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.

Medullary neuroschistosomiasis in adolescence: case report and literature review.

INTRODUCTION: Medullary neuroschistosomiasis is a severe complication of gastrointestinal infection by Schistosoma. There are several endemic areas, wherein the only causative species present is Schistosoma mansoni, which is responsible for the clinical manifestations of all cases in those areas. METHODS: We report the case of a 13-year-old female with lumbar pain and progressive lower limb weakness, with a delayed diagnosis of medullary involvement by the parasite. We also reviewed the literature on the disease. CONCLUSIONS: Although it is related to the less severe forms of schistosomiasis, one should pay attention to the diagnosis of neuroschistosomiasis in cases of transverse myelitis in patients who traveled to endemic areas. The delay in diagnosis and, consequently, the introduction of treatment may result in irreversible neurological sequelae.

Cerebral schistosomiasis: MRI features with pathological correlation.

BACKGROUND: Neuroschistosomiasis is not commonly encountered and is probably underrecognized. We hope these findings can help clinicians and radiologists to raise awareness of this disabling disorder. PURPOSE: To demonstrate the magnetic resonance imaging (MRI) findings of cerebral schistosomiasis and correlate it with pathological findings. MATERIAL AND METHODS: We identified seven patients with cerebral schistosomiasis from radiology and pathology archives of our hospital. Of the seven patients, six were pathologically confirmed. The remaining patient had pathologically confirmed spinal schistosomiasis with MRI findings of cerebral involvement. MRI and pathological findings of these patients were analyzed and correlated. RESULTS: Multiple enhancing nodules at varying size were found in all patients. Prominent leptomeningeal or choroidal veins were found in six patients, four at the center of the foci and two at the periphery. Hemorrhage was identified in two patients. Histology revealed granulomas around ova in six patients. A prominent vein with ova in the lumen and wall of a vein was found in one patient and perivascular ova deposition was found in one patient. CONCLUSION: Multiple enhancing nodules with central or peripheral veins in a circumscribed brain area are important signs to the diagnosis of cerebral schistosomiasis. The inflamed veins involved may be the route taken by the ova to spread to the brain tissue.

Spinal neuroschistosomiasis caused by Schistoma mansoni: cases reported in two brothers.

BACKGROUND: Spinal neuroschistosomiasis (SN) is one of the most severe clinical presentations of schistosomiasis infection and an ectopic form of the disease caused by any species of Schistosoma. In Brazil, all cases of this clinical manifestation are related to Schistosoma mansoni, the only species present in the country. Although many cases have been reported in various endemic areas in Brazil, this is the first time in the literature that SN is described in two brothers. CASE PRESENTATION: Two cases of SN were accidentally diagnosed during an epidemiological survey in an urban area endemic for schistosomiasis transmission. Both patients complained of low back pain and muscle weakness in the lower limbs. Sphincter dysfunction and various degrees of paresthesia were also reported. The patients' disease was classified as hepato-intestinal stage schistosomiasis mansoni at the onset of the chronic form. A positive parasitological stool test for S. mansoni, clinical evidence of myeloradicular damage and exclusion of other causes of damage were the basic criteria for diagnosis. After treatment with praziquantel and corticosteroid, the patients presented an improvement in symptoms, although some complaints persisted. CONCLUSIONS: It is important to consider SN when patients come from areas endemic for transmission of schistosomiasis mansoni. Clinical physicians and neurologists should consider this diagnostic hypothesis, because recovery from neurological injuries is directly related to early treatment. As, described here in two brothers, a genetic predisposition may be related to neurological involvement. Primary care physicians should thus try to evaluate family members and close relatives in order to arrive at prompt schistosomiasis diagnosis in asymptomatic individuals and propose treatment in an attempt to avoid progression to SN.

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.

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.

[Radiculomyelopathy in schistosomiasis]. / Radikulomyelopathie bei Schistosomiasis.

