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

Related Links:

Psoriasis and lupus are autoimmune conditions that can affect the skin. They have some similarities but are different conditions. Learn about the causes, symptoms, diagnosis, and treatment of each condition here.
Source: Health News from Medical News Today - Category: Consumer Health News Tags: Psoriasis Source Type: news
Contributors : Emma E Davenport ; Tiffany Amariuta ; Maria Gutierrez-Arcelus ; Kamil Slowikowski ; Harm-Jan Westra ; Yang Luo ; Ciyue Shen ; Deepak A Rao ; Ying Zhang ; Stephen Pearson ; David von Schack ; Jean S Beebe ; Nan Bing ; Sally John ; Michael S Vincent ; Baohong Zhang ; Soumya RaychaudhuriSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensBackground: Cytokines are critical to human disease and are attractive therapeutic targets given their widespread influence on gene regulation and transcription. Defining the downstream regulatory mechanisms influenced by cytokines is central ...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research
Contributors : Liangyu Lin ; Liming DuSeries Type : Expression profiling by arrayOrganism : Homo sapiensMesenchymal stem cells (MSCs) have been shown to exert therapeutic effects on various autoimmune diseases. However, such therapeutic effect is not always achieved. Among many reasons, MSC culture methodologies may account for the these differences. It is known that oxygen concentration could profoundly affect the properties of MSCs. Therefore, we compared human umbilical cord derived MSCs cultured under hypoxic and normoxic conditions.We used microarrays to profile the gene expression of human umbilical cord derived MSCs...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by array Homo sapiens Source Type: research
This study is registered at ClinicalTrials.gov, number NCT02349061.FindingsBetween Oct 6, 2015, and Nov 30, 2016, 166 patients were screened, of whom 102 were randomly assigned to receive ustekinumab (n=60) or placebo (n=42). At week 24, 37 (62%) of 60 patients in the ustekinumab group and 14 (33%) of 42 patients in the placebo group achieved an SRI-4 response (percentage difference 28% [95% CI 10–47], p=0·006). Between week 0 and week 24, 47 (78%) of 60 patients in the ustekinumab group and 28 (67%) of 42 patients in the placebo group had at least one adverse event. Infections were the most common type of adv...
Source: The Lancet - Category: General Medicine Source Type: research
Anti-AMPA (anti–α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor limbic encephalitis is a rare autoimmune syndrome. It can be associated with an underlying malignancy, such as lung, breast, or thymus. We are reporting a case of a 19-year-old patient who presented with a 2-week history of confusion and short-term memory loss. A magnetic resonance imaging of brain showed increased T2 hyperintensity within the hippocampi bilaterally. Cerebrospinal fluid analysis was positive for anti-AMPA receptor antibodies. A computed tomography revealed multiple pulmonary metastases as well as an expansile lucent ...
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Radiology Corner Source Type: research
This report adds to the previously described paraneoplastic syndromes potentially associated with ovarian germ cell tumor.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Original Articles Source Type: research
Authors: Lee CH, Son JW, Kong EJ PMID: 30238713 [PubMed]
Source: Korean Circulation Journal - Category: Cardiology Tags: Korean Circ J Source Type: research
Conclusion: This series reports a higher diagnostic yield than most other published studies and opens platform for direct comparison of each available needle. It also adds to the safety data for this larger needle. In addition, it raises doubt into utility of TBLB for diagnosis of sarcoidosis, which can increase the procedural complications.
Source: Journal of Bronchology and Interventional Pulmonology - Category: Respiratory Medicine Tags: Original Investigations Source Type: research
Background: The 2016 CHEST consensus guidelines recommend use of either 21- or 22-G needles for endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA). We decided to prospectively compare sample adequacy and diagnostic yield of the 19-G with the 22-G EBUS needle, hypothesizing that a larger gauge difference might magnify the differences between 2 needle sizes. Methods: Twenty-seven patients undergoing EBUS-TBNA at our institution were evaluated. All cases were performed by a single operator formally trained in interventional pulmonology. Both Olympus 19- and 22-G needles were used at each lymph n...
Source: Journal of Bronchology and Interventional Pulmonology - Category: Respiratory Medicine Tags: Original Investigations Source Type: research
Conclusions: In Indian subcontinent, pediatric AILD subjects usually present with advanced hepatic disease, but may have a good outcome if timely therapy can be instituted. Associated autoimmune disorders should be carefully screened. There is no difference in the predictive value of the available diagnostic scores for pediatric AILD.
Source: Journal of Pediatric Gastroenterology and Nutrition - Category: Gastroenterology Tags: Original Articles: Hepatology Source Type: research
More News: Ataxia | Autoimmune Disease | Brain | Children | Gluten | Lupus | Neurology | Paraneoplastic Syndrome | Rare Diseases | Sarcoidosis