Neuroschistosomiasis mansoni: literature review and guidelines.

BACKGROUND: Schistosomiasis is a tropical disease caused by worms of the genus Schistosoma. It is endemic in the Caribbean Islands, the middle east, eastern Asia, South America, and Africa. In nonendemic areas, physicians should be aware of this condition in travelers returning from endemic areas and in immigrants. The main disease-causing species are Schistosoma haematobium, Schistosoma mansoni, and Schistosoma japonicum. Neuroschistosomiasis is an ectopic form of the disease that is mainly associated with S. japonicum infection. Involvement of the central nervous system (CNS) in S. mansoni infection is neglected and underestimated. Neuroschistosomiasis mansoni can be classified into cerebral, spinal, and encephalomyelitic forms in the course of an acute or chronic infection. REVIEW SUMMARY: We review the CNS involvement by S. mansoni infection with an emphasis on life cycle, epidemiology, pathophysiology and immunology, clinical manifestations, diagnostic criteria, differential diagnosis, current treatment guidelines, and prognosis. CONCLUSIONS: Although an underreported CNS infection, found mainly in underdeveloped countries, neuroschistosomiasis mansoni still causes significant incapacity and morbidity. Hence, neurologists should become familiar with this infection worldwide and include it in the differential diagnosis of CNS involvement in travelers returning from endemic areas and in immigrants.

HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) inflammatory network.

HTLV-1 associated myelopathy/ tropical spastic paraparesis (HAM/TSP) is a systemic immune-mediated inflammatory disease and tissues other than nervous can be damaged, mainly ocular, rheumatic and dermatologic. Over 90% of HTLV-1-infected individuals remain lifelong asymptomatic and this retrovirus persists indefinitely in their CD4+ T-lymphocytes. The infection is maintained due to the proliferation of lymphocytes that harbor a provirus and express HTLV-1 proteins, particularly Tax, promoting an active and selective expansion of infected T cells. High proviral load is related to disease progression, which is correlated to disequilibrium between host and virus. Cytotoxic T lymphocytes are abundant and chronically activated in asymptomatic carriers and in HAM/TSP patients. The asymptomatic carriers were shown to have a high frequency of pro-inflammatory monocytes and anti-inflammatory IL-10+CD4+ and IL-10+CD8+ T-cells, as an immunoregulatory mechanism to counterbalance the monocyte-derived TNF-alpha. A putative immunomodulatory event would be the key to control their overall immunological status. In HAM/TSP, a pro-inflammatory microenvironment is the hallmark of the immunological profile. Enhanced frequency of activated CD8+ T-cells (HLA-DR+) in combination with high CD18 surface expression has been seen. In blood and cerebrospinal fluid, increased levels of Type-1 cytokines, as interferon-(IFN)-gamma, Tumor Necrosis Factor (TNF)-alpha, Interleukin (IL)-2, and pro-inflammatory IL-6, can be found. Concerning the progression, HLA polymorphisms may influence HAM/TSP and the allele HLA-A*2 has been associated with protection. The authors showed that HAM/TSP is strongly associated with a decreased percentage of B-cells, with enhanced T/B-cell ratio and activated CD8+ T-cells. These immunological parameters have been proposed as a prognostic biomarker for HAM/TSP.

Proposal for diagnostic criteria of tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM).

After the first description of TSP/HAM in 1985 and the elaboration of WHO's diagnostic criteria in 1988, the experience of the professionals in this field has increased so that a critical reappraisal of these diagnostic guidelines was considered timely. Brazilian neurologists and observers from other countries met recently to discuss and propose a modified model for diagnosing TSP/HAM with levels of ascertainment as definite, probable, and possible, according to myelopathic symptoms, serological findings, and/or detection of HTLV-I DNA and exclusion of other disorders.

Treatment of schistosomal myeloradiculopathy with praziquantel and corticosteroids and evaluation by magnetic resonance imaging: a longitudinal study.

BACKGROUND: The best treatment for schistosomal myeloradiculopathy (SMR) remains undefined. There is also no longitudinal study to estimate the value of magnetic resonance imaging (MRI) in the diagnosis and follow-up of this disease. METHODS: Patients with the following presentation were considered for study: lumbar and/or lower limb pain; lower limb weakness; anesthesia, hypoesthesia, or paresthesia; bladder and/or intestinal dysfunction; and sexual impotence. Sixteen patients with SMR were treated with oral praziquantel (50 mg/kg in a single dose) and methylprednisolone (15 mg/kg/day intravenously for 5 days) followed by prednisone (1 mg/kg/day orally for 6 months). Clinical outcome was prospectively evaluated in months 2 and 6 of treatment. RESULTS: Image alterations were detected by MRI at diagnosis for all patients, and normalization or improvement was reported at the end of treatment. There was statistically significant clinical melioration at both the second and sixth months of therapy for most neurological alterations. However, the best clinical outcome was achieved when the steroid was given for >2 months. CONCLUSIONS: Treatment with praziquantel associated with corticosteroids was successful in all cases. MRI proved to be a good method for the diagnosis of SMR and helpful in the evaluation of response to treatment.

Schistosomal myeloradiculopathy due to Schistosoma mansoni: report on 23 cases

Schistosoma mansoni infection is likely to be responsible for a significant proportion of cases of myelopathy occurring in areas where schistosomiasis is endemic. The aim of this study is to describe the clinical, laboratory and therapeutic data of 23 patients with schistosomal myeloradiculopathy. The medical records of 23 patients with schistosomal myelopathy admitted to two general hospitals of Belo Horizonte (MG), in Brazil, from 1995 to 1999, were reviewed retrospectively. Seventeen patients were male (74 percent). The mean age for the whole group was 27 years. Lower limb weakness and associated lumbar and/or lower limb pain were reported by 20 patients (87 percent), and 16 (70 percent) were unable to walk. All individuals presented urinary retention and 19 (83 percent) complained of intestinal dysfunction. The treatment was based on the association of antischistosomal drugs and corticosteroids. Five patients (22 percent) presented a full response to treatment, 13 (57 percent) partial response without functional limitations and 4 (17 percent) partial improvement with limitations or no response. Three out of the 4 patients who stopped steroids before 45 days of treatment developed recurrence of the symptoms and signs of myelopathy. Our cases demonstrate the severe presentation of the disease and the data disclosed here suggest that treatment with steroids should be kept for months after clinical improvement