Chapter 21 Immune-mediated ataxias
Publication date: 2018 Source:Handbook of Clinical Neurology, Volume 155 Author(s): Bastien Joubert, Kevin Rostásy, Jérôme Honnorat Immune-mediated cerebellar ataxia (CA) comprises a group of rare diseases that are still incompletely described, and are probably underdiagnosed. Both acute and progressive progressions are possible. Different syndromes have been identified, including CA associated with anti-GAD antibodies, the cerebellar type of Hashimoto encephalopathy, primary autoimmune CA, gluten ataxia, opsoclonus-myoclonus syndrome, and paraneoplastic cerebellar degenerations. Most of these syndromes are associated with autoantibodies targeting neuronal antigens. Additionally, autoimmune CA can be triggered by infections, especially in children, and in rare cases occur in the context of an autoimmune multisystem disease, such as systemic lupus erythematosus, sarcoidosis, or Behçet disease. A careful workup is needed to distinguish autoimmune CA from other causes. In adults, a paraneoplastic origin must be ruled out, especially in cases with subacute onset. Neurologic outcome in adults is frequently poor, and optimal therapeutic strategies remain ill defined. The outcome in children is in general good, but children with a poor recovery are on record. The precise pathophysiologic mechanisms even in the presence of detectable autoantibodies are still largely unknown. Further research is needed on both the clinical and mechanistic aspects of immune-...
Introduction: MS is a neurodegenerative, autoimmune disease associated with significant symptom burden such as fatigue, cognitive impairment, motor dysfunction, and depression. Thus, there is a need for therapeutic options for accessible symptom management. tDCS is an emerging neuromodulation treatment that delivers low amperage direct current ( ≈2 mA) to targeted brain regions through scalp electrodes. tDCS is thought to lower the neuronal threshold required for action potentials and is often used to augment the benefit achieved through repetitive stimulation.
We present findings of two ataxic patients following the rTMS intervention
A 62-year-old Japanese male with spinocerebellar ataxia type 6(SCA6) was admitted to our hospital for exacerbation of ataxia. We administered single-pulse transcranial magnetic stimulation (TMS) over the hand motor area (40 single pulses, about 0.3Hz at 100% of RMT) and over the cerebellum (20 single pulses, about 0.5Hz at 50% of RMT) with a circular coil once a day for one session, and for five sessions a week for 2 weeks (10 sessions). This therapy was performed for 2 courses, separated by a rest period of about 2 weeks (total 20 sessions) to reduce ataxia.
Introduction: The standing postural control is dependent upon numerous inputs interacting across multiple temporal-spatial scales. As such, the dynamics of its output, i.e., postural sway, is physiologically complex. Studies have shown the degree of postural sway complexity is often diminished by aging and diseases. Spinocerebellar ataxia (SCA) is a neurodegenerative disorder caused by dysfunction of the cerebellum and its connected neural networks. People with SCA often had impaired standing postural control, and recent studies using repetitive transcranial magnetic stimulation (rTMS) can improve the postural control performance in SCA.
AbstractExercise-associated physiological disturbances alter gastrointestinal function and integrity. These alterations may increase susceptibility to dietary triggers, namely gluten and a family of short-chain carbohydrates known as FODMAPs (fermentable oligo-, di-, monosaccharides and polyols). A recent surge in the popularity of gluten-free diets (GFDs) among athletes without celiac disease has been exacerbated by unsubstantiated commercial health claims and high-profile athletes citing this diet to be the secret to their success. Up to 41% of athletes at least partially adhere to a GFD diet, with the belief that gluten...
(MedPage Today) -- French experts call for new assessment tools and better use of available treatments
ConclusionDiscontinuing a TNFi before gestational week 20 seems feasible in women with RA and JIA who enter pregnancy with well ‐controlled disease.This article is protected by copyright. All rights reserved.
ConclusionOur findings indicate that agingFMR1 premutation carriers show static and dynamic postural control deficits relative to healthy controls implicating degenerative processes of spinocerebellar and cerebellar-brainstem circuits that may be independent of or precede the onset of FXTAS. Our finding that FXTAS+ and FXTAS − premutation carriers differed on their level of intentional AP sway suggests that neural mechanisms of dynamic postural control may be differentially impacted in patients with FXTAS, and its measurement may be useful for rapidly and precisely identifying disease presence and onset.
AbstractThe impairment of regulatory T cells (Tregs) is a characteristic feature of autoimmune hepatitis (AIH), and the degradation of tryptophan (Trp) to kynurenine (Kyn), by gamma interferon-induced indoleamine-2,3-dioxygenase-1 (IDO-1), is a central metabolomics check point in the differentiation of Tregs. For this reason, we investigate whether or not Kyn and IDO activity is potentially useful biomarkers in pediatric AIH.Between January 2016 and January 2017, children of AIH type-1 (AIH-1,n = 37), AIH type-2 with liver kidney microsome-1 autoantibodies (AIH-2-LKM-1,n = 8), and autoantibody-n...
ConclusionsThis first SFGM-TC retrospective report shows long-term benefit of AHSCT in AD patients with acceptable toxicity.