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.

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 ssociated with enteroviral infection

Acute disseminated encephalomyelitis [ADEM] is an acute demyelinating infection of the CNS that is characterized by multifocal white matter involvement often following an infection and vaccination. A 16-year-old child receiving risperidone for 7 years for the treatment of attention-deficit/hyperactivity disorder was hospitalized with complaints of double-vision during the past month and weakness on his right side, ataxia, and vomiting over the past 3 days. The child was diagnosed with ADEM after clinical, laboratory, and cranial MRI was conducted. Following an initial 3-day therapy with pulsed methylprednisolone, the child showed obvious clinical improvement. The treatment was continued with prednisolone and significant improvement was achieved. Enterovirus was detected in the results of the viral examination of the CSF. This child was found to be an interesting case having been diagnosed with ADEM associated with enteroviral infection, because of the rarity of few case reports in the literature

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.

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.