Face-to-Face or Telematic Cognitive Stimulation in Patients with Multiple Sclerosis and Cognitive Impairment: Why Not Both?

INTRODUCTION: Cognitive impairment (CI) affects 40-65% of patients with multiple sclerosis (MS). Few studies address telematic cognitive stimulation (TCS) in MS. The objective of this study is to evaluate the efficacy and impact of telestimulation or distance cognitive stimulation (TCS), with and without the support of face-to-face cognitive stimulation (FCS) in cognitive impairment in MS. METHODS: Multicentre, prospective, randomised, controlled study. We will include 98 MS patients with EDSS ≤ 6, symbol digit modality test (SDMT) ≤ Pc 25, and Multiple Sclerosis Neuropsychological Screening Questionnaire (MSNQ) > 26 points. Patients will be randomised into 3 groups, a TCS group, a mixed TCS/FCS group, and a control group. CS is performed 3 days a week for 3 months. Processing speed, memory, attention, and executive functions will be rehabilitated. FCS will include ecological exercises and strategies. EDSS and a cognitive evaluation (SDMT, CTMT, PASAT, and TAVEC), MSNQ, psychological impact scales (MSIS), and depression (BDI) will be carried out, baseline, postrehabilitation, and also 6 and 12 months later, to evaluate the effect of CS in the longer term. CONCLUSION: This study could help to establish the usefulness of TCS or, in its absence, TCS with face-to-face help for CI in MS. The interest lies in the clear benefits of remote rehabilitation in the daily life of patients.

Afectación visuoespacial/visuoconstrucción en pacientes con esclerosis múltiple. Relación con el volumen lesional regional y la atrofia subcortical / Impairment of visuospatial/visuoconstructional skills in multiple sclerosis patients: the correlation with regional lesion load and subcortical atrophy

Introducción: Solo el 20-26% de los pacientes con esclerosis múltiple presenta déficits en habilidades visuoespaciales-visuoconstructivas (VE-VC) pese a la frecuente afectación témporo-parieto-occipital en resonancia magnética. No hay estudios que analicen la relación entre estas funciones y el volumen lesional (VL) de estas áreas cerebrales. Objetivo: Evaluar la relación del VL parieto-occipito-temporal y la atrofia subcortical con el rendimiento en funciones VE-VC en esclerosis múltiple. Metodología: De 100 pacientes de esclerosis múltiple con evaluación neuropsicológica rutinaria se seleccionan 21 por afectación en habilidades VE-VC, medidas por Figuras incompletas, Cubos (WAIS-III) y Figura compleja de Rey-Osterrieth, y 13 sin déficit cognitivo (grupo control). El VL regional se cuantifica por un método semiautomático en secuencias FLAIR y T1, y la atrofia subcortical por el ratio bicaudado y la anchura del iii ventrículo (AIIIV). Se utilizan correlaciones parciales (controlando con edad y escolarización) y regresión lineal para analizar la relación entre los parámetros de resonancia magnética y el rendimiento cognitivo. Resultados: Todas las medidas de VL y de atrofia cerebral son significativamente mayores en pacientes con deterioro cognitivo. El VL regional en FLAIR, ratio bicaudado y AIIIV muestran significativa correlación inversa con el rendimiento cognitivo, mayor entre la AIIIV y VC (Cubos: p = 0,001; Figura compleja de Rey-Osterrieth: p < 0,000). En el análisis multivariante, la AIIIV influye significativamente en tareas de VC (Cubos: p = 0,000; Figura compleja de Rey-Osterrieth: p = 0,000) y el VL regional en FLAIR en tareas VE (Figuras incompletas; p = 0,002). Conclusiones: Las medidas de atrofia subcortical se relacionan con tareas de visuoconstrucción y el VL regional con tareas VE

