[Anti-NMDA receptor paraneoplastic encephalitis: complete recovery after ovarian teratoma removal]. / Encefalitis paraneoplásica por anticuerpos contra el receptor de NMDA. Remisión completa después de la resección de un teratoma ovárico.

INTRODUCTION: A paraneoplastic syndrome characterized by neuropsychiatric symptoms, involuntary movements and seizures has been recently associated with antibodies targeting NMDA (N-methyl-D-aspartate) receptor in patients with an ovarian teratoma. Severe neurological impairment is frequent and treatment in the intensive care unit is often required because of ventilatory failure and life-threatening autonomic instability. Tumor removal is curative in many cases and neurological improvement is demonstrated shortly after surgery. CASE REPORT: Here we report on a patient with paraneoplastic encephalitis manifested by unconsciousness and coreo-athetosic movements related to NMDA receptor antibodies associated with an immature ovarian teratoma grade III. She made a complete recovery after oophorectomy, intravenous immunoglobulin and corticosteroids. CONCLUSIONS: Treatment of paraneoplastic syndromes is based on specific therapy for underlying tumor associated to immunomodulators. As in this case, anti-NMDA encephalitis may significantly improve after tumor removal and intra-venous immunoglobuline.

[Encephalitis due to antibodies against the NMDA receptor. A case report of a female patient with no associated tumour and a literature review]. / Encefalitis por anticuerpos contra el receptor NMDA. Descripción de una paciente sin tumor asociado y revisión de la bibliografía.

INTRODUCTION: A severe form of acute encephalitis associated to antibodies against the N-methyl D-aspartate (NMDA) receptor has recently been reported. This clinical picture occurs in young people, not always with an underlying tumour and, despite the initial severity, if identified and treated at an early stage, complete recovery without any kind of sequelae can be achieved. We report on a new case and review the body of knowledge currently available on this recently identified condition. CASE REPORT: A 22-year-old female who visited our centre due to a progressive conduct disorder. Over the days that followed, deterioration in the level of consciousness made it necessary to put her on assisted respiration. Magnetic resonance imaging of the head showed hyperintense lesions mainly in deep temporal regions. A study of the cerebrospinal fluid revealed pleocytosis with a predominance of lymphocytes and antibodies against the NMDA receptor. In the complementary study no underlying tumour was observed. Treatment with corticosteroids and immunoglobulins brought about a slow but steady improvement. After a one-year follow-up there have been no recurrences, no tumours have appeared and the patient has gone back to her usual day-to-day activities. Even a significant temporal atrophy that developed at the beginning of the clinical picture has gradually been seen to resolve itself in neuroimaging studies performed as a control measure. CONCLUSION: Encephalitis due to anti-NMDA antibodies is a disease that has only recently been characterised, but which, according to currently available data, is relatively frequent. Clinically well defined, suspicion of this condition will make it possible to reach a definitive diagnosis and to establish early treatment.

Encefalitis por anticuerpos contra el receptor NMDA. Descripción de una paciente sin tumor asociado y revisión de la bibliografía / Encephalitis due to antibodies against the NMDA receptor. A case report of a female patient with no associated tumour and a literature review

Introducción. Recientemente se ha descrito una forma grave de encefalitis aguda asociada a anticuerpos contra el receptor N-metil-D-aspartato (NMDA). Este cuadro ocurre en jóvenes, no siempre con un tumor subyacente y, pese a la gravedad inicial, su identificación y tratamiento precoz pueden llevar a recuperaciones sin secuelas. Presentamos un nuevo caso y revisamos los conocimientos disponibles acerca de esta nueva entidad. Caso clínico. Mujer de 22 años de edad que acudió a nuestro centro como consecuencia de un trastorno progresivo del comportamiento. Durante los días siguientes, un deterioro del nivel de conciencia hizo necesario el soporte respiratorio. Una resonancia magnética craneal mostró lesiones hiperintensas principalmente en regiones temporales profundas. El estudio de líquido cefalorraquídeo reveló pleocitosis con predominio linfocitario y anticuerpos contra el receptor NMDA. En el estudio complementario no se apreció tumor subyacente. El tratamiento con corticoesteroides e inmunoglobulinas dio lugar a una lenta pero continua mejoría. Al cabo de un año de seguimiento no ha presentado recidivas, no ha aparecido tumor alguno y la paciente se ha reintegrado a sus actividades habituales. Incluso una atrofia temporal llamativa desarrollada al inicio del cuadro se ha ido resolviendo en estudios de neuroimagen de control. Conclusión. La encefalitis por anticuerpos anti-NMDA es una enfermedad recientemente caracterizada, pero, de acuerdo con los datos disponibles hasta la fecha, relativamente frecuente. Clínicamente bien definida, la sospecha de esta entidad hará posible un diagnóstico definitivo y la instauración de un tratamiento precoz (AU)

Introduction. A severe form of acute encephalitis associated to antibodies against the N-methyl D-aspartate (NMDA) receptor has recently been reported. This clinical picture occurs in young people, not always with an underlying tumour and, despite the initial severity, if identified and treated at an early stage, complete recovery without any kind of sequelae can be achieved. We report on a new case and review the body of knowledge currently available on this recently identified condition. Case report. A 22-year-old female who visited our centre due to a progressive conduct disorder. Over the days that followed, deterioration in the level of consciousness made it necessary to put her on assisted respiration. Magnetic resonance imaging of the head showed hyperintense lesions mainly in deep temporal regions. A study of the cerebrospinal fluid revealed pleocytosis with a predominance of lymphocytes and antibodies against the NMDA receptor. In the complementary study no underlying tumour was observed. Treatment with corticosteroids and immunoglobulins brought about a slow but steady improvement. After a one-year follow-up there have been no recurrences, no tumours have appeared and the patient has gone back to her usual day-to-day activities. Even a significant temporal atrophy that developed at the beginning of the clinical picture has gradually been seen to resolve itself in neuroimaging studies performed as a control measure. Conclusion. Encephalitis due to anti-NMDA antibodies is a disease that has only recently been characterised, but which, according to currently available data, is relatively frequent. Clinically well defined, suspicion of this condition will make it possible to reach a definitive diagnosis and to establish early treatment (AU)