Positive oligoclonal bands and CSF pleocytosis in narcolepsy type 1: A case report supporting the immune-mediated hypothesis.

Narcolepsy-type 1 is a neurological sleep-disorder caused by a selective loss of hypothalamic orexin/hypocretin-producing neurons whose underlying mechanism is considered to be immune-mediated. We report the case of a 16 year-old girl with excessive daytime sleepiness, hypnagogic/hypnopompic hallucinations and cataplexy, fulfilling narcolepsy-type 1 diagnostic criteria. She was HLA-DQB1*06:02/DQA1*01:02 positive. CSF analysis demonstrated positive IgG oligoclonal bands, pleocytosis and hypocretin-1 below detection limit. Other autoimmune processes were excluded, including autoimmune encephalitis. After treatment with intravenous immunoglobulins sleep-related hallucinations transiently improved for a month. This case's CSF inflammatory findings support the role of neuroinflammation in narcolepsy-type 1 development in genetically predisposed patients.

Integrative group-based cognitive rehabilitation efficacy in multiple sclerosis: a randomized clinical trial.

PURPOSE: This study aimed to determine the efficacy of the integrative group-based cognitive rehabilitation programme, REHACOP, on improving cognitive functions in multiple sclerosis (MS). METHODS: Fourty-two MS patients were randomized to the treatment programme REHACOP (n = 21) or waiting list control condition (n = 21). The REHACOP group received cognitive rehabilitation in group format for three months focused on attention, processing speed, learning and memory, language, executive functioning, and social cognition. Patients completed a neuropsychological assessment at baseline and follow-up, which included tests of attention, processing speed, working memory, verbal memory, verbal fluency, and executive functioning. Repeated measures multivariate analysis of covariance (MANCOVA) was used to determine the efficacy of the cognitive rehabilitation programme. RESULTS: Group × Time interactions revealed significant improvements in the REHACOP group as compared with the control group for processing speed (p = 0.011, np2 = 0.16), working memory (p = 0.014, np2 = 0.15), verbal memory (p = 0.025, np2 = 0.13), and executive functioning (p = 0.024, np2 = 0.13), showing medium-large effect sizes. CONCLUSIONS: Patients receiving REHACOP showed improvements in several cognitive domains. This preliminary study thus provides evidence supporting the efficacy of this integrative group-based cognitive rehabilitation intervention in MS. Future research should confirm these findings, examine the impact of the treatment on everyday life functioning and explore the presence of brain changes associated with cognitive rehabilitation. Implications for rehabilitation This study provides initial evidence for integrative group-based cognitive rehabilitation efficacy in MS patients through the implementation of the REHACOP cognitive rehabilitation programme. Patients received cognitive rehabilitation for three months (3 one-hour-sessions per week) focused on training attention, learning and memory, language, executive functioning, and social cognition. Patients attending REHACOP sessions showed medium to large and statistically significant improvements in processing speed, working memory, verbal memory, and executive functioning.

The influence of posterior visual pathway damage on visual information processing speed in multiple sclerosis.

BACKGROUND: The injury of visual pathway and abnormalities of visual processing speed (VPS) are frequent in MS, but their association remains unexplored. OBJECTIVE: To evaluate the impact of posterior visual pathway structural and functional integrity on VPS of MS patients. METHODS: Cross-sectional study of 30 MS patients and 28 controls, evaluating the association of a VPS tests composite (Salthouse Perceptual Comparison test, Trail Making Test A and Symbol Digit Modalities Test) with 3T MRI visual cortex thickness, optic radiations (OR) diffusion tensor imaging indexes, and medial visual component (MVC) functional connectivity (FC) (MVC-MVC FC (iFC) and MVC-brain FC (eFC)) by linear regression, removing the effect of premorbid IQ, fatigue, and depression. RESULTS: V2 atrophy, lower OR fractional anisotropy (FA) and MVC FC significantly influenced VPS in MS (at none or lesser extent in controls), even after removing the effect of Expanded Disability Status Scale and previous optic neuritis (V2 ( r2 = 0.210): ß = +0.366, p = 0.046; OR FA ( r2 = 0.243): ß = +0.378, p = 0.034; MVC iFC, for example, left cuneus ( r2 = 0.450): ß = -0.613, p < 0.001; MVC eFC, for example, right precuneus-postcentral gyrus ( r2 = 0.368): ß = -0.466, p = 0.002). CONCLUSION: Posterior visual pathway integrity, structural (V2 thickness and OR FA) and functional (MVC FC), may explain respectively up to 24% and 45% of VPS variability in MS.

Situaciones especiales en la esclerosis múltiple / Special cases of multiple sclerosis

La esclerosis múltiple es una enfermedad crónica que afecta, en general, a personas jóvenes y les acompaña a lo largo de toda su vida. Esta enfermedad genera en el paciente y en sus familiares una serie de dudas y preguntas acerca de hechos cotidianos y de todo tipo de situaciones que van a ir apareciendo en las distintas etapas de la vida y que pueden influir en el curso de la enfermedad. El objetivo de esta revisión es dar respuesta concreta a estas cuestiones (AU)

Multiple sclerosis is a chronic disease that usually occurs in young people and affects them for the rest of their lives. Patients and their families usually have a series of doubts and questions on everyday matters and all types of situations that occur during the distinct stages of life and which can influence the course of the disease. The aim of this review is to provide specific answers to these questions (AU)

Mortality and cause of death in multiple sclerosis: findings from a prospective population-based cohort in Bizkaia, Basque Country, Spain.

BACKGROUND: Mortality studies of multiple sclerosis (MS) patients are scarce. OBJECTIVE: To assess mortality of MS patients included in a large MS cohort from the county of Bizkaia in Spain. METHODS: Patients were collected from a dynamic population-based cohort of patients with MS from the county of Bizkaia (named the 'Bizkaia cohort') in the Basque Country (Spain). Data from official registries were used for estimating mortality indicators. RESULTS: During a 24-year period (1987-2011), a total of 1,283 patients were included in the Bizkaia cohort. Eighty-nine patients (6.9%) had died before December 31, 2011. The standardized mortality ratio was 2.78 for the whole group (3.26 for men and 2.73 for women), with a decreased life expectancy of 6.53 years. The mean survival from the onset of MS in deceased patients was 22.4 years. Death was related directly or indirectly to MS in 57 patients and unrelated in 27, while the cause of death was unknown in the remaining 5. Respiratory infection and sepsis were the most frequent MS-related causes of death. Differences in survival according to gender or disease course were not observed. CONCLUSION: Patients with MS included in the 'Bizkaia cohort' had an almost threefold increase in the risk for death. Life expectancy is reduced by 6-10 years.

[Special cases of multiple sclerosis]. / Situaciones especiales en la esclerosis múltiple.

Multiple sclerosis is a chronic disease that usually occurs in young people and affects them for the rest of their lives. Patients and their families usually have a series of doubts and questions on everyday matters and all types of situations that occur during the distinct stages of life and which can influence the course of the disease. The aim of this review is to provide specific answers to these questions.