Mielopatias inflamatórias / Inflammatory myelopathies / Mielopatías inflamatorias

Mielopatia inflamatória ou mielite transversa é uma síndrome neurológica potencialmente incapacitante com uma variedade de etiologias. Episódios únicos ou recorrentes podem resultar em dependência de cadeira de rodas. O quadro clínico de fraqueza, alteração de sensibilidade e disfunção autonômica de início agudo ou subagudo é marca dessa síndrome. Esse cenário é comum às diferentes etiologias, que podem ser de natureza desmielinizante, por doença autoimune sistêmica, paraneoplásica ou infecciosa. A ressonância magnética de coluna é o exame de neuroimagem de escolha. Exames complementares como avaliação do líquido cefalorraquidiano, testes sorológicos e pesquisa de anticorpos dão suporte à investigação. A depender da etiologia, há tratamentos específicos a fim de reduzir incapacidade e chance de novos surtos, além de diferentes prognósticos. Este trabalho objetiva uma revisão de literatura sobre mielopatias inflamatórias e suas principais etiologias, a partir de dados obtidos na plataforma eletrônica PubMed. Para a discussão, foram revisadas as etiologias desmielinizantes (encefalomielite disseminada aguda, esclerose múltipla, doença do espectro, neuromielite óptica e neurite óptica, encefalite e mielite associadas ao MOG-IgG); doenças autoimunes (lúpus eritematoso sistêmico e síndrome de Sjögren); síndromes paraneoplásicas e mielopatias infecciosas (neuroesquistossomose, mielite por HIV e por HTLV-1 e neurossífilis). Concluiu-se com este estudo que a mielopatia inflamatória é uma condição de gravidade variável que produz potencial incapacidade, causada por diferentes etiologias, porém com quadro clínico comum entre elas. Por isso, é importante conhecer cada uma dessas causas, a fim de promover o melhor e mais precoce tratamento e reduzir sequelas.

Inflammatory myelopathy or transverse myelitis is a potentially disabling neurological syndrome with various etiologies. Single or recurrent episodes can result in wheelchair dependence. A clinical picture of weakness, altered sensitivity, and autonomic dysfunction with acute or subacute onset is characteristic of this syndrome. This scenario is common to different etiologies, which can be of a demyelinating nature, due to systemic, paraneoplastic, or infectious autoimmune disease. Spine MRI is the neuroimaging test of choice. Complementary tests such as cerebrospinal fluid evaluation, serological tests and antibody research support the investigation. Depending on the etiology, there are specific treatments to reduce disability and the chance of new episodes, and different prognoses. This study is a literature review on inflammatory myelopathies and their main etiologies, based on data obtained from the PubMed database. Demyelinating etiologies (acute disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optic spectrum disease and optic neuritis, MOG-IgG-associated encephalitis and myelitis), autoimmune diseases (systemic lupus erythematosus and Sjögren's syndrome), paraneoplastic syndromes and infectious myelopathies (neuroschistosomiasis, HIV and HTLV-1 myelitis, and neurosyphilis) were reviewed for discussion. In conclusion, inflammatory myelopathy is a condition of variable severity that produces potential disability, caused by different etiologies, but with a common clinical picture between them. Thus, knowledge on each of these causes is important to promote the best and earliest treatment and reduce sequelae.

La mielopatía inflamatoria o mielitis transversa es un síndrome neurológico potencialmente incapacitante con una variedad de etiologías. Los episodios únicos o recurrentes pueden tener como consecuencia dependencia de silla de ruedas. El cuadro clínico de debilidad, sensibilidad alterada y disfunción autonómica de inicio agudo o subagudo es distintivo de este síndrome. Esto es común a diferentes etiologías, que pueden ser de naturaleza desmielinizante, debido a enfermedades autoinmunes sistémicas, paraneoplásicas o infecciosas. La resonancia magnética de columna es la prueba de neuroimagen de elección. Las pruebas complementarias, como la evaluación del líquido cefalorraquídeo, las pruebas serológicas y la investigación de anticuerpos respaldan la investigación. Dependiendo de la etiología, existen tratamientos específicos para reducir la discapacidad y la posibilidad de nuevos brotes, además de diferentes pronósticos. Este trabajo tiene como objetivo revisar la literatura sobre mielopatías inflamatorias y sus principales etiologías desde los datos obtenidos de la base de datos electrónica PubMed. Se revisaron las etiologías desmielinizantes (encefalomielitis aguda diseminada, esclerosis múltiple, enfermedad del espectro, neuromielitis óptico y neuritis óptica, encefalitis y mielitis asociadas a MOG-IgG), las enfermedades autoinmunes (lupus eritematoso sistémico y síndrome de Sjögren), los síndromes paraneoplásicos y mielopatías infecciosas (neurosquistosomiasis, mielitis por VIH y HTLV-1 y neurosífilis). Se concluyó que la mielopatía inflamatoria es una condición de severidad variable, que produce potencial discapacidad causada por diferentes etiologías, pero tiene un cuadro clínico común entre ellas. Por ello, es importante conocer cada una de las causas para promover el mejor y más precoz tratamiento, además de reducir las secuelas.

