Enfermedades desmielinizantes en niños: Encefalomielitis diseminada aguda y esclerosis múltiple / Demyelinizing diseases in children. Acute disseminated encephalomyelitis and multiple sclerosis

Se revisan dos de las principales enfermedades desmielinizantes en niños, la encefalomielitis aguda diseminada (EAD) y la esclerosis múltiple (EM). Por sus características fisiopatológicas, etiologías probables, manifestaciones clínicas, diagnóstico, tratamiento, pronóstico, evolución, así como alteraciones atípicas que complican su diagnóstico; cuanto más pequeño es el paciente se necesita estudiar más, antes de llegar al diagnóstico. El Grupo Internacional de Estudio de Esclerosis Múltiple Pediátrica publicó las definiciones operativas para enfermedades desmielinizantes adquiridas del sistema nervioso central: la EAD es monofásica, polisintomática y con encefalopatía. Su duración es de hasta 3 meses, con síntomas fluctuantes y hallazgos en resonancia magnética. La EM se define como síndrome aislado monofocal o polifocal, sin encefalopatía. Actualmente se consideran dos enfermedades diferentes y distinguibles desde el inicio de los síntomas.

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.

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.

Fulminant acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis, or post infectious encephalomyelitis is an immunologically mediated demyelinating disorder affecting the central nervous system after infection or vaccination. Young and adolescents are mostly affected with characteristic diffuse neurological signs. Prognosis is generally favorable, however, fulminant acute disseminated encephalomyelitis can be fatal if not diagnosed and treated early. With an increase in intracranial pressure, the treatment of choice is methylprednisolone, followed by immunoglobulin, plasmapheresis or cytotoxic drugs. We present a case of acute disseminated encephalomyelitis in a 17 year male who presented with a sudden onset recurrent generalized tonic-clonic seizures and rapidly become comatose. Magnetic resonance imaging along with other investigations helped in establishing the diagnosis. Treatment resulted in full recovery with uneventful follow up of four years

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.

Cyclosporine in the treatment of a case of fulminant and refractory acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis [ADEM] is a rare, monophasic, demyelinating disease of the CNS which sometimes could be refractory to traditional treatment. We present a case of fulminant ADEM which is treated with combination of corticosteroid, intravenous immunoglobulin and cyclosporine. Immunosuppressive agents such as cyclosporine may be effective especially in fulminant form of the disease

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.

Pediatric optic neuritis.

OBJECTIVE: Describe the clinical characteristics of pediatric optic neuritis. MATERIAL AND METHOD: Retrospective observational case series was performed on patients < or = 12 years of age with optic neuritis at Childrens Hospital Los Angeles. RESULTS: Thirty-one patients (48 eyes) were identified. Mean follow-up was 2.7 years. There were 17 preadolescents (< 10-years-old) in group I, and 14 adolescents (10--12-years-old) in group II. Females comprised 59% of group I, and 71% of group II. Bilateral cases comprised 65% from group I, and 43% from group II. Five patients from group I had acute disseminated encephalomyelitis (ADEM). Two patients from group II had multiple sclerosis (MS). No other patients developed MS. There was no difference in initial or final vision for the eyes with or without steroid treatment. CONCLUSION: Pediatric optic neuritis has no gender or racial predilection, is usually bilateral, and is associated with ADEM rather than MS.

potential diagnostic value of diffusion-weighted imaging in acute disseminating encephalomyelitis

Acute disseminating encephalomyelitis [ADEM] is a treatable inflammatory, demyelinating disorder of the central nervous system. Early application of steroids has been shown to reduce morbidity, therefore, early diagnosis is highly desirable. Magnetic resonance imaging [MRI] is the imaging modality of choice for establishing the diagnosis along with the clinical presentation. The aim of this report is to show the potential diagnostic value of advanced MR techniques such as diffusion-weighted imaging [DWI], which has been found to add to the diagnostic power of MRI in the setting of demyelinating disorder, even in the absence of contrast enhancement. We report a case of ADEM in an 11-year-old boy revealing high signal intensity changes on DWI indicating elevated diffusion

Acute disseminated encephalomyelitis in classic dengue

Neurological manifestation is uncommon in dengue infection. The pathogenesis of central nervous system involvement is controversial. We report a rare case of acute disseminated encephalomyelitis in classic dengue, with isolation of serotype 3 in liquor. This condition was associated with significant structural damage detected by magnetic resonance.

Manifestação neurológica é incomum em dengue. A patogênese do acometimento do sistema nervosa central é controversa. Nós relatamos um raro caso de encefalomielite disseminada aguda em dengue clássica, com isolamento do sorotipo 3 em líquor. O quadro estava associado a significativo dano estrutural detectado pela ressonância magnética.

