Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease. / Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert.

BACKGROUND AND OBJECTIVES: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. MATERIAL AND METHODS: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. RECOMMENDATIONS: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. CONCLUSION: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up.

Platelet-lymphocyte and neutrophil-lymphocyte ratios are prognostic but not predictive of response to abiraterone acetate in metastatic castration-resistant prostate cancer.

PURPOSE: Recently neutrophil-lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) have been reported to be inflammatory parameters that confer poorer outcome in metastatic castration-resistant prostate cancer (mCPRPC). However, these ratios have not been analyzed in patients treated with abiraterone acetate. We explored the relationship between different values of PLR and NLR and survival in mCPRCP treated with abiraterone and their possible relation with a prostate specific antigen (PSA) response. METHODS: We retrospectively analyzed 101 patients with mCRPC treated with abiraterone from January of 2012 to November of 2015 in two different hospitals. A cut-off value of 5 for NLR and 150 for PLR were used to compare survival by Kaplan-Meier method. Moreover, an association between these cut-off values and the PSA response was analyzed by a χ 2 test. RESULTS: In the case of NLR, the median DFS were 12, 1 months for NLR <5 and 7 months for NLR ≥5, p = 0.061. The median OS were 23.9 months for NLR <5 and 16.3 months for NLR ≥5, p = 0.046. In the case of PLR, the median DFS were 11.8 months for PLR <150 and 10.6 months for PLR ≥150, p = 0.549. The median OS were 27.4 months for PLR <150 and 15.9 months for PLR ≥150, p = 0.005. It was not observed a correlation between the different cut-off values of PLR or NLR and a PSA response ≥25% (p = 0.31). CONCLUSIONS: It is shown a better prognostic relationship between PLR and NLR low values and OS that is statistically significant in mCPRC patients treated with abiraterone. Furthermore, it was not shown a relation between PLR and NLR values and PSA response.

GHG and black carbon emission inventories from Mezquital Valley: The main energy provider for Mexico Megacity.

The greenhouse gases and black carbon emission inventory from IPCC key category Energy was accomplished for the Mezquital Valley, one of the most polluted regions in Mexico, as the Mexico City wastewater have been continuously used in agricultural irrigation for more than a hundred years. In addition, thermoelectric, refinery, cement and chemistry industries are concentrated in the southern part of the valley, near Mexico City. Several studies have reported air, soil, and water pollution data and its main sources for the region. Paradoxically, these sources contaminate the valley, but boosted its economic development. Nevertheless, no research has been done concerning GHG emissions, or climate change assessment. This paper reports inventories performed by the 1996 IPCC methodology for the baseline year 2005. Fuel consumption data were derived from priority sectors such as electricity generation, refineries, manufacturing & cement industries, transportation, and residential use. The total CO2 emission result was 13,894.9 Gg, which constituted three-quarters of Hidalgo statewide energy category. The principal CO2 sources were energy transformation (69%) and manufacturing (19%). Total black carbon emissions were estimated by a bottom-up method at 0.66 Gg. The principal contributor was on-road transportation (37%), followed by firewood residential consumption (26%) and cocked brick manufactures (22%). Non-CO2 gas emissions were also significant, particularly SO2 (255.9 Gg), which accounts for 80% of the whole Hidalgo State emissions. Results demonstrated the negative environmental impact on Mezquital Valley, caused by its role as a Megacity secondary fuel and electricity provider, as well as by the presence of several cement industries.

Influence of fatigue on construction workers' physical and cognitive function.

BACKGROUND: Despite scientific evidence linking workers' fatigue to occupational safety (due to impaired physical or cognitive function), little is known about this relationship in construction workers. AIMS: To assess the association between construction workers' reported fatigue and their perceived difficulties with physical and cognitive functions. METHODS: Using data from a convenience sample of US construction workers participating in the 2010-11 National Health Interview Survey two multivariate weighted logistic regression models were built to predict difficulty with physical and with cognitive functions associated with workers' reported fatigue, while controlling for age, smoking status, alcohol consumption status, sleep hygiene, psychological distress and arthritis status. RESULTS: Of 606 construction workers surveyed, 49% reported being 'tired some days' in the past 3 months and 10% reported 'tired most days or every day'. Compared with those feeling 'never tired', workers who felt 'tired some days' were significantly more likely to report difficulty with physical function (adjusted odds ratio [AOR] = 2.03; 95% confidence interval [CI] 1.17-3.51) and cognitive function (AOR = 2.27; 95% CI 1.06-4.88) after controlling for potential confounders. CONCLUSIONS: Our results suggest an association between reported fatigue and experiencing difficulties with physical and cognitive functions in construction workers.

