Towards establishing MS prevalence in Latin America and the Caribbean.

A very high prevalence of multiple sclerosis (MS) has been reported in some Western European and North American countries. The few surveys of MS epidemiology in South America reveal lower prevalence rates, implying that susceptibility varies between distinct ethnic groups, thus forming an important determinant of the geographic distribution of the disease. The objective of this study is to review MS prevalence estimates in different Latin American and Caribbean countries. We reviewed surveys of regional MS prevalence from 1991 to 2011. Sources included an online database, authors' reports and proceedings or specific lectures from regional conferences. We obtained a total of 30 prevalence surveys from 15 countries, showing low/medium MS prevalence rates. Both the number and the quality of prevalence surveys have greatly improved in this region over recent decades. This is the first collaborative study to map the regional frequency of MS. Establishment of standardized methods and joint epidemiological studies will advance future MS research in Latin America and the Caribbean.

Fluctuations of MS births and UV-light exposure.

BACKGROUND: Patients with multiple sclerosis (MS) are more frequently born in spring when compared to autumn. Fluctuation of UV-light has been hypothesized to drive this phenomenon. AIM: To assess the correlation between fluctuation of sunlight and birth season in persons with MS. METHODS: For this record-linkage study, we collected from the international MSBase and the Italian MS iMed-web databases the dates of birth of 11,415 patients with MS from 36 centres from 15 countries worldwide and compared these to dates of live-births from national registries. From all participating sites, we collected data on UV-light fluctuation and assessed its correlation with seasonal fluctuation in MS births. RESULTS: Compared with the reference cohort, an increased proportion of persons with MS were born in spring and a decreased proportion in autumn (odds ratio (OR) to be born in spring versus autumn = 1.158, χ² = 36.347, P < 0.001). There was no significantly increased fluctuation of MS births with increased quartile of ambient UV-light fluctuation (Ptrend = 0.086). CONCLUSION: Seasonal fluctuation of MS births as found in this worldwide cohort of patients with MS did not correlate with variation in seasonal fluctuation of UV-light. Most likely, it results from a complex interplay between fluctuation of sunlight, behavioural factors, other environmental factors and (epi)genetic factors.

Conventional brain MRI in neuromyelitis optica.

BACKGROUND AND PURPOSE: Numerous case series have demonstrated that lesions on brain MRI are common in neuromyelitis optica (NMO), but there has not been an attempt to survey and synthesize the literature on neuro-radiology of brain findings in NMO. OBJECTIVES: To review the studies on conventional brain MRI in NMO and to propose to incorporate characteristic brain MRI lesions into the diagnostic criteria of NMO. METHODS: We searched for articles with descriptions of brain MRI findings in NMO in EBSCO, EMBASE, PubMed/Medline, Science Citation Index, and SCOPUS. RESULTS: Brain abnormalities are seen in majority of NMO patients as disease duration increases. A minority of NMO patients meet Barkhof criteria for space dissemination in multiple sclerosis (MS), and these criteria should not be used to exclude NMO diagnosis. Distinctive brain lesions of NMO are cataloged and described. CONCLUSIONS: Brain lesions in NMO are a consistent feature of the disease. International consensus MRI criteria are needed for NMO analogous to the existing criteria for MS.

Neuromyelitis optica unique area postrema lesions: nausea, vomiting, and pathogenic implications.

