Encefalomielitis aguda diseminada por Mycoplasma pneumoniae en un niño previamente sano / Acute disseminated encephalomyelitis due to Mycoplasma pneumoniae in a previously healthy boy

Se presenta el caso de un niño de 5 años sin antecedentes de enfermedad, que se internó en terapia intensiva por convulsiones tónico-clónicas focalizadas en la cara y en el hemicuerpo derecho, con documentación de temperatura axilar de 37,4°C. Se descartó la presencia de gérmenes comunes y la etiología viral a través de estudios de muestras de líquido cefalorraquídeo (LCR). Se sospechó la presencia de Mycoplasma pneumoniae por comprobarse inmunofluorescencia positiva en suero para anticuerpos de clase IgM. El diagnóstico se confirmó mediante la detección del ADN de dicho patógeno sobre la biopsia cerebral efectuada por el método de la reacción en cadena de la polimerasa (PCR) y una histología compatible con encefalomielitis aguda diseminada. El paciente recibió tratamiento con claritromicina y su evolución fue favorable. Al menos dentro de nuestros conocimientos, este es el primer caso en el que se detectó ADN de M. pneumoniae en una biopsia cerebral por el método de PCR.

We present here the case of a previously healthy 5 year-old boy hospitalized in an intensive care unit due to tonic-clonic seizures focused on the face and right side of the body, and axillary temperature of 37.4 °C. Common bacterial and viral etiology was ruled out through studies of cerebrospinal fluid (CSF) samples. Mycoplasma pneumoniae was suspected by a positive immunofluorescence serum test for IgM class antibodies. Finally, with a brain biopsy, M. pneumoniae was confirmed by polymerase chain reaction (PCR) and acute disseminated encephalomyelitis by pathological anatomy. The patient was treated with clarithromycin and had an uneventful evolution. At least to our knowledge, this is the first case in which M. pneumoniae DNA was detected by PCR in a brain biopsy.

Encefalomielitis diseminada aguda posinfecciosa en un paciente con sospecha de infección por virus dengue / Postinfectious acute disseminated encephalomyelitis in a patient with suspected dengue virus infection

Introducción: La infección por virus dengue es considerada una de las arbovirosis de mayor prevalencia en los países tropicales. La encefalomielitis diseminada aguda es un trastorno inflamatorio desmielinizante y multifocal que afecta al sistema nervioso central, de inicio agudo y curso clínico monofásico. El proceso inflamatorio se encuentra mediado por mecanismos inmunológicos y su relación con infecciones por el virus dengue aún no se establece con claridad. Objetivo: Describir un caso clínico con manifestaciones del sistema nervioso central después de una probable infección por el virus dengue. Presentación del caso: Paciente femenina de 50 años, con antecedentes de hipertensión arterial controlada. Quince días después de un cuadro de fiebre de 4 días de duración, que posiblemente fue por una infección por el virus dengue, comienza con síntomas y signos de afectación neurológica caracterizadas por ligera irritabilidad, dificultad para la concentración en una actividad específica de la vida cotidiana. Progresivamente se nota dificultad motora en el hemicuerpo izquierdo además de encontrarse agitada y distraída, motivo por el cual se decide su ingreso hospitalario. Se realiza el diagnóstico a través de los hallazgos en el examen físico, los estudios positivos de resonancia magnética nuclear y el resultado positivo de la IgM de dengue en sangre. Tanto la evolución clínica como la respuesta al tratamiento con esteroides fueron favorables. Conclusiones: El evento ocurrido en este caso sugiere que los facultativos deben tener presente el diagnóstico de encefalomielitis diseminada aguda en pacientes que han tenido infección previa o alta sospecha de esta por el virus dengue(AU)

