Clinical features of HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) in northeast Iran.

This study aimed to introduce clinical manifestations of patients in northeast Iran with HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and describe the epidemiological features, as well as risk factors for HTLV-1 infection. This is a cross-sectional study of HTLV-1 infected cases and HAM/TSP patients referred by outpatient neurology clinics as well as Mashhad Blood Transfusion Center from 2005 to 2010. The study comprises 513 cases, including 358 healthy carriers (HCs) and 145 HAM/TSP patients. The majority of carriers were male (73.5%), whereas 67.6% of HAM/TSP sufferers were female (P < 0.001). The mean age of HAM/TSP patients and HCs was 45.9 ± 13.6 and 39.5 ± 11.58 years, respectively (P < 0.001). The history of transfusion, surgery, hospitalization and cupping was observed in a significant greater number of HAM/TSP patients than the HCs (P < 0.001, P < 0.001, P < 0.001 and P = 0.029, respectively). Gait disturbance was the most common complaint in HAM/TSP patients (72.4%). This research develops an HTLV-1 data registry in an endemic area such as Mashhad which can serve useful purposes, including evaluation of clinical and laboratory characteristics of HAM/TSP patients and epidemiological data of HTLV-1-infected cases.

Konzo outbreak, in the south-west of the Democratic Republic of Congo, 1996.

In August 1996, cases of poliomyelitis were reported in Kahemba zone, in the south-west of the Democratic Republic (DR) of Congo. The diagnosis was reviewed and charged to Konzo, a spastic paraparesis attributed to food cyanide intoxication. In order to describe the phenomena, a community-based survey took place and found 237 people affected. The highest prevalence was found in the most isolated part of the zone. The patients suffered from an isolated non-progressive spastic paraparesis of abrupt onset. Children and women were the most affected groups, especially women after childbirth. Most of the patients developed the disease after 1990 with 101 cases in 1996. Cassava processing was the same over time and in all the villages. The study did not fully explain the increased number of cases in 1996 but suggested that complementary investigations regarding micronutrient intakes, especially vitamin A, would be necessary.

On the etiology of tropical spastic paraparesis and human T-cell lymphotropic virus-I-associated myelopathy.

The purpose of this review is to present some concepts on the etiology of tropical spastic paraparesis or human T-cell lymphotropic virus-I (HTLV-I)-associated myelopathy (TSP/HAM). The large number of syndromes that have been associated with HTLV-I (60 to date), the existence of TSP/HAM cases associated with other retroviruses (human immunodeficiency virus-2 [HIV-2], HTLV-II), the existence of many TSPs without HTLV-I, and the evidence of clear epidemiologic contradictions in TSP/HAM indicate that the etiopathogenesis of TSP/HAM is not yet clear. Tropical spastic paraparesis/HAM affects patients of all human ethnic groups, but usually in well localized and relatively isolated geographic regions where HTLV-I has been endemic for a long time. Environmental factors and geographic locations appear to be critical factors. Because the neuropathology of TSP/HAM suggests a toxometabolic, rather than a viral cause, it is proposed that an intoxication similar to neurolathyrism could account for some of TSP/HAM cases, mainly in tropical and subtropical countries. If this were the case, HTLV-I could be a cofactor or act as a bystander. it is possible that co-infection with another agent is necessary to produce TSP/HAM and most of the syndromes associated with HTLV-I.

[Coexistence of mass hysteria, konzo and HTLV-1 virus in the Democratic Republic of the Congo]. / Coexistence hystérie collective, konzo et virus HTLV-1 en République Démocratique du Congo.

A cross-sectional study was carried out in Pindi located 115 kilometers from Kikwit, Democratic Republic of the Congo to characterize a local school epidemic involving paralysis of the lower extremities, identify risk factors, and establish differential diagnosis with konzo and spastic paralysis related to human T-lymphotropic virus type 1 (HTLV-1). Data was obtained using a qualitative approach based on records, interviews, focus group technique, and neurological examination. Blood tests using the ELISA and western blot tests were performed to detect HTLV-1 and HIV 1 and 2. A total of 41 cases of paralysis were observed between 1994 and 1998. All patients were female and most (n = 28) were between the ages of 16 and 20 at the time of the study. The majority of cases were recorded in 1998 (31 prevalent cases and 16 incidents). Epidemiological data, clinical findings, and laboratory tests suggested that the etiology was mass hysteria with somatic conversion rather than toxic or viral causes in most cases. The psychosocial environment played an important role in the spread of the epidemic. These findings demonstrate the crucial role of the psychosocial environment in the occurrence of mass hysteria and support use of integrated health programs in developing countries.

An epidemic in Cuba of optic neuropathy, sensorineural deafness, peripheral sensory neuropathy and dorsolateral myeloneuropathy.

An epidemic outbreak of peripheral neuropathy affected Cuba in 1992-93 resulting in 50,862 cases (national cumulative incidence rate (CIR) 461.4 per 100,000). Clinical forms included retrobulbar optic neuropathy, sensory and dysautonomic peripheral neuropathy, dorsolateral myeloneuropathy, sensorineural deafness, dysphonia and dysphagia, spastic paraparesis, and mixed forms. For epidemiological purposes, cases were classified as optic forms (CIR 242.39) or peripheral forms (CIR 219.25). Increased risk was found among smokers (odds ratio (OR) 4.9), those with history of missing meals (OR 4.7) resulting in lower intake of animal protein, fat, and foods that contain B-vitamins, combined drinking and smoking (OR 3.5), weight loss (OR 2.8), excessive sugar consumption (OR 2.7) and heavy drinking (OR 2.3). Optic neuropathy was characterized by decreased vision, bilateral and symmetric central or cecocentral scotomata, and loss of color vision due to selective lesion of the maculopapillary bundles. Peripheral neuropathy was a distal axonopathy lesion affecting predominantly large myelinated axons. Deafness produced selective high frequency (4-8 kHz) hearing loss. Myelopathy lesions combined dorsal column deficits and pyramidal involvement of lower limbs with spastic bladder. Clinical features were those of Strachan syndrome and beriberi. Intensive search for neurotoxic agents, in particular organophosphorus esters, chronic cyanide, and trichloroethylene intoxication, yielded negative results. Treatment of patients with B-group vitamins and folate produced rewarding results. Most patients improved significantly and less than 0.1% of them remained with sequelae; there were no fatal cases. Supplementation of multivitamins to the entire Cuban population resulted in curbing of the epidemic. Overt malnutrition was not present, but a deficit of micronutrients, in particular thiamine, cobalamine, folate and sulfur amino acids appears to have been a primary determinant of this epidemic.