Diagnóstico de SCIWORA asociado a síndrome de Brown Séquard en un adolescente / Diagnosis of SCIWORA associated with Brown Séquard syndrome in an adolescent

Resumen: El término SCIWORA (Spinal Cord Injury Without Radiologic Abnormality) describe una patología poco frecuente que afecta principalmente a pacientes pediátricos. Presentamos el caso de un paciente adolescente diagnosticado con SCIWORA/síndrome de Brown Séquard, el cual presenta múltiples contusiones en cuello y hemicuerpo derecho, de manera inmediata refiere parestesias en las cuatro extremidades y hemiparesia espástica con predominio derecho, incapacidad a la bipedestación y continencia urinaria. Se inicia tratamiento basado en el estudio NASCIS III y posterior a la pruebas de imagen se realizó diagnóstico ya comentado. Exponemos las definiciones más actuales de SCIWORA y las recomendaciones para realizar su diagnóstico así como su relación y coexistencia con síndromes medulares.

Abstract: SCIWORA (Spinal Cord Injury without Radiologic Abnormality) is a rare condition that mainly affects pediatric patients; We present the case of a teenage male patient diagnosed with SCIWORA/Brown Séquard Syndrome. In admission, he has multiple bruises on his neck and right hemibody and immediately he refers paraesthesia in all four limbs and spastic hemiparesis with right predominance, as well as inability to stand up and urinary continence. Treatment based on NASCIS III study is initiated and after the imaging tests, a diagnosis has already been made. We present the most current definitions of SCIWORA and the recommendations to make its diagnosis, as well as its relationship and coexistence with spinal syndromes.

Manejo quirúrgico de ependimomas en médula cervical: A propósito de un caso clínico / Surgical management of ependymomas in the cervical spine: Apropos of a clinical case

Introducción: los ependimomas son tumores cerebrales que surgen de células ependimarias, células de soporte en el cerebro y la médula espinal. Representan entre el 2 y el 3% de todos los tumores cerebrales primarios. Son el cuarto tumor cerebral más común en los niños, donde el 90% de los mismos se localizan en la fosa posterior. En adultos, el 60% de estos tumores se encuentran en la médula espinal pudiendo presentarse a cualquier nivel de ésta; el caso a continuación reporta un ependimoma cervical. Objetivo: reportar un caso de ependimoma medular de ubicación poco frecuente, con resección total, sin recidiva porterior a 2 años de seguimiento.Descripción del caso: se reporta el caso de un paciente adulto, 44 años, masculino, con un síndrome medular cervical completo, progresivo, provocado por un ependimoma cervical. Intervención: se realizó laminectomía de dos espacios [C7 a T1], apertura dural, mielotomía posterior logrando exéresis total de la lesión con durorrafia a sello de agua y posterior cierre por planos sin complicaciones, con seguimiento de 2 años de sobrevida. Conclusiones: la resección total macroscópica de este tipo de tumores es muy importante para poder evitar residiva. El ependimoma cervical puede recidivar, sobre todo cuando existen residuales de la lesión. El manejo oportuno depende de gran manera de que los pacientes acudan tempranamente a valoración especializada; la resección guiada con monitorización neurofisiológica transoperatoria provee mayor oportunidad a los pacientes a no presentar secuelas permanentes y permite asimismo, mejores resultados de la rehabilitación neurológica postoperatoria

Introduction: ependymomas are brain tumors that arise from ependymal cells, supporting cells in the brain and spinal cord. They represent between 2 and 3% of all primary brain tumors. They are the fourth most common brain tumor in children, where 90% of them are located in the posterior fossa. In adults, 60% of these tumors are found in the spinal cord and can occur at any level of the spinal cord; the case below reports a cervical ependymoma. Objective: to report a case of medullary ependymoma of rare location, with total resection, without recurrence after 2 years of follow-up. Case description: a 44-year-old male adult patient with a progressive, complete cervical spinal cord syndrome, caused by a cervical ependymoma, is reported.Intervention: two-space laminectomy [C7 to T1], dural opening and posterior myelotomy were performed, achieving total excision of the lesion with water-seal durorrhaphy and subsequent closure by planes without complications, with a 2-year survival follow-up. Conclusions: macroscopic total resection of this type of tumor is very important to avoid residual. Cervical ependymoma can recur, especially when there is residual lesion. Timely management depends to a great extent on the patients attending early for a specialized evaluation; guided resection with intraoperative neurophysiological monitoring provides a greater opportunity for patients to not present permanent sequelae and also allows better results of postoperative neurological rehabilitation.