Introduction: Dengue virus infection is one of the most prevalent arboviruses in tropical countries. Acute disseminated encephalomyelitis is an inflammatory demyelinating multifocal disorder affecting the central nervous system. Its onset is acute and its clinical course monophasic. The inflammatory process is mediated by immunological mechanisms, and its relationship to dengue virus infections is still not clear. Objective: Describe a clinical case of central nervous system manifestations after probable dengue virus infection. Case presentation: Female 50-year-old patient with a history of controlled hypertension. Fifteen days after a 4-day fever episode, possibly due to dengue virus infection, the patient starts presenting neurological signs and symptoms, such as slight irritability and difficulty to concentrate on a specific activity of daily living. The patient notices progressive motor difficulty in her left hemibody and she feels agitated and distracted. It is therefore decided for her to be hospitalized. A diagnosis is made based on physical examination findings, positive nuclear magnetic resonance studies, and the positive result of the dengue IgM blood test. Both the patient's clinical evolution and her response to treatment with steroids were favorable. Conclusions: The event herein described suggests that physicians should consider the diagnosis of acute disseminated encephalomyelitis in patients with previous infection or high suspicion of infection with dengue virus(AU)

Enfermedades desmielinizantes / Demyelinating disorders

Las enfermedades desmielinizantes constituyen un grupo de afecciones de etiología autoinmune dirigida contra la mielina del sistema nervioso central. En muchos casos, el inicio del cuadro es precedido por una infección viral inespecífica. La esclerosis múltiple evoluciona con recaídas y remisiones con déficit neurológicos polifocales, siendo los más frecuentes la neuritis óptica, la mielitis transversa y el compromiso de tronco encefálico. Se caracteriza por lesiones hiperintensas que se observan en una resonancia magnética nuclear (RMN) en T2 y FLAIR peri-ventriculares y peri-callosas, cerebelo, tronco y médula espinal. La neuromielitis óptica se caracteriza por la presencia de neuritis óptica y mielitis transversa asociada a síndrome de área postrema y diencefálico. Las lesiones en RMN se distribuyen en los sectores ricos en acuaporina-4 (AQP-4): hipotálamo, peri tercer y cuarto ventrículo, nervios ópticos y médula espinal. Los anticuerpos anti AQP4 ayudan al diagnóstico aunque no son esenciales para el mismo. La encefalomielitis diseminada aguda es un cuadro clásicamente monofásico caracterizado por una encefalopatía aguda asociada a lesiones en RMN hiperintensas en T2 y FLAIR bilaterales, asimétricas, de gran tamaño y de bordes irregulares. En los tres casos, el líquido cefalorraquídeo (LCR) puede mostrar pleocitosis e hiperproteinorraquia. La presencia de bandas oligoclonales en LCR es característica de la esclerosis múltiple. En todos los casos, el tratamiento agudo incluye corticoides a altas dosis por vía endovenoso y en caso de no respuesta, plasmaféresis. Tanto la esclerosis múltiple como la neuromielitis óptica requieren tratamiento a largo plazo para evitar nuevas recaídas ya que se trata de enfermedades recurrentes.

Demyelinating diseases are a group of conditions of autoimmune etiology directed against the myelin of the central nervous system. In many cases, the onset of the illness is preceded by a nonspecific viral infection. Multiple sclerosis is a disease that evolves with relapses and remissions with polyfocal neurological deficits, being the most frequent optic neuritis, transverse myelitis and encephalic trunk involvement. Typically, magnetic resonance image (MRI) shows peri-ventricular, peri-callosal, cerebellum, brain stem and spinal cord hyperintensive lesions in T2 and FLAIR weighted images. Optic neuromyelitis is characterized by the presence of optic neuritis and transverse myelitis associated with the postrema and diencephalic area syndrome. MRI lesions are distributed in sectors rich with aquaporine-4 channels (AQP-4): hypothalamus, third and fourth ventricle, optic nerves and spinal cord. Finding anti AQP4 antibodies is useful for the diagnosis although they are not essential for it. Acute disseminated encephalomyelitis is typically a monophasic condition characterized by acute encephalopathy associated with hyperintense MRI large, bilateral and irregular asymmetric lesion in T2 and FLAIR weighted images. In all three cases, cerebral spine fluid (CSF) can show pleocytosis and hyperproteinorrachia. The presence of oligoclonal bands in CSF is characteristic of multiple sclerosis. In all cases, acute treatment includes high dose intravenous corticosteroids and plasmapheresis in non-responsive cases. Both multiple sclerosis and optic neuromyelitis require long-term treatment to prevent relapse and recurrent diseases.

