Síndrome de nieve visual y su relación con la migraña / Visual snow syndrome and its relationship with migraine

Introducción El síndrome de nieve visual (SNV) es un trastorno del sistema nervioso central que implica la visión de forma constante de pequeños puntos blancos y negros en la totalidad del campo visual. Desarrollo El SNV puede presentarse desde la infancia hasta la tercera edad, siendo más frecuente en jóvenes y sin diferencia entre géneros. En sus criterios diagnósticos se incluye la presencia de nieve visual, pero también otros fenómenos visuales como palinopsia, fotofobia, nictalopía y otros fenómenos visuales persistentes. La fisiopatología del SNV es desconocida, pero se postulan como mecanismos la hiperexcitabilidad del córtex visual y una disfunción en el procesamiento visual de orden superior. La prevalencia de migraña en los pacientes con SNV es alta en comparación con la población general y cuando se presentan conjuntamente los síntomas son más severos. No se dispone de un tratamiento eficaz, pero el fármaco con mejores resultados es la lamotrigina, recomendándose únicamente en casos seleccionados con alta limitación funcional. Conclusiones El síndrome de nieve visual es una entidad poco conocida e infradiagnosticada, pero el creciente número de investigaciones durante los últimos años ha permitido definir unos criterios diagnósticos y acercarnos a su fisiopatología. Es una entidad íntimamente relacionada con la migraña, con solapamiento de síntomas y probablemente mecanismos fisiopatológicos comunes. (AU)

Introduction Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. Development VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. Conclusions VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms. (AU)

Visual snow syndrome and its relationship with migraine.

INTRODUCTION: Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. DEVELOPMENT: VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. CONCLUSIONS: VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms.

Visual snow syndrome and its relationship with migraine. / Síndrome de nieve visual y su relación con la migraña.

INTRODUCTION: Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. DEVELOPMENT: VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. CONCLUSIONS: VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms.

B Cell Synovitis and Clinical Phenotypes in Rheumatoid Arthritis: Relationship to Disease Stages and Drug Exposure.

OBJECTIVE: To define the relationship of synovial B cells to clinical phenotypes at different stages of disease evolution and drug exposure in rheumatoid arthritis (RA). METHODS: Synovial biopsy specimens and demographic and clinical data were collected from 2 RA cohorts (n = 329), one of patients with untreated early RA (n = 165) and one of patients with established RA with an inadequate response to tumor necrosis factor inhibitors (TNFi-IR; n = 164). Synovial tissue was subjected to hematoxylin and eosin and immunohistochemical staining and semiquantitative assessment for the degree of synovitis (on a scale of 0-9) and of CD20+ B cell infiltrate (on a scale of 0-4). B cell scores were validated by digital image analysis and B cell lineage-specific transcript analysis (RNA-Seq) in the early RA (n = 91) and TNFi-IR (n = 127) cohorts. Semiquantitative CD20 scores were used to classify patients as B cell rich (≥2) or B cell poor (<2). RESULTS: Semiquantitative B cell scores correlated with digital image analysis quantitative measurements and B cell lineage-specific transcripts. B cell-rich synovitis was present in 35% of patients in the early RA cohort and 47.7% of patients in the TNFi-IR cohort (P = 0.025). B cell-rich patients showed higher levels of disease activity and seropositivity for rheumatoid factor and anti-citrullinated protein antibody in early RA but not in established RA, while significantly higher histologic synovitis scores in B cell-rich patients were demonstrated in both cohorts. CONCLUSION: We describe a robust semiquantitative histologic B cell score that closely replicates the quantification of B cells by digital or molecular analyses. Our findings indicate an ongoing B cell-rich synovitis, which does not seem to be captured by standard clinimetric assessment, in a larger proportion of patients with established RA than early RA.

Life cycle and human health risk assessments as tools for decision making in the design and implementation of nanofiltration in drinking water treatment plants.