Encefalomielitis aguda diseminada de presentación atípica: utilidad de la biopsia estereotáctica para su diagnóstico. Caso clínico / Diagnosis of acute disseminated encephalomyelitis using a stereotaxic biopsy: Report of one case

We report a 15 year-old female presenting with behavioral disturbances, headache, left hemiparesis and paresis of the vertical gaze. CAT scan and magnetic resonance showed an involvement of right thalamus, third ventricle and medial temporal lobe suggesting an encephalitis or lymphoma. 201Thalium SPECT suggested a lymphoma. A stereotaxic biopsy showed a subacute demyelinizing lesion, compatible with an acute disseminated encephalomyelitis. The patient was treated with Methylprednisolone with resolution of symptoms. She remains in good condition after one year of follow-up.

Encefalomielitis aguda diseminada: reporte de un caso y revisión de la literatura / Acute disseminated encephalomyelitis: a case report and review of the literature

Introducción: la encefalomielitis aguda diseminada (EAD) es un trastorno neurológico caracterizado por inflamación del cerebro y médula espinal causado por un daño a la mielina, afectando al sistema nervioso central de manera difusa. Esta afección puede manifestarse de manera espontánea o secundaria a infecciones o a vacunación. La mayoría de las veces evoluciona de manera monofásica con manifestaciones clínicas inespecíficas, por lo que la sospecha diagnóstica es fundamental. Objetivo: Presentar un caso clínico de una patología infrecuente, con una revisión actualizada del tema. Caso clínico: Presentamos el caso de una paciente de 7 años de edad, de sexo femenino, portadora de EAD y se exponen la forma clínica de presentación, los hallazgos neurorradiológicos y el tratamiento realizado. Conclusión: La EAD es una patología poco frecuente, que puede tener un curso mortal. El diagnóstico definitivo se realiza a través de RM y el tratamiento más efectivo pareciera ser los corticoides, aunque un porcentaje de pacientes evoluciona espontáneamente a la mejoría.

Imaging features in acute disseminated encephalomyelitis

The authors report 3 children with the diagnosis of acute disseminated encephalomyelitis, [ADEM] Diagnosis was based on clinical and radiological findings. The most common presenting symptoms were fever and disturbed consciousness, followed by cranial nerve abnormalities and puramidal sings. Brain MRI showed hyperintense signals on T2-weighted images, most commonly in the subcortical and periventricular white matter, brainstem, basal ganglia and thalamus. The lesions were bilateral, asymmetrical and highly variable in size and number. A preceding infection was present in 2 of 3 children. Early high-dose corticosteroids were given to all the patients. One child succumbed to the disease. Two children improved on steroid and immunoglobulin therapy but had residual neurological deficits. Early high-dose steroid therapy is an effective treatment in acute disseminated encephalomyelitis

Biphasic demyelination of the nervous system following anti-rabies vaccination.

Acute disseminated encephalomyelitis (ADEM) is an acute or subacute condition characterized by widespread multifocal neurological deficits of variable severity. We report a case of a 45-year-old man with ADEM following anti-rabies vaccine (ARV).

Ressonância magnética e características clínicas em adultos com doenças desmielizantes monofásicas: encefalomielite aguda disseminada ou uma variante da esclerose múltipla? / Magnetic resonance imaging and clinical features in adults with monophasic demyelinating disease: acute disseminated encephalomyelitis or a variant of multiple sclerosis?

A encefalomielite aguda disseminada (ADEM) é doença monofásica inflamatória difusa do sistema nervoso central, que geralmente ocorre após infecção ou vacinação. Neste estudo, apresentamos a análise da ressonância magnética (RM), líquor e aspectos clínicos de quatro pacientes com diagnóstico presuntivo inicial de ADEM. O achado de lesões desmielinizantes na RM foi importante, mas não por si só suficiente para o diagnóstico definitivo. O seguimento clínico e realização de RM de controle, para excluir o aparecimento de novas lesões e reavaliar as anteriores, assim como análises liquóricas, foram impotantes para o diagnóstico diferencial com outras doenças desmielinizantes, particularmente a esclerose múltipla. Além disso, mostramos que a introdução precoce de metilprednisolona foi eficaz tanto para a melhora do quadro clínico quanto para redução ou desaparecimento das lesões na RM.

Encefalomielite disseminada aguda e vacinaçäo antimeningocócica A e C / Acute disseminated encephalomyelitis: association with meningococcal A and C vaccine: case report

Os autores descrevem o caso clínico de paciente do sexo feminino, de 25 anos, que desenvolveu encefalomielite aguda disseminada (EDA) iniciando-se cinco dias após vacinaçäo para meningococcus A e C (Pasteur-Merieux) na campanha de vacinaçäo realizada em dezembro de 1995 na cidade do Rio de Janeiro. Houve excelente resposta clínica e neurorradiológica após tratamento com corticosteróides em altas doses (pulsoterapia). Näo foram encontrados relatos sobre a associaçäo entre a vacina antimeningocócica e a EDA. A associaçäo entre EDA e leptospirose ou infecçöes por Mycoplasma sugerem porém que a síndrome pode ser precipitada näo só por viroses ou vacinaçäo antiviral como também pela exposiçäo do organismo a proteínas e polissacarídeos de bactérias.