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-...
Source: Handbook of Clinical Neurology - Category: Neurology Source Type: research

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Authors: Astudillo L, Laure A, Fabry V, Pugnet G, Maury P, Labrunée M, Sailler L, Pavy-Le Traon A Abstract Postural tachycardia syndrome (PoTS) is a multifactorial syndrome defined by an increase in heart rate ≥30bpm, within 10minutes of standing (or during a head up tilt test to at least 60°), in absence of orthostatic hypotension. It is associated with symptoms of cerebral hypoperfusion that are worse when upright and improve in supine position. Patients have an intense fatigue with a high incidence on quality of life. This syndrome can be explained by many pathophysiological mechanisms. It can be a...
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AbstractSpinocerebellar ataxia type 8 (SCA8) is a rare autosomal dominant neurodegenerative disease caused by expanded CTA/CTG repeats in theATXN8OS gene. Many patients had pure cerebellar ataxia, while some had parkinsonism, both without causal explanation. We analyzed theATXN8OS gene in 150 Japanese patients with ataxia and 76 patients with Parkinson ’s disease or related disorders. We systematically reassessed 123 patients with SCA8, both our patients and those reported in other studies. Two patients with progressive supranuclear palsy (PSP) had mutations in theATXN8OS gene. Systematic analyses revealed that patie...
Source: The Cerebellum - Category: Neurology Source Type: research
Conclusions With inclusion of LAC, the ELISA and CIA show comparable performance for the diagnosis of APS. However, correlations of APS-specific manifestations were dependent on method of detecting the aPL antibodies suggesting platforms may not be used interchangeable.
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Two UAB researchers have received Novel Research grants from the Lupus Research Alliance.   The grants, which total $100,000 for up to three years, were awarded to Andre Ballesteros-Tato and Frances Lund. The grants are aimed toward developing targeting treatments for lupus, a complex auto-immune disorder that has widespread and varied effects throughout the body. The professors are two of nine researchers nationwide who were awarded these grants to explore lupus from many different perspectives…
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AbstractAnti-U1-RNP positivity remains mandatory for the mixed connective tissue disease (MCTD) diagnosis, reason for which anti-U1-RNP occurrence in patients with lupus clinical features might determine diagnostic issues. Thus, the prevalence of 25 –30% for anti-RNP was reported in John Hopkins and LUMINA lupus cohorts and also 13% prevalence for the anti-U1-RNP in Euro-Lupus cohort. Presence of anti-U1-RNP antibodies in patients fulfilling SLE criteria (but not the MCTD ones) was associated with manifestations such as Raynaud phenomenon, mu sculoskeletal and lung impairment or nail fold capillaroscopy changes, some...
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Source: Anais Brasileiros de Dermatologia - Category: Dermatology Source Type: research
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