Origin of the Posterior Inferior Cerebellar Artery over the C1 Posterior Arch / Origem da artéria cerebelar inferoposterior sobre o arco posterior de C1

Resumo The posterior inferior cerebellar artery usually arises from the intracranial segment (V4) of the vertebral artery. Despite its mean diameter of 2 mm, it usually irrigates important areas of the brain. When occluded, whether due to trauma or surgery, it may cause infarction in the brain stem and cerebellum. The present report describes a case of incidental finding of a posterior inferior cerebellar artery arising from the cervical segment (V3) of the vertebral artery, demonstrated by angiography. The findings were recorded and compared to those of earlier publications. Brief explanations regarding anatomy, vascular anomalies and embryology were provided. A literature review showed that anomalous branches of the cervical segment of the vertebral artery are infrequent andmust be known. A better understanding of anatomy and its variations enables an accurate topographic diagnosis, as well as the planning of the optimal surgical approach and therapy. Knowledge of this anatomical variation is essential because, if it is mistaken for a muscle branch and coagulated, this can cause ischemia and disabling sequelae.

Resumo A artéria cerebelar inferoposterior geralmente nasce do segmento intracraniano (V4) da artéria vertebral. Apesar de ter diâmetro médio de 2 mm, usualmente irriga áreas eloquentes do encéfalo. Quando ocluída, seja por trauma ou cirurgia, pode causar infarto no tronco encefálico e no cerebelo. Apresentamos um caso de artéria cerebelar inferoposterior com origem anômala no segmento cervical (V3) da artéria vertebral, demonstrado por angiografia. Os resultados foram registrados e comparados com os de publicações anteriores. Foram fornecidas breves explicações sobre a anatomia, anomalias vasculares e embriologia. A revisão da literatura mostrou que os ramos anômalos do segmento cervical da artéria vertebral são infrequentes e devem ser conhecidos. Uma melhor compreensão da anatomia e suas variações permite fazer um diagnóstico topográfico preciso, bem como planejar a abordagem e a terapia cirúrgicas ideais. O conhecimento dessa variação anatômica é essencial, pois, se confundida com um ramo muscular e coagulada, pode causar isquemia com sequelas incapacitantes.

An outbreak of compressive myelopathy due to pyogranulomatous reaction to the oily adjuvant of the foot-and-mouth disease vaccine - case report / Surto de mielopatia compressiva associada à reação piogranulomatosa ao adjuvante oleoso da vacina contra Febre Aftosa em Minas Gerais - relato de caso

Cases of compressive myelopathy syndrome associated with post vaccinal pyogranulomas were diagnosed post mortem in three cows from a farm in Minas Gerais state, Brazil. These cows presented ataxia and bilateral paresis of the pelvic limbs, which evolved to paralysis, and sternal recumbence. On necropsy, locally extensive areas of the longissimus dorsi muscle were replaced by pyogranulomas supported by moderate amounts of fibrous connective tissue. On the cut surface, some nodules contained yellowish and viscous fluid (purulent exudate) or whitish fluid (interpreted as the oily adjuvant of a vaccine). In the spinal canal of the subjacent vertebrae, compressing the spinal cord, were pyogranulomas identical to those described in the skeletal muscle. Histologically, the pyogranulomas were composed of a central clear vacuole (consistent with the space left by the oil adjuvant droplets), surrounded by neutrophils and, more externally, by large numbers of epithelioid macrophages and fewer multinucleated giant cells. In the white matter of the spinal cord were numerous well-defined, clear vacuoles (Wallerian degeneration). The association of the clinical history and pathological findings allowed the diagnosis of compressive myelopathy associated with pyogranulomatous reaction to the oily adjuvant of the foot-and-mouth disease vaccine, in this case, due to its inadequate application.(AU)