Neuroesquistosomiasis / Neuroschistosomiasis

RESUMEN La esquistosomiasis es una enfermedad parasitaria causada por helmintos del género Schistosoma. La neuroesquistosomiasis es la afectación del sistema nervioso central (SNQ en la esquistosomiasis. Se trata de una afectación grave, infradiagnosticada y potencialmente letal. La verdadera prevalencia de la neuroesquistosomiasis se desconoce y las cifras obtenidas de los diferentes estudios son muy dispares. Los esquistosomas son trema-todos cuyo hábitat principal se ubica en los vasos sanguíneos de los vertebrados. El sistema nervioso central (SNC) puede afectarse en todas las fases de la esquistosomiasis, incluyendo la fase postinfecciosa precoz, el estadio intermedio paucisintomático y los estadios crónicos avanzados. A la fecha no existe consenso respecto al tratamiento de la neuroesquistosomiasis, pero se cuenta con algunas opciones orales y, en algunos casos, el abordaje quirúrgico.

SUMMARY Schistosomiasis is a parasitic disease caused by helminths of the genus Schistosoma. Neuroschistosomiasis is the involvement of the central nervous system (CNS) in the schistosomiasis. It is a serious, underdiagnosed, and potentially fatal condition. The true prevalence of neuroschistosomiasis is unknown and the figures obtained from the different studies are very disparate. Schistosomes are trematodes whose main habitat is located in the blood vessels of vertebrates. The central nervous system (CNS) can be affected in all phases of schistosomiasis including the early postinfectious phase, the paucisymptomatic intermediate stage and advanced chronic stages. To date, there is no consensus regarding the treatment of neuroschistosomiasis, but there are some oral options and, in some cases, the surgical approach.

Schistosoma mansoni granulomas in the skeletal striated muscles in the murine model of neuroschistosomiasis: histological findings

Schistosomiasis mansoni presents many clinical manifestations during migration of schistosomes in their hosts, including diarrhea, hepatomegaly, splenomegaly, liver abscesses, skinlesions, brain tumors and myeloradiculopathy. No lesions have been reported in skeletal striated muscles due to schistosomiasis mansoni in the literature. This short communication reports the histopathological findings on skeletal musculature in a murine model of neuroeschistosomiasis mansoni. Lesions were found in the tongue, masseter muscle, buccinator muscle, digastric muscle and temporalis muscle. Worm recovery was carried out to confirm the infection. We describe here, for the first time in the literature, injuries in the skeletal musculature due to Schistosoma mansoni nfection.

Schistosomal myeloradiculopathy in a non-endemic area / Mielorradiculopatia esquistossomótica em região não endêmica

ABSTRACT Objective: To report a schistosomal myeloradiculopathy case in a non-endemic area. Case description: A previously healthy 11-year-old boy, stricken by an acute loss of strength on his lower limbs, followed by a loss of strength on his upper limbs and upper body, associated with altered sensitivity of the vesical globe formation. The patient's cerebrospinal fluid analysis showed eosinophilic meningitis, in addition to peripheral eosinophilia. The investigation resulted in a positive serology for Schistosoma mansoni. The treatment included steroids and praziquantel 60mg/kg, with a new dose after a month, as well as physical therapy for rehabilitation. The patient evolved with clinical improvement in the neurological exam, with a medullary section initially at C6, but now at T6. The patient is kept at prednisolone use (30mg/day) and longterm urinary catheter dependence. Comments: The schistosomiasis is endemic in many regions of Brazil; however, it has low incidence in the south of the country. Among its main manifestations, the schistosomal myeloradiculopathy is the most severe ectopic form of the disease, and should be suspected in patients with low back pain, strength and/or sensibility disorder of the lower limbs or urinary tract's disturbance. Early diagnosis and treatment should be done in order to reduce severe neurological sequelae. Treatment includes schistosomiasis drugs, corticosteroids and/or surgery.