OBJECTIVE: To characterize the neuropathologic features of neuromyelitis optica (NMO) at the medullary floor of the fourth ventricle and area postrema. Aquaporin-4 (AQP4) autoimmunity targets this region, resulting in intractable nausea associated with vomiting or hiccups in NMO. METHODS: This neuropathologic study was performed on archival brainstem tissue from 15 patients with NMO, 5 patients with multiple sclerosis (MS), and 8 neurologically normal subjects. Logistic regression was used to evaluate whether the presence of lesions at this level increased the odds of a patient with NMO having an episode of nausea/vomiting. RESULTS: Six patients with NMO (40%), but no patients with MS or normal controls, exhibited unilateral or bilateral lesions involving the area postrema and the medullary floor of the fourth ventricle. These lesions were characterized by tissue rarefaction, blood vessel thickening, no obvious neuronal or axonal pathology, and preservation of myelin in the subependymal medullary tegmentum. AQP4 immunoreactivity was lost or markedly reduced in all 6 cases, with moderate to marked perivascular and parenchymal lymphocytic inflammatory infiltrates, prominent microglial activation, and in 3 cases, eosinophils. Complement deposition in astrocytes, macrophages, and/or perivascularly, and a prominent astroglial reaction were also present. The odds of nausea/vomiting being documented clinically was 16-fold greater in NMO cases with area postrema lesions (95% confidence interval 1.43-437, p = 0.02). CONCLUSIONS: These neuropathologic findings suggest the area postrema may be a selective target of the disease process in NMO, and are compatible with clinical reports of nausea and vomiting preceding episodes of optic neuritis and transverse myelitis or being the heralding symptom of NMO.

Absence of cortical demyelination in neuromyelitis optica.

OBJECTIVE: To asses the presence of cortical demyelination in brains of patients with neuromyelitis optica (NMO). NMO is an autoimmune inflammatory demyelinating disease that specifically targets aquaporin-4-rich regions of the CNS. Since aquaporin-4 is highly expressed in normal cortex, we anticipated that cortical demyelination may occur in NMO. METHODS: This is a cross-sectional neuropathologic study performed on archival forebrain and cerebellar tissue sections from 19 autopsied patients with a clinically and/or pathologically confirmed NMO spectrum disorder. RESULTS: Detailed immunohistochemical analyses of 19 archival NMO cases revealed preservation of aquaporin-4 in a normal distribution within cerebral and cerebellar cortices, and no evidence of cortical demyelination. CONCLUSIONS: This study provides a plausible explanation for the absence of a secondary progressive clinical course in NMO and shows that cognitive and cortical neuroimaging abnormalities previously reported in NMO cannot be attributed to cortical demyelination. Lack of cortical demyelination is another characteristic that further distinguishes NMO from MS.

Multiple sclerosis and solar exposure before the age of 15 years: case-control study in Cuba, Martinique and Sicily.

Few studies report a protective role of childhood solar exposure to multiple sclerosis. Our objective was to confirm the protective role of childhood solar exposure in multiple sclerosis in Cuba, Martinique and Sicily. This was a matched case- control study, and cases met Poser criteria for clinically, laboratory (definite, probable) multiple sclerosis. Controls were resident population, without neurological disorder, living close to cases (within 100 km), matched for sex, age (+/-5 years), residence before age 15. We recruited 551 subjects during a 1-year period (193 cases, Cuba n = 95, Sicily n = 50, Martinique n = 48; 358 controls). Some (89%) met definite clinical multiple sclerosis criteria (relapsing remitting form (with and without sequel) (74%), secondary progressive (21%), primary progressive (5%)). Odds ratios in a uni-variate analysis were: family history of multiple sclerosis (5.1) and autoimmune disorder (4.0); wearing shirt (3.5), hat (2.7), pants (2.4); sun exposure causing sunburn (1.8); sun exposure duration (1 h more/day; weekends 0.91, weekdays 0.86); bare-chested (0.6); water sports (0.2). Independent factors in the multivariate analysis were family history of multiple sclerosis (4.8 (1.50-15.10)), wearing pants under sunlight (1.9 (1.10-3.20)), sun exposure duration (1 h more/ day, weekdays 0.90 (0.85-0.98), weekends 0.93 (0.87-0.99)), water sports (0.23 (0.13-0.40)). We conclude that outdoor leisure activities in addition to sun exposure reports are associated with a reduced multiple sclerosis risk, with evidence of dose response.

Relapsing neuromyelitis optica: long term history and clinical predictors of death.

