A Case in which Eosinophilic Meningoencephalitis Occurred during Oral Corticosteroid Tapering and after Switching from Anti-IL-5 to Anti-IgE Treatment.

A 49-year-old man with severe eosinophilic asthma, sinusitis, and esophagitis was admitted with a sudden severe headache. The patient was diagnosed with eosinophilic meningoencephalitis based on frontotemporal abnormalities on brain magnetic resonance imaging and high eosinophil counts in the cerebrospinal fluid. His allergic-disease control levels were poor, requiring regular oral corticosteroid (OCS) use. He was switched from anti-interleukin (IL)-5 to anti-IgE therapy because of worsening urticaria and asthma symptoms during OCS tapering. We suspect this was a case of complex eosinophilic meningoencephalitis caused by the combination of OCS tapering and anti-IL-5 therapy cessation that acquired anti-IgE antibody sensitization based on positive drug-induced lymphocyte stimulation test results.

IL-33 Enhances the Total Production of IgG, IgG1, and IgG3 in Angiostrongylus cantonensis-Infected Mice.

The purpose of this study is to clarify the role of IL-33 in the immune response to angiostrongyliasis, especially in terms of antibody production and isotype switching. In our experiment, C57BL/6 mice were each infected with 35 infectious larvae and were divided into groups that received an intraperitoneal injection of IL-33, anti-IL-33 monoclonal antibody (mAb), or anti-ST2 mAb 3 days post-infection (dpi) and were subsequently administered booster shots at 5-day intervals with the same dose. Serum samples from each group were collected weekly for ELISA assays. The levels of total IgG, IgG1, and IgG3 were significantly increased in A. cantonensis-infected mice that were treated with IL-33, and the levels decreased significantly in infected groups treated with anti-IL-33 or anti-ST2 mAb. These results suggest that IL-33 may play a critical role in the pathogenesis of human angiostrongyliasis and could be useful for understanding protective immunity against this parasitic infection.

[Health implications of the finding of Angiostrongylus cantonensis, the main cause of eosinophilic meningoencephalitis, in continental Europe (Valencia, Spain)]. / Implicaciones sanitarias del hallazgo de Angiostrongylus cantonensis, causa principal de la meningoencefalitis eosinofílica, en Europa continental (València, España).

The rat pulmonary artery nematode, Angiostrongylus cantonensis (discovered in rats from the province of Canton, southern China, in 1933 ) is the main cause in humans of what is known as eosinophilic meningoencephalitis (EEM), with around of 3,000 confirmed cases in various parts of the world.

El nematodo de las arterias pulmonares de las ratas, Angiostrongylus cantonensis (descubierto en ratas de la provincia de Cantón, en el sur de China, en 1933  es el principal responsable en el ser humano de la conocida como meningoencefalitis eosinofílica (MEE), con alrededor de 3.000 casos confirmados en diversas partes del mundo.

Angiostrongylus cantonensis Infection in Brown Rats (Rattus norvegicus), Atlanta, Georgia, USA, 2019-2022.

Rat lungworm (Angiostrongylus cantonensis), a zoonotic parasite invasive to the United States, causes eosinophilic meningoencephalitis. A. cantonensis harbors in rat reservoir hosts and is transmitted through gastropods and other paratenic hosts. We discuss the public health relevance of autochthonous A. cantonensis cases in brown rats (Rattus norvegicus) in Atlanta, Georgia, USA.

In the Dawn of an Early Invasion: No Genetic Diversity of Angiostrongylus cantonensis in Ecuador?

The nematode Angiostrongylus cantonensis has been reported worldwide. However, some basic questions remain unanswered about A. cantonensis in Ecuador: (1) Was the invasion of A. cantonensis in Ecuador unique, or did it occur in different waves? (2) Was this invasion as recent as historical records suggest? (3) Did this invasion come from other regions of South America or elsewhere? To address these issues, we assessed the genetic diversity of MT-CO1 gene sequences from isolates obtained in 11 of Ecuador's 24 provinces. Our Bayesian inference phylogenetic tree recovered A. cantonensis as a well-supported monophyletic group. All 11 sequences from Ecuador were identical and identified as AC17a. The haplotype AC17a, found in Ecuador and the USA, formed a cluster with AC17b (USA), AC13 (Thailand), and AC12a-b (Cambodia). Notably, all the samples obtained in Ecuadorian provinces' different geographic and climatic regions had no genetic difference. Despite the lack of genetic information on A. cantonensis in Latin America, except in Brazil, our finding differs from previous studies by its absence of gene diversity in Ecuador. We concluded that the invasion of A. cantonensis in Ecuador may have occurred: (1) as a one-time event, (2) recently, and (3) from Asia via the USA. Further research should include samples from countries neighboring Ecuador to delve deeper into this.