Potential role of dengue virus, chikungunya virus and Zika virus in neurological diseases

This study showed that laboratory markers of recent infection by dengue, Zika or chikungunya arboviruses were detected in the biological samples of approximately one-third of patients with encephalitis, myelitis, encephalomyelitis or Guillain-Barré syndrome, in a surveillance programme in Piauí state, Brazil, between 2015-2016. Fever and myalgia had been associated with these cases. Since in non-tropical countries most infections or parainfectious diseases associated with the nervous system are attributed to herpesviruses, enteroviruses, and Campylobacter jejuni, the present findings indicate that in tropical countries, arboviruses may now play a more important role and reinforce the need for their surveillance and systematic investigation in the tropics.

Encefalitis recurrente post vacuna influenza anual / Recurrent encephalitis following annual influenza vaccine: case report

Influenza vaccine is rarely associated with neurological adverse effects such as Guillain Barré syndrome, encephalitis or aseptic meningitis. We report the case of a male patient that presented two episodes of acute encephalitis in consecutive years, 16 and 20 days after his annual influenza vaccine shot, respectively. In both instances, patient required ICU admission and evolved with fast recovery and no sequels. The first episode was managed empirically as herpetic encephalitis but an extensive study was performed in the second episode without identifying any microorganism. Neuroimaging studies also discarded acute disseminated encephalomyelitis. Mild pleocytosis of mononuclear predominance was detected in both cases in CSF. Naranjo score punctuated 8 points indicating a probable causal relationship. Acute encephalitis is a rare adverse effect of influenza vaccine and occurs several days after immunization. It has a broad differential diagnosis, and appears to be of self-limited duration and associated with good prognosis.

La vacuna influenza en raras ocasiones se asocia a efectos adversos neurológicos tales como síndrome de Guillain Barré, encefalitis o meningitis aséptica, entre otros. Presentamos el caso clínico de un paciente de sexo masculino de 66 años que presentó dos episodios de encefalitis en años consecutivos, 16 y 20 días después de recibir la vacuna anual de influenza, respectivamente. En ambos casos requirió ingreso a UCI y evolucionó con una rápida recuperación y sin secuelas. El primer episodio fue manejado empíricamente como una encefalitis herpética y en el segundo se hizo un extenso estudio microbiológico que no identificó microorganismos. Los estudios de neuroimágenes descartaron una encefalomielitis aguda diseminada. En ambos casos se pesquisó pleocitosis de predominio mononuclear en el LCR. El score de Naranjo dio 8 puntos indicando una probable relación causal. La encefalitis aguda es un efecto adverso muy infrecuente tras la vacuna influenza y ocurre varios días después de la inmunización. Tiene un amplio diagnóstico diferencial, parece ser autolimitado y de buen pronóstico.

Acute disseminated encephalomyelitis following inactivated influenza vaccination in the Brazilian Amazon: a case report

Here, we describe a case of acute disseminated encephalomyelitis (ADEM) that occurred during a plausible risk interval following inactivated influenza vaccination in a previously healthy 27-year-old man from Manaus, Brazil. He was treated with intravenous methylprednisolone and immunoglobulin. One-month follow-up revealed resolution of the brain lesions, but not of the spinal cord lesions. No recurrence or progression of the main neurological symptoms was observed. After two years of monitoring, the patient continues to experience weak lower limbs and urinary retention. Thus, we recommend that ADEM should be considered in a patient presenting with neurological symptoms after influenza vaccination.