BACKGROUND: Relapsing neuromyelitis optica (RNMO) is an uncommon but devastating inflammatory disorder of the central nervous system. Long term history in a wide series of RNMO is required for better knowledge of the course of the disease and identification of patients at high risk of death. METHODS: Clinical features of patients with RNMO (88 women/eight men) obtained from the geographic Caribbean database (Cuba and French West Indies) were used to determine the progression of disability and to identify clinical predictors of death. RESULTS: Median age at onset of RNMO was 29.5 years (range 11-74). Median duration of disease was 9.5 years (1-40). Median relapse rate was 0.7 attack/patient/year (0.1-3). 66 patients experienced severe visual loss in at least one eye and 46 in both eyes. Median time from onset to unilateral and bilateral severe visual loss was 3 and 15 years, respectively. Median times to reach Kurtzke Disability Status Scale 3, 6 and 8 from onset of RNMO were 1, 8 and 22 years. There were 24 deaths (25%); within 5 years in 63% of cases. A higher attack frequency during the first year of disease (p = 0.009), blindness (p = 0.04) and sphincter signs at onset (p = 0.02) and lack of recovery of first attack (p = 0.003) were independently associated with a shorter time to death. CONCLUSION: RNMO is a very rapidly disabling disease affecting primarily young women. This study has identified clinical features that predict a poor outcome. These findings suggest that early and aggressive immunotherapy might be warranted in RNMO.

Neuromyelitis optica positive antibodies confer a worse course in relapsing-neuromyelitis optica in Cuba and French West Indies.

BACKGROUND: In Caucasian populations neuromyelitis optica (NMO-IgG) antibody has been detected in 27.1% / 78.2% of patients with relapsing-NMO (R-NMO). The prevalence reported for the disease in the Caribbean is 3.1/100,000 in the French West Indies (FWI) and 0.52 /100,000 in Cuba, but the NMO antibody status is unknown. OBJECTIVE: To assess the NMO-IgG antibody status of Cuban/FWI RNMO patients, comparing with European cases tested at the same laboratories. METHODS: Serum NMO-IgG antibodies were assayed in 48 R-NMO patients (Wingerchucks 1999 criteria): Cuba (24)/FWI (24), employing Lennon et als method. We compared the demographic, clinical, disability and laboratory data between NMO-IgG +/- patients. All the data were reviewed and collected blinded to the NMO-IgG status. RESULTS: Seropositivity of the NMO-IgG antibody demonstrated a lower rate in the Caribbean (33.3%), as compared with Caucasian patients from Spain/Italy (62.5%) and France (53.8%). Caribbean patients with NMO-IgG (+) displayed more attacks, more spinal attacks and a higher EDSS than NMO-IgG (-) cases, while brain and spinal cord MRI lesions were more frequent during remission, with more vertebral segments, more gray, white matter and holocord involvement. CONCLUSIONS: NMO IgG positive antibodies in NMO patients had a lower rate in the Caribbean area - where the population has a predominant African ancestry - than in Caucasian Europeans, suggesting the influence of a possible ethnic factor in the pathogenesis of the disease, but they confer a worse course with more attacks, more disability and MRI lesions.

Neuromyelitis optica and multiple sclerosis in sisters.

INTRODUCTION: The association of neuromyelitis optica (NMO) and multiple sclerosis (MS) has been reported, but details of the cases were not described. We report two Venezuelan Caucasian sisters with human leukocyte antigen (HLA) typing. RESULTS: Patient 1 fulfilled McDonald, et al. criteria with HLA A*24; B*07,*15; DRB1*01,*16 (DR2 positive). Patient 2 fulfilled the NMO revised criteria of Wingerchuck, et al. with HLA A*02,*24; B*07,*40; DRB1*04,*08, similar to Canadian aboriginal NMO cases and the Yukpa population from Venezuela. CONCLUSION: These cases confirmed the coexistence of NMO and MS in sisters, and further studies are needed to understand the genetic linkage between these diseases.

Treatment with type I interferons induces a regulatory T cell subset in peripheral blood mononuclear cells from multiple sclerosis patients.