The metagenomic next-generation sequencing in diagnosing central nervous system angiostrongyliasis: a case report.

BACKGROUNDS: The incidence of angiostrongyliasis is increasing in recent decades due to the expanding endemic areas all over the world. Clinicians face tremendous challenge of diagnosing angiostrongyliasis because of the lack of awareness of the disease and less effective definitive laboratory tests. CASE PRESENTATION: A 27-year-old man initially manifested skin itching, emesis, myalgia and quadriparesis. With progressive weakness of four limbs and elevated protein in the cerebrospinal fluid (CSF), he was diagnosed as Guillain-Barré syndrome and treated with intravenous methylprednisolone and immunoglobulin. However, the patient deteriorated with hyperpyrexia, headache and then persistent coma. The routine tests for Angiostrongylus cantonensis (A. cantonensis) with both the CSF and the serum were all negative. In contrast, the metagenomic next-generation sequencing (mNGS) was applied with the serum sample and the CSF sample in the middle phase. The central nervous system (CNS) angiostrongyliasis was diagnosed by mNGS with the mid-phase CSF, but not the mid-phase serum. At the same time, the CSF analysis revealed eosinophils ratio up to 67%. The discovery of A. cantonensis was confirmed by PCR with CSF later. Unfortunately, the patient died of severe angiostrongyliasis. During his hospitalization, mNGS was carried out repeatedly after definitive diagnosis and targeted treatment. The DNA strictly map reads number of A. cantonensis detected by mNGS was positively correlated with the CSF opening pressure and clinical manifestations. CONCLUSIONS: The case of A. cantonensis infection highlights the benefit of mNGS as a target-free identification in disclosing the rare CNS angiostrongyliasis in the unusual season, while solid evidence from routine clinical testing was absent. The appropriate sample of mNGS should be chosen according to the life cycle of A. cantonensis. Besides, given the fact that the DNA reads number of A. cantonensis fluctuated with CSF opening pressure and clinical manifestations, whether mNGS could be applied as a marker of effectiveness of treatment is worth further exploration.

Particularidades de la meningoencefalitis eosinofílica en la era del caracol gigante africano / Particular features of eosinophilic meningoencephalitis in the age of the giant African snail

Introducción: la meningoencefalitis eosinofílica es una enfermedad inflamatoria infecciosa reportada en Cuba desde la década de los ochenta del siglo pasado y actualmente extendida al continente americano. Es producida por el parásito Angiostrongylus cantonensis. Objetivo: determinar si existen diferencias entre los pacientes que sufrieron meningoencefalitis eosinofílica antes y después de la introducción del caracol gigante africano. Métodos: se estudiaron un total de 19 muestras de líquido cefalorraquídeo y suero tomadas simultáneamente a cada paciente diagnosticados con meningoencefalitis eosinofílica, perteneciente a la seroraquioteca del Laboratorio Central del Líquido Cefalorraquídeo (LABCEL). Para la determinación de las proteínas albúmina e IgG se empleó como método de laboratorio la inmunodifusión radial. Resultados: el 14 por ciento de los pacientes fueron adultos antes de la aparición de este molusco, en contraste con el momento actual, donde el 50 por ciento son pacientes con edades superiores a 18 años. El porcentaje de síntesis de IgG intratecal media fue mayor en los enfermos actuales, aunque no de manera significativa. Conclusiones: . existen diferencias antes y después de la aparición del caracol gigante africano dado por la respuesta de síntesis intratecal en los pacientes asociados con el molusco que denota una mayor agresividad del parásito. La edad promedio mayor de los enfermos confirma que estos son los que más manipulan y dispersan el molusco(AU)

