BACKGROUND: To describe a case of auto-immune encephalitis in an adolescent with favorable outcome despite prolonged status epilepticus. METHODS: A 17 year old Asian man without previous medical history developed alteration of consciousness and partial seizures. The diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was confirmed by the detection of specific antibodies in both cerebrospinal fluid and serum. RESULTS: The clinical course was complicated by prolonged status epilepticus which was refractory to a large number of antiepileptic drugs, including barbiturate coma. Immunomodulatory therapy included steroids, plasma exchanges, and intravenous immunoglobulins. After 86 days of intensive therapy, the patient regained consciousness progressively. Brain magnetic resonance imaging never demonstrated any lesion. Extensive search for a tumor was negative. At 12 month follow-up, the patient had made an excellent recovery. CONCLUSION: Auto-immune encephalitis is likely underdiagnosed in adolescents. In their most severe presentation, seizures may be resistant to a large number of anti-epileptic drugs, and the clinical improvement seems to be mainly because of the immunomodulatory therapy. Relapse is possible, as well as the delayed development of a teratoma or other tumor.
BACKGROUND: To describe a case of auto-immune encephalitis in an adolescent with favorable outcome despite prolonged status epilepticus. METHODS: A 17 year old Asian man without previous medical history developed alteration of consciousness and partial seizures. The diagnosis of anti-N-methyl-D-aspartate receptor encephalitis was confirmed by the detection of specific antibodies in both cerebrospinal fluid and serum. RESULTS: The clinical course was complicated by prolonged status epilepticus which was refractory to a large number of antiepileptic drugs, including barbituratecoma. Immunomodulatory therapy included steroids, plasma exchanges, and intravenous immunoglobulins. After 86 days of intensive therapy, the patient regained consciousness progressively. Brain magnetic resonance imaging never demonstrated any lesion. Extensive search for a tumor was negative. At 12 month follow-up, the patient had made an excellent recovery. CONCLUSION: Auto-immune encephalitis is likely underdiagnosed in adolescents. In their most severe presentation, seizures may be resistant to a large number of anti-epileptic drugs, and the clinical improvement seems to be mainly because of the immunomodulatory therapy. Relapse is possible, as well as the delayed development of a teratoma or other tumor.
Authors: Claudia Poloni; Christian M Korff; Valeria Ricotti; Mary D King; Eliane Roulet Perez; Claire Mayor-Dubois; Charles-Antoine Haenggeli; Thierry Deonna Journal: Dev Med Child Neurol Date: 2009-12-23 Impact factor: 5.449
Authors: Jonathan H Smith; Radhika Dhamija; Brian D Moseley; Paola Sandroni; Claudia F Lucchinetti; Vanda A Lennon; Orhun H Kantarci Journal: Arch Neurol Date: 2011-08
Authors: Josep Dalmau; Erdem Tüzün; Hai-yan Wu; Jaime Masjuan; Jeffrey E Rossi; Alfredo Voloschin; Joachim M Baehring; Haruo Shimazaki; Reiji Koide; Dale King; Warren Mason; Lauren H Sansing; Marc A Dichter; Myrna R Rosenfeld; David R Lynch Journal: Ann Neurol Date: 2007-01 Impact factor: 10.422
Authors: Josep Dalmau; Amy J Gleichman; Ethan G Hughes; Jeffrey E Rossi; Xiaoyu Peng; Meizan Lai; Scott K Dessain; Myrna R Rosenfeld; Rita Balice-Gordon; David R Lynch Journal: Lancet Neurol Date: 2008-10-11 Impact factor: 44.182
Authors: Nicole R Florance; Rebecca L Davis; Christopher Lam; Christina Szperka; Lei Zhou; Saba Ahmad; Cynthia J Campen; Heather Moss; Nadja Peter; Amy J Gleichman; Carol A Glaser; David R Lynch; Myrna R Rosenfeld; Josep Dalmau Journal: Ann Neurol Date: 2009-07 Impact factor: 10.422
Authors: Pitt Niehusmann; Josep Dalmau; Christian Rudlowski; Angela Vincent; Christian E Elger; Jeffrey E Rossi; Christian G Bien Journal: Arch Neurol Date: 2009-04
Authors: Gerard Davies; Sarosh R Irani; Cordelia Coltart; Gordon Ingle; Yogen Amin; Christopher Taylor; Jeremy Radcliffe; Nicholas P Hirsch; Robin S Howard; Angela Vincent; Dimitri M Kullmann Journal: Crit Care Med Date: 2010-02 Impact factor: 7.598