar encephalitis in a young person with JIA on abatacept. Rheumatology 59 (Suppl. 2): ii68 abstr. 803507417 P137, Apr 2020. Available from: URL: http://doi.org/10.1093/rheumatology/keaa111.132 [abstract]

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Editorial comment: Details of this case report have previously been published and processed for Adis PV [see Reactions 1804 p14; 803473779].

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Reactions 17 Oct 2020 No. 1826