São descritos casos de síndrome de compressão medular, associada a granulomas pós-vacinais, em bovinos Nelore, provenientes de uma propriedade em Minas Gerais. Esses bovinos apresentavam ataxia e paresia bilateral dos membros pélvicos, que evoluiu para paralisia e decúbito esternal. Na necropsia, áreas focalmente extensas da musculatura na região torácica dorsal (músculo longissimus dorsi) eram substituídas por numerosos piogranulomas, separados por tecido brancacento e firme (tecido conjuntivo fibroso). Ao corte, alguns nódulos continham material amarelado e viscoso (exsudato purulento) ou material esbranquiçado e fluido (sugestivo de adjuvante de vacina). No canal medular das vértebras subjacentes, havia granulomas idênticos aos observados no tecido muscular. Histologicamente, os piogranulomas continham, no centro, vacúolo, bem delimitado e arredondado (consistente com o espaço deixado pela gotícula de lipídio do adjuvante), circundado por variável quantidade de neutrófilos degenerados e íntegros e, mais externamente, por numerosos macrófagos epitelioides e algumas células gigantes multinucleadas. Nas áreas da medula espinhal, circundadas pelos granulomas, numerosos vacúolos, bem definidos, eram observados na substância branca (degeneração walleriana). A associação do histórico clínico e de achados patológicos permitiu o diagnóstico de mielopatia compressiva associada à reação granulomatosa ao adjuvante oleoso da vacina contra febre aftosa, no caso, induzida pela aplicação inadequada da vacina.(AU)

Síndrome de Opalski / Opalski síndrome

El síndrome de Opalski es la presencia de hemiplejia ipsilateral asociada con síntomas del síndrome medular lateral. Diferentes estudios confirman que el infarto en tales casos ocurre a un nivel más bajo que el encontrado en el síndrome de Wallenberg. Se localiza debido a la presentación característica, el territorio único de suministro de sangre y el área pequeña de lesión. La implicación del tracto corticoespinal ipsilateral después de la decusación piramidal, o compresión de la decusación, podrían causarlo. Presentamos el caso de una mujer de 71 años que desarrolló hemiplejia izquierda y dificultad repentina para caminar, acompañada de diplopía, cefalea, disfagia y vómito recurrente. La imagen de resonancia magnética demostró un infarto lateral medular izquierdo. La angiografía reveló estenosis distal y proximal de la arteria vertebral izquierda e hipoplasia de la arteria vertebral derecha.

Opalski syndrome is the presence of ipsilateral hemiplegia which is associated with symptoms of a lateral medullary syndrome. Studies confirm that the infarction, in such cases, occurs at a lower level than that found in Wallenberg syndrome. This syndrome is localized because of characteristic presentation, the unique territory of blood supply and small area of lesion. The involvement of the ipsilateral corticospinal tract after the pyramidal decussation, or compression of the decussation, could cause the syndrome. We present a case of Opalski syndrome in a 71-year-old woman developed left hemiplegia and sudden difficulty for walking, accompanied by diplopia, headache, dysphagia and recurrent vomiting. Magnetic resonance image demonstrated a left lateral-medullary infarction. Angiography revealed distal and proximal stenosis of left vertebral artery and hypoplasia of right vertebral artery.