RESUMO Objetivo: Relatar um caso de mielorradiculopatia esquistossomótica em área não endêmica. Descrição do caso: Paciente do sexo masculino, 11 anos, previamente hígido, com história aguda de paresia de membros inferiores, que evoluiu para membros superiores e tronco, associada à alteração de sensibilidade e formação de globo vesical. O exame do líquor demonstrava meningite eosinofílica, além de eosinofilia periférica. A investigação resultou em sorologia positiva para Schistosoma mansoni. O tratamento foi realizado com corticoterapia e praziquantel 60 mg/kg, com nova dose após um mês, além de fisioterapia para reabilitação. Evoluiu com melhora clínica no exame neurológico, com nível de secção medular que inicialmente correspondia a C6, encontrando-se atualmente em T6. Mantém uso de prednisolona 30 mg/dia e dependência de sonda vesical de demora. Comentários: A esquistossomose é uma doença endêmica em muitas regiões do Brasil, porém com pouca incidência no Sul do país. Dentre as principais manifestações, a mielorradiculopatia esquistossomótica é a forma ectópica mais grave e deve ser suspeitada na vigência de dor lombar, alteração de força e/ ou sensibilidade de membros inferiores e distúrbio urinário. O diagnóstico e o tratamento devem ser instituídos precocemente para diminuir o risco de sequelas neurológicas graves. O tratamento pode ser realizado com esquistossomicidas, corticosteroides e/ ou cirurgia.

Prevalence of urodynamic changes with risk for upper urinary tract damage in neuroschistosomiasis patients

Abstract INTRODUCTION: This study aimed to identify the prevalence of urodynamic changes with an associated risk of developing upper urinary tract damage in neuroschistosomiasis patients. METHODS: A prospective study was conducted, wherein68 patients were admitted for analysis of urodynamics, urea and creatinine levels, and uroculture. RESULTS: Blood test results did not indicate kidney failure. There were cases of asymptomatic bacteriuria. Common symptoms were frequent nocturia and detrusor overactivity. Results of low compliance and low cystometric capacity were both statistically significant (p = 0.001 and p = 0.002, respectively). CONCLUSIONS: A high prevalence of negative urodynamic changes were found in neuroschistosomiasis patients.

Characterisation of ocular involvement in an experimental model of neuroschistosomiasis mansoni

The Global Burden of Disease Study 2010 listed schistosomiasis among the leading 100 causes of death in Brazil, responsible for 3.6% of the estimated total of deaths globally. Eye and adnexa are very rarely affected by schistosomiasis mansoni, with limited documentation of ocular pathology in this setting. This short communication reports ocular histolopathological findings in a murine model of neuroschistosomiasis mansoni. Lesions were found in the bulbar conjunctiva, lacrimal gland, choroid and corneoscleral limbus.

Schistosomal Cervical Myelopathy

Schistosomiasis is an infectious disease caused by trematode platyhelminths of the genus Schistosoma. The involvement of the cervical spinal cord is rare, with few cases reported in the literature. The management of such patients is particularly challenging, since clinical and radiological findings may be confounded with other inflammatory diseases and/ or spinal cord tumors. We describe a 20-year old male with a history of swimming outdoors. He first presented pain in the back of the neck extending to shoulders and upper limbs paresis associated with four limbs hyperreflexia. The magnetic resonance imaging (MRI) showed a hypointense T1-weighted lesion in the cervical spinal cord, which was hyperintense on T2 images. The serologic testing was negative for schistosomiasis. A cervical cord biopsy at the C5-C6 level showed Schistosoma eggs in the histopathological examination. The treatment was performed using a single dose of praziquantel 50 mg/kg, with prednisone 40 mg/day for 3 weeks. On the follow-up, 1 year later, the patient presented mild reduction of the vibratory sensitivity in the distal third of both legs. Our illustrative case strengthens that, in endemic regions, Schistosoma mansoni infestation should be included in the differential diagnosis of intramedullary expansive lesions.

