Clinical impact of early brain atrophy in clinically isolated syndromes.

BACKGROUND: The impact of global and tissue-specific brain atrophy on conversion to multiple sclerosis (MS) after a clinically isolated syndrome (CIS) is not fully gauged. OBJECTIVES: We aimed to determine the magnitude and clinical relevance of brain volume dynamics in the first year after a CIS. METHODS: We assessed 176 patients with CIS within 3 months of onset, clinically and by conventional magnetic resonance imaging (MRI) scans, at baseline and 1 year after clinical onset. We determined the percentage of brain volume change (PBVC) and the brain parenchymal (BPF), grey matter (GMF) and white matter (WMF) fractions. RESULTS: The mean follow-up time was 53 months (SD = 16.8): 76 patients (43%) experienced a second attack, 32 (18%) fulfilled MRI-only 2005 McDonald criteria and 68 (39%) remained as CIS. Statistically significant decreases in the volume measures tested were observed in patients with a second attack, for BPF and PBVC; in both MS groups for GMF; whereas in all groups, the WMF was unchanged. Patients with a second attack had larger PBVC decreases (- 0.65% versus + 0.059%; p < 0.001). PBVC decreases below - 0.817% independently predicted shorter times to a second attack. CONCLUSIONS: Global brain and grey matter volume loss occurred within the first year after a CIS; brain volume loss predicted conversion to MS.

Diagnosis of epileptic syndrome after a new onset seizure and its correlation at long-term follow-up: longitudinal study of 131 patients from the emergency room.

This study aims to demonstrate the reliability of the diagnosis of epilepsy after a new onset seizure, supported by a detailed anamnesis and the complementary tests accessible at the emergency room (ER), such as CT-scan and video-EEG. It was a prospective study including 131 adult patients (55% males, medium age 52.42 (±21.5)[16-98] years-old, follow-up 25.22 (±13.69)[12-31] months). In half of cases we could not identify any predisposing factor. Within the first 72 h, patients were included into an epileptic syndrome according to the ILAE 1989 classification, if possible. Thereafter, they were followed-up in the outpatient clinic of the Epilepsy Unit, where seizure recurrence was recorded and further diagnostic examinations were performed. 94.1% of patients initially diagnosed of epilepsy were confirmed as epileptics, and up to 57% of patients could be classified into a particular syndrome from the ER. Conversely, 44.6% of patients with the initial diagnosis of isolated seizure and one third of patients with non-epileptic seizures developed recurrence, switching their initial diagnosis to epilepsy. Both CT-scan and early EEG demonstrated its usefulness evaluating the risk of recurrence after a new onset seizure (Positive predictive value 78% and 88%, respectively). Epileptiform activity was a predictor of seizure recurrence (p<0.05), independently to the moment where the EEG was performed. According to our results, it is realistic to perform the diagnosis of epilepsy after a new onset seizure, although many patients still need further specific examinations, or seizure recurrence, to be diagnosed.

An observational study of the effectiveness and safety of natalizumab in the treatment of multiple sclerosis.

AIM: To analyse the safety and effectiveness of natalizumab in the treatment of multiple sclerosis in a real clinical practice setting and according to the approved indications. PATIENTS AND METHODS: All patients with multiple sclerosis treated with natalizumab in our centre were evaluated. The clinical and radiological disease activity during the first year of treatment was analyzed in patients who received at least 12 doses of the drug. The data regarding moderate and severe adverse events in the entire study sample was also evaluated. RESULTS: A total of 112 patients were included in the study, of which 110 had been previously treated with other drugs and 76 had received at least 12 doses of natalizumab. In this group, the annualized relapse rate was reduced by 89% compared to the preceding year and 80% of patients were free from relapses after one year of treatment. Nine percent of patients exhibited 3-month confirmed disability progression. At month 12, the mean number of gadolinium-enhancing lesions on brain MRI was decreased by 99% compared to the pre-treatment MRI. During the first year of treatment, 76% of patients remained free from clinical activity and 33% remained free from both clinical and radiological disease activity. Twenty-nine percent of patients had at least one moderate or severe adverse event, which led to treatment discontinuation in 6%. Four percent of patients experienced immediate hypersensitivity reactions. CONCLUSION: This study suggests that natalizumab is effective in reducing disease activity in patients with relapsing multiple sclerosis and inadequate response to other therapies, with a favorable risk-benefit ratio.