A combined methodology using life cycle assessment (LCA) and human health risk assessment (HHR) is proposed in order to select the percentage of water in drinking water treatment plants (DWTP) that should be nanofiltered (NF). The methodological approach presented here takes into account environmental and social benefit criteria evaluating the implementation of new processes into conventional ones. The inclusion of NF process improves drinking water quality, reduces HHR but, in turn, increases environmental impacts as a result of energy and material demand. Results from this study lead to balance the increase of the impact in various environmental categories with the reduction in human health risk as a consequence of the respective drinking water production and consumption. From an environmental point of view, the inclusion of NF and recommended pretreatments to produce 43% of the final drinking water means that the environmental impact is nearly doubled in comparison with conventional plant in impact categories severely related with electricity production, like climate change. On the other hand, the carcinogenic risk (HHR) associated to trihalomethane formation potential (THMFP) decreases with the increase in NF percentage use. Results show a reduction of one order of magnitude for the carcinogenic risk index when 100% of drinking water is produced by NF.

Afección neurológica en la enfermedad de Whipple en forma de polineuropatía de fibra fina / Small-fibre polyneuropathy in Whipple’s disease

No disponible

Eighteen month study of continuous intraduodenal levodopa infusion in patients with advanced Parkinson's disease: impact on control of fluctuations and quality of life.

Symptom control, daily "on" time, and quality of life (QoL) of nine patients with Advanced Parkinson's Disease was assessed following 18-months treatment with Continuous Intraduodenal Levodopa Infusion (CIDLI). Patients had severe motor fluctuations and dyskinesias and had previously received treatment with oral levodopa and dopamine agonists. There were significant improvements in patients' symptoms on the Unified Parkinson's Disease Rating Scale, and QoL (Parkinson's Disease QoL Questionnaire; Schwab & England Capacity for Daily Living Scale; p < 0.05). Mean (+/-SD) daily "on" time increased from 6.1 +/- 1.9 to 12.0 +/- 3.4 h (p < 0.05). Improved QoL in APD was associated with CIDLI-related improvements in symptom control and increase in daily "on" time.

Asociación de carcinoma renal con neuromiotonía y afectación de neurona motora inferior / Association of renal carcinoma with neuromyotonia and involvement of inferior motor neuron

La neuromiotonía adquirida, definida como actividad muscular continua originada en el nervio periférico debida a alteraciones de la membrana del axón motor, se ha considerado una manifestación paraneoplásica en relación a tumores relacionados con el sistema inmunitario, principalmente linfomas. De forma similar, la afectación de neurona motora ha sido descrita como una complicación paraneoplásica infrecuente también en relación con linfomas. Aportamos un caso excepcional, dada la presencia de dos manifestaciones paraneoplásicas infrecuentes como la neuromiotonía y la enfermedad de neurona motora secundarias a carcinoma renal de células claras

Acquired neuromyotonia is defined as continuous muscle activity originated in peripheral nerve due to alterations of the motor axon membrane. It has been considered a paraneoplastic syndrome in patients with neoplasms of the immune system, mainly lymphomas. Similarly, involvement of the motor neuron has been described as an uncommon paraneoplastic complication, also in relationship to lymphomas. We report a rare case, given the presence of two uncommon paraneoplastic manifestations such as neuromyotonia and reversible paraneoplastic lower motor neuronopathy secondary to clear cell renal carcinoma

[Association of renal carcinoma with neuromyotonia and involvement of inferior motor neuron]. / Asociación de carcinoma renal con neuromiotonía y afectación de neurona motora inferior.

Acquired neuromyotonia is defined as continuous muscle activity originated in peripheral nerve due to alterations of the motor axon membrane. It has been considered a paraneoplastic syndrome in patients with neoplasms of the immune system, mainly lymphomas. Similarly, involvement of the motor neuron has been described as an uncommon paraneoplastic complication, also in relationship to lymphomas. We report a rare case, given the presence of two uncommon paraneoplastic manifestations such as neuromyotonia and reversible paraneoplastic lower motor neuronopathy secondary to clear cell renal carcinoma.

Antibodies to the E2 protein of GB virus C/hepatitis G virus: prevalence and risk factors in different populations in Italy.

