Efficacy of a Gluten-Free Diet in the Gilles de la Tourette Syndrome: A Pilot Study.

The Gilles de la Tourette syndrome (GTS) and Non-Coeliac Gluten Sensitivity (NCGS) may be associated. We analyse the efficacy of a gluten-free diet (GFD) in 29 patients with GTS (23 children; six adults) in a prospective pilot study. All of them followed a GFD for one year. The Yale Global Tics Severity Scale (YGTSS), the Yale-Brown Obsessive-Compulsive Scale—Self Report (Y-BOCS) or the Children’s Yale-Brown Obsessive-Compulsive Scale—Self Report (CY-BOCS), and the Cavanna’s Quality of Life Questionnaire applied to GTS (GTS-QOL) were compared before and after the GFD; 74% of children and 50% of adults were males, not significant (NS). At the beginning of the study, 69% of children and 100% of adults had associated obsessive-compulsive disorder (OCD) (NS). At baseline, the YGTSS scores were 55.0 ± 17.5 (children) and 55.8 ± 19.8 (adults) (NS), the Y-BOCS/CY-BOCS scores were 15.3, (standard deviation (SD) = 12.3) (children) and 26.8 (9.2) (adults) (p = 0.043), and the GTS-QOL scores were 42.8 ± 18.5 (children) and 64 ± 7.9 (adults) (p = 0.000). NCGS was frequent in both groups, with headaches reported by 47.0% of children and 83.6% of adults (p = 0.001). After one year on a GFD there was a marked reduction in measures of tics (YGTSS) (p = 0.001), and the intensity and frequency of OCD (Y-BOCS/CY-BOCS) (p = 0.001), along with improved generic quality of life (p = 0.001) in children and adults. In conclusion, a GFD maintained for one year in GTS patients led to a marked reduction in tics and OCD both in children and adults.

Clinical rating scale for pantothenate kinase-associated neurodegeneration: A pilot study.

BACKGROUND: Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. METHODS: In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment. The scale included 34 items (maximal score, 135) encompassing 6 subscales for cognition, behavior, disability, parkinsonism, dystonia, and other neurological signs. RESULTS: Forty-seven genetically confirmed patients (30 ± 17 years; range, 6-77 years) were examined with the scale (mean score, 62 ± 21; range, 20-106). Dystonia with prominent cranial involvement and atypical parkinsonian features were present in all patients. Other common signs were cognitive impairment, psychiatric features, and slow and hypometric saccades. Dystonia, parkinsonism, and other neurological features had a moderate to strong correlation with disability. The scale showed good internal consistency for the total scale (Cronbach's α = 0.87). On interrater analysis, weighted kappa values (0.30-0.93) showed substantial or excellent agreement in 85% of the items. The scale also discriminated a subgroup of homozygous c.1583C>T patients with lower scores, supporting construct validity for the scale. CONCLUSIONS: The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society.

Gluten ataxia is better classified as non-celiac gluten sensitivity than as celiac disease: a comparative clinical study.

Gluten ataxia (GA) has customarily been considered to be the main neurological manifestation of celiac disease (CD). In recent years, the condition of non-celiac gluten sensitivity (NCGS) has been defined, which includes some patients who are not considered "true celiacs." We performed a comparative clinicopathological study of these three entities. We studied 31 GA, 48 CD and 37 NCGS patients, prospectively in the same center for a period of 7 years. The protocol study included two serological determinations for gluten sensitivity [anti-gliadin IgA and IgG (AGA) and anti-tissue transglutaminase IgA (TG) antibodies], HLA-DQ2 typing, and duodenal histological assessment. Demographics and investigative findings were compared. Females were 55 % in GA, 75 % in CD (p < 0.001), and 47 % in NCGS (N.S.). GA patients were older (59 ± 14 years) than CD (43 ± 13 years) and NCGS (41 ± 8 years) groups (p < 0.001). AGA positivity was higher in GA (100 %) than in CD (48 %) groups (p < 0.001), but similar to NCGS patients (89 %; N.S.); TG positivity was lower in GA (3.2 %) than in CD (33.3 %; p < 0.001), but similar to NCGS (2.7 %; N.S.). DQ2 (+) was lower in GA (32.2 %) than in CD (89.6 %; p < 0.001), but similar to NCGS (29.7 %; N.S.). Lymphocytic enteritis (Marsh type 1) was lower in GA (9.6 %) than in CD (66.7 %; p < 0.001), but similar to NCGS (10.8 %; N.S.). The other gluten sensitivity-related characteristics measured were different to CD patients, but very close to NCGS. We conclude that GA patients are better classified within the NCGS group, than within CD.

