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.

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.

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.

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.

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.

[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.

Cerebral schistosomiasis japonica without gastrointestinal system involvement.

BACKGROUND: Schistosoma japonicum is the most widespread schistosoma in the world. Although gastrointestinal system involvement with S japonicum appears to be considerably common, cerebral schistosomiasis is not frequent. Cerebral schistosomiasis japonica intestinal and hepatosplenic involvement is more rare. We collected 2 cases of cerebral schistosomiasis identified by pathological diagnosis, lacking extracranial involvement. In addition, one of them had multiple lesions, which was also rare. CASE DESCRIPTION: Two male patients came from Dongting Lake region, Hunan province, one of the oldest and most severe endemic areas of China. Their clinical symptoms varied, such as headache, dizziness, seizures, and others. Studies in blood were normal except for eosinophilia. Computed tomography of brains showed hyperdense areas, and MRI showed isointense signal on T1-weighted images, hyperintense signal on T2-weighted images, and heterogeneous enhancement. The definitive diagnosis was cerebral schistosomiasis japonium by biopsy. Standard use of praziquantel and corticosteroid drugs was applied, and the prognosis was good. CONCLUSION: Cerebral schistosomiasis japonica without intestinal and hepatosplenic involvement is exactly rare and easily ignored. The diagnosis sometimes is difficult. Laboratory and imaging examinations are helpful but not specific. Although operation can give the definitive diagnosis, it is not imperative. The administration of praziquantel and corticosteroid drugs in early stages is good for prognosis.

[Sciatica after return from Mali: case report of schistosomal myelopathy]. / Sciatique au retour du Mali.

INTRODUCTION: In spite of the high prevalence of schistosomiasis in Mali, few cases involving neurological complications have been described. The purpose of this report is to present a case associated medullary complications. CASE REPORT: A 29-year-old man was hospitalized for low back pain and difficulty in walking linked to dysesthesia. Five months earlier the patient had been trreated for schistosomiasis contracted during a trip to Dogon region of Mali. Based on radiological and laboratory findings and previous clinical history, the difinitive diagnosis was schistosomal myelopathy. DISCUSSION/CONCLUSION: Neuroschistosomiasis is a rare but serious complication of the schistosomiasis that can only be made after complete parasite identification and careful differential diagnosis. Treatment with antiparasitic agents in association with corticosteroids is mandatory but must only be initiated in state stage of the parasitic infection, i.e., after maturation of larvae into adults.

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.

Cerebral schistosomiasis: Case series from Qatar.

Schistosomiasis is the third most widespread devastating parasitic disease worldwide and has a high mortality burden. Neuroschistosomiasis is one of the rare and most severe clinical presentations of the disease. It is caused by granuloma formation around eggs that lodge in the central nervous system, with Schistosoma japonicum usually causing most reported cerebral disease. Three unusual presentations of schistosomiasis in Qatar are described herein. The three patients were young males who presented with seizures and tumor-like lesions on brain imaging. The diagnosis was confirmed by biopsy, which showed necrotizing granulomas containing Schistosoma eggs. These cases raise awareness of neuroschistosomiasis as a potential cause of tumor-like brain lesions in migrants and returning travelers from endemic areas.

Early complicated schistosomiasis in a returning traveller: Key contribution of new molecular diagnostic methods.

Early schistosomiasis poses a serious diagnostic challenge, because current standard diagnostic techniques based on serology and egg microscopy lack sensitivity at the initial presentation. We report spinal cord neuroschistosomiasis in a traveller developing 6 weeks after exposure. The diagnosis was confirmed by Schistosoma mansoni-targeted real-time PCR in blood and cerebrospinal fluid, before the results of conventional methods became positive. Molecular assays represent a paradigm shift for the difficult diagnosis of early schistosomiasis and related complications.

Neurological complications of Schistosoma infection.

Schistosomiasis is a parasitic disease caused by blood flukes of the genus Schistosoma. Currently more than 200 million people worldwide are affected. Neuroschistosomiasis constitutes a severe presentation of the disease. Neurological symptoms result from the inflammatory response of the host to egg deposition in the brain and spinal cord. Neurological complications of cerebral schistosomiasis include delirium, loss of consciousness, seizures, dysphasia, visual field impairment, focal motor deficits and ataxia. Cerebral and cerebellar tumour-like neuroschistosomiasis can present with increased intracranial pressure, headache, nausea and vomiting, and seizures. Myelopathy (acute transverse myelitis and subacute myeloradiculopathy) is the most common neurological complication of Schistosoma mansoni infection. Schistosomal myelopathy tends to occur early after infection and is more likely to be symptomatic than cerebral schistosomiasis. The conus medullaris and cauda equina are the most common sites of involvement. Severe schistosomal myelopathy can provoke a complete flaccid paraplegia with areflexia, sphincter dysfunction and sensory disturbances. Schistosomicidal drugs, steroids and surgery are the currently available treatments for neuroschistosomiasis. Rehabilitation and multidisciplinary team care are needed in severely disabled patients.

MRI in cerebral schistosomiasis: characteristic nodular enhancement in 33 patients.

OBJECTIVE: The purpose of our study was to describe the characteristic MRI appearance of cerebral infection with Schistosoma japonicum. CONCLUSION: Cerebral infection with S. japonicum can cause a characteristic MRI pattern of a large mass comprising multiple intensely enhancing nodules, sometimes with areas of linear enhancement. The typical appearance may be useful for diagnosis in endemic regions and may potentially be useful in cases imported into countries in which the disease is not endemic.

Spinal cord schistosomiasis japonica: a report of 4 cases.

BACKGROUND: Neuroschistosomiasis is a severe presentation of schistosomal infection. Currently however, spinal cord schistosomiasis japonica is clinically rare, and very few cases are reported. METHODS: The purpose of this study is to retrospectively analyze the diagnosis and treatment of 4 patients who presented with a lower cord syndrome of acute progression characterized by motor, sensory, and autonomic dysfunctions. The patients were examined by MRI, and the biochemical and immunologic changes of blood and CSF of the patients were also analyzed before surgery. Treatments including surgical resection and antischistosomal drugs followed by a histologic examination were used to confirm the diagnosis. RESULTS: Spinal cord schistosomiasis japonica is a very rare disease. Magnetic resonance imaging can obtain precise position fixing, although it is hard to make preoperative qualitative determination. Postoperative follow-up assessment indicated that the symptoms of 4 patients had improved without further treatment. CONCLUSIONS: The main clinical manifestations of spinal cord schistosomiasis japonica have some common features, and MRI is useful and important in diagnosing the disease. The most effective treatment for the disease that shows radiological evidence of spinal cord or conus compression and inefficacious expectant treatment by other methods is to excise it totally and apply antischistosomal drugs.