Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

Neuritis óptica pediátrica: un desafío diagnóstico / Pediatric optic neuritis: a diagnostic challenge Pediatric optic neuritis: a diagnostic challenge

Introducción: la neuritis óptica requiere un diagnóstico y manejo desafiante, se puede presentar de forma aislada o asociada con trastornos inflamatorios, siendo hasta 25% de la clínica de los síndromes desmielinizantes agudos pediátricos. La incidencia anual estimada de neuritis óptica pediátrica es 0.2 por 100.000 niños, con una preponderancia femenina y una edad media de presentación de 9 a 11 años. Presentación del caso: paciente de 12 años con cuadro agudo de diplopía, dolor ocular izquierdo sin antecedentes relevantes, en quien se encontró parálisis del VI par izquierdo y resonancia magnética cerebral (RMc) con realce en nervio óptico, iniciándose tratamiento con corticoterapia endovenosa, previo descarte de patologías infecciosas, con evolución satisfactoria. Conclusiones: en el espectro de la neuritis óptica es importante el conocimiento de sus diferentes etiologías, debido a que el tratamiento y pronóstico dependen de la causa.

Introduction: optic neuritis (ON) requires a challenging diagnosis and management. It can appear as an isolated condition or in association with inflammatory disorders, being 25% of the clinical manifestations of pediatric acute demyelinating syndromes. The estimated annual incidence of pediatric ON is of 0.2 per 100.000, with a female preponderance and a mean age at onset of 9 to 11 years. Case report: a 12-year-old patient presenting with acute diplopia and left ocular pain, referring no relevant past history. Left sixth nerve palsy and brain magnetic resonance imaging (MRI) showing optic nerve enhancement, were evidenced. Therapy with intravenous corticosteroids was started, once an infectious etiology had been ruled out, with satisfactory progression. Conclusions: in the optic neuritis spectrum disorders, knowledge regarding other potential etiologies is key to treatment and prognosis, which depend on the cause.

Perfil clínico y epidemiológico de los pacientes con diagnóstico de neuromielitis óptica / Clinical and epidemiological profile of patients with neuromyelitis optica diagnosis

INTRODUCCIÓN. El trastorno del espectro de neuromielitis óptica, enfermedad inflamatoria, desmielinizante, afecta al sistema nervioso central, frecuente en poblaciones no caucásicas como la ecuatoriana. El retraso en su diagnóstico y tratamiento provoca discapacidad que se puede prevenir. OBJETIVO. Determinar el perfil clínico y epidemiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 45 Historias Clínicas y una muestra de 41 de pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica atendidos en la Unidad de Neurología del Hospital de Especialida-des Carlos Andrade Marín, período enero 2005 a diciembre 2019. Se realizó análisis univarial. Se aplicó el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 25. RESULTADOS. El 76,0% (31; 41) fueron mujeres. Datos promedios: edad 48,9 años; diagnóstico definitivo demoró 4,12 años, desde el inicio de los síntomas; tiempo de diagnóstico fue 3,17 años; 3,7 brotes en total; el 87,8% (36; 41) con un fenotipo recurrente. La media de duración de la enfermedad fue de 6,8 años. En el 70,7% (29; 41), se identificaron anticuerpos anti-AQP4 en suero mediante inmunofluorescencia directa, el 51,2% requirieron para la marcha apoyo uni o bilateral. El 43,9% (18; 41) debutó con neuritis óptica; el 31,7% (13; 41) presentaron mielitis como primer síntoma y el 24,4% (10; 41) la combinación de neuritis óptica y mielitis fueron los síntomas iniciales. CONCLUSIÓN. Se determinó el perfil clínico y epi-demiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. Existió demora en el diagnóstico definitivo de los pacientes desde el inicio de los síntomas, lo que se tradujo en un aumento de la discapacidad.