A esquistossomose é uma doença infecciosa causada por platelmintos trematódeos do gênero Schistosoma. O acometimento da medula espinhal cervical é raro, com poucos casos apresentados na literatura. O manejo desses pacientes é particularmente difícil, uma vez que os achados clínicos e radiológicos podem ser confundidos com outras doenças inflamatórias e/ou tumores da medula espinhal. Descrevemos um homem de 20 anos de idade com história de natação ao ar livre. Primeiramente, ele apresentou cervicalgia que se estendeu até os ombros e paresia dos membros superiores, associada à hiperreflexia de quatro membros. A ressonância magnética (RM) mostrou lesão hipointensa em T1 na medula espinhal cervical, a qual foi hiperintensa nas imagens em T2. O teste sorológico foi negativo para esquistossomose. Uma biópsia da medula cervical ao nível C5-C6 evidenciou ovos de Schistosoma no exame histopatológico. O tratamento foi realizado com dose única de praziquantel 50 mg/ kg, com prednisona 40 mg/dia por 3 semanas. No seguimento de 1 ano, o paciente apresentou discreta redução da sensibilidade vibratória no terço distal de ambas as pernas. Nosso caso ilustrativo reforça que, em regiões endêmicas, a infestação pelo Schistosoma mansoni deve ser incluída no diagnóstico diferencial de lesões expansivas intramedulares.

Brain schistosomiasis in mice experimentally infected with Schistosoma mansoni

Introduction Human neuroschistosomiasis has been reported in the literature, but the possibility of modeling neuroschistosomiasis in mice is controversial. Methods In two research laboratories in Brazil that maintain the Schistosoma mansoni life cycle in rodents, two mice developed signs of brain disease (hemiplegia and spinning), and both were autopsied. Results S. mansoni eggs, both with and without granuloma formation, were observed in the brain and meninges of both mice by optical microscopy. Conclusions This is the first description of eggs in the brains of symptomatic mice that were experimentally infected with S. mansoni. An investigation of experimental neuroschistosomiasis is now feasible. .

Extensive spinal cord involvement in magnetic resonance imaging evaluation on schistosomal myelitis / Envolvimento difuso da medula espinhal na avaliação pela ressonância magnética da mielite esquistossomótica

The diagnosis of schistosomal myelitis (SM) is frequently presumptive because no findings from any complementary examination are pathognomonic for this disease. The present report describes some abnormalities seen on magnetic resonance imaging (MRI) evaluation of a series of SM patients and discusses their etiopathogenesis. Methods: This study evaluated SM patients at the time of their diagnosis. These patients routinely underwent MRI on all segments of the spinal cord. Results: Thirteen patients were evaluated. The MRI was abnormal in 12 (92.3 percent) of them. In 11 patients (84.61 percent), the damage reached two or more spinal segments. Conclusions: MRI was an important diagnostic aid in this sample, because of the high rate of abnormalities detected. The tissue damage observed on MRI was extensive in the majority of the patients.

O diagnóstico da mielite esquistossomótica é frequentemente realizado por presunção, não havendo achado de exame complementar que seja patognomônico à condição. O presente estudo descreve alterações presentes na avaliação desses pacientes pela técnica da ressonância magnética e discute sua etiopatogênese. Métodos: O estudo avaliou pacientes com mielite esquistossomótica no momento do diagnóstico, os quais foram submetidos, rotineiramente, à ressonância magnética de todos os segmentos medulares. Resultados: Foram avaliados 13 pacientes, sendo a ressonância magnética alterada em 92.3 por cento dos casos. Em 11 pacientes (84.61 por cento), o dano abrangeu dois ou mais segmentos espinais. Conclusões: A ressonância magnética espinhal foi um importante auxílio diagnóstico nessa casuística em virtude da alta taxa de alterações detectadas. O dano tecidual observado foi extenso na maioria dos pacientes.

Diffuse pseudotumoral cerebral Schistosomiasis mansoni: a new form of presentation / Forma pseudotumoral difusa da esquistossomose cerebral: uma nova forma de apresentação

We describe two cases of cerebral schistosomiasis mansoni with multiple pseudotumoral lesions diagnosed by stereotactic brain biopsy. Both patients presented with seizures and one with left visual impairment. Imaging techniques revealed multiple brain lesions involving cerebral parenchyma, pons, cerebellum and thalamus. Brain histopathologic specimens of the patients showed multiple schistosomal granulomas in distinct evolutive phases. All patients presented good clinical response to treatment and reversion of the brain lesions. This new form of neuroschistosomiasis must be considered by those who work in the endemic area for Schistosoma mansoni.