Cerebrospinal fluid analysis in the context of CNS demyelinating diseases / Analise do liquido cefalorraquiano no contexto das doencas desmielinizantes do SNC

The central nervous system demyelinating diseases are a group of disorders with different etiologies, characterized by inflammatory lesions that are associated with loss of myelin and eventually axonal damage. In this group the most studied ones are multiple sclerosis (MS), neuromyelitis optic (NMO) and acute disseminated encephalomyelitis (ADEM). The cerebrospinal fluid is essential to differentiate between these different syndromes and to define multiple sclerosis, helping to assess the probability of Clinical Isolated Syndrome turn into multiple sclerosis.

As doenças desmielinizantes do sistema nervoso central são um grupo de desordens de diferentes etiologias, caracterizadas por lesões inflamatórias associadas a perda da mielina e eventualmente dano axonal. Neste grupo de doenças, as mais estudadas são a esclerose múltipla (EM), a neuromielite óptica e a encefalomielite aguda disseminada. O estudo de liquido cefalorraquiano é essencial para o diagnóstico diferencial entre as diferentes síndromes e para a definição de EM, ajudando a estimar a probabilidade da transformação da síndrome clínica isolada em EM.

Acute disseminated encephalomyelitis following falciparum malaria.

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the brain and spinal cord which is preceded by many viral and bacterial infections. We present a rare case of ADEM following falciparum malaria infection. Early identification of ADEM is highly required as it responds to treatment very well and delay in diagnosis may lead to permanent neurological sequelae.

Encefalomielitis aguda diseminada y colitis ulcerosa: descripción de un caso clínico / Acute disseminated encephalomyelitis and ulcerative colitis: description of a clinical case in magnetic resonance

Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterized by an acute or subacute onset of neurological symptoms and focal signs in association with multifocal demyelinating findings on neuroimaging. Spontaneous resolution has been reported within days of onset, corticosteroids therapy accelerates the resolution time. ADEM has been associated with previous infections, and its relationship with other autoinmune disease is unclear. A case of ADEM is reported, in a patient previously diagnosed with ulcerative pancolitis, whose neurological symptoms resolved after 3 days of hospital admission without evident sequelae after receiving a short empirical treatment with ampicilin, ceftriaxone, acyclovir and intravenous steroids.

La encefalomielitis aguda diseminada (EAD) es un trastorno desmielinizante agudo del Sistema Nervioso Central, caracterizado por la presentación aguda de síntomas y signos neurológicos en asociación con hallazgos multifocales de desmielinización en las neuroimágenes. Se describe en la literatura resolución espontánea a los pocos días de presentación, con secuelas variables. La terapia corticoesteroidal aceleraría este tiempo de resolución. Se ha relacionado con eventos infecciosos previos, y su relación con otras enfermedades autoinmunes no es clara. Se presenta un caso de EAD en paciente con diagnóstico previo de pancolitis ulcerosa cuya sintomatología neurológica se resuelve tras 3 días del ingreso hospitalario, sin secuelas evidentes, tras recibir tratamiento empírico corto con Ampicilina, Ceftriaxona, Aciclovir y corticoides endovenosos en dosis de estrés.

A Case of Acute Disseminated Encephalomyelitis Associated with Hepatitis C Virus Infection

Acute disseminated encephalomyelitis (ADEM) is a monophasic autoimmune demyelinating disease of the central nervous system, which typically follows acute viral or bacterial infection or vaccination. We report a case of ADEM associated with hepatitis C virus (HCV) infection with positive serum and cerebrospinal fluid (CSF) anti-HCV antibody. After steroid treatment, neurologic symptoms were improved. Virus triggers autoimmunity or direct viral invasion plays a part in the genesis of ADEM. This is the first reported case of ADEM with anti-HCV antibody in the CSF.