Introduction: About 20% to 26% of patients with multiple sclerosis (MS) show alterations in visuospatial/visuoconstructive (VS-VC) skills even though temporo-parieto-occipital impairment is a frequent finding in magnetic resonance imaging. No studies have specifically analysed the relationship between these functions and lesion volume (LV) in these specific brain areas. Objective: To evaluate the relationship between VS-VC impairment and magnetic resonance imaging temporo-parieto-occipital LV with subcortical atrophy in patients with MS. Methodology: Of 100 MS patients undergoing a routine neuropsychological evaluation, 21 were selected because they displayed VS-VC impairments in the following tests: Incomplete picture, Block design (WAIS-III), and Rey-Osterrieth complex figure test. We also selected 13 MS patients without cognitive impairment (control group). Regional LV was measured in FLAIR and T1-weighted images using a semiautomated method; subcortical atrophy was measured by bicaudate ratio and third ventricle width. Partial correlations (controlling for age and years of school) and linear regression analysis were employed to analyse correlations between magnetic resonance imaging parameters and cognitive performance. Results: All measures of LV and brain atrophy were significantly higher in patients with cognitive impairment. Regional LV, bicaudate ratio, and third ventricle width are significantly and inversely correlated with cognitive performance; the strongest correlation was between third ventricle width and VC performance (Block design: P = .001; Rey-Osterrieth complex figure: P < .000). In the multivariate analysis, third ventricle width only had a significant effect on performance of VC tasks (Block design: P = .000; Rey-Osterrieth complex figure: P = .000), and regional FLAIR VL was linked to the VS task (Incomplete picture; P = .002). Conclusions: Measures of subcortical atrophy explain the variations in performance on visuocostructive tasks, and regional FLAIR VL measures are linked to VS task

Impairment of visuospatial/visuoconstructional skills in multiple sclerosis patients: the correlation with regional lesion load and subcortical atrophy.

INTRODUCTION: About 20% to 26% of patients with multiple sclerosis (MS) show alterations in visuospatial/visuoconstructive (VS-VC) skills even though temporo-parieto-occipital impairment is a frequent finding in magnetic resonance imaging. No studies have specifically analysed the relationship between these functions and lesion volume (LV) in these specific brain areas. OBJECTIVE: To evaluate the relationship between VS-VC impairment and magnetic resonance imaging temporo-parieto-occipital LV with subcortical atrophy in patients with MS. METHODOLOGY: Of 100 MS patients undergoing a routine neuropsychological evaluation, 21 were selected because they displayed VS-VC impairments in the following tests: Incomplete picture, Block design (WAIS-III), and Rey-Osterrieth complex figure test. We also selected 13 MS patients without cognitive impairment (control group). Regional LV was measured in FLAIR and T1-weighted images using a semiautomated method; subcortical atrophy was measured by bicaudate ratio and third ventricle width. Partial correlations (controlling for age and years of school) and linear regression analysis were employed to analyse correlations between magnetic resonance imaging parameters and cognitive performance. RESULTS: All measures of LV and brain atrophy were significantly higher in patients with cognitive impairment. Regional LV, bicaudate ratio, and third ventricle width are significantly and inversely correlated with cognitive performance; the strongest correlation was between third ventricle width and VC performance (Block design: P=.001; Rey-Osterrieth complex figure: P<.000). In the multivariate analysis, third ventricle width only had a significant effect on performance of VC tasks (Block design: P=.000; Rey-Osterrieth complex figure: P=.000), and regional FLAIR VL was linked to the VS task (Incomplete picture; P=.002). CONCLUSIONS: Measures of subcortical atrophy explain the variations in performance on visuocostructive tasks, and regional FLAIR VL measures are linked to VS tasks.

Cytokine IL-1 beta but not IL-1 alpha promoter polymorphism is associated with Alzheimer disease in a population from the Canary Islands, Spain.

AIMS: Previous studies have reported the presence of low-grade inflammation in Alzheimer disease (AD). Based on these data, our work attempts to investigate the effects of some promoter polymorphisms of pro-inflammatory cytokines [interleukin (IL)-1 alpha and IL-1 beta] on AD. PATIENTS AND METHODS: A PCR-RFLP technique was used to analyze the promoter polymorphisms of both IL-1 alpha (-889 C/T) and IL-1 beta (-511 C/T) and the APOE genotype from the DNA samples of 282 patients (according to NINCDS-ADRDA criteria) and 312 control subjects. RESULTS: (i) The risk of developing AD in our population was associated with the IL-1 beta (-511 C/T) promoter polymorphism; (ii) such risk was independent of the risk factor allele in the APOE gene (APOE4); and (iii) the IL-1 alpha promoter polymorphism (-889 C/T) was not associated with the disease. CONCLUSION: In our population, IL-1 beta promoter polymorphism (-511 C/T) is an independent risk factor for AD.