Therapeutic perspectives of epigenetically active nutrients.

Many nutrients are known for a wide range of activities in prevention and alleviation of various diseases. Recently, their potential role in regulating human health through effects on epigenetics has become evident, although specific mechanisms are still unclear. Thus, nutriepigenetics/nutriepigenomics has emerged as a new and promising field in current epigenetics research in the past few years. In particular, polyphenols, as part of the central dynamic interaction between the genome and the environment with specificity at physiological concentrations, are well known to affect mechanisms underlying human health. This review summarizes the effects of dietary compounds on epigenetic mechanisms in the regulation of gene expression including expression of enzymes and other molecules responsible for drug absorption, distribution, metabolism and excretion in cancer, metabolic syndrome, neurodegenerative disorders and hormonal dysfunction.

First clinical experience with a dedicated PET for hanging breast molecular imaging.

AIM: Recently, a high-resolution dedicated PET system for hanging breast imaging (MAMMI PET) has been developed to improve primary tumor detection and characterization. The aim of this pilot study was to assess its feasibility for tumor detection and FDG uptake measurements in patients with stage II and III breast cancer. METHODS: Thirty-two patients with invasive breast cancer (26 ductal, 4 lobular, 2 other), prior to and/or during neoadjuvant chemotherapy, underwent both conventional PET/CT and MAMMI PET in prone position with hanging breasts. Conventional PET/CT and MAMMI PET were performed 60±10 min and 110±10 min after injection of 180-240 MBq of FDG, respectively. Primary tumor detection was assessed and FDG uptake, expressed as maximum standardized uptake value (SUVmax), was calculated. RESULTS: Both MAMMI PET and conventional PET/CT visualized the primary tumor in 31 patients (97%). The mean distance from the tumor to the pectoral muscle was 26.4mm (smallest distance 3.3mm). Agreement in FDG uptake between PET/CT and MAMMI PET was high (r=0.86, 95% CI 0.69-0.94). However, SUVmax as assessed with MAMMI PET was consistently higher than with PET/CT in all patients with an average ratio of 2.7. CONCLUSION: The dedicated high-resolution breast PET with hanging breast technique is able to visualize approximately all breast tumors in stage II and III breast cancer patients, including tumors in the vicinity of the thoracic wall. This may enable its sequential use in the assessment of response in breast cancer patients receiving neoadjuvant systemic therapy, although SUVmax values are not directly comparable to standard PET/CT.

Health status and behaviors among adults residing in rural Dominican Republic.

INTRODUCTION: The rapidly increasing burden of chronic diseases linked to adequacy of healthcare services and individual health behaviors is a key determinant of global public health. Given demographic aging and the accompanying health transition, chronic diseases in low and middle income communities of the Dominican Republic are likely to increase significantly. The objective of this article was to report on efforts in surveillance of health conditions and behaviors in underserved rural Dominican communities. METHODS: A modified 30 item, language-sensitive health survey was randomly administered to 117 adult participants (18 years and older) during a health fair held at three rural villages from March to April 2009 in the rural San Cristobál region of the Dominican Republic. Descriptive analyses of select health conditions and behavior variables from all completed surveys were tabulated. RESULTS: Adult participant ages ranged from 18 to 79 years (mean ± standard deviation; 34.0 ± 2.1), height from 1.4 to 2.0 m (1.7 ± 0.1), weight from 41.8 to 100.0 kg (66.2 ± 1.7) and BMI from 15.2 to 46.2 (24.2 ± 0.7). Overall, 69.2% of the sample self-reported their general health status to be fair to poor. The top three chronic diseases included: high blood pressure (35.8%), diabetes (15.0%), and asthma (14.2%). In all, 33.4% reported current smoker status and 61.7% were classified as heavy alcohol drinkers. CONCLUSION: Considerable variation was found in the self-report of health conditions and behavioral characteristics among those individuals that attended the health fair. Documenting these important health indicators in the rural communities has the potential to inform the development of surveillance activities and prevention efforts for future health education interventions.

