P263 Two children with NMDAr +ve limbic encephalitis

Posters for a week. MRI brain showed bilateral signal changes in thalami and in the brain stem suggestive of rhombencephalitis. Lumbar puncture (LP) showed 38 cells (98% lymphocytes). He received IV Cefatoxime and IV Acyclovir till CSF bacterial and viral cultures were negative. Blood Mp titre by gelatin particle agglutination test (GPAT) was 2560 (normal less than 40). He was treated with Clarithromycin and steroids for three weeks. Following extubation, he demonstrated mutism with extrapyramidal movements for 2 weeks which spontaneously resolved. At 3 months follow up, he is reported to be normal and back at school. Case 2: A sixteen-year old girl with retroviral infection on treatment presented with a five-day history of headache preceded by a cough of two weeks. She had bilateral papilloedema with no focal neurological signs. MRI brain was normal. LP revealed raised opening CSF pressure at 25 cm with normal cells, chemistry and cultures confirming the diagnosis of idiopathic intracranial hypertension. Blood Mp titre (GPAT) was 5120. She received a three week course of Clarithromycin. Follow up at 4 weeks showed normal fundoscopy with disappearance of headaches. Conclusion: Mp infection should be considered as a differential diagnosis in children with an unusual neurological presentation as the good outcome depends on early diagnosis and specific therapy. P263 Two children with NMDAr +ve limbic encephalitis V. Gowda1 *, B. Mukhtyar1 , M.G. Pike1 , J. Hewertson2 , M.A. McShane1 , A. Vincent3 . 1 Paediatric Neurology Department, John Radcliffe Hospital, Oxford, UK; 2 Department of Paediatrics, Northampton General Hospital, Northampton, UK; 3Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, UK Anti-NMDA receptor encephalitis is a newly described form of limbic encephalitis with a characteristic neuropsychiatric presentation. The condition is characterized by initial psychiatric disturbance, florid orofacial dyskinesias, involuntary movements, autonomic dysfunction, and the presence of NMDA receptor antibodies. About 60% of reported cases are paraneoplastic. We describe two cases of Anti-NMDA receptor encephalitis with no identified tumors. Aged thirteen and twelve years, both girls presented with initial psychiatric symptoms followed by florid neurological symptoms and signs. In both cases, there was deterioration with confusion, aggressive behaviour, involuntary movements, impaired cognition, mutism, dysphagia, autonomic dysfunction and impaired mobility. Although the first case had seizure-like episodes, there was no epileptic activity on EEG whereas the second case had seizures in the first week. There were MRI brain changes in the first case, which evolved and then resolved over time. MRI brain was normal in the second case. Anti-NMDA receptor antibodies were present in both. The diagnosis was made retrospectively in the first case from saved blood and CSF samples, prompted by recognizing the clinical pattern in the second case. Neither child responded to treatment with high dose steroids. In the first case, there was slow but definite improvement following empirical treatment with IVIG. IVIG was considered helpful in the second case but no improvement was noted with plasmapheresis. Recovery was almost complete in the first case although there is mild residual cognitive impairment two years after presentation. The second case continues to improve a month after discharge from the hospital, with treatment plans for monthly IVIG for several months. More than 100 cases of anti-NMDA receptor encephalitis have been described but most are adults. The number of case reports since first described in 2007 would suggest this may be a relatively common cause of culture negative encephalitis.

S103 P264 Immunomodulatory drug treatment in childhood MS: Experience from the UK M. Absoud1 *, C. Cummins1 , A. Gika3 , C. Hemingway4 , M. Lim3 , E. Wassmer2 . 1 Institute of Child Health, Birmingham Children’s Hospital, UK; 2 Paediatric Neurology Department, Birmingham Children’s Hospital, UK; 3 Paediatric Neurology Department, The Evelina Children’s Hospital, London, UK; 4 Paediatric Neurology Department, Great Ormond Street Hospital, London, UK Objective: There is evidence that early treatment of childhood Multiple sclerosis (MS) with immunomodulatory treatment (IMT) may be beneficial. Data on use of IMT in children is limited, and there are no large randomised drug trials. We reviewed cases in 3 UK Centres to evaluate efficacy, safety and tolerability of IMT in childhood MS. Methods: Records were reviewed from 3 UK paediatric demyelination clinics (2004 2008). Only children started on IMT for definite MS (age of onset <16 yrs) were included, and information before and after treatment was collected. Data on demographics, symptoms, side effects, compliance, and relapse rate was analysed. Results: 28 cases (22F:6M) had mean age 11 yr 9 mo (SD 3 yr 6 mo) at onset. On presentation 50% had polyfocal clinically isolated episodes (CIS), 39% monofocal CIS and 11% acute disseminated encephalomyelitis. All developed relapsing-remitting MS. Follow up was median 1.0 yr (4 months to 6 years) before treatment and 1.0 yr (6 months to 4 years) after treatment. 26 were treated with rebif/BetaInterferon and 2 with copaxone. One patient stopped shortly afterwards (poor compliance). Relapse rate was mean 2.3/year (95% CI 1.8−2.6) prior to treatment and 0.5/yr (95% CI 0.28 0.80) after treatment (p < 0.001 Wilcoxon signed ranks test). In one centre (12 cases) time from “decision to treat” to administering IMT averaged 5 months due to delays in drug approval and nursing support. There was no change in relapse rate in this group compared to before treatment decision. There were no serious side effects. 29% reported flu-like symptoms, 25% fatigue, 18% transient lymphopaenia, and 11% transient transaminasemia. Conclusions: IMT in this cohort appeared safe, well tolerated, and efficacious as comparable to adult data and observations from similar childhood studies. Children should be offered the choice of IMT in MS, and guidelines are needed. P265 Anti-NMDAR auto-immune encephalitis A. Antunes Martins1 *, J.P. Vieira1 , R. Silva1 , A.I. Dias1 , A. Moreira1 , E. Calado1 , A. Vincent2 . 1 Pediatric Neurology Department, Hospital Dona Estefania, Lisbon, Portugal; 2 ˆ Neuroimmunology group, John Radcliffe Hospital, Oxford, United Kingdom Introduction: anti-NMDAR encephalitis is characterized by hallucinations, involuntary movements, altered consciousness and autonomic changes; it usually (but not always) represents a paraneoplastic picture, though sometimes the tumour is occult. Case report: Nine-year-old male with no relevant antecedents. One week prior to admission started complaints of vomiting, abdominal pain, psychomotor agitation, delirious speech and distonic/choreic left upper limb movements; he was admitted and started oral risperidone. Throughout his admission there have been several clinical stages: 1st week: worsening of psychomotor agitation and delirious speech; haloperidol was added. 2nd week: depressed consciousness and generalized rigidity; stopped haloperidol. 3rd week: refractory psychomotor agitation, absence of eye-contact and verbal response, oral dyskinesias and diffuse hypertonia. 4−6th week: progressive amelioration of psychomotor agitation. 7th week-onwards: slowly progressive improvement: regained sleep-wake rhythm, visual