INTRODUCTION. Neuromyelitis optica spectrum disorder, an inflammatory, demyelinating disease, affects the central nervous system, common in non-Caucasian popu-lations such as Ecuadorians. The delay in its diagnosis and treatment causes disabi-lity that can be prevented. OBJECTIVE. To determine the clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 45 Medical Records and a sample of 41 patients with a diagnosis of neuromyelitis optica spectrum disor-der seen at the Neurology Unit of the Carlos Andrade Marín Specialties Hospital, period from January 2005 to December 2019. Univariate analysis was performed. The statistical program International Business Machines Statistical Package for the Social Sciences, version 25 was used. RESULTS. 76,0% (31; 41) were women. Average data: age 48,9 years; definitive diagnosis took 4,12 years from the onset of symptoms; time to diagnosis was 3,17 years; 3,7 outbreaks in total; 87,8% (36; 41) with a recurrent phenotype. The average disease duration was 6,8 years. In 70,7% (29; 41), anti-AQP4 antibodies were identified in serum by direct immunofluorescence, 51,2% required uni- or bilateral su-pport for walking. Optic neuritis started in 43,9% (18; 41); 31,7% (13; 41) had myelitis as the first symptom and 24,4% (10; 41) the combination of optic neuritis and myelitis were the initial symptoms. CONCLUSION. The clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder was determined. There was delay in the conclusive diagnosis of patients from the beginning of symptoms, which resulted in increased disability.

Epidemiological Study of Patients with Spinal Cord Injury Treated in POLEM, a Specialized Clinic in São Paulo State ­ Brazil

Objective To analyze the epidemiological profile of patients with spinal cord injury treated at POLEM ­ Associação de Apoio às Pessoas comLesão Medular (Association for Supporting People with Spinal Cord Injury). Method The population studied comprised 113 patients with spinal cord injury, of traumatic or nontraumatic etiology, and the data obtained were compared with those of other institutions. Results Of the 113 patients, 70.8% were male and 29.2% female. Traumatic lesions were responsible for 54% of the patients, and nontraumatic for 46%. Of the patients with traumatic injury, 90.2% were male, the main cause being traffic accidents. In nontraumatic lesions, women were the most affected, 51.9%; and dysraphism and myelitis were the main causes (31% and 21%, respectively). Conclusion The results showed an important incidence of spinal cord injury due to trauma,mainly affecting young individuals of productive age and low educational level, representing high economic and social costs. The data found in the present study are similar to those of other studies performed in our country.

Sarcoidose como diagnóstico diferencial de mieloma múltiplo / Sarcoidosis as a differential diagnosis of multiple myeloma

A sarcoidose caracteriza-se como doença granulomatosa que acomete diferentes órgãos humanos, especialmente os pulmões, sendo sua patogênese pouco conhecida. No caso em questão, a paciente iniciou com sintomas inespecíficos, como fraqueza, perda ponderal e tosse seca esporádica, sendo internada para extensão da propedêutica. Sugeriu-se como hipótese diagnóstica inicial possível quadro de mieloma múltiplo, tendo em vista a anemia, a disfunção renal, a hipercalcemia e, sobretudo, as lesões osteolíticas apresentadas pela paciente. Todavia, o diagnóstico de sarcoidose foi selado a partir das biópsias de medula óssea e de linfonodo inguinal, que evidenciaram mielite e linfadenite granulomatosas, respectivamente. A terapêutica instituída baseou-se na administração de corticosteroides e em medidas de redução da calcemia. A paciente recebeu alta, com melhora do quadro clínico, para acompanhamento ambulatorial da doença. Conclui-se que a sarcoidose não possui tratamento curativo, mas a terapêutica imunossupressora é eficaz no controle da progressão da enfermidade, fazendo com que o paciente tenha um prognóstico favorável.

Sarcoidosis is characterized as a granulomatous disease that affects different human organs, especially the lungs, and its pathogenesis is little known. In this case, the patient started with nonspecific symptoms, such as weakness, weight loss, and sporadic dry cough, being hospitalized for extension of the propaedeutics. The initial diagnostic hypothesis suggested was a possible case of multiple myeloma, based on the anemia, renal dysfunction, hypercalcemia and, above all, the osteolytic lesions presented by the patient. However, the diagnosis of sarcoidosis was made after bone marrow and inguinal lymph node biopsies that showed granulomatous myelitis and lymphadenitis, respectively. The therapy instituted was based on the administration of corticosteroids and on measures to reduce the level of calcium. The patient was discharged, with clinical improvement, for outpatient follow-up of the disease. It is concluded that sarcoidosis has no curative treatment, but immunosuppressive therapy is effective in controlling the progression of the disease, giving the patient a favorable prognosis.

