N‐methyl‐D‐aspartate receptor antibodies in pediatric dyskinetic encephalitis lethargica

Encephalitis lethargica (EL) describes an encephalitis with psychiatric, sleep, and extrapyramidal movement disorders. Dyskinetic and Parkinsonian forms have been described. EL shares clinical features with the anti–N‐methyl‐D‐aspartate receptor (NMDAR‐Ab) encephalitis. We studied 20 sera from pediatric patients with contemporary EL. Ten sera (from 2 males and 8 females, aged 1.3–13 years) and 6/6 cerebrospinal fluid samples were positive for NMDAR‐Ab. NMDAR‐Ab–positive patients had dyskinesias, agitation, seizures, and insomnia, whereas Parkinsonism and somnolence dominated in the NMDAR‐Ab–negative children. We were unable to identify any tumors. The dyskinetic form of EL is an NMDAR‐Ab encephalitis and can affect very young children. Ann Neurol 2009;66:704–709

[1]  C. Bien,et al.  Successful treatment of anti-N-methyl-D-aspartate receptor encephalitis presenting with catatonia , 2009, Archives of Disease in Childhood.

[2]  A. Hida,et al.  RESPONSE OF ANTI-NMDA RECEPTOR ENCEPHALITIS WITHOUT TUMOR TO IMMUNOTHERAPY INCLUDING RITUXIMAB , 2008, Neurology.

[3]  R. Balice-Gordon,et al.  Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies , 2008, Lancet Neurology.

[4]  Michael K. Hutchinson,et al.  PROGRESSIVE ENCEPHALOMYELITIS, RIGIDITY, AND MYOCLONUS: A NOVEL GLYCINE RECEPTOR ANTIBODY , 2008, Neurology.

[5]  P. Thompson,et al.  The distinctive movement disorder of ovarian teratoma‐associated encephalitis , 2008, Movement disorders : official journal of the Movement Disorder Society.

[6]  Jacqueline A Palace,et al.  Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis. , 2008, Archives of neurology.

[7]  R. Dale,et al.  Contemporary encephalitis lethargica presenting with agitated catatonia, stereotypy, and dystonia‐parkinsonism , 2007, Movement disorders : official journal of the Movement Disorder Society.

[8]  P. Kempster,et al.  Sporadic encephalitis lethargica , 2007, Journal of Clinical Neuroscience.

[9]  Josep Dalmau,et al.  Paraneoplastic anti–N‐methyl‐D‐aspartate receptor encephalitis associated with ovarian teratoma , 2007, Annals of neurology.

[10]  G. Giovannoni,et al.  Neuronal surface glycolytic enzymes are autoantigen targets in post-streptococcal autoimmune CNS disease , 2006, Journal of Neuroimmunology.

[11]  C. de Sousa,et al.  Immune mediated chorea encephalopathy syndrome in childhood. , 2002, Developmental medicine and child neurology.

[12]  H. Okamura,et al.  An acutely confused 15-year-old girl , 1997, The Lancet.

[13]  H. Koga,et al.  Reversible limbic encephalitis caused by ovarian teratoma , 1997, Acta neurologica Scandinavica.

[14]  N. Turjanski,et al.  Clinical features and management of two cases of encephalitis lethargica , 1997, Movement disorders : official journal of the Movement Disorder Society.

[15]  Constantin von Economo Encephalitis Lethargica, Us Sequelae and Treatment , 1931 .

[16]  N. Omori,et al.  Steroid-responsive encephalitis lethargica syndrome with malignant catatonia. , 2007, Internal medicine.

[17]  Gavin Giovannoni,et al.  Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. , 2004, Brain : a journal of neurology.

[18]  M. Esiri,et al.  A contemporary case of encephalitis lethargica. , 2001, Clinical neuropathology.