Síndrome de Opalski, una variante del síndrome de Wallenberg: reporte de caso y revisión de la literatura / Opalski Syndrome, a variant of Wallenberg Syndrome: Case report and literature review

RESUMEN Se presenta un caso local de la variante de Opalski, del síndrome medular lateral (síndrome de Wallenberg) y se hace una revisión de la literatura sobre los síntomas de este cuadro clínico, así como las posibles razones que explican esta modificación en la expresión clínica. La variante de Opalski fue descrita en 1948, ochenta y nueve años después de la descripción original del síndrome por Adolf Wallenberg, su principal característica es la presencia de hemiparesia o hemiplejía del cuerpo del mismo lado de la lesión del bulbo raquídeo. La etiología es vascular en la mayoría de casos, por obstrucción de la arteria cerebelosa postero-inferior o de los ostium de vasos penetrantes que se originan en la arteria. No hay estudios que reporten la incidencia exacta de este cuadro clínico a nivel mundial, sin embargo, su presentación no es tan infrecuente. Lo más interesante al analizar cada caso, es que los hallazgos semiológicos son una muestra de la profunda organización del sistema nervioso.

SUMMARY A clinical case of the Opalski variant of the lateral medullary syndrome (Wallenberg's syndrome) is presented and a literature review about the symptoms of this clinical picture is done, as well as the possible reasons explaining this modification in the clinical expression. The Opalski variant was described in 1948, eighty-nine years after the original description of the syndrome by Adolf Wallenberg, its main feature is the presence of hemiparesis or hemiplegia of the ipsilateral side of the injury in the medulla oblongata. In most cases the etiology is vascular when the postero-inferior cerebellar artery is occluded or there is an obstruction of the ostium of penetrating vessels originating in the artery. There are no studies that accurately report the worldwide incidence of this clinical variant, however, its presentation is not uncommon. The most interesting discovery of each case is that semiological findings are a good example of the profound organization of the nervous system.

Significance of SCIWORA in adults / Significância da SCIWORA em adultos / Trascendencia del cuadro SCIWORA en adultos

Recognizing the importance of SCIWORA in adult age; analyze the usefulness of complementary studies; evaluating therapeutic options; learn about the evolution of the treated patients.

Reconhecer a importância de SCIWORA na idade adulta; analisar a utilidade dos estudos complementares; avaliar as opções terapêuticas; conhecer a evolução dos pacientes tratados.

Reconocer la importancia de SCIWORA en la edad adulta; analizar la utilidad de los estudios complementarios; evaluar las opciones terapéuticas; conocer la evolución de los pacientes tratados.

Predictores en el cuadro SCIWORA del adulto / Preditores no quadro SCIWORA do adulto / Predictors in adult SCIWORA

OBJETIVOS: Analizar cinemática, reconocer cuadro clínico de presentación, describir imágenes, considerar posibilidades terapéuticas y evaluar evolución. MÉTODO: Se analizan 13 pacientes del sexo masculino que presentaron SCIWORA (en inglés Spinal cord injury without radiographic abnormality) entre el 2005 al 2012. Se evalúan cuadro clínico, tratamiento, complicaciones y evolución. RESULTADOS: 10 pacientes mayores de 45 años presentaban signos de espondiloartrosis con mínimos síntomas. De los tres menores de esta edad solo uno presentaba estenosis de canal constitucional asintomática. Todos sufrieron trauma de baja energía. En la resonancia magnética prevaleció el hematoma intramedular y clínicamente todos los pacientes presentaban un síndrome medular central, con cuadro severo (ASIA A-B) en los mayores de 45 años. Siete pacientes fueron tratados inicialmente en forma conservadora; un paciente empeoró y tuvo que ser sometido a intervención quirúrgica 18 meses después y otro falleció en las primeras horas, el resto de los pacientes tuvieron buena evolución. Seis pacientes requirieron cirugía (laminoplastía) en los primeros 10 días; tres fallecieron y el resto mejoro por lo menos un nivel ASIA. CONCLUSIONES: La menor edad, los cuadros leves y el edema intramedular son factores de buen pronóstico y se ven favorecidos con el tratamiento conservador. La mayor edad, la espondiloartrosis y los cuadros severos o progresivos, son factores de mal pronóstico y puede ser necesario su tratamiento quirúrgico.