Introduction: eosinophilic meningoencephalitis is an infectious inflammatory disease reported in Cuba since the 1980s and currently extended to the American continent. This condition is caused by the parasite Angiostrongylus cantonensis. Objective: determine whether there are differences between the patients suffering from eosinophilic meningoencephalitis before and after the introduction of the giant African snail. Methods: a study was conducted of a total 19 cerebrospinal fluid and serum samples taken simultaneously from each of the patients diagnosed with eosinophilic meningoencephalitis and kept at the sample collection of the Central Cerebrospinal Fluid Laboratory (LABCEL). Radial immunodiffusion was the laboratory method used for determination of the proteins albumin and IgG. Results: of the patients studied, 14 percent were adults before the appearance of this mollusc, in contrast with the present moment, when 50 percent are patients aged over 18 years. The percentage of mean intrathecal synthesis of IgG was higher in the current sufferers, though not significantly. Conclusions: there are differences before and after the appearance of the giant African snail, given the intrathecal synthesis response of patients associated to the mollusc, which denotes greater aggressiveness by the parasite. The higher mean age of sufferers confirms that these are the ones who most often handle and disperse the mollusc(AU)

Evidencias clínico-epidemiológicas vinculadas a Lissachatina fulica en un adulto con meningoencefalitis eosinofílica causada por Angiostrongylus cantonensis / Clinical-epidemiological evidence related to Lissachatina fulica in an adult with eosinophilic meningoencephalitis caused by Angiostrongylus cantonensis

Introducción: El helminto Angiostrongylus cantonensis es un parásito habitual en los pulmones de la rata y puede ocasionar meningoencefalitis eosinofílica en el hombre cuando se pone en contacto con las larvas por ingestión accidental. En Cuba es endémico y el riesgo de contraerlo aumenta con la entrada del caracol gigante africano en el país. Objetivo: Describir las acciones desarrolladas por las autoridades sanitarias locales para el control del caracol gigante africano entre 2016 y 2018 y la aparición de un paciente en 2018 con meningoencefalitis eosinofílica causada por Angiostrongylus cantonensis vinculado epidemiológicamente con la presencia de este caracol. Métodos: Se realizó un estudio cualitativo a partir de un grupo focal con el que se trabajó ante la aparición del caracol gigante africano en un área de salud del municipio San Miguel del Padrón. Se realiza una encuesta semi-estructurada. Resultados: Se trazaron las estrategias para la erradicación de la especie invasora a partir de un trabajo comunitario. Dos años después, se observa nuevamente el caracol gigante africano y un paciente con meningoencefalitis eosinofílica epidemiológicamente asociado a Angiostrongylus cantonensis. Conclusiones: Las acciones realizadas entre 2016 y 2018 resultaron ser insuficientes por el nuevo avistamiento del caracol en el área, con el agravante de encontrar un paciente con meningoencefalitis eosinofílica epidemiológicamente asociado con el molusco(AU)

Introduction: Helmint Angiostrongylus cantonensis is a natural parasite in the lungs of ratas. Ocassionally it can produced an eosinophilic meningoencephalitis in men by larvae accidental ingestion. Methods: A qualitative study was performed from a focal group by a semi-structural survey in a health area from San Miguel del Padrón municipality. Objectives: To describe the actions developed by the local sanitarian authorities for the control of African giant snails between 2016 and 2018 and the appearance of a patient suffering from eosinophilic meningoencephalitis due to Angiostrongylus cantonensis linked to the presence of this snail. Results: It has been established an eradication strategy for the elimination of this invasive species based on a community work. Two years later, it was observed again the giant African snail in the area with a patient suffering from Angiostrongylus cantonensis eosinophilic meningoencephalitis epidemiologically associated. Conclusions: The 2016 actions were not efficient due to the emerging vector and the further finding of a patient linked with the parasite(AU)

Diagnóstico neuroinmunológico de meningoencefalitis eosinofílica producida por Angiostrongylus cantonensis / Neuroimmunological diagnosis of eosinophilic meningoencephalitis caused by Angiostrongylus cantonensis

El diagnóstico de la meningoencefalitis por Angiostrongylus cantonensis se establece por la presencia de las larvas del helminto en el líquido cefalorraquídeo, pero esta evidencia es muy difícil de encontrar ya que las larvas son lábiles en este medio y no se hallan con frecuencia. Debido a que en Cuba, la presencia del parásito se remonta a 1981 y este parásito es el único que puede provocar esta enfermedad en el país, se realiza una revisión con el objetivo de revisar la literatura publicada sobre el tema para acopiar toda la evidencia que ayude al diagnóstico auxiliar de meningoencefalitis eosinofílica. Se propone que el estudio de la síntesis intratecal de IgE y C3c, unido al patrón de síntesis local de IgA+IgG principalmente y de IgG1+ IgG2 resultan las más indicadas. Existen otras proteínas que pudieran auxiliar como la síntesis intratecal de C4 y en menor proporción MBL(AU)

