Mansonic neuroschistosomiasis in the childhood: an undiagnosed pathology?

Schistosomiasis is an endemic parasitic disease in several tropical countries. In Brazil, the only prevalent species of parasite responsible for schistosomiasis is Schistosoma mansoni. Neuroschistosomiasis is the second most frequent form of infection and the primary ectopic manifestation, with predominant involvement of the lower thoracic spinal cord and lumbar and lumbosacral regions. The frequent contact of children with contaminated ponds and the immaturity of their immune systems make this age group especially susceptible to infection by this parasite. Therefore, neuroschistosomiasis mansoni should always be considered in cases of transverse myelitis in children from endemic regions. The treatment for this condition is quite simple and effective, resulting in total recovery of neurological deficits if the diagnosis is made early.

SCHISTOSOMAL MYELORADICULOPATHY IN A NON-ENDEMIC AREA.

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.

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 Myeloradiculopathy - A case report.

The most common neurological impairments related to schistosomiasis involve the lower portions of the medulla and the cauda equina. A 22-year-old woman, with no history, signs, or symptoms of hepatointestinal schistosomiasis, presented with lumbar pain associated with acute paresthesia and paresis of the right lower limb. Spinal schistosomiasis was suspected based on the disease progression and radiological findings, and the diagnosis was confirmed after cerebrospinal fluid analysis. The authors emphasize this pathology as important as a differential diagnosis in similar clinical scenarios, especially in endemic areas, because both early diagnosis and treatment are essential to avoid permanent sequelae.

Schistosomal myeloradiculopathy - A case report

Abstract The most common neurological impairments related to schistosomiasis involve the lower portions of the medulla and the cauda equina. A 22-year-old woman, with no history, signs, or symptoms of hepatointestinal schistosomiasis, presented with lumbar pain associated with acute paresthesia and paresis of the right lower limb. Spinal schistosomiasis was suspected based on the disease progression and radiological findings, and the diagnosis was confirmed after cerebrospinal fluid analysis. The authors emphasize this pathology as important as a differential diagnosis in similar clinical scenarios, especially in endemic areas, because both early diagnosis and treatment are essential to avoid permanent sequelae.

Vestibular Evoked Myogenic Potential (VEMP) Triggered by Galvanic Vestibular Stimulation (GVS): A Promising Tool to Assess Spinal Cord Function in Schistosomal Myeloradiculopathy.

BACKGROUND: Schistosomal myeloradiculopathy (SMR), the most severe and disabling ectopic form of Schistosoma mansoni infection, is caused by embolized ova eliciting local inflammation in the spinal cord and nerve roots. The treatment involves the use of praziquantel and long-term corticotherapy. The assessment of therapeutic response relies on neurological examination. Supplementary electrophysiological exams may improve prediction and monitoring of functional outcome. Vestibular evoked myogenic potential (VEMP) triggered by galvanic vestibular stimulation (GVS) is a simple, safe, low-cost and noninvasive electrophysiological technique that has been used to test the vestibulospinal tract in motor myelopathies. This paper reports the results of VEMP with GVS in patients with SMR. METHODS: A cross-sectional comparative study enrolled 22 patients with definite SMR and 22 healthy controls that were submitted to clinical, neurological examination and GVS. Galvanic stimulus was applied in the mastoid bones in a transcranial configuration for testing VEMP, which was recorded by electromyography (EMG) in the gastrocnemii muscles. The VEMP variables of interest were blindly measured by two independent examiners. They were the short-latency (SL) and the medium-latency (ML) components of the biphasic EMG wave. RESULTS: VEMP showed the components SL (p = 0.001) and ML (p<0.001) delayed in SMR compared to controls. The delay of SL (p = 0.010) and of ML (p = 0.020) was associated with gait dysfunction. CONCLUSION: VEMP triggered by GVS identified alterations in patients with SMR and provided additional functional information that justifies its use as a supplementary test in motor myelopathies.

Mansonic neuroschistosomiasis.

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.

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.

Diagnóstico diferencial das lesões inflamatórias e infecciosas do cone medular utilizando a ressonância magnética / Magnetic resonance imaging in the differential diagnosis of infectious and inflammatory conus medullaris lesions

The conus medullaris is frequently affected by inflammatory and infectious lesions which many times are hardly differentiated because of the similarity of their clinical history and physical examination among the different etiologies. Magnetic resonance imaging presents high sensitivity in the detection of these lesions and plays a relevant role in the diagnosis as well as in the evolutive control of the condition. The present pictorial essay with selected cases from the archives of the authors' institution is aimed at demonstrating imaging findings which might help in the diagnosis of a specific etiology amongst inflammatory an infectious conditions and in the differentiation with diseases of neoplastic and vascular etiologies, for example. Findings such as enhancement pattern, presence of cysts, edema and involvement of other regions of the central nervous system are important for this differentiation, and may define a specific etiology as associated with clinical and laboratory tests findings.

O cone medular é frequentemente acometido por lesões de etiologia inflamatória e infecciosa, muitas vezes de difícil diferenciação devido a história clínica e exame físico semelhantes entre as diversas entidades. A ressonância magnética apresenta alta sensibilidade na detecção de lesões no cone medular e tem importante papel no diagnóstico e controle evolutivo. Este ensaio iconográfico com casos selecionados dos arquivos do nosso serviço tem como objetivo demonstrar achados de imagem que possam auxiliar no diagnóstico de uma etiologia específica entre as doenças inflamatórias e infecciosas e na diferenciação com doenças de outras etiologias como neoplasias e causas vasculares. Características como padrão de realce, presença de cistos, edema, além do acometimento de outras regiões do sistema nervoso central são importantes para esta diferenciação, podendo definir uma etiologia específica quando associadas ao quadro clínico e laboratorial.