Trastornos relacionados con el gluten y enfermedades desmielinizantes / Gluten-related disorders and demyelinating diseases

Los trastornos relacionados con el gluten forman un espectro de enfermedades inmunomediadas que aparecen a cualquier edad, en sujetos portadores de una susceptibilidad genética, al ingerir o exponerse al contacto con el gluten. Los más frecuentes son la enfermedad celíaca y la sensibilidad al gluten. Ambas entidades pueden asociarse a otras enfermedades autoinmunitarias, como la esclerosis múltiple y la neuromielitis óptica. Los pacientes que tienen un trastorno relacionado con el gluten y a la vez una de esas 2 enfermedades desmielinizantes del sistema nervioso central podrían beneficiarse de la dieta sin gluten para ambos procesos (AU)

Gluten-related disorders are a spectrum of systemic immune mediated conditions that occur at any age in genetically susceptible individuals upon ingesting gluten. Celiac disease and gluten sensitivity are the most important conditions of the spectrum. They may be associated with other autoimmune diseases, such as multiple sclerosis and neuromyelitis optica. Treatment with a gluten-free diet can provide considerable benefits to the patients having both a gluten-related disorder and one of these 2 demyelinating diseases of the central nervous system (AU)

[Gluten-related disorders and demyelinating diseases]. / Trastornos relacionados con el gluten y enfermedades desmielinizantes.

Gluten-related disorders are a spectrum of systemic immune mediated conditions that occur at any age in genetically susceptible individuals upon ingesting gluten. Celiac disease and gluten sensitivity are the most important conditions of the spectrum. They may be associated with other autoimmune diseases, such as multiple sclerosis and neuromyelitis optica. Treatment with a gluten-free diet can provide considerable benefits to the patients having both a gluten-related disorder and one of these 2 demyelinating diseases of the central nervous system.

[Endovascular treatment of carotid stenosis: an experience in the Hospital Universitario Central de Asturias]. / Tratamiento endovascular de la estenosis carotídea: experiencia en el Hospital Universitario Central de Asturias.

INTRODUCTION: Carotid stenosis accounts for about 25% of all ischaemic cerebrovascular events. Carotid angioplasty and stenting (CAS) is a minimally invasive procedure used as an alternative to carotid endarterectomy, especially in high surgical risk patients. AIM: To analyse the effectiveness and safety of the endovascular treatment of carotid stenosis in the Hospital Universitario Central de Asturias. PATIENTS AND METHODS: The study consisted in a retrospective analysis of the carotid stenoses treated by means of CAS between February 2005 and April 2010, and the following information was recorded: demographic data, clinical diagnosis, indication of treatment, time between the onset of symptoms and beginning of treatment, angiographic findings, complications and long-term follow-up (including the rate of restenosis). RESULTS: Altogether 121 patients were treated (77.8% males and 22.2% females), with a mean age of 70.8 ± 10.7 years. The main vascular risk factors were arterial hypertension (65.3%), smoking (61.2%) and dyslipidaemia (42.1%). In 86% of cases the stenoses were symptomatic and in the remaining 14% they were asymptomatic. In 60.3% of cases they were stenoses > 70%, in 30.6% they were preocclusive stenoses and in 9.1% they were recanalisations of unstable carotid occlusions. The mean treatment time was 17.0 ± 8.3 days after the ischaemic event. The residual stenosis was less than 30% in all cases. The morbidity and mortality rate at 30 days was 4.1% and the rate of restenosis throughout a mean follow-up of 31.2 ± 10.8 months was 2.4%. CONCLUSIONS: In our hospital CAS is considered an effective and safe technique, with a rate of complications that is within the parameters that justify its indication.

Prevalence of celiac disease in multiple sclerosis.

BACKGROUND: Celiac disease (CD) is a common systemic disease related to a permanent intolerance to gluten and is often associated with different autoimmune and neurological diseases. Its mean prevalence in the general population is 1-2% worldwide. Our aim was to study the prevalence of celiac disease in a prospective series of Multiple Sclerosis (MS) patients and their first-degree relatives. METHODS: We analyzed the prevalence of serological, histological and genetic CD markers in a series of 72 MS patients and in their 126 first-degree relatives, compared to 123 healthy controls. RESULTS: Tissue IgA-anti-transglutaminase-2 antibodies were positive in 7 MS patients (10%), compared to 3 healthy controls (2.4%) (p < 0.05). OR: 5.33 (CI-95%: 1.074-26.425). No differences were found in HLA-DQ2 markers between MS patients (29%) and controls (26%) (NS).We detected mild or moderate villous atrophy (Marsh III type) in duodenal biopsies, in 8 MS patients (11.1%). We also found a high proportion of CD among first-degree relatives: 23/126 (32%). Several associated diseases were detected, mainly dermatitis 41 (57%) and iron deficiency anemia in 28 (39%) MS patients. We also found in them, an increased frequency of circulating auto-antibodies such as anti-TPO in 19 (26%), ANA in 11 (15%) and AMA in 2 (3%). CONCLUSIONS: We have found an increased prevalence of CD in 8 of the 72 MS patients (11.1%) and also in their first-degree relatives (23/126 [32%]). Therefore, increased efforts aimed at the early detection and dietary treatment of CD, among antibody-positive MS patients, are advisable.