Type I Interferon (IFN-alpha/beta) therapy has altered the natural course of multiple sclerosis. In this paper we evaluate the possible molecular mechanisms involved in the in vitro effects of IFN-alpha/beta on peripheral blood mononuclear cells from patients with clinically definite Relapsing-Remitting Multiple Sclerosis. The total RNA from IFN-alpha, IFN-beta treated cells and untreated cells was extracted and amplified for CD86, CD28, CTLA-4, TNF-alpha, IFN-gamma, CCL2, CCR5, IL-13, MMP-9, TIMP-1, CD25, TGF-beta, IL-10 and the transcriptional factor Foxp3 by Reverse Transcription-Polymerase Chain Reaction and the CD4+CD25high subset was evaluated using flow cytometry. In general, there were no significant differences concerning the modulation of the genes studied in the response to IFN-alpha and IFN-beta treatments, which suggest a similar mechanism of action for both interferons. However, we found a significant increment in IFN-gamma expression after IFN-alpha but not after IFN-beta treatments. The in vitro treatment of mononuclear cells from multiple sclerosis patients with both interferons significantly increased the CD25 mRNA. Furthermore, we observed a CD25/Foxp3 correlation and an increment of the CD4+CD25high subset, indicating that the induction of regulatory T cells could be a crucial mechanism involved in the type I interferon effects.

Intrathecal polyspecific immune response to neurotropic viruses in multiple sclerosis: a comparative report from Cuban patients.

OBJECTIVE: Intrathecal measles(M)- rubella(R)- and varicella zoster(Z)-antibody synthesis in German and Cuban multiple sclerosis (MS) patients are compared considering the different rubella epidemiology in the tropics. PATIENTS AND METHODS: Twenty-three Cuban MS patients with a representative age distribution and gender ratio like the group of 177 German MS patients were analysed for albumin, IgG, IgA IgM, oligoclonal IgG and MRZ- antibodies in cerebrospinal fluid (CSF) and serum. RESULTS: Cuban MS patients show similar CSF data patterns like German patients and high frequencies of intrathecal measles- (78/78%) and varicella zoster- (59/55%) antibody synthesis correspondingly. A lower frequency of intrathecal rubella antibody synthesis (rubella-AI >or= 1.5) in Cuban patients (30%, gender ratio of increased rubella - AI m:f = 1:6) compared with German patients (60%, m:f = 1:1.8) is explained by low incidence of rubella infections in Cuba. Only about 10% of the male population (not immunized before 1986, in contrast to females) had rubella antibodies compared to at least 60% in a European male population, representing the relation of increased rubella-AI in male MS patients. CONCLUSION: In MS the frequency of intrathecal antibody synthesis is limited by the fraction of seropositives in the population. Natural infection or vaccination are a necessary and equivalent precondition contributing to the arguments against microorganisms as a cause of MS.

Down's syndrome and neuromyelitis optica (Devic's disease). An autopsy-proven case.

Neuromyelitis optica (NMO) has been attributed to different underlying pathological events. The aim of this paper is to present the first case report of a patient with Down's syndrome (DS) who died of a fulminant NMO. A 29-year-old woman with DS developed acute transverse myelitis, with complete visual loss and swollen optic discs. Two days later, she developed quadriplegia, respiratory arrest and died. The anatomical study demonstrated typical findings of DS in the brain without demyelinating lesions. A severe destruction of medulla and cervical cord with a very high degree of demyelination of the optic nerves was typical of monophasic NMO (Devic's disease). Most of the cases of NMO in Cuba are of the relapsing form, but this case report is the first one with monophasic NMO and DS with a very aggressive course. The link of the pathogenetic relationship between DS and NMO remains unclear; it may well be coincidence but the fact that the patient died very shortly after the onset suggests, at least on clinical grounds, that the presence of DS could have accelerated the fatal evolution of NMO.

Hereditary Melkersson-Rosenthal syndrome and multiple sclerosis.

UNLABELLED: The revision of MEDLINE from 1966 to 2003 did not report any association between multiple sclerosis (MS) and Melkersson-Rosenthal syndrome (MRS). This is a case report of a 51-year-old woman, with history of four recurrent Bell's palsies. In 1999 she developed a right facial paralysis due to a supranuclear pyramidal lesion with right monoparesis. The family history showed five relatives with recurrent Bell's paralysis and plicata tongue. PHYSICAL EXAMINATION: right Bell's paralysis, left supranuclear facial paralysis, furrowed tongue, right hemiparesis with pallor of the optic disks. Brain magnetic resonance imaging (MRI) demonstrated the typical lesions of MS and CSF oligoclonal bands. This is the first observation of a patient with hereditary MRS and MS. The link between both diseases is discussed.