São descritos dois casos de esquistossomose mansônica cerebral com lesões pseudotumorais múltiplas diagnosticadas por biópsia estereotáxica. Ambos os pacientes apresentaram-se com crises epilépticas e um deles com distúrbio visual. Estudos de neuroimagem revelaram múltiplas lesões cerebrais envolvendo parênquima cerebral, ponte, cerebelo e tálamo. Espécimes histopatológicos cerebrais dos pacientes demonstraram múltiplos granulomas esquistossomóticos em distintas fases evolutivas. Ambos os pacientes apresentaram boa resposta clínica ao tratamento e reversão das lesões cerebrais. Essa nova forma de euroesquistossomose deve ser considerada por aqueles que trabalham em área endêmica para Schistosoma mansoni.

Radicular dysfunction preponderance at early phase clinical evaluation in myelitis by Schistosoma mansoni / Preponderância de disfunções radiculares na avaliação clínica precoce da mielite pelo Schistosoma mansoni

In neuroschistosomiasis, the spinal cord is the most common place of the disease. In high prevalent areas for schistosomiasis mansoni, the clinical alertness is important for an early diagnostic, in order to decrease the final neurological damage. This study provides some useful neurologic information about a series of patients with schistosomal myelitis. METHOD: The sample consisted of 13 schistosomiasis mansoni carriers examined at the moment of the diagnosis of myelitis. RESULTS: The classical triad (lumbago, weakness at the lower limbs and urinary dysfunctions) was documented in 11 (86.61 percent) patients. The distribution of the clinical forms was: myeloradicular in six patients (46.15 percent), radicular in four (30.76 percent) and myelitic in three (23.07 percent). CONCLUSION: The radicular dysfunction and their clinical associated forms were the most prominent pattern during the early phase of this disease.

Na neuroesquistossomose, a medula espinhal é o sítio de predileção da doença. Em áreas de alta prevalência para esquistossomose mansoni, o conhecimento clínico dessa condição é importante para o seu diagnóstico precoce e consequente redução da lesão neurológica definitiva. Este estudo provê informações neurológicas relevantes pertinentes a uma série clínica de pacientes com mielite esquistossomótica. MÉTODO: A amostra consistiu de 13 pacientes portadores de esquistossomose mansoni examinados no momento do diagnóstico de sua forma mielítica. RESULTADOS: A tríade clássica (lombalgia, fraqueza nos membros inferiores e disfunções urinárias) foi documentada em 11 (86,61 por cento) pacientes. Quanto à distribuição das formas clínicas, se observou a ocorrência da mieloradicular em seis pacientes (46,15 por cento), da radicular em quatro (30,76 por cento) pacientes e da mielítica em três (23,07 por cento) pacientes. CONCLUSÃO: A disfunção radicular e suas formas clínicas associadas foram o padrão mais frequente durante a avaliação na fase precoce da doença.

Schistosomal myeloradiculopathy in a low-prevalence area: 27 cases (14 autochthonous) in Campinas, São Paulo, Brazil

Schistosomal myeloradiculopathy (SMR) is a form of schistosomiasis that is not linked with a high worm burden but rather is found in patients who have been sporadically exposed to Schistosoma mansoni. This paper aims to determine the occurrence of SMR in a low-endemic area with urban transmission in Campinas, São Paulo, Brazil. A retrospective study was performed, identifying confirmed cases in the two largest public hospitals on the region. Patients were diagnosed with SMR using standardised criteria, common clinical parameters, evidence of schistosomal infection and exclusion of other causes of myelopathy. A total of 27 patients were identified; 19 (85.2 percent) were men and four (14.8 percent) were women, ranging from 13-57 years of age (mean = 31.2; standard deviation = 12.8). Patients were classified as autochthonous (n = 14; 51.9 percent) or allochthonous (n = 11; 40.7 percent) and epidemiological data could not be obtained for two patients (7.4 percent). The clinical parameters of these patients were not different from previous studies. The sensitivity of serum immune reactions, cerebrospinal fluid immune reactions and parasitological stool examinations in identifying infected individuals was 87.5 percent, 93.8 percent and 40 percent, respectively. The epidemiological importance of these findings and their relationship with the control policies of schistosomiasis are discussed.