Parálisis fláccida aguda por enterovirus con resolución completa del cuadro neurológico. A propósito de un caso pediátrico / Acute flaccid paralysis related to enterovirus with complete resolution of the neurological symptoms. A pediatric case report

Desde la eliminación de la circulación del virus polio salvaje, disminuyeron los casos de parálisis fláccida aguda. Sin embargo, continúan ocurriendo casos asociados a otros enterovirus no polio y virus neurotropos. Se presenta el caso de una paciente de 9 años con diagnóstico de meningitis y mielitis con compromiso motor en los miembros inferiores y vejiga neurogénica asociado a enterovirus, con resolución completa del cuadro neurológico posterior a la administración de gammaglobulina hiperinmune.

Since the wild poliovirus no longer circulates, the number of cases of acute flaccid paralysis decreased. However, cases related to non-polio enteroviruses and neurotrope viruses continue to occur. We present a nine-year-old patient with meningitis and myelitis with motor involvement in the lower limbs and neurogenic bladder associated with enterovirus, with complete resolution of the neurological symptoms following the administration of hyperimmune gammaglobulin.

Mielitis isquémica como presentación de aorta shaggy en paciente con síndrome aórtico agudo concomitante / Ischaemic myelitis as a presentation of a shaggy aorta in a patient with concomitant acute aortic syndrome

Resumen La aorta shaggy se define como una degeneración ateromatosa agresiva y extensa de la aorta, cuya friabilidad predispone a ulceración y complicaciones embólicas, cursa con alto riesgo de embolia sistémica y no se conocen prevalencia ni incidencia en poblaciones de riesgo. La mayoría de casos publicados hacen referencia a complicaciones en procedimientos quirúrgicos. En la literatura no se hallaron reportes que asocien ateroembolia aórtica con obstrucción de la arteria de Adamkiewicz, cuyo diagnóstico no siempre es posible visualizando su oclusión por angiotomografía o por angiorresonancia, pues el defecto puede ser evanescente o puede existir compromiso distal con obstrucción microvascular, difícilmente aparente con arteriografía selectiva. Se presenta un caso de mielopatía isquémica embólica asociada a aorta shaggy con probable oclusión de la arteria de Adamkiewicz como responsable del deterioro neurológico agudo de la paciente, confirmado por resonancia magnética nuclear. Para caracterizar mejor esta enfermedad y para tener las estrategias diagnósticas y terapéuticas apropiadas en su abordaje oportuno, consideramos importante el reporte de casos similares aumentando así su sospecha diagnóstica.

Abstract A shaggy aorta is defined as an aggressive and extensive atheromatous degeneration of the aorta. Its friability predisposes to ulceration and embolic complications. It carries a high risk of systemic embolisms, and its prevalence and incidence in risk populations is unknown. The majority of published cases mention complications in surgical procedures. No reports have been found in the literature that associate aortic atheroembolism with obstruction of the artery of Adamkiewicz. Its diagnosis is not always possible by visualising its occlusion by computed tomography angiography or by magnetic resonance angiography, since the defects may be evanescent, or there may be a distal compromise with a microvascular obstruction, hardly apparent with selected angiography. A case is presented of embolic ischaemic myelitis associated with a shaggy aorta, with probable occlusion of the artery of Adamkiewicz being responsible form the acute neurological deterioration of the patient, confirmed by a nuclear magnetic resonance scan. In order to better describe the features of this disease and to have the appropriate diagnostic and therapeutic strategies for its timely approach, it is considered important to report all similar cases, thus increasing its diagnostic suspicion.