Lack of toxicity to adults of the Mexican fruit fly (Diptera: Tephritidae) of beta-exotoxin in Bacillus thuringiensis endotoxin preparations.

beta-Exotoxin (thuringiensin) was found in high titers in centrifugation supernatants and acetone/lactose powders produced from centrifugation pellets of strains Guat 1 and HD 2 of Bacillus thuringiensis (Berliner). Diets containing powders of either strain were toxic, diets containing Guat 1 supernatant were not toxic, diets containing HD 2 supernatant were slightly toxic, and diets containing powders or supernatants from uninoculated culturing medium spiked with beta-exotoxin were not toxic. Most mortality occurred within 3 d when flies fed on powders but not until 6-7 d when flies fed on HD 2 supernatant. These results indicated that the primary toxic principals of the powders were endotoxins/spores and that beta-exotoxin alone was not toxic to adult flies at the concentrations found in the supernatants or powders.

Inhibition of peridural fibrosis after laminectomy using low-dose external beam radiation in a dog model.

OBJECTIVE: Clinical studies have demonstrated a significant association between the presence of extensive postlumbar discectomy peridural scar formation and the recurrence of low back and radicular pain. Low-dose perioperative radiation therapy has previously been demonstrated to inhibit peridural fibrosis after laminectomy in a rat model. The current study was designed to measure the effect of low-dose radiation on postlaminectomy peridural fibrosis development in a larger animal model. METHODS: Three dogs underwent a total of 12 lumbar hemilaminectomies. For each animal, two levels received 1) external beam radiation 24 hours before surgery, 2) surgery alone, or 3) radiation alone. Radiation was administered in a single fraction of 700 cGy using computed tomographic guidance for dosimetry planning. The isodose distribution was such that the dose conformed to the posterior epidural space with minimal exit dose to normal tissue. Port films were used to confirm the correct levels. Gadolinium-enhanced magnetic resonance imaging (MRI) of the lumbar spines was obtained before the animals were killed 12 weeks after surgery. The spines were harvested, and axial sections through the laminectomy defect were stained with hematoxylin and eosin and Masson's trichrome. All specimens were evaluated for extent of fibrosis along the dura, density of fibrosis, nerve root entrapment, and sublaminar fibrosis. RESULTS: There were no complications from the surgery, and no new neurological deficits were noted. There was a statistically significant difference between the irradiated and nonirradiated groups regarding the extent of fibrosis (P = 0.001) and the density of fibroblasts (P = 0.001). There was also a marked difference in nerve root entrapment (P = 0.182) and the presence of sublaminar fibrosis (P = 0.061) between the treatment and control groups. MRI revealed less gadolinium enhancement at the irradiated levels compared with the nonirradiated levels, confirming the usefulness of MRI in predicting the degree of epidural fibrosis. CONCLUSION: Low-dose external beam radiation therapy administered 24 hours before laminectomy in a dog model significantly decreased the extent and density of peridural fibrosis as well as nerve root entrapment and sublaminar fibrosis. This treatment strategy may be efficacious in patients with recurrent radicular pain after lumbar discectomy that is thought to be secondary to peridural fibrosis on the basis of gadolinium-enhanced MRI studies, and who might benefit from reoperation for nerve root decompression.

Use of cytological preparations for the intraoperative diagnosis of stereotactically obtained brain biopsies: a 19-year experience and survey of neuropathologists.

OBJECT: The goals of this study were to analyze the accuracy of cytological techniques, consisting of touch and smear preparations, for the intraoperative diagnosis of stereotactically obtained brain biopsy samples, and to determine the prevalence of the use of these methods among neuropathologists. METHODS: A survey regarding preferred methods for intraoperative diagnosis of stereotactically obtained brain biopsy samples was completed by 92 (62%) of 148 neuropathologists. Twenty-three percent of respondents chose frozen-section examination alone; 13% chose one or more cytological methods alone; and the remainder (64%) chose a combination of frozen-section examination and cytology. At the University of Pittsburgh, the neuropathology records for all stereotactic brain biopsies performed from May 1979 through May 1998 were retrospectively reviewed. Of the 946 stereotactic brain biopsies, 316 cases were excluded because the intraoperative neuropathological consultation was not recorded. Thirty-five cases were excluded because frozen-section examinations were performed. Therefore, a total of 595 cases were suitable for analysis. Intraoperative cytological investigation correlated with the final diagnosis in 90% of cases (52% complete correlation and 38% partial correlation). In 11% of cases there was no correlation between the intraoperative and final diagnoses. Intraoperative diagnoses were most accurate in cases of abscess, germinoma, lymphoma, metastasis, and malignant glioma. Overall, 91% of biopsy specimens were diagnostic when examined using the paraffin-embedded section technique. The sensitivity of cytological preparations in detecting a diagnostic specimen was 96% and the specificity in detecting a nondiagnostic specimen was 75%. CONCLUSIONS: Intraoperative cytological preparations correlated with the final diagnoses in 90% of stereotactic biopsies and had a 96% sensitivity in detecting diagnostic specimens. The highest rate of correlation was noted in cases of abscess, germinoma, lymphoma, metastasis, and malignant glial tumor.