Acute schistosomiasis mansoni: revisited and reconsidered

Acute schistosomiasis is a systemic hypersensitivity reaction against the migrating schistosomula and eggs. A variety of clinical manifestations appear during the migration of schistosomes in humans: cercarial dermatitis, fever, pneumonia, diarrhoea, hepatomegaly, splenomegaly, skin lesions, liver abscesses, brain tumours and myeloradiculopathy. Hypereosinophilia is common and aids diagnosis. The disease has been overlooked, misdiagnosed, underestimated and underreported in endemic areas, but risk groups are well known, including military recruits, some religious congregations, rural tourists and people practicing recreational water sports. Serology may help in diagnosis, but the finding of necrotic-exudative granulomata in a liver biopsy specimen is pathognomonic. Differentials include malaria, tuberculosis, typhoid fever, kala-azar, prolonged Salmonella bacteraemia, lymphoma, toxocariasis, liver abscesses and fever of undetermined origin. For symptomatic hospitalised patients, treatment with steroids and schistosomicides is recommended. Treatment is curative in those timely diagnosed.

Clinical-epidemiologic profile of the schistosomal myeloradiculopathy in Pernambuco, Brazil

This was a retrospective descriptive study on a series of cases of schistosomal myeloradiculopathy (SMR) and the aim was to investigate the incidence of this disease and its clinical and epidemiological characteristics in cases diagnosed at three healthcare units in Pernambuco, Brazil between 1994-2006. The data were collected by reviewing the medical records from both the neurological and paediatric outpatient clinics and wards of the Hospital Clinics, Hospital of the Restoration and Pernambuco Mother and Child Institute. To gather the data, a spinal cord schistosomiasis evaluation protocol was used. The diagnoses were based on positive epidemiological evidence of schistosomiasis, clinical findings and laboratory tests (stool parasitological examination or rectal biopsies, magnetic resonance imaging findings and cerebrospinal fluid investigations). A total of 139 cases aged between 2-83 years were found. The most important determinants of SMR were male sex (66.2 percent), contact with fresh water (91 percent), origin in endemic regions (39.5 percent), lower-limb muscle weakness (100 percent), sensory level at the lower thoracic medulla (40.3 percent), myeloradicular form (76 percent) and presence of eggs in the stool parasitological examination (48 percent). This sample indicates the need for intervention policies guided by diagnostic standardization, thereby avoiding disease under-notification.

Portal Doppler ultrasound evaluation in myelitis by Schistosoma mansoni / Avaliação pela ultrassonografia com Doppler portal na mielite pelo Schistosoma mansoni

The mechanism for development of schistosomal myelitis has not yet been completely understood. Few publications have tried to establish a link between the general clinical presentation of the illness and its neurological manifestations. Objective: To evaluated the portal blood flow in patients with schistosomal myelitis and controls. Method: Forty-three individuals were evaluated using portal Doppler ultrasound. Group I consisted of 13 patients with the spinal form of schistosomiasis. Group II included 10 patients with intestinal and hepatointestinal forms of schistosomiasis, but no neurological symptoms; while group III involved 20 healthy controls. Results: Portal venous speed and portal venous flow were significantly elevated in group I (p<0.001) compared with the others. Conclusion: Doppler ultrasound demonstrated a situation of high flow within the portal venous system of patients with schistosomal myelitis.

A fisiopatogênese da mielopatia esquistossomótica não é completamente entendida, sendo escassas as publicações que tentam inferir a relação entre a forma clínica da esquistossomose e suas manifestações neurológicas. Objetivo: Avaliar o fluxo sanguíneo do sistema porta em pacientes com mielite esquistossomótica, contribuindo no entendimento dos mecanismos que favorecem a chegada dos parasitas e/ou seus derivados ao sistema nervoso. Método: Foram avaliados 43 indivíduos pela Dopplerfluxometria portal. O grupo I constou de 13 pacientes com esquistossomose medular, sendo o grupo II composto por 10 portadores de formas intestinal e hepatointestinal (sem doença neurológica), e o grupo III, por 20 controles sadios. Resultados: A velocidade venosa portal e o fluxo venoso portal foram significativamente elevados (p-valor<0,001) no grupo I. Conclusão: A Dopplerfluxometria demonstrou a presença de estados hiperdinâmicos do leito vascular porta em pacientes com mielite pelo Schistosoma mansoni.