Diagnosis of meningoencephalitis due to Angiostrongylus cantonensis is based on the presence of helminth larvae in cerebrospinal fluid, but such evidence is very hard to find, since the larvae are labile in this medium and cannot be spotted easily. Based on the fact that presence of the parasite in Cuba dates back to 1981, and this is the only agent of the disease in the country, a review was conducted with the purpose of going over the published literature about the topic and gather evidence leading to the auxiliary diagnosis of eosinophilic meningoencephalitis. The study of the intrathecal synthesis of IgE and C3c, alongside the local synthesis pattern for IgA+IgG mainly and IgG1+IgG2, are proposed as the most appropriate. Other useful proteins are the intrathecal synthesis of C4 and to a lesser extent MBL(AU)

Angiostrongylus cantonensis in North African hedgehogs as vertebrate hosts, Mallorca, Spain, October 2018.

In October 2018, two Atelerix algirus hedgehogs were admitted to the Wildlife Rehabilitation Hospital in Mallorca (Balearic Islands, Spain) with signs of acute neurological disease. Necropsy detected immature, fully developed nematodes in the subarachnoid space of both hedgehogs, including a gravid female worm. DNA-based molecular tools confirmed the nematode as Angiostrongylus cantonensis, an important aetiological agent of eosinophilic meningitis in humans. So far this zoonotic parasite in has not been reported in western European wildlife.

Cross-reactivity of the 31 kDa antigen of Angiostrongylus cantonensis - Dealing with the immunodiagnosis of meningoencephalitis.

The primary causative agent of eosinophilic meningoencephalitis (EoM) in endemic regions is the nematode Angiostrongylus cantonensis. The occurrence of EoM was previously restricted to countries in Southeast Asia and the Pacific Islands; however, more recently, it has been reported from other regions, including Brazil. The commonly used diagnosis is detection of specific antibody reactivity to the 31 kDa antigen, which is derived from female worm somatic extracts. Here we report the occurrence of cross-reactivity to this antigen in sera from other parasitic infections, especially those that may cause EoM, such as gnathostomiasis, toxocariasis, hydatidosis and strongyloidiasis. We also demonstrated that the cross-reactivity, in part, is dependent of the concentration of antigen used in Western blot assays. We discuss the importance of these findings on the interpretation of this test.

Meningoencefalitis Eosinofílica por Angiostrongylus Cantonensis y variables meteorológicas / Eosinophilic meningoencephalitis due to Angiostrongylus cantonensis and meteorological variables

Introducción: Angiostrogylus cantonensis es un helminto que vive en las arterias pulmonares de dos especies de ratas: Rattus rattus y Rattus norvergicus. El hombre es un hospedero accidental que se infecta al ingerir las larvas de tercer estadio a través de vegetales o frutas mal lavados y por la ingestión de diferentes especies de moluscos, que constituyen los hospederos intermediarios. Objetivo: caracterizar las condiciones de humedad, temperatura y lluvia caída en las zonas donde se reportaron los pacientes y su vinculación con la aparición de los casos de meningoencefalitis eosinofílica por Angiostrongylus cantonensis. Material y Métodos: se estudiaron 34 pacientes diagnosticados con esta enfermedad que fueron ingresados en el Hospital Pediátrico San Miguel del Padrón, en La Habana, Cuba, en el período de enero de 1991 hasta enero del 2014. Para la obtención de los datos correspondientes a las variables meteorológicas se acudió con la fecha de ingreso de los enfermos a los registros diarios y mensuales del Instituto de Meteorología. Se calcularon los coeficientes de correlación para cada variable meteorológica y el número de casos y se ajustaron las rectas de regresión correspondientes. Resultados: los enfermos se concentraron en períodos en que la humedad promedio anual osciló entre 75 y 76 por ciento, mientras que la humedad ambiental varío entre 65 y 80 por ciento. La probabilidad de encontrar casos enfermos es mayor entre los años con lluvia anual promedio entre 68 y más de 120 mm. Para la temperatura, la correlación fue negativa, a medida que se registra un aumento de temperatura hay una disminución del número de enfermos. Conclusiones: el mayor número de casos de enfermos coincide con las condiciones de humedad ambiental y temperatura optimas para el desarrollo de las larvas de tercer estadio en los caracoles(AU)

