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.

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.

Mansonic neuroschistosomiasis / Neuroesquistossome mansonica

Mansonic neuroschistosomiasis (MN) is not only the most common but also the most serious ectopic presentation of the infection by Schistosoma mansoni. Both, brain and spinal cord can be independently affected by the infection, but the later is more frequently affected. Brain MN by itself is due to the presence of eggs and/or adult worms in situ and can be symptomatic or asymptomatic. Unlike the brain MN, spinal cord mansonic neuroschistosomiasis is more frequently symptomatic. In both forms the intensity, the seriousness and also the clinical characteristics of signs and symptoms depend on the amount of eggs in the compromised region and on the intensity of the inflammatory reaction surrounding the eggs. Cerebrospinal fluid examination and magnetic resonance imaging are important diagnostic tools. Both corticosteroids and drugs against S. mansoni are used in the treatment. The outcome may largely depend upon the prompt use of these drugs.

A neuroesquistossome mansônica (NM) é não apenas a mais comum, mas também a mais grave apresentação da infecção pelo Schistosoma mansoni. Tanto o encéfalo quanto a medula podem ser independentemente afetadas pela doença, embora a última o seja de forma mais frequente. A NM encefálica é secundária à presença dos ovos e/ou da forma adulta do verme in situ, e pode ser sintomática ou não. Ao contrário da forma encefálica, a NM medular é mais frequentemente sintomática. Em ambas as formas a gravidade dos sintomas dependerá na quantidade de ovos na região comprometida e na intensidade da reação inflamatória ao seu redor. Os exames do líquido cefalorraquiano e de imagem por ressonância magnética são importantes ferramentas diagnósticas. Corticosteróides e drogas parasiticidas são usadas no tratamento desta doença, e seu prognóstico dependerá diretamente do rápido uso destas drogas.

Schistosomiasis of the spinal cord: report of 5 cases from Sudan

Schistosomiasis of the spinal cord is an uncommon but potentially curable form of schistosomiasis, if diagnosed and managed early. The spinal cord is more frequently affected in Schistosomo mansoni or S. hoemotobium infections. This paper describes the clinical manifestations, diagnosis and management of schistosomiasis of the spinal cord in 5 patients attending Shaab and Ibn Khuldoun Hospitals, Khartoum from 1997 to 2007. There were 4 males and 1 female aged 9-45 years. They presented with symptoms and signs due to cord compression at the lower thoracic and lumbar vertebrae. Imaging studies revealed intramedullary masses compressing the cord. Biopsy showed ova of 5. mansoni with surrounding inflammatory reaction. The cord showed demyelination nearthe ova and an associated inflammatory reaction. Patients responded well to surgical decompression and treatment with praziquantel and oral steroids

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.

Clinical-epidemiological profile of children with schistosomal myeloradiculopathy attended at the Instituto Materno-Infantil de Pernambuco

The most critical phase of exposure to schistosomal infection is the infancy, because of the more frequent contact with contaminated water and the immaturity of the immune system. One of the most severe presentations of this parasitosis is the involvement of the spinal cord, which prognosis is largely dependent on early diagnosis and treatment. Reports on this clinical form of schistosomiasis in children are rare in the literature. We present here the clinical-epidemiological profile of schistosomal myeloradiculopathy (SMR) from ten children who were admitted at the Instituto Materno-Infantil de Pernambuco over a five-year period. They were evaluated according to an investigation protocol. Most of these patients presented an acute neurological picture which included as the main clinical manifestations: sphincteral disorders, low back and lower limbs pain, paresthesia, lower limbs muscle weakness and absence of deep tendon reflex, and impairment of the gait. The diagnosis was presumptive in the majority of the cases. This study emphasizes the importance of considering the diagnosis of SMR in pediatric patients coming from endemic areas who present a low cord syndrome, in order to start the appropriate therapy and avoid future complications.

Schistosomal myeloradiculopathy due to Schistosoma mansoni: report on 23 cases

Schistosoma mansoni infection is likely to be responsible for a significant proportion of cases of myelopathy occurring in areas where schistosomiasis is endemic. The aim of this study is to describe the clinical, laboratory and therapeutic data of 23 patients with schistosomal myeloradiculopathy. The medical records of 23 patients with schistosomal myelopathy admitted to two general hospitals of Belo Horizonte (MG), in Brazil, from 1995 to 1999, were reviewed retrospectively. Seventeen patients were male (74 percent). The mean age for the whole group was 27 years. Lower limb weakness and associated lumbar and/or lower limb pain were reported by 20 patients (87 percent), and 16 (70 percent) were unable to walk. All individuals presented urinary retention and 19 (83 percent) complained of intestinal dysfunction. The treatment was based on the association of antischistosomal drugs and corticosteroids. Five patients (22 percent) presented a full response to treatment, 13 (57 percent) partial response without functional limitations and 4 (17 percent) partial improvement with limitations or no response. Three out of the 4 patients who stopped steroids before 45 days of treatment developed recurrence of the symptoms and signs of myelopathy. Our cases demonstrate the severe presentation of the disease and the data disclosed here suggest that treatment with steroids should be kept for months after clinical improvement

Esquistossomose do sistema nervoso central / Schistosomiasis mansonic case report of the central nervous system

A esquistossomose do Sistema Nervoso Central é relativamente pouco diagnosticada. Relatamos o caso de um paciente procedente de área näo endêmica, com manifestaçöes meningorradiculares da esquistosomose mansônica, comprovada por reaçäo de imunofluorescência no líquido cefalorraquidiano, tratado com praziquantel associado à corticoterapia. Ressaltamos a necessidade do diagnóstico etiológico específico e a instituiçäo precoce do tratamento com drogas antiinflamatórias e drogas específicas para a melhor recuperaçäo do paciente.

Spinal cord schistosomiasis / Neuroesquistossomose medular

The authors present a case of spinal cord schistosomiasis which simulated a tumoral lesion determining spastic paraparesis with a sensitive-motor level at T1. The patient was treated with surgery, praziquantel and dexamethasone, with ?restitutio ad integrum?. Seven years later the patient presented recrudescence of the symptoms, without a good response to the medication and without a plausible explanation. The authors wonder about the reasons of the low incidence of central nervous system schistosomosis in an endemic area such as the Northeast of Brazil. Abrief review of the literature is done.

Os autores apresentam um caso de esquistossomose medular simulandoprocesso tumoral, manisfestando-se com paraparesia espástica e nívelsensitivo em T1. O tratamento cirúrgico, complementado pela administraçãode praziquantel e dexametasona, proporcionou recuperação total do quadro neurológico. Após sete anos, houve recidiva dos sintomas, de causa não totalmente esclarecida e sem resposta ao tratamento medicamentoso. Chamam atenção à baixa incidência de neuroesquistossomose em regiões endêmicas, como o Nordeste do Brasil.