Mielitis parcial excéntrica como primera manifestación de trastornos del espectro de neuromyelitis óptica: presentación de casos y revisión de la literatura / Eccentric partial myelitis as the first manifestation of optic neuromyelitis spectrum disorders

RESUMEN El entendimiento de las caracteristicas clínicas del espectro de trastornos de Neuromielitis óptica (NMOSD) con mielitis parcial y neuritis óptica típica ha ampliado el diagnóstico en casos atípicos. Presentamos el caso de una mujer de 47 años que debuta con neuritis óptica atípica y mielitis parcial. Resonancia magnética cerebral y órbitas con realce de nervio óptico, quiasma óptico y tracto óptico derecho, de columna cervical y torácica contrastada con mielitis parcial a nivel C4 y T2. Fue tratada con bolos de metilprednisolona y plasmaferesis, con buena respuesta clínica. Se realizó anticuerpos aquaporina 4 sérico positivos.

SUMMARY The understanding of the clinical characteristics of the spectrum of optic neuromyelitis disorders (NMOSD) with partial myelitis and typical optic neuritis has extended the diagnosis in atypical cases. We present the case of a 47-year-old woman who debuts with atypical optic neuritis and partial myelitis. Magnetic resonance imaging and orbits with optic nerve enhancement, optical chiasm and right optic tract, cervical and thoracic spine contrasted with partial myelitis at level C4 and T2. I t was treated with boluses of Methylprednisolone and plasmapheresis, with good clinical response. Aquaporina 4 Serum positive antibodies were performed.

Degeneración combinada subaguda en un paciente sin anemia y con hipertensión severa / Subacute combined degeneration in a patient without anemia but with severe hypertension

RESUMEN La degeneración combinada subaguda, es una patología de tipo neuropsiquiatrica asociada al déficit de vitamina B12. Se manifiesta clínicamente por ataxia sensorial, neuropatía periférica, disfunción cognitiva y neuropatía óptica; estas manifestaciones generalmente son atribuidas a la síntesis anormal de mielina. Puede tener una presentación clínica inespecífica, pero la medición de los niveles de vitamina B12, algunos metabolitos séricos, y el uso de métodos de neuroimagen, ayudan a confirmar el diagnóstico ante su sospecha. A continuación se describe el caso de un paciente con un cuadro de degeneración combinada subaguda, quien consultó por síntomas neurológicos e hipertensión severa, quien luego del tratamiento presentó mejoría de su sintomatologia neurológica y vascular.

SUMMARY Sub-acute combined degeneration is a neuropsychiatrical pathology associated with vitamin B12 deficiency It is clinically manifested through sensorial ataxia, peripheral neuropathy, cognitive dysfunction and optical neuropathy; these manifestations are generally attributed to the abnormal synthesis of myelin. It can have a nonspecific clinical presentation but the measurement of the levels of vitamin B12, some serum metabolites, and the use of neuroimaging methods help to confirm the diagnose when suspected. The case of a patient with sub-acute combined degeneration is described below. The patient was attended because of neurological symptoms and severe hypertension, and after the treatment, the patient's neurological and vascular symptomatology improved.

Aumento inusual de casos de mielitis en un hospital pediátrico en Argentina / Unusual increase of cases of myelitis in a pediatric hospital in Argentina

Introducción: La asociación entre enterovirus D68 y cuadros de mielitis aguda fláccida ha sido descrita en Estados Unidos, en 2014. Desde ese año, se han reportado casos esporádicamente en Canadá y Europa. Se describe, en este estudio, una serie de casos con mielitis aguda fláccida en el Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en Buenos Aires, Argentina, en 2016. Métodos: Estudio descriptivo, retrospectivo. Se incluyeron todos los pacientes internados desde el 1/04/2016 al 1/07/2016 con mielitis fláccida aguda con lesiones en la médula espinal que comprometieran la sustancia gris en la resonancia magnética nuclear. Se procesaron, para la búsqueda etiológica, muestras de secreciones nasofaríngeas, hisopados de materia fecal y líquido cefalorraquídeo. Resultados: Se incluyeron 10 pacientes. La mediana de edad fue 4 años (rango de 3 meses a 5 años). Ocho pacientes tuvieron una enfermedad febril autolimitada antes del inicio de los síntomas neurológicos. Los hallazgos neurológicos fueron debilidad fláccida de, al menos, un miembro, cervicoplejia (n= 2) y parálisis facial (n= 2). Todos los pacientes presentaron lesiones longitudinales en la médula espinal, con compromiso de sustancia gris, predominantemente, en el asta anterior. En todos los casos, se realizó una punción lumbar. En 7 pacientes, se observó pleocitosis. En cuatro niños, se identificó enterovirus D68 en secreciones nasofaríngeas y, en uno, se identificó el enterovirusD68 en el líquido cefalorraquídeo. Todos los pacientes persistieron con déficits neurológicos al momento del alta. Conclusiones: Se reporta el primer brote de mielitis aguda fláccida asociada a enterovirusD68 en Argentina. La vigilancia epidemiológica activa permitirá conocer la verdadera incidencia, epidemiología y etiología de esta enfermedad.