Epidemiological aspects in neuroschistosomiasis / Aspectos epidemiologicos na neuroesquistossomose

The urbanization process of schistosomiasis has been described in the literature. The present report shows and discusses some epidemiological aspects of neuroschistosomiasis that emphasizes this trend. Method: The study was done between July, 2005 and June, 2006, and consisted of spinal cord schistosomiasis carriers at the moment of diagnosis. These patients were evaluated whatever the origin and period of the year that they developed the symptoms. Results: A total of 13 patients were evaluated. Ten cases (76.92 percent) came from the Metropolitan Region of Recife, while the other zones (Zona da Mata, Agreste e Sertão) contributed with just three cases (23.07 percent). October, November and December (post rainy period) concentrated the majority of the cases (61.54 percent). Conclusion: The high spatial and temporal concentration of cases in relation to a recent endemic area for Schistosomiasis suggests that the immunological status may be an important factor for installation of Sschistosomal myelitis.

O processo de urbanização da esquistossomose vem sendo descrito na literatura. O presente estudo apresenta e discute alguns aspectos epidemiológicos na neuroesquistossomose que confirmam essa tendência. Método: O estudo foi realizado entre julho de 2005 e junho de 2006, sendo composto por portadores de mielite esquistossomótica no momento do diagnóstico. Os pacientes foram avaliados quanto à procedência e ao período do ano que desenvolveram a doença. Resultados: No total foram 13 pacientes avaliados, sendo 10 casos (76,92 por cento) provenientes da Região Metropolitana do Recife, enquanto as demais regiões (Zona da Mata, Agreste e Sertão) contribuíram com apenas um caso cada. Os meses de outubro, novembro e dezembro concentraram a maioria dos pacientes (61,54 por cento). Conclusão: A alta concentração espacial e temporal de casos em relação a uma área endêmica recente para esquistossomose sugere que a participação de fatores imunológicos possa ser importante para o desenvolvimento de sua forma medular.

Simultaneous occurrence of brain tumor and myeloradiculopathy in schistosomiasis mansoni: case report / Ocorrência simultânea de tumor cerebral e mielorradiculopatia na esquistossomose mansoni: relato de caso

Simultaneous occurrence of brain tumor and myeloradiculopathy in cases of Manson's schistosomiasis have only rarely been described. We report the case of a 38-year-old man who developed seizures during a trip to Puerto Rico and in whom a brain tumor was diagnosed by magnetic resonance imaging: brain biopsy revealed the diagnosis of schistosomiasis. He was transferred to a hospital in the United States and, during hospitalization, he developed sudden paraplegia. The diagnosis of myeloradiculopathy was confirmed at that time. He was administered praziquantel and steroids. The brain tumor disappeared, but the patient was left with paraplegia and fecal and urinary dysfunction. He has now been followed up in Brazil for one year, and his clinical state, imaging examinations and laboratory tests are presented here.

Tem sido descrita, raramente, na esquistossomose mansônica, a ocorrência simultânea de tumor cerebral e mielorradiculopatia. Relatamos aqui o caso de um homem de 38 anos que desenvolveu convulsões, durante viagem a Porto Rico, e um tumor cerebral foi diagnosticado à ressonância magnética: a biópsia do cérebro revelou o diagnóstico de esquistossomose. Ele foi transferido para hospital na América do Norte e durante a hospitalização desenvolveu súbita paraplegia. O diagnóstico de mielorradiculopatia foi confirmado na ocasião. Ele recebeu praziquantel e esteróides. O tumor cerebral desapareceu, mas o paciente permaneceu com paraplegia, disfunção urinária e fecal. Ele tem sido acompanhado no Brasil no último ano e o seu estado clínico, os métodos de imagem e os exames de laboratório são apresentados aqui.

Diagnóstico intraoperatório de esquistossomose mansônica cerebelar pseudotumoral / Intraoperative diagnosis of pseudotumoral cerebellar neuroschistosomiasis

A esquistossomose é uma doença parasitária causada por vermes trematódios do gênero Schistosoma. Esses vermes habitam ramos da veia mesentérica inferior e, por meio da ovoposição, disseminam seus ovos por várias regiões do organismo humano. O sistema nervoso central (SNC) pode ser afetado no decorrer de qualquer forma clínica da infecção, principalmente pela embolização dos ovos ocorrida após a migração anômala dos vermes adultos para locais próximos ao tecido cerebral. No SNC, os ovos induzem reação granulomatosa periovular intensa, respondendo pela produção do efeito de massa com sinais neurológicos de aumento de pressão intracraniana. É relatado o caso de um homem de 27 anos cujo diagnóstico de esquistossomose mansônica cerebelar foi obtido pelo método de esmagamento linear (squash), durante o ato cirúrgico. Uma biópsia retal realizada posteriormente confirmou a presença de ovos viáveis de S. mansoni. No conhecimento dos autores, este é o primeiro relato de neuroesquistossomose diagnosticado por esta técnica.

