Recent advances in autoimmune autonomic ganglionopathy.
Summary of "Recent advances in autoimmune autonomic ganglionopathy."
PURPOSE OF
REVIEW:
This review highlights recent advances in our understanding of autoimmune autonomic ganglionopathy (AAG) including serological diagnosis, clinical observations, pathophysiology, and treatment. RECENT
FINDINGS:
Classical AAG is a subacute disorder associated with antibodies against ganglionic acetylcholine receptors. Recent observations demonstrate that these antibodies cause a specific inhibition of synaptic transmission in autonomic ganglia and antibody levels correlate in a predictable way with severity of autonomic failure. Ganglionic acetylcholine receptor antibodies can be associated with other neurological disorders including chronic or restricted forms of autonomic dysfunction and with neurological or paraneoplastic disorders unrelated to the autonomic nervous system. Treatment of AAG is based on case reports. It is clear that AAG patients respond to immunomodulatory treatments but combination immunotherapy may be required to achieve a clinical response. Recently, additional clinical observations indicate that AAG patients may also show signs of treatment-responsive cognitive impairment.
SUMMARY:
The pathophysiology and varied clinical presentation of AAG are becoming more clear. AAG is a treatable antibody-mediated disorder of autonomic ganglionic synaptic transmission. Future studies will continue to define the optimal diagnostic and therapeutic approach to autoimmune autonomic disorders.
Affiliation
Department of Neurology, UT Southwestern Medical Center, Dallas, Texas, USA.
Journal Details
This article was published in the following journal.
Name: Current opinion in neurology
ISSN: 1473-6551
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20634694
- DOI: http://dx.doi.org/10.1097/WCO.0b013e32833d4c7f
Medical and Biotech [MESH] Definitions
Education, Dental, Continuing
Educational programs designed to inform dentists of recent advances in their fields.
Education, Medical, Continuing
Educational programs designed to inform physicians of recent advances in their field.
Education, Nursing, Continuing
Educational programs designed to inform nurses of recent advances in their fields.
Education, Pharmacy, Continuing
Educational programs designed to inform graduate pharmacists of recent advances in their particular field.
Education, Continuing
Educational programs designed to inform individuals of recent advances in their particular field of interest. They do not lead to any formal advanced standing.
PubMed Articles
Autoimmune autonomic ganglionopathy after a CNS infection.
We report the first Greek case of autoimmune autonomic ganglionopathy seropositive for antibodies against ganglionic acetylcholine receptors, unique for an antecedent viral cerebellitis and long, slow...
Sudomotor dysfunction in autoimmune autonomic ganglionopathy: a follow-up study.
OBJECTIVE: We have previously shown that sudomotor dysfunction in autoimmune autonomic ganglionopathy is severe, widespread, and predominantly post-ganglionic. However, the long-...
Acute pandysautonomia - restitutio ad integrum by high prednisolone therapy.
Acute pandysautonomia is a rare disease defined as acute widespread and severe sympathetic and parasympathetic failure and sparing of somatic nerve fibers. The causes of this syndrome are often an aut...
Sensory ganglionopathy due to gluten sensitivity.
OBJECTIVES: Gluten sensitivity can engender neurologic dysfunction, one of the two commonest presentations being peripheral neuropathy. The commonest type of neuropathy seen in the context of gluten s...
Clinical Trials
The Relative Prevalence and Severity of Autonomic Nervous System Dysfunction
The purpose of this study is to measure the prevalence and severity of cardiac autonomic neuropathy (CAN), diabetic autonomic neuropathy (DAN) which in non-diabetics is termed advanced ner...
Autonomic Nervous System and Nitric Oxide Interactions
The investigators are interested in the role of the autonomic nervous system in the regulation of endothelial function. In particular, the investigators will study how endothelial function...
The heart rate variability assessment of the sympathetic-parasympathetic balance is a strong analytical tool in the autonomic nervous system (ANS) physiology, at each end of life. In neon...
The purpose of the study is to determine the role special antibodies play in possibly identifying Autoimmune Hepatitis through the following: Identify the response of specific T cells to...
Post-marketing Safety Study of Autoimmune Diseases Following Cervarix® Vaccination
The purpose of this post-marketing study is to evaluate the incidence of autoimmune diseases (AIDs) following females who have received at the least the first dose of Cervarix® as part of...