Introduction: The association between enterovirus D68 and acute flaccid myelitis was first described in the United States in 2014. Since then, sporadic cases have been reported in Canada and Europe. This study describes a series of cases of acute flaccid myelitis at Hospital de Pediatría "Prof. Dr. Juan P. Garrahan," in Buenos Aires, Argentina, during 2016. Methods: Descriptive, retrospective study. All patients with acute flaccid myelitis and lesions in the spinal cord involving the gray matter, as observed in the magnetic resonance imaging (MRI) scan, hospitalized from 04/01/2016 to 07/01/2016, were included in the study. Samples of nasopharyngeal secretions, fecal swabs and cerebrospinal fluid were collected and processed to look for the causative agent. Results: Ten patients were included. The median age was 4 years old (range from 3 months to 5 years old). Eight patients had a self-limiting febrile condition before the onset of neurological symptoms. Neurological findings were flaccid weakness in, at least, one limb, cervical paralysis (n= 2) and facial paralysis (n= 2). All patients had longitudinal lesions in the spinal cord, with gray matter involvement, mainly in the anterior horn. In all cases, a lumbar puncture (spinal tap) was performed. Pleocytosis was observed in 7 patients. In four children, enterovirus D68 was identified in nasopharyngeal secretions, and in one, it was detected in the cerebrospinal fluid. Neurological deficit persisted in all patients at the time of discharge. Conclusions: The first outbreak of acute flaccid myelitis associated to enterovirus D68 is reported in Argentina. Active epidemiological surveillance will help to determine the true incidence, epidemiology and etiology of this disease.

Infecciones por el virus EV-D68 en niños hospitalizados en un hospital de alta complejidad / EV-D68 virus infections in children admitted to a tertiary-care hospital

Introducción: Los reportes de infecciones por enterovirus D68 (EV-D68) han aumentado en los últimos años. Material y métodos: Cohorte prospectiva. Se realizó la búsqueda de EV-D68 en niños internados en el Hospital de Pediatría Juan P. Garrahan entre 1-5-2016 y 30-9-2016 con: infección respiratoria aguda baja (IRAB) que requirieran cuidados intensivos, parálisis aguda fláccida (PAF) asimétrica con compromiso de sustancia gris en resonancia magnética nuclear (RMN) o identificación de cualquier enterovirus con cuadro clínico compatible. La identificación de EV-D68 se realizó en el Servicio de Neurovirus, Instituto Nacional de Enfermedades Infecciosas INEI-ANLIS "Dr. CG. Malbrán". Resultados: n: 6. PAF: cuatro niños presentaron PAF asimétrica, con arreflexia y RMN compatible con mielitis. Requirieron ventilación mecánica en unidades de cuidados intensivos (UCI) dos de los 4 niños. Todos presentaron parálisis residual. Se identificó EV-D68 en secreciones nasofaríngeas (SNF) de todos ellos. En líquido cefalorraquídeo sólo en uno. Miocarditis: Una niña sana de 5 años se internó en UCI por disfunción miocárdica y fiebre. Presentaba además derrame pericárdico moderado. Recibió gamaglobulina e.v. con buena evolución. En SNF se identificaron virus sincicial respiratorio (VSR) y EV-D68. IRAB grave: se identificó EV-D68 en un paciente de 14 meses que permaneció en UCI por IRAB grave con requerimientos de ventilación no invasiva por 72 hs, con buena evolución posterior. Se constató coinfección VSR y EV-D68 en SNF. Conclusiones: Se reportan 6 pacientes internados con infección por EV-D68. La vigilancia epidemiológica activa es esencial para identificar la circulación, las características clínicas y el pronostico de las infecciones por virus emergentes (AU)