The increasing migration of people from their homeland in far distant regions to Europe in the last few years has strongly influenced the rise of previously rarely seen diseases. They not only originate from the respective homeland but also from the transit countries during the migration process. We report the case of a 27-year-old male migrant from Eritrea, who after months of flight as a refugee travelling through various African countries, presented at our hospital with a progressive, painful radiculopathy. Whole spine magnetic resonance imaging (MRI) showed a focus located in the myelon, extending from T11 to the medullary conus. The differential diagnostic clarification ultimately revealed an infection with Schistosoma mansoni. After guideline-conform treatment with praziquantel for 3 days and additional administration of corticosteroids for 3 months, a slow regression of the findings and improvement of the symptoms could be shown clinically and by MRI. This case study shows the importance of taking the medical history and that a closer look at the potential exposure in the homeland and transit countries should be of great benefit in reaching the diagnosis, especially in patients with a migration background.

Immunological Profile in Individuals with Schistosomal Myeloradiculopathy.

BACKGROUND: Schistosomal myeloradiculopathy (SMR) is the most serious ectopic presentation of Schistosoma mansoni infection. The pathogenesis occurs mainly via the host inflammatory response to the eggs of the parasite that are stuck in the central nervous system, and the diagnosis is generally made by the exclusion of other neurological diseases. OBJECTIVE: We aimed to evaluate the immune status of SMR patients and to identify a marker for SMR diagnosis. METHODS: We enrolled 15 patients with a presumptive diagnosis of SMR, and the control groups included 17 patients with myelopathy associated with human T cell lymphotropic virus type 1 (HTLV-1) and 11 with other neurological disorders. The determination of soluble egg antigen-specific IgE and the levels of cytokines from Th1, Th2, Th17 and T-regulatory cell profiles and the chemokines MIP-1a and RANTES were measured in the cerebrospinal fluid (CSF) and serum using an ELISA technique. RESULTS: We observed that SMR leads to an increase in IgE levels in the CSF compared to serum, and the levels of IL-13 and MIP-1α were significantly higher in the CSF and serum of the SMR patients than in the patients with HTLV-1-associated myelopathy. The levels of MIP-1α and RANTES were higher in the CSF than in the serum of the SMR group. The ratio between levels of IL-13, MIP-1α and RANTES over IL-10 was positive in the CSF of the SMR patients. CONCLUSIONS: These results indicate that S. mansoni-specific IgE in the CSF is a promising marker for the diagnosis of SMR and that the cytokines and chemokines associated with the Th2 profile may be important factors in the immunopathogenesis of SMR.

Spinal neuroschistosomiasis.

We report a 20-year-old woman with a diagnosis of spinal neuroschistosomiasis that was confirmed histologically. Magnetic resonance imaging demonstrated an expanded, oedematous conus with intrinsic heterogeneous enhancement, which was initially thought to be a neoplastic lesion. This case demonstrates an alternative diagnosis to oncological lesions of the spinal cord which should be considered in patients who have recently travelled to endemic areas.

Pseudotumoral Encephalic Schistosomiasis: A Literature Review.

Pseudotumoral encephalic schistosomiasis (PES) is the chronic form of cerebral neuroschistosomiasis, and is rarely encountered in clinical practice. Clinically, PES closely resembles other intracranial space-occupying lesions including brain tumors. Laboratory investigations are usually inconclusive, and neuroradiologic findings are frequently reported as non-specific. Such diagnostic difficulties may result in delayed diagnosis and treatment. Across the literature, there is a paucity of information about and controversy over many aspects of the disease. Particularly, inconsistent magnetic resonance imaging (MRI) findings, a wide variation of medical treatment protocols, lacking consensus regarding the indications of surgery, and undetermined information regarding the impact of the extent of resection on prognosis. We herein review the pertinent literature with the aim of providing focused information regarding the pathogenesis of PES, its currently identified more distinctive neuroimaging features, and the indications and extent of surgery in light of the state-of-the-art operative neurosurgical practice. A distinctive multinodular arborizing pattern of PES lesions can often be observed on MRI in patients with PES. Praziquantel is considered by many authors to be the drug of choice in all cases, and seems to be effective at variable dose regimens. Although lesion excision utilizing current technology is generally safe, the indications and extent of surgery are still undetermined and should be decided on a case-by-case basis. Multicenter collaborative research is further needed to fill the existing gaps in the current knowledge on PES.