BACKGROUND: Many aspects of the epidemiology of GB virus C/hepatitis G virus (GBV-C) infection have not been fully elucidated. The purpose of this study was to assess the prevalence of GBV-C antibodies and risk factors in different subjects living in Italy. MATERIALS AND METHODS: A total of 1,005 sera were tested for the presence of antibodies to the of GBV-C E2 protein using a recently developed enzyme immunoassay. RESULTS: A high prevalence of GBV-C antibodies was found in healthy blood donors (12.6%). Hemodialysis patients and drug users showed higher rates of GBV-C seropositivity (22% and 39%, respectively). Immigrants from sub-Saharan Africa and South Asia had anti-GBV-C prevalence comparable to that found in Italian blood donors, whereas higher and lower rates were detected in immigrants from Latin America and the Caribbean (19.5%) and from Mediterranean Africa (5.6%). CONCLUSION: GBV-C infections are widespread in the general population in Italy and particularly common in risk groups. The different prevalence of GBV-C antibodies detected in third world immigrants is likely to reflect the prevalence in the countries of origin. However, the observation that the length of residency in Italy is a significant risk factor may suggest that at least some GBV-C infections are contracted in Italy.

A seroepidemiologic survey on brucellosis antibodies in southern Italy.

This study investigated the prevalence of Brucella antibodies in the general population in two regions in southern Italy. A total of 1,294 subjects were recruited from January to June 1996 among patients attending randomly selected public and private laboratories and pediatric outpatient clinics. Information on sex, age, residence, and occupation was recorded. Seroprevalence of brucellosis was studied by the safranin O-stained antigen microagglutination test. The chi-square test and multiple logistic regression were performed. An overall prevalence of brucellosis of 3.1% was recorded; no significant difference was found between the two regions, with values of 2.7% in Campania and 3.8% in Calabria. Multiple logistic regression analysis indicated that positivity to brucellosis was significantly associated with the province the subjects reside in, ranging from 0 in Salerno to 6.2% in Caserta, and that it significantly increased with age, ranging from 1% in persons under 16 to 4.2% in those over 65 (OR = 1.7; 95% CI = 1.04-2.6), whereas it was not significantly associated with sex. When analysis was conducted after restriction to subjects reporting to be working, occupation was found not to be significantly associated with brucellosis. Since the spread of the disease is still of concern, though circulation of Brucella is decreasing, strict application of measures for the eradication of brucellosis from livestock, pasteurization of milk and dairy products, and education regarding eating habits must be pursued.

Persistence of immunity to poliomyelitis among a southern population that received four doses of OPV 5 to over 15 years before.

The immune status against polioviruses was investigated in a population of 545 students aged 11-20 years residing in the Neapolitan area, who had completed the vaccination cycle with four doses of OPV 5 to over 15 years before. Assuming as unprotected those individuals without detectable neutralizing antibodies at the dilution 1:2, nobody resulted without protection against all types of poliovirus; 0.7% lacked antibodies only against type 1, 0.6% only against type 3 and none against type 2. A very slight decreasing trend was observed for GMT values in function of the distance from the last dose of OPV for polio 1 and 2, but not for polio 3. As expected, GMT values for polio 2 resulted higher than those for polio 1 and both were higher than those for polio 3, when calculated by age groups as well as by distance groups. The last four Italian cases of autochthonous paralytic poliomyelitis, occurred in the period 1981/83, regarded unvaccinated children aged 6 months-2 years, residing in the same geographical area to which the study population belong. In the same area a delay of immunization practices was also ascertained in the recent past. Results of this study confirm that a priority for public health services is to devote their human and economic resources to reduce the vaccination delay more than administrate a further fifth dose of OPV at the age of twelve.

Effective antibody response in newborn babies living in Maldives to simultaneous vaccination against hepatitis B, poliomyelitis, diphtheria and tetanus.

The antibody responses of Maldivian infants early in their life to simultaneous immunization against hepatitis B virus, poliomyelitis, diphtheria and tetanus were investigated. The vaccines were given at 6, 10 and 14 weeks of age. Among 243 newborn babies from HBsAg-negative mothers, 103 received three doses of oral poliomyelitis (OPV) and diphtheria and tetanus (DTV) vaccines; 105 were similarly immunized but received in addition the recombinant hepatitis B vaccine (HBV); 35 were immunized with the HBV recombinant vaccine alone. The antibody response to all of the vaccines was effective. No significant differences among the groups were observed. Hepatitis B vaccination of infants neither affected nor was affected by the contemporary administration of OPV and DTV vaccines.