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Neuroschistosomiasis due to Schistosoma mansoni: a review of pathogenesis, clinical syndromes and diagnostic approaches.

Neuroschistosomiasis (NS) is the second most common form of presentation of infection by the trematode, Schistosoma mansoni. Granulomatous inflammatory reaction occurs as a result of schistosome eggs being transmitted to spinal cord or brain via the vascular system, or by inadvertent adult worm migration to these organs. The two main clinical syndromes are spinal cord neuroschistosomiasis (acute or subacute myelopathy) and localized cerebral or cerebellar neuroschistosomiasis (focal CNS impairment, seizures, increased intracranial pressure). Presumptive diagnosis of NS requires confirming the presence of S. mansoni infection by stool microscopy or rectal biopsy for trematode eggs, and serologic testing of blood and spinal fluid. The localized lesions are identified by signs and symptoms, and confirmed by imaging techniques (contrast myelography, CT and MRI). Algorithms are presented to allow a stepwise approach to diagnosis.

Algorithms↗

An unusually late presentation of neuroschistosomiasis.

Schistosomiasis is one of the most widespread parasitic infections in man. Neuroschistosomiasis, referring to schistosomal involvement of the central nervous system, is an uncommon but well recognised complication of schistosomal infection. The duration between time of infection and onset of neurological symptoms typically varies between weeks to months. We describe a case of transverse myelitis secondary to neuroschistosomiasis, presenting more than three years after the time of initial schistosomal infection, diagnosed and treated in the district general hospital setting.

Adult↗

Experimental neuroschistosomiasis: inadequacy of the murine model.

Neuroschistosomiasis is rarely observed in human pathology, but it is of considerable importance. To investigate its pathogenesis, consequences and response to treatment, an experimental model would be desirable, but is not yet available, in spite of a few indications of a suitable mouse model in the literature. Severe, recent and late Schistosoma mansoni infections in outbred and inbred strains of mice revealed widespread distribution of parasite eggs in several organs, but only exceptionally did eggs reach the encephalus, thus revealing the inadequacy of the mouse as an experimental model for neuroschistosomiasis.

Animals↗

Neuroschistosomiasis myelopathy: case report.

A case of intramedullary neuroschistosomal granuloma in a 15-year-old girl is reported. Despite the fact that Saudi Arabia is endemic for schistosomiasis, neuroschistosomiasis has rarely been reported from that country. A review of the literature on the incidence, pathogenesis, clinical patterns and management of neuroschistosomiasis, particularly myelopathy, is given.

Adolescent↗

Clinical and cerebrospinal fluid findings in patients less than 20 years old with a presumptive diagnosis of neuroschistosomiasis.

CNS manifestations are rarely attributable to Schistosoma mansoni infection although systemic distribution of S. mansoni eggs is well recorded. We describe 73 patients aged less than 20 years who presented S. mansoni eggs in the stool or rectal biopsy and IFA and/or HAI positive CSF tests to S. mansoni, and who had neurological signs and symptoms. Paraparesis (54.8 per cent), urinary retention (53.4 per cent), and paraplegia (20.5 per cent) were the most commonly observed CNS manifestations. In the CSF, pleocytosis (range 7-560 WBC/mm3) associated with eosinophilia (85.7 per cent vs. 50.0 per cent, p = 0.02) and elevated protein concentration (96.8 per cent vs. 40.0 per cent, p = 0.00003) were observed. We conclude that in areas of the world where infection by S. mansoni is common, neuroschistosomiasis should be an important diagnostic consideration in children with neurological signs and symptoms.

Adolescent↗

[Cerebrospinal fluid syndrome in neuroschistosomiasis].

A study was made of 220 samples of cerebrospinal fluid (CSF) from patients who suffered from several diseases of the central nervous system. In all samples immunological reactions for syphilis, cysticercosis, toxoplasmosis, Chagas' disease and schistosomiasis were studied comparatively. Immunofluorescent reactions for schistosomiasis were made by indirect antiglobulin technic with two types of antigen: the worm and the liver granuloma of hamster infected by Schistosoma mansoni. Emphasis is given on data concerning to 16 cases in which these reactions were reagent. The importance of routine search in the CSF for schistosomiasis antibodies is discussed. The concept of a 'CSF neuroschistosomiasis syndrome' is discussed as the main aspect of diagnosis "in vivo" of the disease. It is supported by the demonstration of specific antibodies in the CSF. Hypercytosis of lymphomononuclear type associated to the presence of eosinophil cells, protein concentration increase and gamma globulins increase are other characteristics found in the CSF in this syndrome.

Adolescent↗

[Neuroschistosomiasis].

The low incidence of mansoni schistosomiasis involving the nervous system calls our attention in a country where 12 million people carry the intestinal form of the disease. Also, during autopsies the incidence of the parasites eggs occurs three to four times more often than the cases diagnosed in life. Therefore, new investigations become necessary around endemic areas. The cerebrospinal fluid syndrome of the neuroschistosomiasis must be searched among patients from endemic areas who present neurological symptoms and which cause is unknown. The probable protein liberation giving signs in the CSF and causing the demyelinization or the axonal degeneration at a distance is emphasized among the physiopathological factors. Two cases of neuroradiculomyelitis with positive copro analysis and CSF syndrome for mansoni schistosomiasis are presented and discussed; both were treated with success using praziquantel in association with steroids for a period of fourteen days.

