Fechamento de hemicorporectomia com utilização do retalho subtotal unilateral da coxa: relato de caso / Closure of hemicorporectomy using a subtotal unilateral tight flap: a case report

A hemicorporectomia ou amputação translombar foi primeiramente descrita em 1950 por Kredel como método curativo para neoplasia localmente avançada de pelve. Trata-se de um procedimento cirúrgico extenso, que quando bem indicado é capaz de prover a cura oncológica, além de possibilitar a melhora clínica e de qualidade de vida. Os autores apresentam o relato de caso de um paciente de 34 anos, com carcinoma espinocelular em úlcera de pressão crônica, acometendo períneo, glúteo e coxa posterior à direita. O paciente foi submetido à hemicorporectomia com reconstrução utilizando retalho subtotal da coxa esquerda. Observou-se boa evolução no pós-operatório e a utilização do retalho subtotal da coxa para fechamento da hemicorporectomia se mostrou como método seguro, eficiente e de técnica reprodutível.

Hemicorporectomy or translumbar amputation was first described in 1950 by Kredel to treat locally advanced pelvic neoplasia. This extensive surgical procedure can achieve oncological cure and improve clinical status and quality of life. The authors present a case report of a 34-yearold patient with squamous cell carcinoma in a chronic pressure ulcer affecting the right perineum, gluteus, and posterior thigh. The patient underwent hemicorporectomy with reconstructive surgery using a partial-thickness flap of the left thigh. The postoperative course was good, and the use of a partial-thickness flap of the thigh to close the hemicorporectomy proved to be safe, efficient, and reproducible.

Translation and validation into Brazilian Portuguese of the Spastic Paraplegia Rating Scale (SPRS) / Tradução e validação da escala de classificação de paraplegia espástica (SPRS) para a versão brasileira

ABSTRACT Hereditary spastic paraplegias (HSP) are characterized by progressive lower limb weakness and spasticity. There are no validated instruments to quantify disease severity in Portuguese. Objective To translate and validate the Spastic Paraplegia Rating Scale (SPRS) into Brazilian-Portuguese. Method Two experienced and English-fluent neurologists translated SPRS into Portuguese, creating SPRS-BR. We then assessed inter and intra-rater reliability of this version using coefficients of correlation and variability in a cohort of 30 patients. Results Mean age of patients and disease duration were 47.7 ± 10.5 and 17.0 ± 10.6 years, respectively. Twenty-one had pure HSP and SPG4 was the most frequent genotype. Mean Rankin and SPRS-BR scores were 2.2 ± 0.9 and 19.9 ± 9.9, respectively. Mean intra and inter-rater correlation coefficients of SPRS-BR scores were 0.951 and 0.934, whereas coefficients of variation were 11.5% (inter-rater) and 9.9% (intra-rater). Cronbach’s alpha for the whole SPRS-BR scale was 0.873. Conclusion SPRS-BR is a useful, reliable and valid clinical instrument.

RESUMO As paraparesias espásticas hereditárias (PEH) apresentam progressiva espasticidade e fraqueza dos membros inferiores. Não existem escalas validadas em língua portuguesa para quantificar a gravidade da doença. Objetivo Traduzir e validar para o português do Brasil a Spastic Paraplegia Rating Scale (SPRS). Método Dois neurologistas experientes em neurogenética e fluentes em inglês traduziram a SPRS para o português, criando a SPRS-BR. Em seguida, checamos a reprodutibilidade da escala usando coeficientes de correlação e variabilidade em um grupo de 30 pacientes. Resultados As médias de idade e duração de doença foram de 47,7 ± 10,5 e 17,0 ± 10,6 anos, respectivamente. Vinte e um eram portadores da forma pura de PEH, sendo que SPG4 foi o genótipo mais frequente. A pontuação das escalas Rankin e SPRS-BR foi, respectivamente, 2,2 ± 0,9 e 19,9 ± 9,9. Os coeficientes de correlação inter e intraexaminador da SPRS-BR foram 0,934 e 0,951, enquanto que os coeficientes de variação foram de 11,5% (interexaminador) e 9,9% (intraexaminador). O coeficiente alfa de Cronbach’s para a escala SPRS-BR foi de 0.873. Conclusão A SPRS-BR é uma escala útil clinicamente, fácil de aplicar e que apresentou boa reprodutibilidade e validade.