Fuerte modulación del sexo sobre el riesgo conferido por el gen de la apolipoproteína E para la enfermedad de Alzheimer en la población de las Islas Canarias, España / Gender has a strong modulating effect on the risk of Alzheimer's disease conferred by the apolipoprotein e gene in the population of the canary islands, Spain

Objetivos. En la mayoría de las poblaciones estudiadas se ha encontrado que el gen de la apolipoproteína E (ApoE) es un factor de riesgo para la enfermedad de Alzheimer (EA). El objetivo del presente trabajo es el estudio de los polimorfismos de dicho gen, tanto de las variantes exónicas como los polimorfismos de promotor, en nuestra población canaria. Sujetos y métodos. Hemos analizado, por medio de técnicas de PCR-RFLP, tanto las variantes exónicas como tres de los polimorfismos de promotor más comunes del gen de la ApoE, sobre el ADN de 131 pacientes diagnosticados clínicamente de probable EA, según criterios NINCS-ADRDA, y 85 controles sin déficit cognitivo medido por el test de CAMCOG. Resultados. Hemos encontrado que las variantes alélicas exónicas del gen de la ApoE se relacionan fuertemente con la EA, presentan un claro efecto de dosis sobre la susceptibilidad a la enfermedad, mientras que no muestran ninguna asociación con la enfermedad las variantes de promotor. En nuestra muestra analizada, el gen de la ApoE no parece influir en la edad de presentación de la enfermedad, mientras que el sexo de los pacientes confiere características de susceptibilidad distintivas. Conclusión. En nuestra población, el gen de la ApoE se relaciona con la EA, y está condicionado por una fuerte modulación del sexo de los pacientes (AU)

Objectives. Apolipoprotein E (ApoE) gen has been found to confer risk for Alzheimer disease in every population studied. We are interested in analyzed the exonic variants and the promoter polymorphisms in our Canary population. Subjects and methods. By means of PCR-RFLP analysis of DNA from patients (NINCS-ADRDA criteria) and controls (cognitive state CAMCOG test measured) we analyzed the known exonic and promoter polymorphism of ApoE gen. Results. We have found an association of Alzheimer disease risk based on exonic variants of ApoE gen, with a clear cut dose-effect on susceptibility and no risk conferred by the promoter polymorphisms. Age at onset are not affected by variants of ApoE gen, and patients gender strongly modulate the disease susceptibility. Conclusion. We have found in our Canary population an association between Alzheimer disease with exonic variants of ApoE gen with a strong modulation by the patients gender (AU)

[Guillain-Barré syndrome in the northern area of Gran Canaria and the island of Lanzarote]. / Sindrome de Guillain-Barré en el area norte de Gran Canaria e isla de Lanzarote.

AIMS: The objective of this study is to analyse the incidence and clinical characteristics of Guillain Barre syndrome (GBS) in the Canary Islands. PATIENTS AND METHODS: We conducted a retrospective study of GBS patients (according to diagnostic criteria from the National Institute of Neurological and Communicative Disorders and Stroke) treated in the Ntra. Sra. del Pino Hospital in Gran Canaria between 1983 and 1998. Annual incidence, seasonal distribution, preceding infection, clinical and electrophysiological data, and evolution were all evaluated. Prognostic factors were studied by means of a univariate analysis. RESULTS: A total of 81 patients were selected for the study. The raw incidence was 1.04/100,000 inhab./year (CI 95%: 0.83 1.29; adjusted for age to the European population: 1.5). The rates of incidence were higher in men and increased lineally with age in both sexes. We observed an upward tendency during the winter months. 48% of the patients displayed serious motor deficits in the nadir of the disease, and 17.8% required assisted ventilation. After one year s evolution 74% were seen to experience an excellent recovery. The mortality rate was 8.2% and 37% received immunomodulatory treatment. The main variables associated with a bad prognosis at 3 and 12 months were: serious deficits in muscular balance, the need for assisted ventilation and very reduced amplitude of evoked motor potential. CONCLUSIONS: GBS incidence in the Canary Islands is similar to that found in other countries. An increase with age and an upward tendency during the winter months was observed. No differences were found in the clinical data as compared with other series.