Schistosomiasis is a parasitic infection caused by trematode platyhelminths of the genus Schistosoma. These worms live in branches of the inferior mesenteric vein and disseminate its eggs to several regions of the human organism through circulation. Central nervous system (CNS) involvement may occur in the development of any clinical infection, mainly by in-situ egg deposition following anomalous migration of adult worms to sites near the cerebral tissue. The presence of eggs in the CNS induces a severe periovular granulomatous reaction, which is responsible for the mass effect and the increased intracranial pressure. A case of cerebellar schistosomiasis caused by S. mansoni in a 27-year-old man is diagnosed by squash (smear) technique. A rectal biopsy performed later showed viable S. mansoni eggs. This is, probably, the first report of neuroschistosomiasis diagnosed by the squash (smear) technique.

Guidelines for the diagnosis and treatment of schistosomal myeloradiculopathy

Schistosomal myeloradiculopathy is the most severe and disabling ectopic form of Schistosoma mansoni infection. The prevalence of SMR in centres in Brazil and Africa that specialise in attending patients with non traumatic myelopathy is around 5 percent. The initial signs and symptoms of the disease include lumbar and/or lower limb pain, paraparesis, urinary and intestinal dysfunctions, and impotence in men. The cerebrospinal fluid of SMR patients shows an increase in protein concentration and in the number of mononuclear cells in 90 percent of cases; eosinophils have been reported in 40 percent. The use of magnetic resonance imaging is particularly valuable in the diagnosis of Schistosomal myeloradiculopathy. The exclusion of other myelopathies and systemic diseases remains mandatory. Early diagnosis and treatment with steroids and schistosomicides provide a cure for most patients, whilst delayed treatment can result in irreversible physical disabilities or death. To improve awareness concerning Schistosomal myeloradiculopathy amongst public health professionals, and to facilitate the control of the disease, the Brazilian Ministry of Health has launched a program of education and control of this ectopic form of schistosomiasis. The present paper reviews current methods for the diagnosis of SMR and outlines protocols for treatment of the disease.

A mielorradiculopatia esquistossomótica é a forma ectópica mais grave da infecção pelo Schistosoma mansoni. A prevalência da mielorradiculopatia esquistossomótica em centros médicos no Brasil e em África, especializados no atendimento de pacientes com mielopatia, encontra-se em torno de 5 por cento. Os sintomas e sinais iniciais da doença incluem: dor lombar e/ou dor em membros inferiores, paraparesia, disfunções urinária e intestinal, e impotência no homem. A análise do líqüor destes pacientes revela aumento na concentração de proteínas e no número de células mononucleares em 90 por cento dos casos; a presença de eosinófilos foi documentada em 40 por cento. O uso rotineiro da ressonância magnética tornou-se obrigatório na definição diagnóstica. A exclusão de outras mielopatias e doenças sistêmicas é mandatória. O diagnóstico precoce e o tratamento com corticoesteróides e esquistossomicidas curam a maioria dos pacientes, enquanto o atraso em iniciar o tratamento resulta em seqüelas irreversíveis ou morte. Para melhorar a percepção da importância da mielorradiculopatia associada à esquistossomose, o Ministério da Saúde do Brasil lançou programa de controle dessa forma ectópica da esquistossomose. Nesta revisão, descrevem-se os métodos diagnósticos atuais para o diagnóstico e os protocolos para o tratamento da doença.

Pseudotumoral form of neuroschistosomiasis: report of three cases

Central nervous system (CSN) involvement in schistosomiasis is an ectopic manifestation with a large variety of clinical forms, including pseudotumoral, which occurs in isolated cases and is rare. Three patients with epidemiological indications of this pathology were examined; the clinical picture included lower-back pain irradiating to lower limbs, associated with progressive flaccid paraparesis and sphincterial disturbances in cases in which the spinal chord was involved; while in cases with encephalitic impairment, headache, dizziness and cerebellar syndrome, characterized by dysarthria and right-side dysgraphia, were present. Magnetic resonance imaging (MRI) showed a growing process in all cases; cerebrospinal fluid (CSF) characteristics and biological markers were compatible with neuroschistosomiasis (NS). Biopsy of the lesions confirmed this diagnosis in one case. After specific treatment with schistosomicides and corticosteroids, clinical, radiological and laboratorial improvement was observed.