Schistosoma antigens downmodulate the in vitro inflammatory response in individuals infected with human T cell lymphotropic virus type 1.

UNLABELLED: Human T cell lymphotropic virus type 1 (HTLV-1) is the causal agent of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). While the immune response to HTLV-1 infection is polarized to the Th1-type, chronic helminth infections drive the Th2- and T regulatory-type, and are able to downregulate the inflammatory response in some autoimmune diseases. OBJECTIVE: To evaluate whether Schistosoma spp. antigens alter the in vitro cytokine response in HTLV-1 infection. METHODS: The recombinant Schistosoma antigens Sm29 and ShTSP2 (tetraspanin) and PIII, a fraction of the Schistosoma mansoni adult worm antigen were added to peripheral blood mononuclear cell (PBMC) cultures of HTLV-1-infected individuals and the levels of interferon (IFN)-γ and interleukin (IL)-10 in the supernatants were measured using the ELISA sandwich technique. RESULTS: Compared to the levels of cytokine in nonstimulated cultures, the levels of IFN-γ were reduced in 50, 47 and 50% of patients by the presence of Sm29, ShTsp2 and PIII, respectively. The downregulation of IFN-γ production in the presence of Sm29 antigen was observed mainly in subjects who had lower basal levels of this cytokine. The levels of IL-10, however, increased by the addition of the three antigens in the cultures in 74, 62 and 44% of individuals, respectively. In addition, there was a decrease in the ratio of IFN-γ/IL-10 levels in cultures stimulated with Sm29 and ShTSP2 when compared to nonstimulated ones. CONCLUSIONS: The Schistosoma spp. antigens used in this study were able to downmodulate IFN-γ production in vitro in HTLV-1 infection. This may be associated with the increased levels of IL-10 induced by the antigens.

Cerebral and spinal schistosomiasis.

Cerebral schistosomiasis and spinal schistosomiasis are severe underrecognized complications of Schistosoma sp. infection, and can occur at any time during the parasitic infection. Neuroschistosomiasis has been increasingly reported not only in endemic areas but also in Western countries owing to immigration and international travel. Immunogenic interaction between schistosome egg deposition and the delayed hypersensitivity reaction of the host are the main neuropathogenic mechanisms involved. Eggs induce a periovular granulomatous reaction in the tissues. In some cases, schistosome adult worms may aberrantly migrate to the central nervous system via the vertebral venous plexus and place the ova at an ectopic site. Headache and seizures are common in cerebral schistosomiasis, and intracranial hypertension and hydrocephalus may occur in tumour-like and cerebellar schistosomiasis. Spinal schistosomiasis may manifest itself as acute myelitis and/or myeloradiculopathy. Recognition of neuroschistosomiasis is important so that early treatment with praziquantel and steroids can be started in an attempt to prevent severe disability.

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 Schistosoma mansoni or S. haematobium 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 S. mansoni with surrounding inflammatory reaction. The cord showed demyelination near the ova and an associated inflammatory reaction. Patients responded well to surgical ecompression and treatment with praziquantel and oral steroids.

Are seizures associated with neuroschistosomiasis mansoni? An exploratory survey in an endemic area of Brazil.

BACKGROUND: Deposition of Schistosoma mansoni eggs in the brain of patients with hepatosplenic schistosomiasis (HS-SM) is frequent and usually asymptomatic. However, it is questioned whether it could cause seizures. Thus, we investigated the occurrence of seizures in these patients and also searched for parameters associated with this disorder. METHODS: In a cross-sectional survey, we compared 128 patients with HS-SM with 102 patients with portal hypertension due to compensated chronic hepatic disease of other etiologies. A standardized questionnaire, emphasizing epilepsy-related parameters, was applied to all participants. RESULTS: Eight (6.3%) patients with HS-SM had a history of seizures, whereas this condition was reported by three (2.9%) individuals from the comparison group (p=0.354). None of the variables were associated with the occurrence of seizures, either in univariate or in multivariate analysis. CONCLUSIONS: The frequency of seizures was similar in both study groups. However, it was higher than that described in population-based studies. Thus, we hypothesize that HS-SM individuals may have a higher frequency of seizure. The lack of difference between the two study groups may be explained by the inclusion of cases of HS-MS overlapping other chronic liver diseases in the comparison group, because this finding is relatively common in schistosome-endemic areas.