Introduction: Reports on enterovirus D68 (EV-D68) infections have increased over the past years. Material and methods: A prospective cohort study. A search for EV-D68 infection was conducted in children hospitalized at Hospital de Pediatría Juan P. Garrahan between 1-5-2016 and 30-9-2016 with: acute lower respiratory infection (ALRI) requiring intensive care unit (UCI) admission, acute flaccid paralysis (AFP), asymmetry with grey matter involvement on magnetic resonance imaging (MRI), or identification of any enterovirus associated with compatible features. The identification of EV-D68 was performed at the Department of Neuroviruses of the InstitutoNacional de EnfermedadesInfecciosas INEI-ANLIS "Dr. CG. Malbrán". Results: n: 6. AFP: four children had asymmetric AFP with areflexia and MRI compatible with myelitis. Two of four required mechanical ventilation in the ICU. All of them presented with residual paralysis. EV-D68 was identified in the nasopharyngeal swab (NPS) in all of them and in the cerebrospinal fluid in only one. Myocarditis: A 5-year-old healthy girl was admitted to the ICU because of myocardial dysfunction and fever associated with moderate pericardial effusion. She was put on IV gamma globulin with a good response. In the NPS respiratory syncytial virus (RSV) and EV-D68 were identified. Severe ALRI: EV-D68 was identified in a 14-month-old patient who was admitted to the UCU because of severe ALRI requiring non-invasive ventilation for 72 hours with a good outcome. A RSV and EV-D68 coinfection was found in the NPS. Conclusions: We report six inpatients with a EV-D68 infection. Active epidemiological surveillance is crucial to identify circulation of the virus, clinical features, and prognosis of emerging viruses (AU)

Neuropsychiatric Lupus in clinical practice / Lúpus neuropsiquiátrico na prática clínica

ABSTRACT Systemic lupus erythematosus (SLE) is a chronic autoimmune disease involving multiple organs, characterized by the production of autoantibodies and the development of tissue injury. The etiology of SLE is partially known, involving multiple genetic and environmental factors. As many as 50% of patients with SLE have neurological involvement during the course of their disease. Neurological manifestations are associated with impaired quality of life, and high morbidity and mortality rates. Nineteen neuropsychiatric syndromes have been identified associated with SLE, and can be divided into central and peripheral manifestations. This article reviews major neuropsychiatric manifestations in patients with SLE and discusses their clinical features, radiological findings and treatment options.

RESUMO Lúpus eritematoso sistêmico (LES) é uma doença autoimune crônica que envolve múltiplos órgãos e sistemas, caracterizada pela produção de auto anticorpos e lesão tecidual. A etiologia do LES é parcialmente conhecida e envolve interação entre fatores genéticos e ambientais. Até 50% dos pacientes com LES apresentam envolvimento neurológico no decorrer da doença. Manifestações neurológicas estão associadas a prejuízo na qualidade de vida e altas taxas de mortalidade e morbidade. Foram identificadas 19 síndromes neuropsiquiátricas em pacientes com LES, divididas entre manifestações do sistema nervoso central e periférico. O objetivo deste artigo é revisar as manifestações neuropsiquiátricas mais importantes. Serão abordadas as características clínicas, os aspectos radiológicos e opções de tratamento dos eventos neuropsiquiátricos.