Striatal Dopaminergic Functioning in Patients with Sporadic and Hereditary Spastic Paraplegias with Parkinsonism

Sporadic spastic paraplegia (SSP) and hereditary spastic paraplegia (HSP) belong to a clinical and genetically heterogeneous group of disorders characterized by progressive spasticity and weakness in the lower extremities. The symptoms are associated with pyramidal tract dysfunction and degeneration of the corticospinal tracts. Parkinsonism is uncommon in SSP/HSP patients. However, both disorders are associated with damage to the nigrostriatal dopaminergic system. In the present study, the clinical features of patients with SSP/HSP were investigated, and nigrostriatal dopaminergic binding potential was assessed using dopamine transporter (DAT) single-photon emission computer tomography (SPECT). Nine patients with spastic paraplegia participated in the present study. The subjects underwent DAT SPECT using the agent [2-[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3,2,1]oct-2-yl]methyl](2-mercaptoethyl)amino]ethyl]amino]ethanethiolato (3-)-N2,N20,S2,S20]oxo-[IR-(exo-exo)])-[99mTc]technetium ([99mTc]TRODAT-1). The [99mTc]TRODAT-1 SPECT images of five patients appeared normal, whereas the images of four patients revealed reduced striatal ligand uptake. Among the four patients with reduced uptake, two had parkinsonism, and one exhibited periodic limb movements and restless leg syndrome. Our DAT SPECT imaging study shows that reduced DAT density may be observed in patients with parkinsonism. The results of the present study offer an explanation for the spectrum of spastic paraplegia symptoms and the progression of the disorder.

Profile and Outcome of Non-Traumatic Paraplegia in Kano; Northwestern Nigeria

Aim: This study was aimed to identify the clinical and radiological profile of non-traumatic paraplegia and the various etiologies associated with the condition. Materials and Methods: A review of the clinical and radiological presentations of adult patients presenting with non-traumatic paraplegia managed at the Aminu Kano Teaching Hospital (AKTH) and Murtala Specialist Hospital (MMSH); Kano; from June 2006 to November 2009 was carried out. Patients underwent a detailed clinical evaluation followed by laboratory investigation and neuroimaging studies and were followed up for 9 months to asses outcome and complications. Results: 98 patients with non-traumatic paraplegia consisting of 71 males and 27 females (M:F: 5:2) were seen. The age range of the patients was between 16 and 76 years; with a mean age of 40 years (SD = 15.3) years; 54 (55) of the patients presented after 2 months of the onset of paraplegia. The commonest symptoms were weakness of the lower limbs (100); loss of sensation (55); sphincteric disturbance (50) radicular pain and paresthesia (38.4); back pain (21.4) and erectile dysfunction (40). All the patients had X-ray of the spine; 26.3had Magnetic Resonance Imaging (MRI) spine. The commonest etiological factors were tuberculosis (TB) (44.4); transverse myelitis (13.1); Guillain-Barre syndrome (9.1); metastatic spinal disease (4); and HIV myelopathy (4). However; the cause could not be identified in 14 (14) of the patients. The commonest site of affectation in those with TB spine was lower thoracic (53.8) and upper lumbar (23.1) vertebrae. Conclusion: Clinical profile of non-traumatic paraplegia in Kano; northwestern Nigeria; is similar to that reported elsewhere in Africa; with spinal tuberculosis and transverse myelitis accounting for over half the cases

Manejo de la espasticidad en pacientes con parálisis cerebral infantil en el Hospital Leonarso Martínez Valenzuela / Management of spasticity in patients with cerebral palsy in children at the Hospital Leonarso Martínez Valenzuela

Esta investigaci ón se basa en el trabajo que se est á llevando a cabo en el Hospital Leo- nardo Mart ínez por el servicio de Ortopedia Pedi átrica para el manejo de la espasticidad en los ni ños con Par álisis Cerebral Infantil indepe ndientemente del dañ o fisiol ógico o topogr áfico. Desde Julio del 2010 hasta Enero del 2011 se han realizado 16 cirugí as en ni ños con par álisis cerebral. Los proce dimientos realizados han ido desde tenoto- mías y alargamientos tendinosos multinive- les, hasta transferencias tendinosas y co rrecci ón del pie plano espá stico. Los resul tados al momento han sido alentadores evaluando la mejora de la calidad de vida de los ni ños al igual que la mejora en sus habi lidades motoras. Los casos se han manejado de manera conjunta con el servicio de Cardiologí a Pedi átrica del Hospital Leonardo Mart ínez, servicio de Neurologí a Pedi átrica del IHSS, y por diversas organi - zaciones que proveen servicios de rehabili - taci ón como ser Telet ón, FUNHCAE y el Centro de Rehabili taci ón del IHSS...(AU)

[ report of a case with Gorham's disease]

