Schistosomal Myeloradiculopathy - A case report.

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

Schistosomal myeloradiculopathy - A case report

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

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.

Identification and characterization of immune complexes in the cerebrospinal fluid of patients with spinal cord schistosomiasis.

The pathogenesis of neuroschistosomiasis is largely unknown. Available evidence suggests that it depends on the presence of parasite eggs in the nervous tissue and on the host's immune response. We investigated the presence of immune complexes (ICs) in the cerebrospinal fluid (CSF) of four patients with spinal cord schistosomiasis (SCS), and performed their characterization. ICs containing soluble egg antigen of Schistosoma mansoni (SEA) were found in the CSF of all the SCS patients. To our knowledge, this is the first evidence of ICs containing schistosomal antigens in the CSF of patients with SCS. Further studies are necessary to confirm our findings and investigate the possible roles of ICs in the pathogenesis of this disease.

A laboratory test for the diagnosis of neuroschistosomiasis.

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

Magnetic resonance imaging and cerebrospinal fluid immunoassay in the diagnosis of cerebral schistosomiasis: experience in southwest China.

We analysed the magnetic resonance imaging (MRI) clinical characteristics as well as serum and cerebrospinal fluid (CSF) immunoassay results in 41 patients with cerebral schistosomiasis. Thirty-five cerebral schistosomiasis patients were diagnosed by imaging and immunoassay, five by post-operative pathological examination and one by diagnostic treatment with praziquantel. We found that MRI showed specific enhancement, forming the 'Buddha's hand' appearance. Although this pattern of MRI enhancement may not be present in all cases of cerebral schistosomiasis, when it is observed a diagnosis of cerebral schistosomiasis should be considered. Meanwhile, CSF immunoassay may also play an important role in the differential diagnosis of cerebral schistosomiasis.

Pseudotumoral form of neuroschistosomiasis: report of three cases.

Central nervous system (CSN) involvement in schistosomiasis is an ectopic manifestation with a large variety of clinical forms, including pseudotumoral, which occurs in isolated cases and is rare. Three patients with epidemiological indications of this pathology were examined; the clinical picture included lower-back pain irradiating to lower limbs, associated with progressive flaccid paraparesis and sphincterial disturbances in cases in which the spinal chord was involved; while in cases with encephalitic impairment, headache, dizziness and cerebellar syndrome, characterized by dysarthria and right-side dysgraphia, were present. Magnetic resonance imaging (MRI) showed a growing process in all cases; cerebrospinal fluid (CSF) characteristics and biological markers were compatible with neuroschistosomiasis (NS). Biopsy of the lesions confirmed this diagnosis in one case. After specific treatment with schistosomicides and corticosteroids, clinical, radiological and laboratorial improvement was observed.

Pseudotumoral form of neuroschistosomiasis: report of three cases

Central nervous system (CSN) involvement in schistosomiasis is an ectopic manifestation with a large variety of clinical forms, including pseudotumoral, which occurs in isolated cases and is rare. Three patients with epidemiological indications of this pathology were examined; the clinical picture included lower-back pain irradiating to lower limbs, associated with progressive flaccid paraparesis and sphincterial disturbances in cases in which the spinal chord was involved; while in cases with encephalitic impairment, headache, dizziness and cerebellar syndrome, characterized by dysarthria and right-side dysgraphia, were present. Magnetic resonance imaging (MRI) showed a growing process in all cases; cerebrospinal fluid (CSF) characteristics and biological markers were compatible with neuroschistosomiasis (NS). Biopsy of the lesions confirmed this diagnosis in one case. After specific treatment with schistosomicides and corticosteroids, clinical, radiological and laboratorial improvement was observed.

Myeloradiculitis: a rare event in schistosoma infection.

Schistosomiasis a parasitic disease caused by trematodes is widely distributed in (sub-)tropical countries. Depending on the species the infection manifests clinically as gastrointestinal (preferentially Schistosoma mansoni and S. japonicum) or urinary (preferentially S. haematobium) disorders. Here we present an uncommon case of myeloradiculitis leading to bladder palsy and sensory loss at the lower limbs.

Serum and cerebral spinal fluid levels of chemokines and Th2 cytokines in Schistosoma mansoni myeloradiculopathy.

Schistosomal myeloradiculopathy (SMR) is the most common neurological form of Schistosoma mansoni infection. In this study we investigated the expression of chemokines and Th2 cytokines in serum and cerebral spinal fluid (CSF) of SMR patients. SMR patients presented increased serum levels of CCL11/eotaxin and CCL24/eotaxin-2 when compared to controls. SMR patients also had higher levels of IL-13 in CSF. Thus, SMR patients present enhancement of both IL-13 and CCR3 acting chemokines, both of which may facilitate the expression of a Th2 response and Th2-dependent damage to the spinal cord. As this cytokine is responsible for promoting Th2 responses, this finding is in accordance to the view that Th2 cells are important in the immunological process against the S. mansoni.

Intrathecal cytokines in spinal cord schistosomiasis.