[Clinical features of multiple sclerosis in Western Cuba. A comparison with two other regions in the country]. / Características clínicas de la esclerosis múltiple en el Occidente de Cuba. Comparación con otras dos regiones del país.

INTRODUCTION: Multiple sclerosis (MS) is an inflammatory disease in its early stages whose primary or secondary immunological mechanisms produce reversible or irreversible lesions in the myelin and axons in the central nervous system. The first case of MS in Cuba was reported in 1965. Current prevalence of MS is considered to be 10 cases/100,000 inhabitants. AIMS: The aim of this study was to characterise MS in Western Cuba from a clinical point of view and in comparison with other similar studies carried out in two other regions in the country. PATIENTS AND METHODS: 50 patients living in the western region were clinically assessed. Statistical tests were carried out to compare this survey with two similar studies conducted in the central and eastern regions. RESULTS: 80% of our patients were females, predominantly white skinned, the main events in their family histories were neurological diseases and psychiatric diseases, essentially schizophrenia, the chief triggering event being psychic tension, the most frequent form of progression was the remittent recurring form, followed by secondary progressive form, and then the primary progressive; the main symptoms at onset were visual, followed by pyramidal and sensory; the most strongly affected functional system was the pyramidal and then the sensory system; the functional systems are more affected in the primary progressive form, except the visual and the brainstem; the largest group of patients corresponded to those that had a history of over 10 years with the disorder. By far the majority of results compared with the series from the central region and from Santiago de Cuba were similar, but some significant differences did appear on comparing these two series. CONCLUSIONS: The study shows the characteristics of the disease in the Western region valuated using distinct parameters and several differences between the three series can be observed fundamentally with regard to skin colour, triggering events, symptoms at onset and functional involvement in the forms of progression.

[The influence of geographical background on the clinical and neurophysiological characteristics of patients with multiple sclerosis in Cuba]. / Influencia del origen geográfico en las características clínicas y neurofisiológicas de pacientes con esclerosis multiple en Cuba.

INTRODUCTION: Multiple sclerosis (MS) was first described by Charcot in 1868 and the first case in Cuba was reported in 1965. Prevalence rate is now considered to be about 10 cases/100,000 inhabitants. Cuba is an island that can be divided into three different geographical regions and a comparison of these areas is interesting because it is known that geographical features exert an influence on MS. Demographic differences are also present. AIMS: The purpose of this study was to evaluate a sample of cases from the Western part of the country as regards their results on two important scales and the results of evoked potentials (EP), and to compare them with two samples of patients from the other two regions. PATIENTS AND METHODS: The first sample was made up of 50 patients living in the western region; their diagnosis was confirmed, the scales were applied and the EP test was performed because of its high degree of sensitivity, objectivity and reproducibility. Results were then compared with the other two studies that had already been reported. RESULTS: Most cases had a score of between 0.5 and 5.5 points on the EDSS and only 6% scored above 7.0 points. Patients with PP type MS obtained higher scores. More than half the cases had more than 80 points on the Scripps scale and the second largest group had between 61 and 80 points; the two progressive forms behaved in a similar manner. The most sensitive EP are visual, followed by somatosensory and, lastly, by brain stem auditory EP (BAEP). The vast majority of the results compared with the series from the central region and from Santiago de Cuba were similar, yet some significant differences were found: progression time is longer in the West, EDSS scores were higher in the primary progressive form in the East and the BAEP were less sensitive in the West. CONCLUSIONS: Our findings correspond to those available from other countries around the world and it can be seen that some of the parameters differ between the three regions.

[Interferon-alpha2b recombinant improved the cognitive dysfunction in patients with relapsing remitting multiple sclerosis]. / El interferón-alpha2b recombinante mejora la disfunción cognitiva en pacientes con esclerosis múltiple.