Asociación de encefalomielitis diseminada aguda y síndrome de Guillain-Barré en un adulto / Association between acute disseminated encephalomyelitis and Guillain Barré syndrome in an adult

La encefalomielitis diseminada aguda (EMDA) y el síndrome de Guillain-Barré (SGB) son reconocidas como entidades distintas, que afectan diferentes sectores del sistema nervioso, pero que comparten varias características tales como la patogenia autoinmune, el impacto sobre la mielina y el antecedente de infección viral o vacunación una a cuatro semanas previas al cuadro clínico. Se presenta un paciente varón de 41 años de edad que consultó por presentar fiebre, debilidad en miembros inferiores y somnolencia dos semanas posteriores a episodio agudo de gastroenteritis. Al ingreso se constató deterioro del sensorio (obnubilación) hiperreflexia patelar, Babinski bilateral y vejiga neurogénica. Veinticuatro horas después desarrolló paraplejía flácida y arreflexia generalizada, requiriendo asistencia respiratoria mecánica por insuficiencia respiratoria. El líquido cefalorraquídeo mostró pleocitosis mononuclear e hiperproteinorraquia. El estudio electrofisiológico evidenció importante disminución de las velocidades de conducción en ambos nervios ciáticos poplíteos externos, compatible con polineuropatía desmielinizante. La resonancia magnética nuclear mostró imágenes compatibles con desmielinización en cerebro, protuberancia y segmentos medulares dorsales. Se realizó diagnóstico de ASEMDA-SGB e inició tratamiento con metilprednisolona e inmunoglobulina intravenosa. Evolucionó favorablemente, recuperando las funciones motoras, vesical y la sensibilidad, siendo capaz de deambular luego de seis meses. La asociación de EMDA y SGB (ASEMDA-SGB) es una condición infrecuente, generalmente señalada como de mal pronóstico, en la cual un diagnóstico precoz y un rápido y enérgico tratamiento pueden mejorar substancialmente la evolución.

Acute disseminated encephalomyelitis (ADEM) and Guillain-Barré Syndrome (GBS) are commonly recognized as separated entities involving different parts of the nervous system. However, they share some features such as: autoimmune pathogenesis, myelin injury and previous history of viral infections or vaccination. We report the case of a 41 year-old man who developed fever, lower limbs weakness and obtundation fifteen days after an acute gastroenteritis. Neurological examination showed patellar hypereflexia, bilateral Babinski and neurogenic bladder. Twenty-four hours later he developed flaccid paraparesis, generalized areflexia and respiratory failure that was supported by mechanical ventilation. Cerebrospinal fluid showed mononuclear pleocytosis and elevated proteins. Electrodiagnosis showed important reduction of conduction velocity on both peroneal nerves. Magnetic Resonance Imaging revealed white matter lesions in brain, pons and thoracic levels of the spinal cord. Diagnosis of the association between ADEM and GBS (ASADEM-GBS) was made and treatment with corticosteroids and intravenous immunoglobulin was started. The patient recovered motor, sensory and bladder functions and he was able to walk six months later. ASADEM-GBS is an uncommon entity generally considered of poor outcome; however a rapid diagnosis and treatment can substantially improve the prognosis.

Encefalopatía desmielinizante aguda en el adulto: presentación y algoritmo diagnóstico / Acute disseminated encephalomyelitis in the adulthood

Objetivo: estandarizar la metodología diagnóstica diferencial de lesiones pseudotumorales. Analizar a partir de este caso la aparición de esta entidad poco habitual en la población adulta. Material y métodos: se realiza historia epidemiológica de un paciente masculino de 51 años de edad que concurre a través de consultorios externos presentando inestabilidad en la marcha y diparesia braquial de 7 días de evolución. Como antecedentes refiere episodio febril de origen desconocido y resolución espontánea 20 días previos. Resultados: se realizaron estudios de tomografía axial computada y resonancia magnética nuclear de encéfalo evidenciando lesiones bilaterales. Se completa estudios con screening oncológico negativo. Líquido cefalorraquídeo: bandas oligoclonales negativas, proteinorraquia, PCR + para VEB. PESS, PEV, PEA: negativos. Se somete a tratamiento médico con metilprednisolona 1 gramo diario durante 3 días. Conclusiones: el ADEM (Encefalopatía Desmielinizante Aguda) es una entidad inflamatoria inmunomediada de diversas formas de presentación. Cumple un rol importante en el diagnóstico, la correlación de antecedentes epidemiológicos del paciente como así también la evolución progresiva del cuadro clínico y estudios complementarios.