The neuropathology of intestinal failure and small bowel transplantation.

The aim of this study was to elucidate the neuropathological substrate of intestinal failure before and after small bowel transplantation (SBT). Retrospective analysis of complete autopsy or brain biopsy specimens of 17 patients with intestinal failure (12 children and 5 adults) were studied. Patients were divided into two groups. Group I (transplanted group; n = 13) included those patients who underwent intestinal transplantation under tacrolimus and steroids immunosuppressive therapy. Group II (control group) included 4 children with intestinal failure who were candidates for SBT and died while awaiting an intestinal allograft. Central nervous system (CNS) abnormalities were seen in 92% of the SBT recipients and in 100% of SBT candidates. The neuropathological lesions of SBT recipients included: (a) vascular lesions: global brain ischemia, infarcts, intracranial hemorrhage and edema (7 children/2 adults; 69%); (b) cerebral atrophy (6 children; 46%); (c) Alzheimer type II gliosis (5 children/4 adults; 69%); (d) infection (3 patients; 23%) due to cytomegalovirus (1 child), Aspergillus fumigatus (1 adult) and progressive multifocal leukoencephalopathy (PML)-like (1 adult); (e) Epstein-Barr virus-related cerebral post-transplant lymphoproliferative disorder (2 children; 15%); and (f) central pontine and extrapontine myelinolysis (1 child; 7.5%). The neuropathological lesions of SBT candidates were Alzheimer type II astrocytosis (4 patients), vascular changes (4 patients), brain atrophy (4 patients) and cerebral candidiasis (1 patient). CNS vascular, metabolic and infectious pathology are significant causes of morbidity and mortality in patients suffering intestinal failure, both before and after SBT. Brain atrophy was a frequent finding and may be related to nutritional and developmental inadequacy of long-term total parenteral nutrition.

Beneficial effects of the radioprotectant 21-aminosteroid U-74389G in a radiosurgery rat malignant glioma model.

PURPOSE: To evaluate the radioprotectant effects of the 21-aminosteroid U-74389G on the rat C6 glioma model after stereotactic radiosurgery. Because radiosurgery causes both tumor cytotoxicity, as well as regional brain edema, we hypothesized that this drug might exhibit advantageous or deleterious effects on healthy and neoplastic tissue. METHODS: Rats were implanted with 10(6) C6 glioma cells into the right frontal brain and randomized to a Control Group (n = 18), radiosurgery on Day 14 (50% isodose = 35 Gy) (n = 15), or radiosurgery preceded by a single 15 mg/kg intravenous dose of 21-aminosteroid (n = 27). All animals were killed by 90 days and evaluated for survival, tumor size, the presence or absence of regional parenchymal edema, or radiation-induced vasculopathy. RESULTS: After tumor implantation, median survival in the Control Group was 23 days. Significant improvements in median survival were noted after RS alone (median, 31 days; p = 0.02), and RS plus 21-aminosteroid (median, 59 days; p < 0.0001). In the Control Group, mean tumor diameter was 5.4 mm. After RS alone, the mean diameter was 3.2 mm (p = 0.002), and after RS plus 21-aminosteroid, 2.9 mm (p = 0.0002). In the Control Group, the tumor grew as a hypercellular, compact mass. Only 3 of 18 animals had peritumoral edema. In contrast, 7 of 15 animals in the RS group had evidence of edema (p = 0.006), but rats that received 21-aminosteroid showed no increase compared to controls (p = 0.38). Similarly, 6 of 15 animals that had radiosurgery alone showed evidence of vasculopathy (p = 0.005) compared to no animals in the control group and only 2 of 27 aminosteroid-treated animals. CONCLUSIONS: The 21-aminosteroid U-74389G exhibits a radioprotectant effect on normal brain tissue, but does not appear to protect the tumor in an in vivo rat radiosurgery model. We believe that the observed beneficial effects on healthy brain led to significant prolongation of animal survival; perhaps, by limiting the adverse effects of high-dose radiosurgery. This radioprotectant should now be evaluated in randomized clinical trials in patients with malignant brain tumors.