Estudio observacional sobre la efectividad y seguridad del natalizumab en el tratamiento de la esclerosis múltiple / An observational study of the effectiveness and safety of natalizumab in the treatment of multiple sclerosis

Objetivo. Analizar la seguridad y efectividad del natalizumab en el tratamiento de la esclerosis múltiple según las indicaciones autorizadas en nuestro ámbito y en condiciones de uso real. Pacientes y métodos. Evaluamos todos los pacientes con esclerosis múltiple tratados con natalizumab en nuestro centro. Se analizó la actividad clínica y radiológica de la enfermedad durante el primer año de tratamiento en los pacientes que recibieron 12 o más dosis. Se evaluó la información relativa a los acontecimientos adversos moderados y graves en toda la muestra. Resultados. Se incluyeron 112 pacientes, de los que 110 habían sido tratados anteriormente con otros fármacos y 76 habían recibido 12 o más dosis de natalizumab. En este grupo, la tasa anualizada de brotes se redujo un 89% respecto al año previo y el 80% de los pacientes permaneció libre de brotes después de un año de tratamiento. El 9% de los pacientes presentó progresión de la discapacidad confirmada a los tres meses. En el mes 12, el número medio de lesiones que realzaban con gadolinio en la resonancia magnética cerebral disminuyó un 99% respecto a la resonancia magnética pretratamiento. Durante el primer año de tratamiento, el 76% de los pacientes no presentó actividad clínica y el 33% no presentó actividad clínica ni radiológica. Se observó al menos un acontecimiento adverso moderado o grave en el 29% de los casos, que obligó a interrumpir el tratamiento en el 6%. El 4% de los pacientes tuvo reacciones de hipersensibilidad inmediata. Conclusión. Este estudio sugiere que el natalizumab es efectivo en la reducción de la actividad de la enfermedad en pacientes con formas recurrentes de esclerosis múltiple con respuesta inadecuada a otras terapias, con una relación beneficio- riesgo favorable (AU)

Aim. To analyse the safety and effectiveness of natalizumab in the treatment of multiple sclerosis in a real clinical practice setting and according to the approved indications. Patients and methods. All patients with multiple sclerosis treated with natalizumab in our centre were evaluated. The clinical and radiological disease activity during the first year of treatment was analyzed in patients who received at least 12 doses of the drug. The data regarding moderate and severe adverse events in the entire study sample was also evaluated. Results. A total of 112 patients were included in the study, of which 110 had been previously treated with other drugs and 76 had received at least 12 doses of natalizumab. In this group, the annualized relapse rate was reduced by 89% compared to the preceding year and 80% of patients were free from relapses after one year of treatment. Nine percent of patients exhibited 3-month confirmed disability progression. At month 12, the mean number of gadolinium-enhancing lesions on brain MRI was decreased by 99% compared to the pre-treatment MRI. During the first year of treatment, 76% of patients remained free from clinical activity and 33% remained free from both clinical and radiological disease activity. Twentynine percent of patients had at least one moderate or severe adverse event, which led to treatment discontinuation in 6%. Four percent of patients experienced immediate hypersensitivity reactions. Conclusion. This study suggests that natalizumab is effective in reducing disease activity in patients with relapsing multiple sclerosis and inadequate response to other therapies, with a favorable risk-benefit ratio (AU)

Brainstem lesions in clinically isolated syndromes.

BACKGROUND: Number of baseline lesions has been shown to predict future attacks and disability in clinically isolated syndromes (CIS). OBJECTIVE: To investigate the role of baseline infratentorial lesions in long-term prognosis. METHODS: Subjects were included in a prospective cohort of patients with CIS. Patients underwent brain MRI within 3 months after CIS onset. Number and location of lesions at baseline were prospectively studied. Retrospective scan analysis was conducted to specifically look at number and location of infratentorial lesions. We analyzed the time to a second attack and to reach EDSS 3.0. RESULTS: We included 246 patients with CIS followed for a median of 7.7 years. Patients with infratentorial lesions had both a higher risk of conversion (71.4% vs 29.6%; hazard ratio [HR] 3.3; 95% confidence interval [CI] 2.2-4.8; p < 0.001) and of developing disability (32.5% vs 12.4%; HR 2.4; 95% CI 1.3-4.3; p = 0.003). Presence of at least one cerebellar lesion was associated with an increased risk of conversion (HR 2.4; 95% CI 1.3-4.5; p = 0.007). Presence of at least one brainstem lesion increased both the risk of conversion (HR 2.9; 95% CI 1.7-5.0; p < 0.001) and disability (HR 2.5; 95% CI 1.1-5.4; p = 0.026). Broken down into number of lesions, the presence of infratentorial lesions increased both the risk of conversion (83% vs 61%) (HR 22.3; 95% CI 9.7-51.1; p < 0.001) and of reaching EDSS 3.0 (40% vs 19%) (HR 3.2; 95% CI 1.3-7.4; p = 0.008) only in patients with 9 or more lesions. CONCLUSIONS: Presence of infratentorial lesions increases the risk for disability. Brainstem rather than cerebellar lesions may be responsible for poor prognosis.

[A study of right-left shunt in transient global amnesia]. / Estudio de shunt derecha-izquierda en la amnesia global transitoria.