Objective: To standardize the differential diagnosis method of pseudotumoral lesions. To analyze from this case the occurrence of this unusual condition in adults. Material and methods: An epidemilogical history is conducted in a 51 year old male patient who comes to the out-patient offices showing walking instability and brachial cliparesis during 7 days. The patient reports a febrile state with spontaneous resolution 20 days before the consultation as background. Results: A computerized axial tomography and an encephalic nuclear magnetic resonance were performed showing bilateral lesions. The studies were complete with an oncologic screening of negative results. Cephalorachidian liquid: negative oligocional bands, proteinorachia, detection of EBV (Epstein-Barr Birus) by PCR (Polymerase Chain Reaction). SEPs (Somatosensitive Evoked Potentials), VEP (Visual Evoked Potentials), PEA (Auditory Evoked Potentials): NEGATIVE. The patient receives medical treatment with 1 gram daily methylprednisolone for 3 days. Conclusions: ADEM (Acute Disseminated Encephalomyelitis) is an immune mediated inflammatory condition that can appear in several ways. The patient's epidemiological background correlation, as well as the progressive evolution of the medical condition and complementary analyses play an important role in diagnosis.

Childhood multiple sclerosis mimicking acute disseminated encephalomyelitis.

Multiple sclerosis is a demyelinating disease affecting people in early to middle adulthood and is uncommon in childhood. Here we report a patient of multiple sclerosis who presented much before 10 years of age with features suggestive of acute disseminated encephalomyelitis and altered sensorium which is quite different from the features of multiple sclerosis in adults. Diagnosis in this patient was made on the basis of predominant white matter involvement with clinically remitting and relapsing course of the disease on follow up over 31/2 years, substantiated by finding multiple areas of demyelinating plaques in magnetic resonance imaging, while visual evoked potential and cerebrospinal fluid study supported demyelination. Hence, children with initial diagnosis of acute disseminated encephalomyelitis need long term follow up as they may develop multiple sclerosis.

Evaluation of the cases with acute disseminated encephalomyelitis.

All of the cases were reevaluated with systemic and neurological examinations, serologic tests, cerebrospinal fluid investigations, magnetic resonance imaging. Age ranged between 2.5 and 16 years. Five of the cases had initial infections. Patients presented most often with motor deficits (75%), secondly with loss of consciousness (33%), and seizures (33%). Spinal fluid abnormalities occurred in 41.6%. Cranial, and spinal magnetic resonance imaging (MRI) revealed hyperintense signal changes mainly in basal ganglia and thalamus (58%), cortical and subcortical areas (33) in T2 weighted images. Myelitis was determined in two cases. Six patients were treated with steroid, and 3 were treated with intravenous immunoglobulin. Ten patients recovered completely. We observed relapse in one case and recurrence in two cases. These cases responded well to high dose intravenous prednisolone followed by oral prednisolone for 6 months.

The evaluation of the cases with acute disseminated encephalomyelitis.

OBJECTIVE: We aimed to describe the epidemiologic, clinical, laboratory features, neuroimaging, treatment, and outcome of children with acute disseminated encephalomyelitis in a cohort study. METHODS: In this study, twelve children who were diagnosed as acute disseminated encephalomyelitis were reviewed retrospectively. All of the cases were reevaluated with systemic and neurological examinations, serologic tests, cerebrospinal fluid investigations, magnetic resonance imaging. RESULT: Their age ranged between 2.5 and 16 years. Five of the cases had initial infections. Patients presented most often with motor deficits (75%), secondly with loss of conscious (33%), and seizures (33%). Spinal fluid abnormalities occurred in 41.6%. Cranial, and spinal magnetic resonance imaging (MRI) revealed hyperintense signal changes mainly in basal ganglia and thalamus (58%), cortical and subcortical areas (33) in T2 weighted images. Myelitis was determined in two cases. Six patients were treated with steroid, and 3 were treated with intravenous immunoglobulin. Ten patients recovered completely. We observed relapse in one case and recurrence in two cases. These cases responded well to high dose intravenous prednisolone followed by oral prednisolone for 6 months. CONCLUSION: Outlook recovery is generally good in acute disseminated encephalomyelitis. Recurrence and neurological deficits are rarely seen. Early treatment of prednisolone is one of the most important factors to determine the prognosis in this disease.