INTRODUCTION: Some experimental, Phase II clinical trials and the preliminary reports of the Cuban Phase III clinical trial indicate that alpha-IFN (IFN) may be useful in relapsing remitting (RR) multiple sclerosis (MS). The reports in Cuba showed that 70% of the MS patients have cognitive dysfunction. OBJECTIVE: To assess the efficacy of IFN-alpha2b recombinant in the cognitive dysfunction of RR MS. PATIENTS AND METHODS: 57 RR-MS clinical definite patients from the randomised, double blind, placebo controlled study of 225 patients with RR-MS and brain MRI confirmed. Patients were randomly assigned to receive intramuscular IFN-alpha2b (Heberon R) 10 million IU (high dose), 3 million IU (low dose) or placebo twice week for 2 years. Outcome results were blinding evaluated considering changes in the following tests: Luria, WAIS, Benton and PASAT-3. Adverse events and side effects were not evaluated to maintain physician blinding. RESULTS: The initial comparison of the groups did not show any differences among the placebo (n=20), low dose (n=18) and high dose (n=19) considering age (p=0.234), gender, ethnic group (p=0.012), years ill (p=0.787), EDSS (p=0.203) and rate of relapses (p=0.432). The Luria's Test showed an improved in the low dose group from 2.50 +/- 1.34 to 1.39 +/- 1.85 (p=0.029) and in the high dose group from 3.22 +/- 1.89 to 2.17 +/- 1.50 (p=0.006) vs placebo 2.85 +/- 1.66 to 2.90 +/- 1.97 (p=0.723). The results of the Benton's test demonstrated that the low dose group had an improved from 5.50 +/- 1.10 to 6.22 +/- 1.31 (p=0.047), in the high dose group from 4.87 +/- 1.85 to 5.78 +/- 1.35 (p=0.005) where as in the placebo group worse from 5.15 +/- 1.76 to 5.05 +/- 2.11 (p=0.893). The WAIS test showed the same results, the low dose group increased from 5.17 +/- 1.34 to 6.06 +/- 1.21 (p=0.022), the high dose group from 4.56 +/- 1.38 to 5.39 +/- 1.29 (p=0.007) and the placebo group worse from 5.25 +/- 1.25 to 5.05 +/- 1.57 (p=0.354). Finally, the PASAT-3 test increased in the IFNs groups: from 45.72 +/- 10.61 to 49.94 +/- 11.68 (p=0.015) in the low dose group, from 42.67 +/- 11.04 to 48.72 +/- 8.84 (p=0.03) in the high dose group, but in the placebo group worse from 44.55 +/- 10.86 to 41.95 +/- 13.74 (p=0.655). CONCLUSION: IFN-alpha improved the cognitive dysfunction in RR-MS patients. The higher dose is more beneficial.

[A severe episode in a patient with recurrent disseminated acute encephalitis due to vaccination against hepatitis B. For or against vaccination?]. / Brote grave en paciente con encefalitis aguda diseminada recurrente por vacuna contra LA hepatitis B: A favor o en contra de la Vacunación?

INTRODUCTION: Several reports of new cases of CNS demyelination or reactivation of MS after hepatitis B vaccination have raised the possibility of a causal relationship. Conversely, some authors have concluded that the risk of developing a demyelinating CNS event after a hepatitis B vaccination is unknown. PATIENTS AND METHODS: We have observed a 40 year old man, with diabetes mellitus (DM) type 1 and a previous history of recurrent acute demyelinating encephalomyelitis (rADEM). The patient had experienced three episodes of neurological dysfunction and he fulfilled the criteria for definite clinical MS but MRI showed demyelinating lesions in the pons and cerebellum without MRI criteria of MS. CSF analysis showed oligoclonal banding. The patient had been clinically stable during the last 6 years. Yearly MRI during this period had not shown any new disease activity. He was admitted in our MS Clinic due to dizziness, nausea, vomiting and diplopia, 6 weeks after the first of the two injections for hepatitis B vaccine after participating in the national programme of vaccination in DM type 1 patients. Clinical examination showed intranuclear ophtalmoplegia, visual loss in the left eye and worsening of the previous cerebellar and pyramidal signs. MRI showed an increase in the old lesions with high intensity signals on T2 weighted sequences with post gadolinium enhancement on T1 weighted sequences located in the brainstem and mesencephalon. The patient s diabetes mellitus deteriorated with ketoacidosis that needed increased doses of insulin. His condition worsened and he developed partial motor seizures. He improved 15 days later but he still had involvement of the cerebellar and pyramidal systems and occasional dizziness. CONCLUSIONS: As pointed out by some authors and in view of this observation, it would seem reasonable, as a precautionary measure, to avoid hepatitis B vaccination in patients with a personal or family history of symptoms suggestive of a demyelinating disease of the CNS.

