PT  - JOURNAL ARTICLE
AU  - Bigi, Sandra
AU  - Hladio, Manisha
AU  - Twilt, Marinka
AU  - Dalmau, Josep
AU  - Benseler, Susanne M.
TI  - The growing spectrum of antibody-associated inflammatory brain diseases in children
AID  - 10.1212/NXI.0000000000000092
DP  - 2015 Jun 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e92
VI  - 2
IP  - 3
4099  - http://nn.neurology.org/content/2/3/e92.short
4100  - http://nn.neurology.org/content/2/3/e92.full
SO  - Neurol Neuroimmunol Neuroinflamm2015 Jun 01; 2
AB  - Objective: To describe the clinical spectrum, diagnostic evaluation, current management, and neurologic outcome of pediatric antibody-associated inflammatory brain diseases (AB-associated IBrainD).Methods: We performed a single-center retrospective cohort study of consecutive patients aged ≤18 years diagnosed with an AB-associated IBrainD at The Hospital for Sick Children, Toronto, Ontario, Canada, between January 2005 and June 2013. Standardized clinical data, laboratory test results, neuroimaging features, and treatment regimens were captured.Results: Of 169 children (93 female, 55%) diagnosed with an IBrainD, 16 (10%) had an AB-associated IBrainD. Median age at presentation was 13.3 years (range 3.1–17.9); 11 (69%) were female. Nine patients (56%) had anti–NMDA receptor encephalitis, 4 (25%) had aquaporin-4 autoimmunity, 2 (13%) had Hashimoto encephalitis, and 1 (6%) had anti–glutamic acid decarboxylase 65 (GAD65) encephalitis. The key presenting features in children with anti–NMDA receptor encephalitis, Hashimoto encephalopathy, and anti-GAD65 encephalitis included encephalopathy, behavioral symptoms, and seizures; patients with aquaporin-4 autoimmunity showed characteristic focal neurologic deficits. Six patients (38%) required intensive care unit admission at presentation. Median time from symptom onset to diagnosis was 55 days (range 6–358). All but 1 patient received immunosuppressive therapy. One child with anti–NMDA receptor encephalitis died due to multiorgan failure. At last follow-up, after a median follow-up time of 1.7 years (range 0.8–3.7), 27% of the children had function-limiting neurologic sequelae.Conclusions: Children with AB-associated IBrainD represent an increasing subgroup among IBrainD; 1 in 4 children has function-limiting residual neurologic deficits. Awareness of the different clinical patterns is important in order to facilitate timely diagnosis and initiate immunosuppressive treatment.AB-associated IBrainD=antibody-associated inflammatory brain diseases; AQP4=aquaporin-4; CRP=C-reactive protein; ESR=erythrocyte sedimentation rate; FLAIR=fluid-attenuated inversion recovery; GAD65=glutamic acid decarboxylase 65; IBrainD=inflammatory brain diseases; ICU=intensive care unit; IgG=immunoglobulin G; LETM=longitudinally extensive transverse myelitis; NMO=neuromyelitis optica; ON=optic neuritis; PSOM=Pediatric Stroke Outcome Measure; TPO=thyroperoxidase