Neuroschistosomiasis mansoni: literature review and guidelines.

BACKGROUND: Schistosomiasis is a tropical disease caused by worms of the genus Schistosoma. It is endemic in the Caribbean Islands, the middle east, eastern Asia, South America, and Africa. In nonendemic areas, physicians should be aware of this condition in travelers returning from endemic areas and in immigrants. The main disease-causing species are Schistosoma haematobium, Schistosoma mansoni, and Schistosoma japonicum. Neuroschistosomiasis is an ectopic form of the disease that is mainly associated with S. japonicum infection. Involvement of the central nervous system (CNS) in S. mansoni infection is neglected and underestimated. Neuroschistosomiasis mansoni can be classified into cerebral, spinal, and encephalomyelitic forms in the course of an acute or chronic infection. REVIEW SUMMARY: We review the CNS involvement by S. mansoni infection with an emphasis on life cycle, epidemiology, pathophysiology and immunology, clinical manifestations, diagnostic criteria, differential diagnosis, current treatment guidelines, and prognosis. CONCLUSIONS: Although an underreported CNS infection, found mainly in underdeveloped countries, neuroschistosomiasis mansoni still causes significant incapacity and morbidity. Hence, neurologists should become familiar with this infection worldwide and include it in the differential diagnosis of CNS involvement in travelers returning from endemic areas and in immigrants.

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.

Neuroschistosomiasis: clinical symptoms and pathogenesis.

Neuroschistosomiasis, referring to schistosomal involvement of the CNS, when symptomatic, is a severe disorder in which prognosis depends largely on early diagnosis and treatment. It is an underdiagnosed disorder, but has been increasingly reported in populations in endemic areas and in tourists. CNS involvement can occur at any time during schistosomal infection. Both the brain and the spinal cord can be affected. Schistosoma mansoni and Schistosoma haematobium usually cause myelopathy, whereas Schistosoma japonicum usually causes encephalic disease. There are substantial differences in the pathogenesis, clinical presentation, and outcome of the neurological disorder, depending on the phase and clinical form of schistosomiasis in which it occurs.

Neuroesquistossomose: estudo do perfil clínico-patológico e critérios diagnósticos / Neuroschistosomiasis: profile study of pathological and clinical diagnostic criteria

A presente revisão tem por objetivo realizar um estudo da forma ectópica da esquistossomose, enfatizando o acometimento do sistema nervoso pelo S. mansoni. A neuroesquistossomose é uma doença considerada rara, embora seja a segunda forma mais comum de apresentação da doença. Considerando as formas sintomáticas da neuroesquistossomose relacionada com o S. mansoni, a medula espinhal é afetada com maior frequência do que o cérebro. A apresentação neurológica da neuroesquistossomose é variável e não existe uma manifestação típica para orientar o diagnóstico, podendo ser confundido com o de outras etiologias. Considerando que o tratamento precoce dessa doença é fundamental para se evitar sequelas e o uso indiscriminado de medicamentos pelo paciente, é necessário um diagnóstico seguro e preciso.

This review aims to conduct a study of ectopic form of schistosomiasis with emphasis on the nervous system by S. mansoni. Neuroschistosomiasis The disease is considered rare, although it is the second most common form of disease presentation. Considering the symptomatic forms of neuroschistosomiasis related to S. mansoni the spinal cord is affected more frequently than the brain. The neurological presentation of neuroschistosomiasis is variable and there is a typical manifestation to guide diagnosis and may be confused with other causes. Whereas the early treatment of this disease is critical to prevent sequelae and indiscriminate use of drugs to the patient, you need a safe and accurate diagnosis.

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%) were men and four (14.8%) 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%) or allochthonous (n = 11; 40.7%) and epidemiological data could not be obtained for two patients (7.4%). 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%, 93.8% and 40%, respectively. The epidemiological importance of these findings and their relationship with the control policies of schistosomiasis are discussed.

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%), contact with fresh water (91%), origin in endemic regions (39.5%), lower-limb muscle weakness (100%), sensory level at the lower thoracic medulla (40.3%), myeloradicular form (76%) and presence of eggs in the stool parasitological examination (48%). This sample indicates the need for intervention policies guided by diagnostic standardization, thereby avoiding disease under-notification.

A laboratory test for the diagnosis of neuroschistosomiasis.

OBJECTIVE: To provide a general description of the studies on spinal cord schistosomiasis that our team has developed in Brazil, with an emphasis on how we have planned the investigations and managed the problems. METHODS: Schistosoma mansoni is endemic in extensive areas in Brazil. We review the subject and discuss the design, development and initial evaluation of a diagnostic cerebrospinal fluid enzyme-linked immunoassay test for spinal cord schistosomiasis. RESULTS: Since the late 1980s, several clinical investigations have led to a better understanding of the natural history of this disorder and an improved characterization of its clinical presentations. Spinal cord schistosomiasis is a severe condition that typically presents as an acute/subacute low cord syndrome generally associated with the involvement of the cauda equina roots. Cerebrospinal fluid examination usually shows an inflammatory pattern, and MRI demonstrates findings of an inflammatory spinal cord lesion. Its diagnosis is largely based on clinical evidence. There is no definitive consensus on the treatment of neuroschistosomiasis, but most authors recommend the administration of praziquantel and a steroid. The outcome is variable and chiefly dependent on early treatment. The results of the cerebrospinal fluid enzyme-linked immunoassay test for schistosomal myeloradiculopathy allow the confirmation or exclusion of this disorder in a large proportion of suspected cases. DISCUSSION: Although knowledge on schistosomiasis has improved during the last two decades, several aspects of this disorder are still unknown and deserve investigation. The enzyme-linked immunoassay test in cerebrospinal fluid showed promising results, but needs validation in a new sample of subjects.