Gorham's disease is rare and is associated with progressive bone destruction. The first case was reported in 1838 and until now 175 cases with Gorham's disease have been reported in the literature. In this article a patient with concurrent involvement of lumbar spine and trochanteric region is reported. A 32 years old woman with intensive back pain and paraplegia was admitted to our center. Radiological and laboratory studies were done. Spinal x-rays revealed L3 collapse and a potential metastatic lesion in L3 and trochanteric region. Patient underwent cord decompression and stabilization of the spine and biopsy was done. Histological studies demonstrated that the lesion is due to Gorham's disease. Gorham's disease can involve spine and cause paraplegia and in this conditions, decompression can be helpful. Also, our case portrayed that this disease can involve distant bones concurrently

Espondilolistese em frango de corte no Brasil / Spondylolisthesis (\"Kinky Back\") in broiler chickens in Brazil

Eight 28-day-old broiler chickens of both sexes were examined. Clinical signs, gross findings, radiological, and histopathological changes were described. Spondylolisthesis was characterized by dorsal displacement of the sixth and seventh thoracic vertebrae, resulting in compression of the spinal cord. The major clinical manifestation was paraplegia. Risk factors such as genetics, nutrition, stress, rate of growth, and age were discussed.

Pott's spine and paraplegia.

Spinal tuberculosis is usually secondary to lung or abdominal involvement and may also be the first manifestation of tuberculosis. Spinal tuberculosis (often called Pott's disease) is by definition, an advanced disease, requiring meticulous assessment and aggressive systemic therapy. Physicians should keep the diagnosis in mind, especially in a patient from a group with a high rate of tuberculosis infection. This review aims on updating the knowledge on spinal tuberculosis and its management. Skeletal involvement has been reported to occur in approximately 10% of all patents with extrapulmonary tuberculosis, and half of these patients develop infection within the spinal column. Symptoms of spinal tuberculosis are back pain, weakness, weight loss, fever, fatigue, and malaise. It is much more prone to develop neurological manifestation, paraplegia of varying degree. The palpation of spinous process in routine clinical examination is the most rewarding clinical method and is an invaluable measure for early recognition. Diagnosis of spinal tuberculosis is made on the basis of typical clinical presentation along with systemic constitutional manifestation and the evidence of past exposure to tuberculosis or concomitant visceral tuberculosis. Magnetic resonance imaging can define the extent of abscess formation and spinal cord compression. The diagnosis is confirmed through percutaneous or open biopsy of the spinal lesion. Surgery is necessary as an adjunct to antibiotic therapy if the vertebral infection produces an abscess, vertebral collapse, or neurologic compression. Some patents need aggressive supportive care owing to tuberculous meningitis or encephalopathy. Moreover, the importance of immediate commencement of appropriate treatment and its continuation for adequate duration along with the proper counseling of the patient and family members should not be underestimated for successful and desired outcome.

Foix-Alajouanine syndrome: case report

Em mulher de 52 anos, malformação artério-venosa medular (MAV) associava-se com a síndrome de Foix-Alajouanine. A fisiopatologia da fístula artéria-venosa provavelmente está relacionada com a pressão venosa aumentada, a partir da MAV associada com o processo trombótico. Os sintomas iniciais mais comuns são distúrbio sensorial, dor e fraqueza do membro inferior. O diagnóstico definitivo de MAV medular requer evidências radiográficas da anomalia vascular. De qualquer modo, neste caso, defeitos sugestivos de malformações não foram detectados, contrastando com os achados de ressonância magnética e lesões macroscópicas e anátomo-patológicas. Estes resultados chamaram a atenção e excluíram outras causas. A alta suspeita clínica de MAV nos levou a uma conduta cirúrgica precoce em benefício do paciente.

Paraplejía producida por schistosomiasis de la médula espinal: presentación de 4 pacientes con estudio histopatológico / Paraplegia produced by schistosomiasis in spinal cord: report of 4 patients with histopathological study