OBJETIVOS: Análise cinemática, reconhecer apresentação clínica, descrever imagens, considerar as possibilidades terapêuticas e avaliar a evolução. MÉTODO: Análise de 13 pacientes do sexo masculino com SCIWORA (em ingles Spinal cord injury without radiographic abnormality) entre 2005 e 2012. Avalia-se quadro clínico, tratamento, complicações e resultado. RESULTADOS: Dez pacientes com mais de 45 anos apresentaram sinais de espondiloartrose com sintomas mínimos. Dos três pacientes com menos de 45 anos, apenas um tinha estenose de canal constitucional assintomática. Todos haviam sofrido trauma de baixa energia. Na ressonância magnética prevaleceu hematoma intramedular e clinicamente todos os pacientes com mais de 45 anos tinham síndrome medular central grave (ASIA A-B). Sete pacientes foram inicialmente tratados de forma conservadora; um paciente piorou e teve que ser submetido a cirurgia 18 meses depois e outro morreu nas primeiras horas, o restante teve bom desempenho. Seis pacientes necessitaram de cirurgia (laminoplastia) nos primeiros 10 dias; três morreram e os pacientes restantes melhoraram pelo menos um nível ASIA. CONCLUSÕES: A idade mais baixa, os casos não graves e o edema intramedular são bons fatores prognósticos e são favorecidos pelo tratamento conservador. A idade mais elevada, a espondiloartrose e os quadros graves ou progressivos, são fatores de mau prognóstico e podem necessitar de tratamento cirúrgico.

OBJECTIVES: Kinematic analysis, recognize the clinical presentation, describe image tests, consider therapeutic possibilities and assess progress. METHOD: Analysis of 13 male patients with SCIWORA (Spinal cord injury without radiographic abnormality) between 2005 and 2012. Evaluates clinical presentation, treatment, complications and outcome. RESULTS: 10 patients over 45 years showed signs of spondyloarthritis with minimal symptoms. Of the three with less than 45 years, only one had a constitutional asymptomatic stenosis. All had suffered low-energy trauma. In the magnetic resonance prevailed intramedullary haematoma and clinically all patients over 45 years had a central cord syndrome with severe symptoms (ASIA A-B). Seven patients were initially treated conservatively; one patient worsened and had to undergo surgery after 18 months and another died in the early hours; the remaining patients had good performance. Six patients required surgery (laminoplasty) in the first 10 days; three died and the remaining patients improved at least one ASIA level. CONCLUSIONS: The lower age, the mild cases and intramedullary edema are good prognostic factors and are favored by conservative treatment. The higher age, spondyloarthritis and severe or progressive conditions are factors of poor prognosis and may require surgical treatment.

Síndrome de Wallenberg: hallazgos en resonancia magnética con correlación clínica / Wallenberg syndrome: magnetic imaging findings and clinical correlation

El síndrome de Wallenberg es una de las entidades clínicas más frecuentemente reconocidas como parte de la patología vascular del tallo cerebral. En la actualidad puede ser identificada con rapidez gracias a las modernas técnicas en neuroimagen. Presentamos un paciente con cuadro clínico de miosis, disfonía, disfagia, seguido de ataxia. Se efectuó evaluación con resonancia magnética utilizando técnicas de difusión, coeficiente aparente de difusión, FLAIR (fast fluid-attenuated inversion recovery) y perfusión. Se describe la correlación clínica de los síntomas con la localización anatómica de la lesión, y se discuten los hallazgos de imagen. La descripción de este caso enfatiza la utilidad de la resonancia magnética para una adecuada evaluación y correlación clínica de los hallazgos en imagen con la exploración neurológica.

Wallenberg's syndrome is one of the most common clinically recognized conditions due to brain stem infarct, which can nowadays be identified by modern neuro-imaging techniques. We describe a patient complaining of miosis, dysphonia, and dysphagia followed by ataxia. An MRI evaluation was performed including diffusion-weighted imaging, apparent diffusion coefficient, T2-weighted images, fluid attenuated inversion recovery (FLAIR) and perfusion. A brief discussion of imaging findings is presented as well as a clinical correlation of the symptoms with the anatomic location of the lesion. This case report emphasizes the importance of imaging findings and their clinical correlation with neurological examination.