Adult↗

[Mansonic neuroschistosomiasis: clinical, laboratory and therapeutic aspects].

The authors report 16 patients bearing the meningomyeloradicular form of Mansonic neuroschistosomiasis diagnosed according to clinical, laboratorial and image criteria. Patients have been observed at the Neurology Ambulatory of the Federal University of Bahia, within the period of April/91 to December/93. They have been treated with praziquantel, associated to corticosteroids. The aim has been to evaluate the drug's efficiency and safety in decreasing the neurological signs and symptoms.

Adolescent↗

Neuroschistosomiasis.

Schistosomiasis is an infection caused by digenetic trematode platyhelminths of the genus Schistosoma. These blood flukes use man and other mammals as definitive hosts and aquatic and amphibious snails as intermediate hosts. Of the schistosomal species, S. mansoni, S haematobium and S. japonicum are the most important to man and the most widely distributed. The infection affects about 200 million individuals in 74 countries of Latin America, Africa and Asia. Far less commonly, schistosomes reach the central nervous system (CNS). This may occur at any time from the moment the worms have matured and the eggs have been laid. For this reason, CNS involvement may be observed with any of the clinical forms of schistosomal infection. The presence of eggs in the CNS induces a cell-mediated periovular granulomatous reaction. When eggs reach the CNS during the early stages of the infection or during evolution of the disease to its chronic forms, large necrotic-exudative granulomas are found. In-situ egg deposition following the anomalous migration of adult worms appears to be the main, if not the only, mechanism by which Schistosoma may reach the CNS in these stages. The mass effect produced by the heavy concentration of eggs and the presence of large granulomas in circumscribed areas of the brain and spinal cord explains, respectively, 1) the signs and symptoms of increased intracranial pressure and focal neurological signs; and 2) the signs and symptoms of rapidly progressing transverse myelitis, usually affecting the lumbosacral segments of the spinal cord. Most of the cases of CNS involvement associated with the hepatosplenic and cardiopulmonary chronic forms, or with severe urinary schistosomiasis, though more frequent, are asymptomatic. In the patients with these clinical forms, the random and sparse distribution of eggs in the CNS indicates that the embolization of eggs from the portal mesenteric system to the brain and spinal cord constitutes the main route of CNS invasion by Schistosoma. The discrete inflammatory reaction elicited by the sparsely distributed eggs in the CNS explains the lack of neurological symptoms that could be produced by egg deposition.

Animals↗

[Spinal cord neuroschistosomiasis: clinical and laboratory evaluation of 5 cases].

Schistosomiasis mansoni is an endemic disease in Brazil. Cases of nervous system schistosomiasis have been referred in our medical literature for the past forty years. In this report five cases of schistosomiasis of the spinal cord are studied. A review of the literature has been made and some clinical, laboratory and therapeutical aspects are discussed.

Adolescent↗

Schistosoma mansoni: assessment of morbidity before and after control.

The literature on the assessment of morbidity due to Schistosoma mansoni infection is updated. Imaging techniques such as ultrasonography, echodoppler cardiography, computerized tomography (CT scan) and magnetic resonance imaging (MRI) introduced a new perspective, and expanded our knowledge on morbidity. Three well-defined syndromes caused by schistosomiasis mansoni have been described: the stage of invasion, acute schistosomiasis (Katayama fever), and chronic schistosomiasis. Complications of the acute and chronic syndromes have also been reported: pulmonary hypertension, neuroschistosomiasis, association with Salmonella, association with Staphylococci, viral hepatitis B, glomerulonephritis. In most individuals with hepatosplenic schistosomiasis the spleen is increased in size. Hepatosplenic schistosomiasis can, however, occur without splenomegaly. The definition of hepatosplenic schistosomiasis in endemic areas as the finding of S. mansoni eggs in the stools in an individual with hepatosplenomegaly is not satisfactory anymore. Many aspects of morbidity are expected to change after schistosomiasis control. Some are expected to change quickly (worm burden, Salmonella bacteremia, hepatosplenic schistosomiasis in children) whereas others shall remain for years (pulmonary hypertension, glomerulonephritis, neuroschistosomiasis). Intestinal schistosomiasis in individuals with low worm burdens is very difficult to diagnose and therefore laborious to control.

Brazil↗

Parasitic worms of the central nervous system: an Australian perspective.

The diagnosis and management of parasitic diseases of the central nervous system (CNS) is difficult, even for infectious diseases physicians and neurologists. Furthermore, few overviews of the spectrum of causative helminths and clinical syndromes have been published. In the present study, we review the seven most common parasitic diseases of the CNS: (i) cysticercosis, (ii) neuroschistosomiasis, (iii) paragonimiasis, (iv) angiostrongyliasis, (v) hydatid disease, (vi) sparganosis and (vii) gnathostomiasis. Major syndromes of parasitic disease of the CNS and their differential causes are discussed, including: (i) cystic lesions, (ii) enhancing granulomas (with and without creeping subcutaneous eruptions), (iii) eosinophilic meningoencephalitis and (iv) spinal cord disease. Specific risk factors that predispose to these infections are also discussed and particular attention is drawn to the situation in Australia.