Brote grave en paciente con encefalitis aguda diseminada recurrente por vacuna contra la hepatitis B.¿A favor o en contra de la vacunación? / A severe episode in a patient with recurrent disseminated acute encephalitis due to vaccination against hepatitis b. For or against vaccination?

Introducción. Varios estudios han informado de casos nuevos de desmielinización del SNC o de reactivación de la esclerosis múltiple (EM) después de una vacunación contra la hepatitis B (HB). A la inversa, algunos autores han llegado a la conclusión de que se desconoce el riesgo de desarrollar un fenómeno de desmielinización del SNC tras una vacunación HB. Pacientes y métodos. Hemos observado el caso de un hombre de 40 años de edad con diabetes mellitus (DM) del tipo I e historia previa de encefalomielitis con desmielinización aguda recurrente (EDA-r). Había experimentado tres episodios de disfunción neurológica y cumplido los criterios de EM clínicamente definida, pero la RM mostró lesiones en el tallo cerebral y en el cerebelo sin criterio de EM por RM. El análisis del LCR mostró bandas oligoclonales. Había permanecido estable clínicamente durante los últimos seis años, sin mostrar actividad alguna de la enfermedad. Ingresó en clínica por EM porque padecía mareos, náuseas, vómitos y diplopía, seis semanas después de las dos primeras inyecciones contra la hepatitis, dentro del programa nacional de vacunación de los pacientes de DM del tipo I. El examen clínico mostró oftalmoplejía intranuclear, pérdida de visión del ojo izquierdo y afectación de los signos cerebelares y piramidales previos. La RM mostró un aumento de las viejas lesiones, con señales de alta intensidad en las secuencias de imágenes en T2 y aumento de posgadolinio en los sitios de las secuencias de imágenes en T1 del tronco cerebral y el mesencéfalo. La DM se deterioró con cetoacidosis, por lo que se aumentaron las dosis de insulina. Empeoró y desarrolló ataques convulsivos motores parciales. A los 15 días mejoró, pero todavía sufría afectación de los sistemas cerebelares y piramidales, y mareos ocasionales. Conclusiones. Parece razonable, como medida de precaución, evitar la vacunación HB en pacientes con historia personal o familiar de síntomas sugestivos de una enfermedad desmielinizante del SNC (AU)

Factores exógenos en la etiología de la esclerosis múltiple en Cuba. Estudio de casos y controles / Exogenous factors in the aetiology of multiple sclerosis in Cuba. A study of cases and controls