A case of tumor-like inflammatory demyelinating disease with progressive brain and spinal cord involvement / Um caso de doença desmielinizante inflamatória semelhante a tumor com envolvimento progressivo de cérebro e medula espinhal

CONTEXT: Tumor-like inflammatory demyelinating disease (TIDD) usually occurs in the brain and rarely occurs in the spinal cord. TIDD appears to be very similar to tumors such as gliomas on imaging, which may lead to incorrect or delayed diagnosis and treatment. CASE REPORT: Because of headache and incoherent speech, a 24-year-old Chinese male presented to our hospital with a two-week history of respiratory infections. After dexamethasone treatment, his symptoms still got worse and surgery was performed for diagnostic purposes. Histological examination revealed that the lesion was inflammatory. Further lesions appeared in the spine (T3 and T4 levels) after two months and in the right occipital lobe after three months. After intravenous immunoglobulin (IVIG) and methylprednisolone treatment, his symptoms improved. CONCLUSION: Progressive lesions may damage the brain and spinal cord, and long-term prednisolone and IVIG therapy are beneficial in TIDD patients.

CONTEXTO: A doença desmielinizante inflamatória tumoral (DDIT) geralmente ocorre no cérebro e raramente na medula espinhal. A DDIT é muito semelhante a tumores tais como gliomas em exames de imagem, o que pode conduzir a diagnóstico e tratamento tardios e incorretos. RELATO DO CASO: Por causa de dor de cabeça e discurso incoerente, um homem chinês de 24 anos de idade foi ao hospital com história de duas semanas de infecções respiratórias. Após o tratamento com dexametasona, seus sintomas ficaram ainda piores e a cirurgia foi realizada para fins de diagnóstico. O exame histológico revelou que a lesão era inflamatória. Mais lesões apareceram na coluna vertebral (níveis T3 e T4) após dois meses, e no lobo occipital direito depois de três meses. Depois de tratamento com imunoglobulina intravenosa (IGIV) e metilprednisolona, seus sintomas melhoraram. CONCLUSÃO: Lesões progressivas podem danificar o cérebro e a medula espinhal, e prednisolona a longo prazo e terapia de IGIV são benéficas em pacientes DDIT.

Síndrome del "hombre dentro de un barril". Reporte de un caso / Man in the barrel syndrome. Case report

Introducción: La diplejía braquial bilateral a predominio proximal conocida como síndrome del "hombre dentro de un barril" tiene múltiples causas, entre ellas las post infecciosas, vasculares y neurodegenerativas. Caso clínico: Varón de 75 años que dos semanas antes de su ingreso al servicio presento en forma brusca sensación de "electricidad" y severa dificultad para movilizar miembros superiores. Examen físico: Fuerza muscular bíceps derecho 1 +/5, izquierdo 1-/5, tríceps derecho 2-/5, izquierdo 2+/5, arreflexia bicipital y tricipital, e hipotrofia supra e infra escapular bilateral. Exámenes auxiliares: anticuerpos Ig G anti-CMV en suero: reactivo > 500UI/ml, anticuerpos Ig G anti-Herpes 1 en suero: reactivo I 53U/ml, electromiografía evidencio signos de compromiso radicular crónico C5 a C8 bilateral, resonancia magnética de columna cervical mostro lesiones desmielinizantes que comprometían principalmente los cordones anteriores desde C3 a C6 con realce posterior a la administración del contraste. Conclusión: Se presenta un caso poco frecuente de diplejía braquial por mielitis post herpética.

Introduction: Bilateral brachial diplegia with proximal predominance known as "man in a barrel" syndrome has multiple causes, including post infectious, vascular and neurodegenerative. Clinical case: A 75- year-old-patient who 2 weeks before its entry into the service presented suddenly like-electricity feelings and severe difficulty upper limbs movements. Physical exam: Muscle strength: Right biceps 1 +/5, left 1-/5, right triceps 2-/5, left 2+/5, bilateral biceps and triceps areflexia and scapula muscles hypotrophy. Ancillary tests: Ig G serum anti-CMV: reactive> 500UIIml, Ig G serum anti-Herpes 1: reactive 153U/ml, electromyography showed bilateral chronic involvement from C5 to C8 roots, MRl cervical spinal cord showed demyelinating lesions in anterior spinal cord column from C3 to C6 with enhancement after contrast administration. Conclusion: We present a rare of post herpetic myelitis brachial diplegia case.