Síndrome de Guillain-Barré en el área norte de Gran Canariae isla de Lanzarote / Guillain-Barré syndrome in the northern area of Gran Canaria and the island of Lanzarote

Objetivo. Analizar la incidencia y características clínicas del síndrome de Guillain-Barré (SGB) en Canarias. Pacientes y métodos. Estudio retrospectivo de pacientes con SGB (criterios diagnósticos del National Institute of Neurological and Communicative Disorders and Stroke) atendidos en el H. Ntra. Sra. del Pino de Gran Canaria, entre 1983 y 1998. Se evaluaron la incidencia anual, distribución estacional, infección precedente, los datos clínicos y electrofisiológicos y la evolución. Se investigaron factores pronósticos mediante un análisis univariante. Resultados. Se seleccionaron 81 pacientes. La incidencia cruda fue 1,04/100.000 hab/año (IC 95 por ciento: 0,83-1,29) (ajustada por edad a la población europea: 1,5). Las tasas de incidencia fueron más altas en el hombre y aumentaban linealmente con la edad en ambos sexos. Observamos predilección por los meses de invierno. El 48 por ciento de los pacientes alcanzaron déficit motores graves en el nadir de la enfermedad, y un 17,8 por ciento precisó ventilación asistida. Al año de evolución, el 74 por ciento tuvo una excelente recuperación. La mortalidad fue del 8,2 por ciento. El 37 por ciento recibió tratamiento inmunomodulador. Las principales variables asociadas a mal pronóstico a los tres y 12 meses fueron: déficit graves en el balance muscular, necesidad de ventilación asistida y una amplitud muy reducida de potencial motor evocado. Conclusiones. La incidencia del SGB en Canarias es similar a la que se encontró en otros países. Observamos un aumento de la incidencia con la edad y predilección por los meses de invierno. No encontramos diferencias en los datos clínicos con otras series (AU)

[Sweet's syndrome and motor neuron disease associated with esophageal carcinoma]. / Síndrome de Sweet y afectación de motoneurona asociados a carcinoma de esófago.

The paraneoplastic syndromes are an a group of clinical manifestations of uncommon frequency that they are associated with tumors and they often are precursors of these. The Sweet's syndrome is a dermatosis characterized by fever, erythematous plaques and infiltrate consisting of mature neutrophils. It occurs occasionally in association with hematologic malignancies and is very rare with solid tumors. The isolated motor neuron disease is rare like paraneoplastic syndrome. We report the case of a patient with epidermoid carcinoma of esophagus that it was diagnosed after beginning clinically with two paraneoplastic syndromes: Sweet's syndrome and motor neuron disease.

Síndrome de Sweet y afectación de motoneurona asociados a carcinoma de esófago / Sweet´s syndrome and neuron motor disease associated with esophagus carcinoma

Los síndromes paraneoplásicos son cuadros clínicos infrecuentes que se asocian a neoplasias, siendo a menudo precursores de éstas. El síndrome de Sweet es una dermatosis caracterizada por fiebre y placas eritematosas con infiltrado de neutrófilos maduros. Ocasionalmente acompaña a tumores de tipo hematológicos y más raramente a tumores sólidos. La lesión aislada de motoneurona es rara como síndrome paraneoplásico. Presentamos el caso de un paciente con carcinoma epidermoide de esófago que se diagnosticó tras debutar clínicamente con dos síndromes paraneoplásicos: dermatosis neutrofílica febril y afectación de motoneurona (AU)

[Acute cerebral ischemia in patients under 45 years of age: a study in a series of 68 patients]. / Isquemia cerebral aguda en pacientes menores de 45 años: estudio de una serie de 68 pacientes.