Introduction: Angiostrongylus cantonensis is a helmint that lives in the pulmonary arteries of two rats species: Rattus rattus and Rattus norvergicus. Human is an accidental host that is infected by ingestion of third larvae stage in bad washed vegetables, fruit and different species of mollusks which constitute the intermitted hosts. Objective: to characterize the humidity, temperature and rain fall in zones where were reported the patients and its relation with the eosinophilic meningoencephalitis cases by Angiostrongylus cantonensis. Material and Methods: 34 patients were diagnosed with this illness. They were admitted at Hospital Pediatric San Miguel del Padrón, Havana, Cuba from January 1991until January 2014. In order to obtain of meteorological variables data we remit to the admission patient date and dairy and monthly register of the Meteorology Institute. Were calculated the correlation coefficient for each meteorological variable and the number of cases related to the different variables and the regression lines were performed. Results: the more quaintly of patients were grouped in periods where annual average fluctuated between 75 y 76 % and environment humidity oscillated between 65 y 80 percent.The probability to find illness cases is major in the years with average annual rain between 68 and more of 120mm. An inverse correlation was observed for temperature. Conclusions: the larger number of reported cases coincides with the best conditions of environmental humidity and temperature for the development of the third stage larvae in mollusks(AU)

Occam's razor in the management of ventriculoperitoneal shunt dysfunction: Diagnosis and management of an unusual pediatric case.

Ventriculoperitoneal (VP) shunt surgery is probably the commonest surgical procedure in neurosurgery. Belying its technical simplicity is the myriad complications associated with it. Shunt malfunction is a common complication associated with this surgery, second only to shunt related infections, which may be associated with it. Sterile cerebrospinal fluid (CSF) eosinophilia (CE) has been reported with VP shunts, which may or may not be related to the dysfunction. Eosinophilia in the CSF has also been associated with a number of other conditions including parasitic infestations in the brain. This may be unrelated to the shunt surgery. We present a case of a child, operated earlier for hydrocephalus, who presented with sub-acute loss of vision and bilateral oculomotor paresis. CSF from a chamber tap revealed eosinophilia. The commonest presenting symptom of shunt malfunction is raised intracranial pressure. There are no reports in the literature of VP shunt malfunction presenting with bilateral oculomotor paresis and decreased visual acuity. The associated CE complicated the clinical picture, especially since the initial brain radiology was normal. We discuss the clinical differential diagnosis of this very interesting presentation, management dilemmas and outcome in this child. This rare clinical presentation was found to be the result of a shunt malfunction and not due to any rare parasitic infestation of the brain. Occam's razor dictates that the simplest explanation in a given situation is usually the most accurate, as is seen in this case.

A Case of Ocular Angiostrongyliasis with Molecular Identification of the Species in Vietnam.

A 23-year-old female residing in a village of Cao Bang Province, North Vietnam, visited the Hospital of Hanoi Medical University in July 2013. She felt dim eyes and a bulge-sticking pain in her left eye for some days before visiting the hospital. In the hospital, a clinical examination, an eye endoscopy, and an operation were carried out. A nematode specimen was collected from the eye of this patient. The body of this worm was thin and long and measured 22.0×0.3 mm. It was morphologically suggested as an immature female worm of Angiostrongylus cantonensis. By a molecular method using 18S rRNA gene, this nematode was confirmed as A. cantonensis. This is the first molecular study for identification of A. cantonensis in Vietnam.

A Case of Ocular Angiostrongyliasis with Molecular Identification of the Species in Vietnam

A 23-year-old female residing in a village of Cao Bang Province, North Vietnam, visited the Hospital of Hanoi Medical University in July 2013. She felt dim eyes and a bulge-sticking pain in her left eye for some days before visiting the hospital. In the hospital, a clinical examination, an eye endoscopy, and an operation were carried out. A nematode specimen was collected from the eye of this patient. The body of this worm was thin and long and measured 22.0x0.3 mm. It was morphologically suggested as an immature female worm of Angiostrongylus cantonensis. By a molecular method using 18S rRNA gene, this nematode was confirmed as A. cantonensis. This is the first molecular study for identification of A. cantonensis in Vietnam.

Clinical aspects of eosinophilic meningitis and meningoencephalitis caused by Angiostrongylus cantonensis, the rat lungworm.