INTRODUCTION: Transient global amnesia (TGA) is a disorder of unknown aetiology. In recent studies, TGA was associated with a right to left shunt (RLS). We studied the presence of the RLS in patients with TGA and we compared this series with patients who had suffered a transient ischaemic attack (TIA). PATIENTS AND METHODS: We included 66 consecutive TGA patients. In these patients a transcranial Doppler was performed to determine the presence of a RLS. We collected data on the TGA episode, vascular risk factors, migraine history, recurrence of TGA and neuroimaging in patients with and without RLS. We compared the prevalence of the RLS in TGA series with 59 patients with TIA. RESULTS: The prevalence of RLS was 21.2% in patients with TGA. The RLS was associated with the migraine history (40% versus 13%; p = 0.014) and a Valsalva manoeuvre as a triggering factor (50% versus 14.5%; p = 0.022). A greater prevalence of RLS was detected in patients with TIA (55.9% versus 21.2%; p < 0.001). CONCLUSIONS: The RLS prevalence in TGA patients is similar to the general population but significantly lower than the prevalence in TIA patients. The association with a Valsalva manoeuvre as a precipitating factor in the TGA patients with RLS could play a role in the aetiopathogenesis of the TGA.

Genetic association of CASP8 polymorphisms with primary progressive multiple sclerosis.

We investigated caspase 8 (CASP8) as a candidate gene for multiple sclerosis (MS) susceptibility. Three SNPs (rs2037815, rs12990906 and rs1035140) were genotyped in 546 MS patients and 547 controls. For SNP rs2037815, GG homozygosity was associated with primary progressive multiple sclerosis (PPMS) when compared with relapse-onset MS and controls. We identified risk (GCA) and protective (ACT) haplotypes associated with PPMS when compared with relapse-onset MS and controls. GG homozygosity for SNP rs2037815 in PPMS patients was associated with a trend towards faster disease progression. These findings point to a role of CASP8 polymorphisms in the MS genetic risk in PPMS patients.

Estudio de shunt derecha-izquierda en la amnesia global transitoria / study of right-left shunt in transient global amnesia

Introducción: La amnesia global transitoria (AGT) es una entidad de etiopatogenia incierta. En recientes estudios se cita la posible asociación entre la AGT y el shunt derechaizquierda (SDI). Por ello estudiamos la presencia de SDI en una serie de pacientes con AGT de nuestra población y la comparamos con otra población de pacientes con ataque isquémico transitorio (AIT). Pacientes y métodos: Recogimos de forma consecutiva 66 pacientes con AGT en los que se realizó un estudio de SDI mediante Doppler transcraneal. Comparamos las características clínicas del episodio, los factores de riesgo vascular, el antecedente de migraña, la recurrencia de AGT y la neuroimagen entre las AGT con y sin SDI. Comparamos la prevalencia de SDI con la de una serie de 59 casos de AIT de origen indeterminado. Resultados: En el grupo de AGT la prevalencia de SDI fue del 21,2%. La presencia de SDI se asoció con el antecedente de migraña (el 40 frente al 13%; p = 0,014) y una maniobra de Valsalva como factor desencadenante (el 50 frente al 14,5%; p = 0,022). Se observó una mayor frecuencia de SDI en el grupo de AIT (el 55,9 frente al 21,2%; p < 0,001). Conclusiones: La prevalencia de SDI en los pacientes con AGT es similar a la descrita en la población general, pero significativamente inferior a su prevalencia en aquellos con AIT de origen indeterminado. La asociación con una maniobra de Valsalva como desencadenante del episodio en las AGT con SDI podría implicar un mecanismo etiopatogénico en este subgrupo (AU)

Introduction: Transient global amnesia (TGA) is a disorder of unknown aetiology. In recent studies, TGA was associated with a right to left shunt (RLS). We studied the presence of the RLS in patients with TGA and we compared this series with patients who had suffered a transient ischaemic attack (TIA). Patients and methods: We included 66 consecutive TGA patients. In these patients a transcranial Doppler was performed to determine the presence of a RLS. We collected data on the TGA episode, vascular risk factors, migraine history, recurrence of TGA and neuroimaging in patients with and without RLS. We compared the prevalence of the RLS in TGA series with 59 patients with TIA. Results: The prevalence of RLS was 21.2% in patients with TGA. The RLS was associated with the migraine history (40% versus 13%; p = 0.014) and a Valsalva manoeuvre as a triggering factor (50% versus 14.5%; p = 0.022). A greater prevalence of RLS was detected in patients with TIA (55.9% versus 21.2%; p < 0.001). Conclusions: The RLS prevalence in TGA patients is similar to the general population but significantly lower than the prevalence in TIA patients. The association with a Valsalva manoeuvre as a precipitating factor in the TGA patients with RLS could play a role in the aetiopathogenesis of the TGA (AU)

Gliomatosis cerebri / Cerebral gliomatosis

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