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.

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.

[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 / 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.

Pediatric Neuroschistosomiasis: A Case Report and Review of the Literature.

Neuroschistosomiasis is a rare but severe manifestation of Schistosoma infection. Diagnosis is challenging and surgical biopsy is often required to confirm diagnosis and exclude malignancy. We present a pediatric case of presumed pseudotumoral cerebral schistosomiasis secondary to Schistosoma mansoni with an excellent therapeutic response to empirical praziquantel and corticosteroid treatment.

Neuroschistosomiasis: An Unusual Intracranial Space Occupying Lesion.

BACKGROUND: Neuroschistosomiasis is an uncommonly reported disease. CASE CHARACTERISTICS: An adolescent Indian boy residing in Kenya presented with headache, visual symptoms and seizures, with MRI showing space-occupying lesions in the occipital lobe and cerebellum. OBSERVATION: Brain biopsy was diagnostic of neuro-schistosomiasis; complete recovery was seen with praziquantel and corticosteroid therapy. MESSAGE: This case highlights the importance of considering epidemiology in differential diagnosis and establishing definitive diagnosis even if it is by invasive methods.

[Myelitis transversa caused by neuroschistosomiasis]. / Myelitis transversa door schistosomiasis.

BACKGROUND: Neuroschistosomiasis is a severe complication of an infection with Schistosoma; this infection can lead to myelitis transversa. Acute myelitis transversa is a rare disorder of the spinal cord, which can present with muscular weakness, sensory disturbance and intestinal or bladder dysfunction. CASE DESCRIPTION: A 17-year-old refugee from Eritrea, who had been in the Netherlands for 3 weeks, suffered from back pain and progressive weakness of both legs for one week. Both the clinical presentation and the MRI images were consistent with myelitis transversa. Schistosomamansoni eggs were found in the faeces, and antibodies to Schistosoma eggs and worms were found in both liquor and serum, leading to a diagnosis of neuroschistosomiasis. The patient recovered completely following treatment with praziquantel and prednisone. CONCLUSION: Schistosomiasis is a commonly occurring parasitic disease in sub-Saharan Africa, which can lead to myelitis transversa if it spreads to the spinal cord. Early detection and treatment are necessary to prevent lasting damage. A good geographical case history is essential for this process.

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.

[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.

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.

[Myeloradiculitis due to Schistosoma haematobium: about an observation in Dakar (Senegal)]. / Myéloradiculite à Schistosoma haematobium : à propos d'une observation à Dakar (Sénégal).

Nervous localisations of schistosomiasis are rare. We report the case of a 25 year-old Senegalese patient admitted for a progressive myeloradiculitis onset, over a one week period. The diagnosis of Schistosoma haematobium myeloradiculitis was made in front of a positive serum serology for S. haematobium, presence of S. haematobium eggs in urine, hyperproteinorachia, endemicity of S. haematobium in the region where the patient was originating and a past medical history of macroscopic hematuria in a context of river bathing. There was also no arguments for another cause to these neurological manifestations. Our patient was treated with praziquantel, prednisone and physiotherapy. Evolution was marked 6 weeks after the beginning of treatment by a significant improvement of motor deficit, enabling the patient to walk again. There was also a regression of genitosphincter dysfunction. Work-up for patients presenting with paraplegia in tropical countries, should also include search for S. heamatobium infection.

[Diagnosis and treatment of cerebral schistosomiasis: a report of 166 cases].

OBJECTIVE: To discuss the clinical features, diagnosis and treatment of cerebral schistosomiasis. METHODS: A total of 166 patients with cerebral schistosomiasis were treated, and their clinical data were collected and analyzed retrospectively. RESULTS: In 166 cases of cerebral schistosomiasis, the confirmative diagnoses of 156 cases were diagnosed according to the clinical manifestation, etiology, immunology and auxiliary examination (CT, MRI). In among, 74 cases were confirmed by pathological examination, 10 cases were diagnosed through to the diagnostic treatment. Totally 102 patients received the oral medication of praziquantel, and they all improved and discharged 14-16 days later; 64 patients received the craniotomy and praziquantel medication after the operation, and 48 patients significantly improved, others did not improve or aggravated. There was no operative mortality. CONCLUSIONS: Neuroimaging and laboratory tests are valuable in the diagnosis of cerebral schistosomiasis. The praziquantel treatment is selected firstly when the diagnosis was established. However, in the case of serious intracranial hypertension, intractable epilepsy and praziquantel treatment fails, the surgical treatment is required.

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.

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.

Pseudotumoral form of neuroschistosomiasis: report of three cases in Ganzi, China.

The authors report three rare cases of neuroschistosomiasis lacking extracranial involvement. No parasitic eggs were detected in the stool with the Kato-Katz thick smear methods. Computed tomography of the brains showed hypodense signals, and magnetic resonance imaging showed isointense signals on T1-weighted images, hyperintense signals on T2-weighted images, and intensely enhancing nodules in the brain after intravenous administration of gadolinium. High-grade gliomas were suspected, and operations or radiosurgery was performed. Cerebral schistosomiasis was confirmed in all cases by biopsy of the brain lesions, revealing granulomas containing embedded Schistosoma japonicum eggs. All cases were definitively diagnosed as brain schistosomiasis japonica. Praziquantel and corticosteroids were administered, and the prognoses were good for all case patients. Although the aforementioned pattern of imaging examinations is not present in all cases of neuroschistosomiasis, a diagnosis of neuroschistosomiasis should be considered when this pattern of imaging is observed; cerebrospinal fluid serological exams are also recommended.

Neuroschistosomiasis.

Schistosomiasis (bilharzia) is a neglected tropical disease caused by digenetic trematode platyhelminths of the genus Schistosoma. Neuroschistosomiasis is one of the most severe clinical outcomes associated with schistosome infection. Neurological complications early during the course of infection are thought to occur through in situ egg deposition following aberrant migration of adult worms to the brain or spinal cord. The presence of eggs in the CNS induces a cell-mediated Th2-driven periovular granulomatous reaction. The mass effect of thousands of eggs and the large granulomas concentrated within the brain or spinal cord explain the signs and symptoms of increased intracranial pressure, myelopathy, radiculopathy and subsequent clinical sequelae. Myelopathy (acute transverse myelitis and subacute myeloradiculopathy) of the lumbosacral region is the most common neurological manifestation of S. mansoni or S. haematobium infection, whereas acute encephalitis of the cortex, subcortical white matter, basal ganglia or internal capsule is typical of S. japonicum infection. Cerebral complications include encephalopathy with headache, visual impairment, delirium, seizures, motor deficits and ataxia, whereas spinal symptoms include lumbar pain, lower limb radicular pain, muscle weakness, sensory loss and bladder dysfunction. The finding of eggs in the stool or a positive serology, provides supportive but not direct evidence of neuroschistosomiasis. A definitive diagnosis can only be made with histopathological study showing Schistosoma eggs and granulomas. Schistosomicidal drugs (notably praziquantel), steroids and surgery are currently used for the treatment of neuroschistosomiasis. During the 'acute phase' of the disease, neuroschistosomiasis is treated with corticosteroids which are augmented with a course of praziquantel once female worm ovipositioning commences. Surgery should be reserved for special cases such as in those with evidence of medullary compression and in those who deteriorate despite clinical management.