Inhibition of peridural fibrosis after laminectomy using low-dose external beam radiation in a rat model.

OBJECTIVE: Clinical studies have revealed a significant association between the presence of extensive postlumbar discectomy peridural scar formation and the reoccurrence of low back and radicular pain. Low-dose perioperative radiation therapy has been shown to inhibit scar formation. Its effect on peridural fibrosis, however, has not been studied. METHODS: Thirty male Sprague-Dawley rats underwent L5 laminectomies. Ten rats each received a single fraction of 700-cGy external beam radiation to the lumbar spine 24 hours before surgery; 10 rats each received 700 cGy 24 hours after surgery. The remaining 10 rats served as a control group. All of the rats were killed 30 days after surgery. The spines were harvested, and axial histological sections through the laminectomy defect were evaluated. Each specimen was scored for extent, density, and arachnoidal involvement by fibrosis. RESULTS: There was a statistically significant difference between the treatment and control groups regarding the extent of fibrosis along the dura (P < 0.001), the density of fibroblasts (P < 0.005), and the arachnoid involvement (P < 0.01). There was no difference in fibrosis reduction between the groups receiving pre- and postlaminectomy radiation. CONCLUSION: Low-dose external beam radiation therapy administered before or after laminectomy in a rat model significantly decreases the extent, density, and arachnoidal involvement of peridural fibrosis. This technique may improve the outcome of patients who undergo reoperations for recurrent radicular and/or low back pain after successful lumbar discectomy in whom there is a significant amount of peridural fibrosis.

Radiotherapy for nonfunctional pituitary adenoma: analysis of long-term tumor control.

OBJECT: The authors studied outcomes in patients who had undergone radiotherapy for nonfunctional pituitary adenoma to assess long-term tumor control and to identify factors affecting tumor control such as higher radiation doses, improved imaging, and histological characteristics of the tumor. METHODS: In this retrospective study, the authors evaluated 120 patients who received radiotherapy for nonfunctional pituitary adenomas between 1960 and 1991. The median follow-up period was 9 years (range 1 month-32 years). Radiation doses varied between 37.6 and 65.6 Gy (median 46.7 Gy). Tumors progressed in 15 of the 120 patients by 1 to 25 years after radiotherapy. Actuarial tumor control rates at 10, 20, and 30 years were 87.5+/-3.6%, 77.6+/-6.3%, and 64.7+/-12.9%, respectively. Tumor progression after radiotherapy occurred significantly more often (p=0.0397) in patients with oncocytoma than in patients with nononcocytic null cell adenoma. No other factors correlated significantly with tumor control. One case of optic and oculomotor neuropathy developed 4.5 years after a maximum dose of 50 Gy in 25 fractions. Radiation-induced neoplasms (meningioma and glioblastoma multiforme) developed at a rate of 2.7% at 10 and 30 years. CONCLUSIONS: The oncocytic variant of null cell pituitary adenoma appears less sensitive to control by radiotherapy than nononcocytic undifferentiated cell adenoma. A follow-up period extending beyond 20 years is needed adequately to assess the efficacy of radiotherapy for tumor control. Doses of 40 or 45 Gy in 20 or 25 fractions, respectively, appear optimal.

Death from a malignant cerebellopontine angle triton tumor despite stereotactic radiosurgery. Case report.

Malignant vestibular nerve tumors are rare: to date, only three cases have been reported in the literature. The authors report a case of an eighth cranial nerve tumor that progressed 5 years after stereotactic radiosurgery. The patient was a 44-year-old man who underwent stereotactic radiosurgery for a 27-mm cerebellopontine angle tumor that was discovered on investigation of tinnitus and hearing loss. He developed facial weakness after 5 years, and repeated imaging revealed tumor enlargement. Despite complete microsurgical excision, the tumor rapidly recurred locally and subsequently disseminated within the neuraxis. The patient died 1 year after tumor progression was detected. Histopathological analysis revealed a malignant spindle cell neoplasm with frequent mitotic figures. The presence of positive rhabdoid elements on immunohistochemical studies confirmed that it was a triton tumor. The authors review the relevant literature concerning the classification and management of malignant vestibular nerve tumors and discuss the implications of tumor progression after stereotactic radiosurgery.