Angiostrongylus Eosinophilic Meningitis is caused by human infection with larvae of the rat lungworm, Angiostrongylus cantonensis. The clinical presentation includes a spectrum of disease, from meningitis through radiculitis, cranial nerve abnormalities, ataxia, encephalitis, coma, and rarely death. The condition is diagnosed by recognizing the triad of: the clinical syndrome, eosinophils in the cerebrospinal fluid or blood, and exposure history. A history of eating raw or poorly cooked snails is classic, but ingestion of other intermediate hosts or unwashed produce (such as lettuce) harboring hosts is not uncommon. Several serologic tests exist but none has yet been fully validated. There is good evidence that a 2 week course of high dose corticosteroids shortens the duration and severity of symptoms. There is somewhat weaker evidence that albendazole reduces symptoms. The combination of prednisolone and albendazole is being used more commonly for treatment. Some suggestions for future research are given.

Intoxicação por sal em suínos: aspectos epidemiológicos, clínicos e patológicos e breve revisão de literatura / Salt poisoning in swine: epidemiological, clinical and pathological aspects and brief review of the literature

Salt poisoning occurs commonly in pigs by excessive intake of sodium chloride or by a period water deprivation for followed by free access to water. The objective of this work is to aggregate data from cases of salt poisoning, combining existing data in the literature and describe the main clinical and pathological features observed. We reviewed five outbreaks, one of which was carefully monitored. In three of them the intake of sodium chloride had been determined. Clinical signs were basically seizures with the lateral decubitus with paddling movements. Circling was observed in some cases. Sodium determination in muscle of and liver fragments, serum, cerebrospinal fluid and aqueous humor showed increased concentrations of this ion. There was eosinopenia characterizing increased recruitment eosinophils from the circulation into the brain. In all outbreaks eosinophil infiltration was observed in the meninges and the Virchow-Robin space of the cerebral cortex. Cortical laminar necrosis was more pronounced in the brain of pigs from one of the outbreaks in which animals were sick for six days. The combination of these two lesions characterizes the disease. The changes observed result from high concentrations of sodium in the brain causing cause edema that leads to increased intracranial pressure and decreased perfusion to the brain tissue causing diffuse ischemia and neuronal necrosis, with consequent malacia.

Intoxicação por sal ocorre comumente em suínos por ingestão excessiva de cloreto de sódio ou por privação de água por um período de tempo, seguido de um livre acesso a água abundante. O objetivo deste trabalho é agregar dados de casos de intoxicação por sal, diagnosticados, compilar dados já existentes na literatura e caracterizar as principais alterações clínicas e patológicas observadas. Foram revisados cinco surtos, sendo que um deles foi minuciosamente acompanhado. Em três deles a ingestão de cloreto de sódio foi determinada. Os sinais clínicos eram basicamente convulsões, com intensos tremores musculares e desenvolvimento de opistótono. Os animais permaneciam em decúbito lateral, fazendo movimentos de pedalagem. Alguns andavam em círculos. Dosagens de sódio em fragmentos de músculo e de fígado, no soro, líquor e humor aquoso revelaram concentrações aumentadas do íon. A quantidade de eosinófilos circulantes foi baixa caracterizando grande recrutamento dessas células para o encéfalo. Em todos os surtos foi observada infiltração de eosinófilos nas leptomeninges e no espaço de Virchow-Robin do córtex cerebral. Necrose cortical laminar foi observada mais detalhadamente em um dos surtos onde os suínos estavam doentes há seis dias. A combinação dessas duas lesões caracteriza a doença. Todas as alterações observadas podem ser explicadas pela provável patogenia da doença em que as elevadas concentrações de sódio causam edema cerebral que leva ao aumento da pressão intracraniana e decréscimo da perfusão para o cérebro causando isquemia difusa e necrose neuronal, com consequente malacia.

Rapid progression and brain atrophy in anti-AMPA receptor encephalitis.

Anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor encephalitis is an anti-neuronal surface antigen autoimmune encephalitis that is rarely reported. Our study evaluated the first known patient who developed anti-AMPA receptor encephalitis during pregnancy. Initial brain MRI revealed bilateral limbic encephalitis. However, rapid brain atrophy on MRI with extensive hypometabolism of cerebral cortices, caudate nuclei and brain stem hypoperfusion on (18)F-FDG PET developed when clinically progressed. IgG index of serial CSF studies reflected the clinical improvements after plasmapheresis and plasma exchange. The clinical spectrum of anti-AMPA receptor encephalitis may be expanded from limited limbic involvement to extended central nervous system.