ALS, Amyotrofisk lateralskleros, är en neurodegenerativ sklerotisk nervsjukdom som leder till att de nervceller som styr kroppens muskler gradvis förtvinar. Den är den allvarligaste sjukdomen i gruppen motorneuronsjukdomar och kallas för djävulens sjukdom.
Den drabbar oftast helt friska personer i övre medelåldern. Många är mycket fysiskt aktiva. Sjukdomen orsakar tilltagande kraftnedsättning följt av borttynande muskler (muskelatrofi) och slutligen generell förlamning. Den direkta dödsorsaken är ofta förlorad andningsförmåga.
Det debatteras huruvida det är en autoimmun sjukdom eller inte. Flera studier har visat att NKT-celler och cytotoxiska CD8 T-lymfocyter är förhöjda vid ALS, vilket talar för inflammation och kanske autoimmunitet.
Nu kommer ytterligare ett tecken, både på att det finns en autoimmun koppling, och att autoimmunitet är kopplat till gluten.
Det är nämligen en ny studie som visar att ALS har en koppling till autoimmunitet och överkänslighet mot gluten. Patienterna med ALS hade i högre utsträckning transglutaminas 6-antikroppar i blodet, vilket är ett tecken på att deras immunförsvar reagerar mot gluten.
Citerar sammanfattningen av studien:
“Importance Celiac disease is an autoimmune disorder triggered by gluten in genetically predisposed individuals. Gluten sensitivity can cause neurologic manifestations, such as ataxia or neuropathy, with or without gastrointestinal symptoms. Many patients with gluten ataxia produce antibodies toward the newly identified neuronal transglutaminase 6 (TG6). Two case reports described patients initially diagnosed with amyotrophic lateral sclerosis (ALS) and ultimately with celiac disease who improved with a strict gluten-free diet.
Objective To evaluate the prevalence of celiac disease–related antibodies and HLA antigen alleles, as well as TG6 antibodies, in patients with ALS and healthy individuals serving as controls to determine whether a neurologic presentation of a gluten-related disorder mimicking ALS might occur in some patients.
Design, Setting, and Participants In a case-control study conducted in an ALS tertiary center, we measured serum levels of total IgA antibodies, IgA antibodies to transglutaminase 2 (TG2) and endomysium, as well as IgA and IgG antibodies to deamidated gliadine peptide and TG6 and performed HLA antigen genotyping in 150 consecutive patients with ALS and 115 healthy volunteers of similar age and sex. Participants did not have any known autoimmune or gastroenterologic disorder and were not receiving any immunomodulatory medications. The study was conducted from July 1, 2010, to December 31, 2012.
Main Outcomes and Measures Antibody levels and frequency of individuals with abnormal antibody values as well as frequency of HLA antigen alleles were compared between patient and control groups.
Results All patients and control group participants were seronegative to IgA antibodies to TG2, endomysium, and deamidated gliadine peptide. Twenty-three patients (15.3%) were seropositive to TG6 IgA antibodies as opposed to only 5 controls (4.3%) (P = .004). The patients seropositive for TG6 showed a classic picture of ALS, similar to that of seronegative patients. Fifty patients and 20 controls were tested for celiac disease–specific HLA antigen alleles; 13 of 22 TG6 IgA seropositive individuals (59.1%) were seropositive for celiac disease–related alleles compared with 8 (28.6%) of the 28 seronegative individuals (P = .04). Mean (SD) levels of IgA antibodies to TG2 were 1.78 (0.73) in patients and 1.58 (0.68) in controls (normal, <10). In a subset of study participants, mean levels of deamidated gliadin peptide autoantibodies were 7.46 (6.92) in patients and 6.08 (3.90) in controls (normal, <16). Mean levels of IgA antibodies to TG6 were 29.3 (30.1) in patients and 21.0 (27.4) in controls (P = .02; normal, <26).
Conclusions and Relevance The data from this study indicate that, in certain cases, an ALS syndrome might be associated with autoimmunity and gluten sensitivity. Although the data are preliminary and need replication, gluten sensitivity is potentially treatable; therefore, this diagnostic challenge should not be overlooked.”
http://archneur.jamanetwork.com/article.aspx?articleid=2241558
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