-Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory disorder of the central nervous system with both poor prognosis and high mortality. The disease has been related to a persistent and aberrant measles virus infection and no effective treatment has been available. We report a case of SSPE with atypical features including seizures at onset and a fulminant course in a 8 years-old boy who had been previously immunized against measles.KEY WORDS: subacute sclerosing panencephalitis, measles, immunization.Panencefalite esclerosante subaguda: relato de caso atípico RESUMO -Panencefalite esclerosante subaguda (PES) é uma doença inflamatória e progressiva do sistema nervoso central com prognóstico reservado e alta mortalidade. A doença tem sido relacionada com a infecção persistente e anormal pelo vírus do sarampo e não há tratamento específico disponível. Relatamos um caso de PES com características atípicas representadas por início do quadro com crises convulsivas e apresentação fulminante em menino de 8 anos previamente imunizado contra o vírus do sarampo.PALAVRAS-CHAVE: panencefalite esclerosante subaguda, sarampo, imunização.

A trombose venosa cerebral (TVC) é doença vascular com diferentes manifestações clínicas e várias causas possíveis (locais, sistêmicas ou idiopáticas). A trombose da veia de Galeno (TVG) é causa rara de TVC e geralmente está associada a alguma malformação vascular. Relatamos o caso de uma paciente de 16 anos que apresentou TVG sem malformação vascular, porém associada a trombose de seio reto e infarto venoso talâmico. Discutem-se também aspectos importantes do diagnóstico clínico, radiológico e laboratorial da TVC.

RESUMO -Meningoceles intra-sacrais são lesões raras, que podem determinar sintomas de compressão radicular. Descrevemos o caso de uma paciente de 18 anos, com queixa de incontinência urinária, acometida por esta patologia. Após avaliação neuro-urológica e estudo uro-dinâmico, foi demonstrada arreflexia do detrusor, sendo aventada a hipótese de disrafismo espinhal. O diagnóstico de meningocele intra-sacral oculta foi evidenciado através de ressonância magnética. A paciente foi submetida a abordagem cirúrgica com boa recuperação pós-operatória. No presente artigo, é feita breve revisão da literatura, e são discutidas as apresentações clínicas possíveis, os achados neuro-radiológicos e o tratamento cirúrgico. PALAVRAS-CHAVE: coluna, cirurgia, meningocele intra-sacral. Occult intrasacal meningocele: case reportABSTRACT -Intrasacral meningoceles are rare conditions that can cause symptoms of nerve root compression. We report a case of a 18-year-old female, who presented with urinary incontinence. The neurological and urological evaluation revealed lack of detrusor muscle reflex. This finding supported the hypothesis of spinal disraphism. Magnetic resonance image revealed an intrasacral meningocele. The patient was submitted to surgical excision of the lesion without postoperative complications. In the present paper, the pathogenesis, clinical picture, neuroradiological findings and surgical treatment are discussed. O termo meningocele intra-sacral oculta foi utilizado pela primeira vez na literatura por Enderle (1932) 1 , para descrever uma dilatação meníngea intra-sacral, identificada à mielografia. Embora considerado inadequado por alguns autores 2-6 , seu uso foi consagrado para definir lesões císticas intra-sacrais compostas de tecido fibroso semelhante a duramáter, geralmente acompanhado pela aracnóide, ocupando o canal sacral dilatado e ligado à extremidade do saco dural por trajeto fistuloso que, usualmente, permite fluxo livre de líquido cefaloraquidiano (LCR) [5][6][7] . Vários sinônimos têm sido utilizados para definir esta mesma lesão: meningocele intrasacral oculta 3,4,[8][9][10][11][12] , cisto intra-sacral 4,6,9 , cisto intraespinhal sacral 9 , expansão do espaço subaracnóideo 3,4,6,9 , cisto extradural meníngeo com ausência de fibras nervosas 7,9,12 , meningocele intra-sacral 2,4,9,10,12 . Mais frequentemente, a lesão torna-se sintomática na idade adulta, sugerindo crescimento lento após o nascimento 2,4,10 . A compressão radicular pelo cisto parece ser a causa dos sintomas, determinando dor lombar baixa, eventualmente com irradiação no trajeto radicular. Os fatores compressivos ou displásicos incidentes na região pélvica que prejudicam a função neural autonômica, produzem o complexo sintomático, podendo ocorrer disfunção vesical e disfunção erétil [2][3][4]7,8,10,11 . A estocagem e eliminação periódica da urina deve-se à complacência ou contração da bexiga, à função dos esfincteres externo e interno e à contração da musculatura da uretra e do assoalho pélvico. A motilidade da bexiga é controlada pelo ...