Los autores describen y comentan las historias clínicas de 4 pacientes que fueron admitidos al Hospital Central de Valencia, Venezuela, por presebtar cuadro de paraplejía, demostrándose posteriormente que tenían localización de huevos del Schistosoma mansoni en la médula espinal. En 2 pacientes las manifestaciones clínicas fueron de un síndrome mielomeníngorradicular con parálisis de los miembros inferiores, hipotrofia muscular, signo de Babinski bilateral, compromiso de los esfínteres urinario y rectal, alteraciones objetivas de la sensibilidad, nivel sensitivo anestésico o hipoestético. Los otros 2 pacientes desarrollaron síntomas y signos de comprensión de la médula espinal. se realizó maniobra de Queckenstedt-Stookey. La mielografía fue normal en los 2 primeros casos y en el tercero se comproaron signos compatibles con aracnoiditis o de posible tumor intramedular. En el cuarto paciente se practicó resonancia magnetica nuclear revelándose señales hiperintensas T1 a T4 sugestivas de tumor intranedular. Las lesiones encontradas variaron desde una mielitis necrotizante a una reacción granulomatosa producida por los huevos, de tipo inmunológico, de hipersensibilidad reatrdada. Todos los pacientes fueron tratados mediante extirpación quirúrgica de los granulomas seguidos de tratamiento con medicación anti-Schistosoma: hycantone (3 mg/kg peso, diario por un día), en el paciente Nº4 se adminsitró praziquantel 40 mg/kg peso en dosis única, 2 400mg dosis total. La acción farmacológica produce bloqueo de las vías metabólicas del parásito como es la inhibición de la fosfructoquinasa y también toxicidad para algunas organelas específicas como es la glándula vitelogénica, produciendo la destrucción de la misma. Los esteroides han sido usados con resultados poco convencentes, sin embargo, publicaciones de casos enecdóticos de mejoría con el uso precoz de los corticoesteroides suprarrenales justifican el tratamiento de la mielopatía producida por el Schistosoma mansoni con dexametasona o prednisona en el momento mismo en que se diagnóstica el compromiso de la médula espinal. Estos casos fueron tratados con dexametasona a la dosis de 8 mg IV cada 8 h. El pronóstico de la forma mielomeningorradicular fue peor que la de los pacientes con el síndrome de compresión medular, ya que los 2 pacientes con esta última forma clínica obtuvieron recuperación completa de la sintomatología neurológica, incluso uno de ellos (el paciente Nº4) obtuvo erección peniana completa

Giant cell tumour of soft tissue--a case report.

Giant cell tumor is seen in late adolescence or in the third or fourth decade of life. It arises from epiphysis of long bones, the commonest site being the distal end of the femur and the proximal end of tibia. This paper presents a case report of giant cell tumor of soft tissue.

Síndrome de Munchausen e pseudoparaplegia: relato de caso / Syndrome of Munchausen and pseudoparaplegia: case report

Apresentamos uma paciente com "paraplegia" de oito anos de evolução, que se internou para retirada de agulhas em região lombar e apresentava história de insuficiência renal, câncer de esôfago, ovários e mama, suspeita de tubercolose pulmonar e várias internações em hospitais clínicos e psiquiátricos. No exame psiquiátrico encontramos indiferença à sua doença, prolixidade e descrição detalhada de seus problemas, falando mal dos atendimentos anteriores. Não encontramos evidências de processos neoplásicos. Tinha movimento em bloco de membros inferiores, com preservação dos reflexos e sensibilidade. A eletromiografia, a tomografia de crânio, a ultra-sonografia abdominal e o RX de tórax normais. No RX e na tomografia de coluna lombo-sacra observamos 16 agulhas, semelhantes às de costura, em partes moles. Diagnosticada como síndrome de Munchausen, depois de 15 dias de internação com tratamento antidepressivo, psicoterapia e fisioterapia, melhorou. Três meses depois, a paciente encontrava-se assintomática, não voltando mais ao ambulatório.

Detección de necesidades en relación con la sexualidad en pacientes con lesión medular / Detection of patients' needs in relationship to their sexuality after medullary injury

Se trata de un estudio epidemiológico de detección de los problemas que sufren 35 pacientes con paraplejía en relación con manejo de su sexualidad. Se encontró que el 58 por ciento conservaron su pareja estable: el 34 por ciento consideró que su relación sexual no se había afectado; el grado de satisfacción sexual fue muy alto en 11 por ciento y alto en el 37 por ciento. Por otra parte el 80 por ciento no tiene ni siquiera el nivel mínimo de conocimientos sobre el desempeño de la sexualidad

Ostectomia subtrocanterea como tratamiento alternativo en parapléjicos con luxación abierta posterior de cadera: reporte de un caso / Ostectomy subtrochanteric how treatment alternative in paraplegics with dislocation of open lip posterior: report a case

En el presente trabajo se presenta un caso de luxación posterior abierta de cadera espontánea en paciente parapléjico con ulceras crónicas de decúbito, planteándose como tratamiento alternativo la ostectomía subtrocanterea en virtud de haberse resuelto en forma exitosa mediante esta intervención tanto la luxación de cadera como la ulcera de decúbito.

Ocorrência de paraplegia associada a infecçäo com Schistosoma mansoni näo autóctone no estado de Goiás / Occurrence of paraplegia associated with non autochthnous Schistosoma mansoni - infection in the State of Goiás

Um caso de paraplegia em paciente de 36 anos é associado a infecçäo näo autóctone de Schistosoma mansoni. A possibilidade de presença de fatores que possibilitaräo o estabelecimento desta parasitose no Estado de Goiás é discutida