OBJECTIVE: To study acute cerebrovascular ischemia in young persons in our environment. PATIENTS AND METHODS: A retrospective study was made of 68 patients, aged between 15 and 45, with transient ischemic accidents (TIA) or cerebral infarcts. Etiological factors, clinical features, diagnostic groups according to the TOAST classification and prognosis were analyzed. RESULTS: In our series there was a ratio of 1.26 in favor of the women. TIAs made up 29.4% of the cases. The most commonly affected territory was that of the carotid artery. In persons over the age of 30 there was greater prevalence of this pathology and of the risk factors diabetes, arterial hypertension and dyslipaemia. There was a significant association with migraine in those aged under 30. The biggest diagnostic group was that of infarct of unknown origin (39.7%), probably because of the specificity of the classification. The functional condition on follow-up was good: only 4.4% of the patients had a score greater than 3 on the Rankin scale. There were no deaths either during the acute phase or subsequently. Recurrences were seen in 10.2% of the patients. CONCLUSIONS: The results obtained in our series are similar to those published in the literature. In acute cerebrovascular ischaemia in young persons, full diagnostic investigations should be carried out in order to give suitable treatment.

[Spontaneous dissection of extra-intracranial intern carotid]. / Disección espontánea de arteria carótida interna extraintracraneal.

Dissection of internal carotid artery is an unusual cause of stroke. It generally affects the extracranial portion of the vessel, rarely the intra-cranial portion and exceptionally both sections simultaneously. We present two cases of spontaneous dissection with extra and intra-cranial involvement. Two females, 46 and 36 years old, presented as stroke of the right internal carotid (ICA) associated with headaches and ipsilateral Horner's syndrome. An echo-Doppler was done on the first patient, which turned to be normal, and carotid angiography was done to both patients. The first patient showed a filiform stenosis of the right ICA that ran from the origin to the carotid siphon. The second patient showed a longitudinal stenosis of the right ICA 2 cm from the origin, which ended in an obstruction of the terminal branches. The control angiographs at five and six months respectively, showed partial re-channelling or complete re-channelling. The first case was treated with anti-aggregants and the second with anticoagulants. There were no new episodes in either cases. Dissection of the ICA usually only affects the extracranial portion of the artery, stopping in the petrous portion. We do not know why dissection also affected the intra-cranial section of the artery in these two cases.

[Listeriosis in the nonpregnant adult during an epidemic outbreak on Grand Canary]. / Listeriosis en el adulto no gestante durante un brote epidémico en Gran Canaria.

Twenty-two cases of community-acquired epidemic listeriosis were recorded from December 31st, 1991, to May 15th, 1993, at the Nuestra Señora del Pino Hospital, Las Palmas. The incidence during this outbreak was 31 times higher than the corresponding incidence in the last few years. Twelve cases occurred in pregnant women and/or neonates and ten in non-pregnant adult individuals. Our aim was to study the clinical, biological, radiological, and evolutive issues in non-pregnant adult patients. Six patients had some immunosuppressive condition: cancer, chemotherapy, AIDS, diabetes, and alcoholism. Eight patients had documented involvement of central nervous system: 6 cases of meningitis and 3 of cerebritis (one case had both meningitis and cerebritis); in the remaining two patients associated with seizures and acute confusional states, respectively. A neurological involvement was not documented because of the fulminant clinical course. CSF examination revealed mononuclear predominance in half of meningitis cases and was normal in two of the three cerebritis cases. The mean time from admission to diagnosis was 3.5 days. All patients but the two who died in the first hours of the disease received ampicillin and an aminoglycoside. The response to therapy was excellent with exception of one patient with meningitis who died in the fourth day of therapy. The clustering of listeriosis cases should alert physicians about the possibility of an epidemic outbreak. Listeria infection in non-pregnant adult individuals in this outbreak showed a high rate of neurological involvement, with focal cerebritis and pleocytosis with a mononuclear predominance in meningitis.

[Paroxysmal dystonia as the first manifestation of multiple sclerosis]. / Distonía paroxística como primera manifestación de esclerosis múltiple.

We discuss the case of a patient with episodes of left unilateral paroxysmal dystonia as first manifestation of multiple sclerosis, secondary to a demyelinization lesion in posterior arm of right internal capsule, detected by nuclear magnetic resonance (NMR). These episodes diminished with carbamazepine at low doses.