Ossification of the posterior longitudinal ligament (OPLL) has been a relatively common cause for myelopathy in Korea and Japan since its first description by Tsukimoto in 1960 1,2 . OPLL occurs in perhaps only 0.2% of the Caucasian population 3 , whereas its prevalence in Japan has been reported as of 1.5-2.4% in adults 4 . The rarity of the condition in non-Oriental populations often leads to delay and/or failure of recognition. We report an OPLL in a 54-year-old Afro-Brazilian woman who had tetraparesis and a diagnosis based on clinical and radiological investigation. The aim of this report is to discuss the relevant aspects of OPLL, mainly its clinical presentation and diagnosis. CASEA 54-year-old hypertensive woman was hospitalized (HU-UFJF-SAME 347836) with approximately four months of gait impairment. On orthopedical evaluation, spastic tetraparesis and trunk instability were observed. In addition to this, on neurologic consultation, increased deep tendon reflexes of both upper and lower extremities were detected. There were no abnormalities on upper-and-lower-limb cerebelar and sensitive examinations. Nor family background or spondylosis, diabetes, trauma, irradiation history were positive.Radiographs revealed a hyperdense image at the cervical vertebral canal (Fig 1). A computed tomogram (CT) showed a mass of ossification compressing fifty percent of the spinal cord in the posterior aspect, from the 3 rd to the Tetraparesia secundária a ossificação do ligamento longitudinal posterior cervical: relato de caso RESUMO -Ossificação do ligamento longitudinal posterior (OLLP) é causa rara de mielopatia na população não Oriental e relativamente subdiagnosticada por clínicos gerais. Relata-se um caso de mulher de 54 anos descendência Afro-Brasileira com tetraparesia associada a OLLP cervical. Enfatiza-se a inclusão da OLLP como diagnóstico diferencial de mielopatia cervical compressiva.PALAVRAS-CHAVE: ossificação do ligamento longitudinal posterior, compressão da medula espinhal, estenose espinhal.

Infarction of the anterior cerebral artery (ACA) territory accounts for only 0.3-4.4% of cerebral infarctions. We report an unusually prolonged progressing stroke of the ACA in a 58-yearold patient who had his diagnosis based on neuroimaging investigation and in anatomopathological exclusion of neoplastic disorder.

The human T-lymphotropic virus type I (HTLV-I) is the etiologic agent of tropical spastic paraparesis / HTLV-I associated myelopathy (TSP/HAM), adult T cell leukemia/lymphoma (ATLL) as well as of other immunomediated systemic diseases. Contrary to other pathogenic viruses to man HTLV-I apparently causes diseases in the minority of the infected population. The real prevalence of HTLV-I neurologic manifestations is still reason for debate in the literature with percentages ranging from 0.07% to 2.6%. The relation between an increased risk for development of disease and other contributing cofactors is also speculated. The continuous prospective observation of infected individuals has suggested to us that the prevalence of neurological manifestations might be higher than what is presently assumed. In order to evaluate the prevalence of signs and symptoms of neurological involvement in a population of blood donors from the largest blood bank from the city of Rio de Janeiro, Brazil (HEMORIO) and also the possible correlation of epidemiological variables which could possibly contribute to a higher risk of development of neurological disease we designed the present study.Between May 1995 to December 1997, 392 blood donors from HEMORIO were selected by a cross sectional study. Fifty percent of these individuals were HTLV-I positive. For the prevalence study, neurologically impaired HTLV-I positive individuals were considered cases and HTLV-I positive individuals with a normal neurological examination, controls. Cases were classified in symptomatics and asymptomatics, and according to the topographic diagnosis (involvement of the central [CNS], peripheral [PNS] nervous system or muscle). Patients underwent specific laboratory investigations. The method of case-control study was employed for the evaluation of risk factors for the presence of neurological disease. For this part of the study HTLV-I positive patients were classified as cases, and seronegative asymptomatic individuals as controls.Neurological disease was found in 71 (36.2%) out of 196 seropositive individuals. Of these, 34 (48%) individuals had involvement of the CNS (11 [32.3%] TSP/HAM cases); 33 (46%) patients with involvement of PNS (18 [54.5%] cases of polyneuropathy) and 4 (6%) patients with muscular disease (one case of polymyositis). Previous use of intravenous drugs (OR 9.9 and CI95% 1.9-68.5), family history of either HTLV-I infection or HTLV-I associated disease (OR 7.5 and CI95% 3.1-18.8), risky sexual behavior (OR 6.9 and CI95% 2.5-20.4), hemotransfusion in the past , and a history of either HTLV-I infection or HTLV-I associated diseases (OR 9.9 and CI95% 1.8-11.6) were significantly associated to the presence of neurological disease among HTLV-I seropositives. Hypergammaglobulinemia and ophthalmologic abnormalities were also significantly associated to the presence of those neurologic disorders.Our data suggest that the exposition to HTLV-I was significantly associated to the development of neurological disease in a higher proportion than what has...