We investigate the cytokine profile in the cerebrospinal fluid (CSF) and serum of patients with spinal cord schistosomiasis (SCS). Increased levels of IL-1beta, IL-4, IL-6 and IL-10 and low concentrations of TNF-alpha and IFN-gamma were observed in both CSF and serum. CSF showed higher levels of IL-4 and IL-6 when compared to the paired serum samples. A negative correlation between the concentrations of IL-10 and IFN-gamma was observed in the CSF. These findings suggest an inflammatory as well as a skewed type-2 immune response that probably occur both locally and systemically and may be involved in the pathogenesis of SCS.

Lumbar intramedullary spinal schistosomiasis presenting as progressive paraparesis: case report.

OBJECTIVE AND IMPORTANCE: Blood fluke infection or schistosomiasis affects up to 300 million people worldwide. Although infection of the hepatic and urogenital systems commonly occurs, central nervous system involvement is rare. When presenting in the spinal cord, schistosomiasis can be difficult to diagnose because it can present as mass lesion or transverse myelitis. We describe a patient with lumbar intramedullary spinal schistosomiasis who presented to a tertiary medical center in the United States and discuss the diagnosis and treatment of this rare disease. CLINICAL PRESENTATION: A 25-year-old Brazilian man presented with progressive, subacute, bilateral lower extremity motor and sensory deficits and disturbances in bladder function. Magnetic resonance imaging revealed a diffusely enhancing mass expanding the region of the conus medullaris. INTERVENTION: Laboratory investigation disclosed serum and cerebrospinal fluid eosinophilia. Ultimately, cerebrospinal fluid serology demonstrated the presence of Schistosoma mansoni. The patient was treated with praziquantel and steroids and made a marked recovery. CONCLUSION: The clinical and radiographic manifestations of spinal schistosomiasis can mimic those of intra-axial spinal tumors and transverse myelitis. To avoid unnecessary surgery or delay in treatment, the clinician must have knowledge of this type of presentation. The increasing volume of international travel and high prevalence of the disease worldwide increases the possibility that the practicing neurosurgeon in the United States may encounter this rare but treatable disease.

[Cerebrospinal fluid in the diagnosis of spinal schistosomiasis]. / Líquido cefalorraquidiano no diagnóstico da esquistossomose raquimedular.

Cerebrospinal fluid (CSF) changes in spinal schistosomiasis have been described. Its characteristic features are mild to moderate pleocytosis, presence of eosinophils, slight to moderate protein increase, elevated gamma globulin concentration and a positive immune assay. Nevertheless, these abnormalities are not always present together and therefore difficulties may arise in the assessment of the diagnosis. The purpose of this paper is to evaluate the importance of each CSF alteration concerning the diagnosis in 22 cases of spinal schistosomiasis. According to the results, only 20% of the cases had all the five feature that are considered to be characteristic of spinal schistosomiasis. Abnormal cell count was present in 86%, protein increase in 77.3%, immunoglobulin G increase in 60,8%, eosinophils were present in 36.8% and indirect fluorescent antibody test was positive in 68.2%. In three cases all CSF parameters studied were within the normal limits. As the most specific test among those described was the indirect fluorescent antibody test, it should be regarded for the diagnosis.

Líquido cefalorraquidiano no diagnóstico da esquistossomose raquimedular / Cerebrospinal fluid in the diagnosis of spinal schistosomiasis

As três espécies de esquistossoma podem comprometer o sistema nervoso. O S. mansoni é responsável pela esquistossomose no Brasil, sendo a mielopatia uma forma grave desta helmintose. O propósito deste trabalho é analisar as alterações do líquido cefalorraquidiano (LCR) para dar mais subsídios para o diagnóstico da esquistossomose raquimedular. Fizeram parte deste estudo 22 amostras de LCR de pacientes com esquistossomose espinal. Os resultados das análises destas amostras mostraram que a associação de alterações do LCR com quadro inflamatório e RIFI-IgM positiva ocorreu em 88 por cento dos pacientes, que o eosinófilo esteve presente em apenas 7 amostras (36,8 por cento), e que 3 dos 22 pacientes estudados apresentaram LCR normal. Conclui-se que o exame de LCR é coadjuvante muito útil para o diagnóstico da neuroesquistossomose.

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.

[Myelopathy: clinical considerations and etiological aspects]. / Mielopatias: considerações clínicas e aspectos etiológicos.

We studied 97 inpatients with myelopathy at the University Hospital Lauro Wanderley from 1985 to 2002. Ages ranged from 10 to 78 years and average age was 39.8 years. All patients were examined by neurologist. Cerebrospinal fluid (CSF) was analysed in 44 (45.3%) cases and immunofluorescence reactions and/or ELISA in CSF were made in 32 (32.9%) cases. Radiologic investigation of the vertebral spine and spinal cord of the 32 (32.9%) cases consisted of 8 radiographies, 10 computed tomography and 14 magnetic resonance image. In 41 (42.2%) cases the lesions were situated in the lower dorsal and lumbar level and complete transverse lesion were registred in 61 (62.8%) cases. There was predominance of transverse myelitis (43.2%) and spinal cord compression (15.46%). Spinal cord schistosomiasis was the form more often of the transverse myelitis (13.4%).

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.