RT Journal Article
SR Electronic
T1 NMDA receptor antibodies associated with distinct white matter syndromes
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e2
DO 10.1212/NXI.0000000000000002
VO 1
IS 1
A1 Yael Hacohen
A1 Michael Absoud
A1 Cheryl Hemingway
A1 Leslie Jacobson
A1 Jean-Pierre Lin
A1 Mike Pike
A1 Sunil Pullaperuma
A1 Ata Siddiqui
A1 Evangeline Wassmer
A1 Patrick Waters
A1 Sarosh R. Irani
A1 Camilla Buckley
A1 Angela Vincent
A1 Ming Lim
YR 2014
UL http://nn.neurology.org/content/1/1/e2.abstract
AB Objective: To report the clinical and radiologic findings of children with NMDA receptor (NMDAR) antibodies and white matter disorders.Method: Ten children with significant white matter involvement, with or without anti-NMDAR encephalitis, were identified from 46 consecutive NMDAR antibody–positive pediatric patients. Clinical and neuroimaging features were reviewed and the treatment and outcomes of the neurologic syndromes evaluated.Results: Three distinct clinicoradiologic phenotypes were recognized: brainstem encephalitis (n = 3), leukoencephalopathy following herpes simplex virus encephalitis (HSVE) (n = 2), and acquired demyelination syndromes (ADS) (n = 5); 3 of the 5 with ADS had myelin oligodendrocyte glycoprotein as well as NMDAR antibodies. Typical NMDAR antibody encephalitis was seen in 3 patients remote from the first neurologic syndrome (2 brainstem, 1 post-HSVE). Six of the 7 patients (85%) who were treated acutely, during the original presentation with white matter involvement, improved following immunotherapy with steroids, IV immunoglobulin, and plasma exchange, either individually or in combination. Two patients had escalation of immunotherapy at relapse resulting in clinical improvement. The time course of clinical features, treatments, and recoveries correlated broadly with available serum antibody titers.Conclusion: Clinicoradiologic evidence of white matter involvement, often distinct, was identified in 22% of children with NMDAR antibodies and appears immunotherapy responsive, particularly when treated in the acute phase of neurologic presentation. When observed, this clinical improvement is often mirrored by reduction in NMDAR antibody levels, suggesting that these antibodies may mediate the white matter disease.Ab=antibody; ADEM=acute disseminated encephalomyelitis; ADS=acquired demyelination syndromes; AQP4=aquaporin-4; HSV=herpes simplex virus; HSVE=HSV encephalitis; IVIg=IV immunoglobulin; MOG=myelin oligodendrocyte glycoprotein; MS=multiple sclerosis; NMDAR=NMDA receptor; PLEX=plasma exchange