Oriental schistosomiasis with neurological complications: case report.

We describe a case of cerebral schistosomiasis, caused by Asian (oriental) Schistosoma japonicum trematode blood flukes, in a young Chinese patient and its management. We also provide a brief update for physicians on the clinical features, diagnosis and treatment of schistosomiasis, with particular emphasis on neuroschistosomiasis, the most severe clinical outcome associated with this parasitic infection.

Delayed diagnosis of spinal cord schistosomiasis in a non-endemic country: A tertiary referral centre experience.

BACKGROUND: Neuroschistosomiasis is a severe complication of schistosomiasis, triggered by the local immune reaction to egg deposition, with spinal cord involvement the most well recognised form. Early treatment with praziquantel and high dose steroids leads to a reduction of neurological sequelae. The rarity of this condition in returning travellers to high income countries can result in delayed diagnosis and treatment. We aimed to evaluate the diagnosis and management of neuroschistosomiasis in a UK national referral centre. MATERIALS/METHODS: A retrospective review of confirmed clinical cases of spinal schistosomiasis referred to the Hospital for Tropical Diseases, UK, between January 2016 and January 2020 was undertaken. Electronic referral records were interrogated and patient demographic, clinical, laboratory, and radiological data collected. RESULTS: Four cases of neuroschistosomiasis were identified. The median age at diagnosis was 28 (range 21 to 50) with three male patients. All patients had epidemiological risk factors for schistosomiasis based on travel history and freshwater exposure; two in Uganda (River Nile), one in Malawi and one in Nigeria. All patients presented with features of transverse myelitis including back pain, leg weakness, paraesthesia and urinary dysfunction. The mean time from presentation to health services to definitive treatment was 42.5 days (range 16-74 days). Diagnosis was confirmed with CSF serology for schistosomiasis in all cases. Radiological features on MRI spine included enhancement focused predominantly in the lower thoracic spinal cord in three cases and the conus in one patient. All patients received a minimum of three days of oral praziquantel and high dose steroids. At three-month follow-up, one patient had complete resolution of symptoms and three had residual deficit; one patient was left with urinary and faecal incontinence, another had urinary retention, and the final patient has persistent leg pains and constipation. CONCLUSION: We observed a marked delay in diagnosis of neuroschistosomiasis in a non-endemic country. We advocate undertaking a thorough travel history, early use of imaging and CSF schistosomal serology to ensure early diagnosis of neuroschistosomiasis in patients presenting with consistent symptoms. If schistosomal diagnostics are not immediately available, presumptive treatment under the guidance of a tropical medicine specialist should be considered to minimize the risk of residual disability. We advocate for consensus guidelines to be produced and reporting to be performed in a uniform way for patients with spinal schistosomiasis.

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.

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.

[Histopathology of a rare case of intramedullary schistosomiasis and literature review]. / Histopathologie d´un rare cas de schistosomiase intramédullaire et revue de la littérature.

Schistosomiasis is a major public health problem in sub-Saharan Africa and, in particular, in Cameroon. It is the second parasitic disease endemic after malaria and it is favoured by the coexistence of bioclimatic zones. We report the case of a 6-years old girl presenting with clinical deficit syndrome and medullary infiltration mimicking tumor on medical imaging. Surgery helped to clarify the diagnosis after histopathological examination of the biopsic specimens. The patient had also received a dose of Praziquantel. Regression of symptoms as well as favorable progression of the operative wound facilitated discharge from hospital. The patient was lost to follow-up for three years. Effective management of neuromeningeal bilharziosis should be multidisciplinary.

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.