Encefalitis por anticuerpos contra receptor NMDA. A propósito de un caso / Encephalitis by antibodies against NMDA receptor. About a case

Introducción: En la encefalitis por anticuerpos contra receptores N-Metil-D-Aspartato (NMDA) se genera una disfunción neuronal del ácido gamma-aminobutírico (GABA), con desregulación del glutamato y la dopamina. Los teratomas y las infecciones virales se presumen causales de la respuesta autoinmunitaria. La clínica evoluciona por etapas con un pródromo similar a una infección viral con posteriores manifestaciones psiquiátricas y convulsiones, seguidas de disfunción motora, cognitiva y autonómica. El diagnóstico se basa en la clínica y la presencia de anticuerpos del receptor NMDA. El tratamiento incluye inmunoterapia y eventual eliminación del tumor. La enfermedad puede ser letal o provocar daño irreversible en regiones corticales. Objetivo: Destacar la importancia del diagnóstico precoz en los casos de encefalitis autoinmune para una mayor efectividad de los tratamientos postulados. Reporte de caso: Paciente femenina de 13 años de edad, con diagnóstico de diabetes tipo I; presenta dolor y pérdida de fuerza con movimientos involuntarios en miembro superior izquierdo con dificultad en la deambulación, más episodio convulsivo tónico clónico generalizado seguido de manifestaciones neuropsiquiátricas. Se sospecha encefalitis autoinmune, se dosan anticuerpos anti ácido glutámico descarboxilasa (GAD) y anti NMDA, con resultados positivos. Recibe corticoterapia, inmunoglobulina endovenosa, rituximab y plasmaferesis. Presenta escasa mejoría clínica, con persistencia de síntomas secuelares psiquiátricos y neurológicos. Discusión: Es importante sospechar esta entidad aunque las manifestaciones clínicas iniciales sugieran otras etiologías. El tratamiento inmunosupresor agresivo no debería demorarse aun cuando no se haya confirmado la positividad de los anticuerpos NMDA. El buen pronóstico depende del inicio precoz del tratamiento.

Introduction: The encephalitis by antibodies against NMDA receptors, a neuronal dysfunction of gamma-aminobutyric acid (GABA) is generated, with deregulation of glutamate and dopamine. Teratomas and viral infections are presumed to be the cause to the autoimmune response. The clinic evolves in stages with a prodrome similar to a viral infection with subsequent psychiatric manifestations and seizure, followed by motor, cognitive and autonomic dysfunction. Diagnosis is based on clinical symptoms and the presence of NMDA receptor antibodies. The treatment includes immunotherapy and, eventually, elimination of the tumor. The disease can be lethal or cause irreversible damage in cortical regions. Objective: Highlight the importance of early diagnosis in cases of autoimmune encephalitis for greater effectiveness of postulated treatments. Case report.:13 year old female patient diagnosed with type I diabetes; presents pain, loss of strength and involuntary movements of the upper left limb and ambulation difficulties, associeted with a generalized tonic-clonic seizure episode followed by neuropsychiatric manifestations. Autoinmune encefalitis was suspected so antiglutamic acid decarboxylase (GAD) and anti-NMDA antibodies were dosed, which throw a positive result. The patient receives corticotherapy, intravenous immunoglobulin, rituximab and plasmapheresis. Presenting little clinical improvement, with persistence of psychiatric and neurological sequelae symptoms. Discussion: It is important to suspect this entity although the initial clinical manifestations suggests other etiologies. Aggressive immunosuppressive therapy should not be delayed even when the positivity of NMDA antibodies has not been confirmed. The good prognosis depends on the early start of the treatment.

Aportaciones de la neuropsicología a la encefalitis por anticuerpos antirreceptoContributions of neuropsychology to anti-NMDA receptor antibody encephalitis: a literature reviewr de NMDA: revisión de la bibliografía / Contributions of neuropsychology to anti-NMDA receptor antibody encephalitis: a literature review

La encefalitis límbica generada por anticuerpos antirreceptor de N-metil-D-aspartato (NMDA) es una entidad neurológica aguda y grave, más prevalente en las mujeres jóvenes y asociada a un tumor subyacente. El curso con déficit cognitivo grave hace necesario reflexionar sobre las aportaciones de la neuropsicología al diagnóstico, evolución y tratamiento de la enfermedad, poco estudiadas hasta el momento. Se revisa la bibliografía precedente, criticando la medición de los síntomas cognitivos (predominantemente mnésicos y ejecutivos) asociados a esta enfermedad. Se proponen instrumentos neuropsicológicos válidos y fiables, y se plantea que las medidas neuropsicológicas pueden servir como pará- metros de seguimiento de estos pacientes que ayuden a monitorizar su funcionalidad en la vida diaria una vez recuperados de la fase aguda, así como convertirse en una base sobre la que articular programas de rehabilitación que favorezcan el logro de la autonomía personal y la reinserción comunitaria de los pacientes. Con todo, se subraya la necesidad de incluir a neuropsicólogos y neuropsiquiatras no sólo en la detección, sino en el tratamiento de los pacientes, en pro de alcanzar la recuperación de la independencia personal y la readaptación a sus entornos naturales (AU)

Limbic encephalitis generated by anti-N-methyl-D-aspartate (NMDA) receptor antibodies is an acute and severe neurological entity, which is more prevalent in young females and is associated to an underlying tumour. Since it leads to severe cognitive impairment, thought needs to be given to the contributions of neuropsychology to the diagnosis, development and treatment of the disease, which have received little attention from researchers to date. A review is conducted of the prior literature, evaluating the measurement of the cognitive symptoms (predominantly mnemonic and executive) associated to this disease. Valid, reliable neuropsychological instruments are proposed, and it is suggested that neuropsychological measures may be used as parameters to follow up these patients which help monitor their functionality in daily living once they have recovered from the acute phase. Similarly they can become a basis on which to assemble rehabilitation programmes that favour the accomplishment of personal autonomy and the patients’ reintegration in the community. Nevertheless, we stress the need to include neuropsychologists and neuropsychiatrists in not only the detection but also the treatment of these patients so as to enable them to recover their personal independence and readapt to their natural settings (AU)

Virus and Immune-Mediated Encephalitides: Epidemiology, Diagnosis, Treatment, and Prevention.

Virus encephalitis remains a major cause of acute neurological dysfunction and permanent disability among children worldwide. Although some disorders, such as measles encephalomyelitis, subacute sclerosing panencephalitis, and varicella-zoster virus-associated neurological conditions, have largely disappeared in resource-rich regions because of widespread immunization programs, other disorders, such as herpes simplex virus encephalitis, West Nile virus-associated neuroinvasive disease, and nonpolio enterovirus-induced disorders of the nervous system, cannot be prevented. Moreover, emerging viral disorders pose new, potential threats to the child's nervous system. This review summarizes current information regarding the epidemiology of virus encephalitis, the diagnostic methods available to detect central nervous system infection and identify viral pathogens, and the available treatments. The review also describes immune-mediated disorders, including acute disseminated encephalomyelitis and N-methyl-D-aspartate receptor antibody encephalitis, conditions that mimic virus encephalitis and account for a substantial proportion of childhood encephalitis.