The neuropathology of organ transplantation: comparison and contrast in 500 patients.

The main objective of this report is to compare and contrast the type and frequency of neuropathological findings following liver, heart, lung, heart-lung, kidney and bone marrow transplantation and to provide an overview of the major systemic complications in patients that received allografts. This is a retrospective analysis of the complete autopsy records and clinical histories of 500 adults who underwent organ transplantation at the University of Pittsburgh Medical Center during the interval of March, 1981 through July, 1997. This study is based on the neuropathological and systemic findings among 225 liver, 101 heart, 40 lung, 28 heart-lung, 74 kidney and 32 bone marrow transplants. Clinico-pathological correlations were made. All patients received base-line immunosuppressive therapy with one or more of the following drugs: cyclosporine, corticosteroids and azathioprine. Since August, 1989, the primary immunosuppressive agent is FK-506 (Tacrolimus). Some patients received antilymphocyte globulin (OKT3), when acute rejection was imminent. Light microscopic examination of tissue sections, stained with hematoxylin and eosin and in some cases with special stains were made. Ultrastructural evaluation were also performed in selected cases. All of the studies were carried out at the University of Pittsburgh Medical Center, Department of Pathology, Neuropathology Division. Cerebrovascular complications were the most frequent and were seen in 51% of the liver, 59% of the heart, 58% of the lung, 50% of the heart-lung, 49% of the kidney and 44% of the bone marrow allografts. Aspergillus sp. infection was the most common of all CNS infections followed by viral, bacterial and protozoal. Primary Central Nervous System Lymphoma (PCNSL) was seen in 2% of the liver, and 2% of the heart recipients. Post transplant lymphoproliferative disorder (PTLD) involving the brain was seen in 2% of the liver allografts, 3% of the heart, and 7% of the heart-lung recipients. PTLD systemically was seen in 6% of the liver, 7% of the heart, 5% of the lung, 11% of the heart-lung and 4% of the kidney allografts. Graft-versus-host disease was seen only in 41% of the bone marrow recipients. There was no statistically significant difference between the incidence of the total CNS complications among the different organ transplant groups (p value > 0.10), but there was a statistically significant difference in the systemic complications among the organ transplant groups (p value < 0.001). In conclusion that immunocompromised patients with impaired cellular and humoral immunity are at risk for the development of opportunistic infections and hematologic abnormalities. PTLD appears to be different in its pathogenesis from that of PCNSL which occurs anew in the brain of these patients. The neurological complications may be reduced by earlier recognition and better understanding of their etiopathogenesis.

[Central nervous system infection by free-living amebas: report of 3 Venezuelan cases]. / Infección del sistema nervioso central por amebas de vida libre: comunicación de tres nuevos casos venezolanos.

INTRODUCTION: Infection of the Central Nervous System by free living amebas is an unusual event, 344 cases have been reported to date. The disease becomes evident in two different clinical fashions: Primary Amebic Meningoencephalitis (PAM) caused by Naegleria fowleri and Granulomatous Amebic Encephalitis (GAE) induced by Spp. of Acanthamoeba and Balamuthia. CLINICAL CASES: The authors report three new cases from Venezuela. Case 1. 34 years old man, with a chief complaint of general malaise, headache and fever, a diagnosis of common cold was made and the patient was treated as such, he did not improve and was admitted to the hospital with deterioration of his clinical status; the patient died 10 days after the onset of his illness which was determined to be GAE produced by Balamuthia mandrillaris. Case 2. 8 years old female admitted to the hospital because of fever, headache and generalized seizures of sudden onset; neurocysticercosis was diagnosed and following improvement the patient was discharged and readmitted on two occasions because of relapse and worsening of her illness, she died 2 months after the onset of her disease that was diagnosed by autopsy as GAE due to Balamuthia mandrillaris. Case 3. 16 years old male, previously healthy, who following immersion in a water tank was admitted to the hospital because of meningeal irritation that progressed to coma and death in a 7 day lapse; autopsy revealed PAM by Naegleria fowleri. The two cases of GAE due to Balamuthia mandrillaris occurred in apparently immunocompetent individuals, contrary to the statement that these microorganisms are opportunistic. CONCLUSION: We believe that neurological infection by amphizoic amebas is being underdiagnosed, probably due to ignorance regarding this pathology or because of a very low autopsy rate in most countries.