Introducción. Los estudios de epidemiología descriptiva indican que la prevalencia de la esclerosis múltiple (EM) en Cuba está en la zona media de riesgo, y se desconocen los posibles factores exógenos relacionados con la etiología de la EM. Objetivo. Estudiar los posibles factores exógenos relacionados con la etiología de la EM en las provincias centrales de Cuba. Pacientes y métodos. Se estudiaron todos los pacientes con EM clínica definida (Poser et al) con RM positiva procedentes de las provincias de Cienfuegos, Villa Clara y Sancti Spíritus. El protocolo fue aprobado por los Comités de Ética Médica de cada una de las tres instituciones y revisado por dos autoridades internacionales en la materia. A cada uno de los pacientes (casos) y a uno de sus familiares (controles), previo consentimiento por escrito y que cumplían los criterios de inclusión, se les realizó por personal entrenado una entrevista personal para llenar el cuestionario de estudios de casos y controles de Boiko et al, al que se le hicieron adaptaciones para su utilización en Cuba. Los datos principales de la encuesta incluyeron: datos demográficos, historia ocupacional y residencial, historia médica personal, historia médica familiar, exposición a posibles sustancias tóxicas, dieta y nutrición, contacto con animales y estilo de vida. Para la medición del riesgo se utilizó el riesgo de las razones de productos cruzados odds ratio (OR) con su intervalo de confianza (IC), todos con una exigencia de precisión del 95 por ciento. Resultados. El nivel educacional alto (OR= 2,293; 95 por ciento IC= 0,9-5,4); el número de 2 o más hijos (OR= 2,84; 95 por ciento IC= 1,226,59); cuatro o más embarazos (OR= 3,60; 95 por ciento IC= 0,69-18,7); tres o más partos (OR= 1,939; 95 por ciento IC= 0,44-8,4); infecciones previas por sarampión (OR= 2,042; 95 por ciento IC= 0,76-5,47); antecedentes de padecer migraña (OR= 2,538; 95 por ciento IC= 0,81-7,94); estrés (OR= 3,768; 95 por ciento IC= 1,64-8,62) e insomnio (OR= 2,95; 95 por ciento IC= 1,147,14) fueron los factores relacionados con la EM. Conclusiones. Este estudio confirma la existencia de factores exógenos en la etiología en los pacientes con EM clínica definida y con RM positiva residentes en las provincias estudiadas en Cuba. Los factores exógenos encontrados en esta población de pacientes con EM fueron: el nivel educacional alto, el número de dos o más hijos, cuatro o más embarazos, tres o más partos, las infecciones previas por sarampión, los antecedentes de padecer de migraña, el estrés y el insomnio (AU)

[Exogenous factors in the aetiology of multiple sclerosis in Cuba. A study of cases and controls]. / Factores exógenos en la etiología de la esclerosis múltiple en Cuba. Estudio de casos y controles.

INTRODUCTION: Descriptive epidemiological studies indicate that the prevalence of multiple sclerosis (MS) in Cuba is in the mid range of risk. It is not known what exogenous factors may be related to the aetiology of MS. OBJECTIVE: To study the exogenous factors possibly related to the aetiology of MS in the central provinces of Cuba. PATIENTS AND METHODS: We studied all the patients with definite clinical MS (Poser et al) with positive MR who came from the provinces of Cienfuegos, Villa Clara and Sancti Spiritus. The protocol was approved by the Medical Ethics Committees of each of the three institutions and reviewed by two international authorities on the subject. Each of the patients (cases) and one member of their family (control), after written consent, when they fulfilled the criteria for inclusion in the study, were personally interviewed by trained persons and the questionnaire for studying cases and controls of Boiko et al was completed. The questionnaire had been adapted for use in Cuba. The main data obtained were: demographic data, history of occupation and residence, personal medical history, exposure to possibly toxic substances, diet and nutrition, contact with animals and life style. For measurement of risk we used the risk of the results of the crossed products odds ratio (OR) with its confidence interval (CI), always insisting on precision of 95%. RESULTS: High educational level (OR= 2.293; 95% CI= 0.9 5.4), two or more children (OR= 2.84; 95% CI= 1.22 6.59), four or more pregnancies (OR= 3.60; 95% CI= 0.69 18.7), three or more deliveries (OR= 1.939, 95% CI= 0.44 8.4); previous measles infection (OR= 2.042; 95% CI= 0.76 5.47), a history of migraine (OR= 2.538; 95% CI= 0.81 7.94); stress (OR= 3.768; 95% CI= 1.64 8.62) and insomnia (OR= 2.95; 95% CI= 1.14 7.14) were the factors related to MS. CONCLUSIONS: This study confirmed the existence of exogenous factors in the aetiology of patients with clinically defined MS and positive MR findings who lived in the provinces studied in Cuba. The exogenous factors found in this population of patients with MS were: high educational level, two or more children, four or more pregnancies, three or more deliveries, previous measles, a history of migraine, stress and insomnia.