Encefalomielitis difusa aguda en la edad pediátrica / Acute diffuse encephalomyelitis in chilhood

La encefalomielitis diseminada aguda (EMDA) es una enfermedad inflamatoria aguda del sistema nervioso central, inmunomediada, que es más frecuente en la edad pediátrica. Su diagnóstico se basa en la clínica, en la confirmación del proceso desmielinizante agudo a través de la resonancia magnética y en la exclusión de otras posibles etiologías de acuerdo con los criterios propuestos en el consenso de expertos del “International Pediatric MS Study Group”. La evolución de los conocimientos y los nuevos criterios diagnósticos justifican la revisión del tema. Se realizó un estudio descriptivo y retrospectivo sobre la base de la revisión de las historias clínicas de una serie consecutiva de 12 pacientes menores de 15 años que ingresaron al Hospital Pediátrico del Centro Hospitalario Pereira Rossell con diagnóstico de EMDA desde el 1 de enero del 2000 al 31 de diciembre del 2007 y su seguimiento posterior en la Cátedra de Neuropediatría de la Facultad de Medicina y el Servicio de Neuropediatría de dicho hospital. Si bien se presentaron con una encefalopatía aguda severa, la evolución fue buena en la mayoría de los pacientes, aunque 2 pacientes presentaron secuelas motoras y uno epilepsia secundaria y compromiso cognitivo. En 11 pacientes la enfermedad tuvo un curso monofásico. La importancia del seguimiento clínico e imagenológico radica en que existen formas menos frecuentes con recaídas o recurrencias. También debe considerarse la posibilidad de que un primer evento desmielinizante, indistinguible de una EMDA, constituya el debut de una esclerosis múltiple, como ocurrió en un caso de esta serie.

Acute disseminated encephalomyelitis (ADEM) is an acute inflammatory disease of the central nervous system, immune mediated, which is more common in childhood. Its diagnosis is based on clinical confirmation of acute demyelinating process through the MRI and the exclusion of other diagnoses according to the criteria proposed in the consensus of experts of the International Pediatric MS Study Group. The evolution of knowledge and new diagnostic criteria justify a revision of the item. We performed a retrospective descriptive study based on review of medical records of a consecutive series of 12 patients under age 15 admitted to the Pediatric Hospital Pereira Rossell Hospital Center with diagnosis of ADEM from 1 January 2000 to December 31, 2007 and its subsequent monitoring at the Chair of Pediatric Neurology, Faculty of Medicine and the Pediatric Neurology Service of the hospital. While presented with severe acute encephalopathy, the outcome was good in most patients, although two patients had motor sequelae and one secondary epilepsy and cognitive impairment. In 11 patients the disease had a monophasic course. The importance of clinical and imaging follow-up is that there are ways less frequent relapse or recurrence. It should also be considered a first demyelinating event, indistinguishable from ADEM, constitutes the debut of multiple sclerosis, as in a case of this series.

Acute disseminated encephalomyelitis presenting as acute psychotic disorder.

A 5 year old boy and a 10 year old girl presented with acute onset of psychotic disorder, which occurred one week after an upper respiratory infection. MRI images of brain were consistent with the diagnosis of acute disseminated encephalomyelitis (ADEM) in both cases. ADEM is one of the differential diagnoses to be considered when acute psychotic disorder occurs during childhood.