Angiostrongylus↗

Neuroinflammatory implication of Schistosoma mansoni infection in the mouse.

Schistosomiasis is a parasitic disease due to Schistosoma mansoni. Schistosome infection is known to induce granulomas not only in the spleen, bladder, liver and intestine but also in the brain and spinal cord resulting in severe neuropathological and psychiatric disorders though the interaction between Schistosoma mansoni infection and the nervous system has received on the whole little attention. In the present review it has been discussed recent findings from experimental Schistosoma mansoni infection in mouse nervous system. We show that brain granulomas are associated with a significant alteration in the constitutive levels of nerve growth factor (NGF), a trophic factor playing an essential role in nerve growth and differentiation and in preventing neuronal damages. Animals infected with schistosomes suffered also of increased pain sensitivity which was inhibited by TNF-alpha antibody injections and not by anti-NGF. These findings suggest that the neuropathological dysfunctions in neuroschistosomiasis may be linked to changes in the basal levels and/or activity of neurotrophic factors caused by local formation of granulomas.

Animals↗

Schistosoma mansoni myeloradiculopathy in an 8-year-old Omani boy.

An 8-year-old Omani boy presented with progressive ascending weakness of the lower limbs with bowel and bladder incontinence. A diagnosis of myeloradiculopathy was made on neurologic examination. Eosinophilia in the peripheral blood count and suspected endemicity in his region of residence raised the diagnostic possibility of neuroschistosomiasis. Cerebrospinal fluid findings, high schistosoma antibody titre, magnetic resonance imaging of the spine, and detection of Schistosoma mansoni eggs in the stool confirmed the diagnosis. The boy recovered significantly after receiving praziquantel and methylprednisolone. Neurological symptoms at presentation could be due to parasitic infection and should be considered in an endemic region. Eosinophilia in the peripheral blood count may be the first indicator.

Animals↗

Clinical and cerebrospinal fluid (CSF) profile and CSF criteria for the diagnosis of spinal cord schistosomiasis.

OBJECTIVES: To describe the clinical and CSF findings among patients with presumptive neuroschistosomiasis (NS) and to suggest a classification for the CSF diagnosis of presumptive NS. METHOD: The charts of all patients whose CSF exam was performed at the CSF Lab, Jos Silveira Foundation, Salvador, Brazil, from 1988 to 2002 were reviewed. Those with clinically suspected NS whose indirect fluorescent antibody test (IFA) and or hemagglutination-inhibiting antibodies test (HAI) were positive to S. mansoni were identified. RESULTS: Of 377 patients, 67.9% were males; the median age was 36 years (mean 37 + 16 yrs, range 3-82 yrs). The most frequent complaints were paraparesis (59.9%), urinary retention (36.2%), lower limb pain (22.8%). WBC of CSF (count/mm ) was > 4 in 66.0% (mean 83 + 124, median 40, range 4.3-1,100), protein (mg/dl) was > 40 in 84.6% (mean 185 + 519, median 81, range 41-6,800) and eosinophils were present in 46.9%. IFA and HAI were positive in 75.3%. WBC > 4 and presence of eosinophils were associated with IFA and HAI positive (67.3% versus 51.4%, p 0.014; 49.1% versus 23.0%, p 0.0001, respectively) and protein > 40 was not (85.4% versus 77.0%, p 0.09). Presence of WBC > 4, protein > 40 and eosinophils was associated with IFA and HAI positive (71.6% versus 38.2%, p 0.0003) but presence of eosinophils and any other combination of WBC and protein were not. CONCLUSION: NS should be considered as a possible diagnosis in patients who had had contact with schistosome-infected water and present with spinal cord compromising. Presence of IFA and HAI positive to S. mansoni, WBC > 4, protein > 40 and presence of eosinophils in the CSF may be considered as a criterium of highly probable presumptive diagnosis.

Adolescent↗

Involvement of central nervous system in the schistosomiasis.

The involvement of the central nervous system (CNS) by schistosomes may or may not determine clinical manifestations. When symptomatic, neuroschistosomiasis (NS) is one of the most severe presentations of schistosomal infection. Considering the symptomatic form, cerebral involvement is almost always due to Schistosoma japonicum and the spinal cord disease, caused by S. mansoni or S. haematobium. Available evidence suggests that NS depends basically on the presence of parasite eggs in the nervous tissue and on the host immune response. The patients with cerebral NS usually have the clinical manifestations of increased intracranial pressure associated with focal neurological signs; and those with schistosomal myeloradiculopathy (SMR) present rapidly progressing symptoms of myelitis involving the lower cord, usually in association with the involvement of the cauda esquina roots. The diagnosis of cerebral NS is established by biopsy of the nervous tissue and SMR is usually diagnosed according to a clinical criterion. Antischistosomal drugs, corticosteroids and surgery are the resources available for treating NS. The outcome is variable and is better in cerebral disease.

Animals↗