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The Veterinary Clinics of North... Apr 2018Equine dysautonomia (ED; also known as equine grass sickness) is a neurological disease of unknown cause, which primarily affects grazing adult horses. The clinical... (Review)
Review
Equine dysautonomia (ED; also known as equine grass sickness) is a neurological disease of unknown cause, which primarily affects grazing adult horses. The clinical signs reflect degeneration of specific neuronal populations, predominantly within the autonomic and enteric nervous systems, with disease severity and prognosis determined by the extent of neuronal loss. This review is primarily focused on the major clinical decision-making processes in relation to ED, namely, (1) clinical diagnosis, (2) selection of appropriate ancillary diagnostic tests, (3) obtaining diagnostic confirmation, (4) selection of treatment candidates, and (5) identifying appropriate criteria for euthanasia.
Topics: Animals; Horse Diseases; Horses; Primary Dysautonomias
PubMed: 29398183
DOI: 10.1016/j.cveq.2017.11.010 -
Parkinsonism & Related Disorders Aug 2020
Review
Topics: Amyloidosis; Autonomic Nervous System; Autonomic Nervous System Diseases; Humans; Male; Middle Aged; Multiple System Atrophy; Parkinsonian Disorders; Primary Dysautonomias
PubMed: 31097298
DOI: 10.1016/j.parkreldis.2019.05.005 -
The Lancet. Neurology Sep 2017A substantial minority of patients who survive an acquired brain injury develop a state of sympathetic hyperactivity that can persist for weeks or months, consisting of... (Review)
Review
A substantial minority of patients who survive an acquired brain injury develop a state of sympathetic hyperactivity that can persist for weeks or months, consisting of periodic episodes of increased heart rate and blood pressure, sweating, hyperthermia, and motor posturing, often in response to external stimuli. The unifying term for the syndrome-paroxysmal sympathetic hyperactivity (PSH)-and clear diagnostic criteria defined by expert consensus were only recently established. PSH has predominantly been described after traumatic brain injury (TBI), in which it is associated with worse outcomes. The pathophysiology of the condition is not completely understood, although most researchers consider it to be a disconnection syndrome with paroxysms driven by a loss of inhibitory control over excitatory autonomic centres. Although therapeutic strategies to alleviate sympathetic outbursts have been proposed, their effects on PSH are inconsistent between patients and their influence on outcome is unknown. Combinations of drugs are frequently used and are chosen on the basis of local custom, rather than on objective evidence. New rigorous tools for diagnosis could allow better characterisation of PSH to enable stratification of patients for future therapeutic trials.
Topics: Autonomic Nervous System Diseases; Brain Injuries; Humans; Sympathetic Nervous System
PubMed: 28816118
DOI: 10.1016/S1474-4422(17)30259-4 -
The Veterinary Clinics of North... Jan 2002The autonomic nervous system can be affected as part of a more diffuse peripheral nerve disease such as inflammatory polyneuropathy or diabetes, or as a primary disease,... (Review)
Review
The autonomic nervous system can be affected as part of a more diffuse peripheral nerve disease such as inflammatory polyneuropathy or diabetes, or as a primary disease, such as dysautonomia. Dysautonomia is being diagnosed with increasing frequency in dogs and other species in the Midwest. Affected animals present with absence of parasympathetic and autonomic ganglia and brainstem nuclei degenerate with minimal inflammation. The cause is unknown and treatment symptomatic.
Topics: Animals; Autonomic Nervous System Diseases; Cat Diseases; Cats; Diagnosis, Differential; Dog Diseases; Dogs; Neurologic Examination
PubMed: 11785731
DOI: 10.1016/s0195-5616(03)00087-1 -
Seminars in Neurology Oct 2020Paroxysmal sympathetic hyperactivity (PSH) is a relatively common, but often unrecognized, complication of acute diffuse or multifocal brain diseases, most frequently... (Review)
Review
Paroxysmal sympathetic hyperactivity (PSH) is a relatively common, but often unrecognized, complication of acute diffuse or multifocal brain diseases, most frequently encountered in young comatose patients with severe traumatic brain injury. It is presumed to be caused by loss of cortical inhibitory modulation of diencephalic and brain stem centers and possible additional maladaptive changes in the spinal cord that combine to produce exaggerated sympathetic responses to stimulation. The syndrome consists of repeated sudden episodes of tachycardia, tachypnea, hypertension, sweating, and sometimes fever and dystonic posturing. The diagnosis is clinical. Treatment includes reducing any external stimulation that can trigger the episodes, and starting abortive (e.g., intravenous morphine) and preventive medications (e.g., gabapentin, propranolol, clonidine). Prompt and adequate treatment of PSH may reduce the likelihood of secondary complications, such as dehydration, weight loss and malnutrition, and muscle contractures.
Topics: Autonomic Nervous System Diseases; Humans; Sympathetic Nervous System
PubMed: 32906174
DOI: 10.1055/s-0040-1713845 -
Current Opinion in Cardiology Mar 2001Syncope in childhood is a common problem. In most children, syncope is benign, secondary to a disturbance in autonomic control of heart rate and blood pressure. It is... (Review)
Review
Syncope in childhood is a common problem. In most children, syncope is benign, secondary to a disturbance in autonomic control of heart rate and blood pressure. It is increasingly evident that neurally mediated syncope is a heterogeneous group of conditions, necessitating a reclassification of autonomic disorders. New entities, such as postural orthostatic tachycardia and cerebral vasoconstrictive syncope, are recognized. The key to the diagnosis of syncope is a careful history. Tilt testing can be useful when the history is unclear. Unfortunately tilt test protocols vary, affecting specificity and sensitivity. The mainstay of therapy is reassurance. If symptoms are troublesome, Fludrocortisone and B-blockers remain the favored drugs. The efficacy of Midodrine and Serotonin Uptake Inhibitors is currently under review. Cardiac pacing is effective for those patients with severe episodes and demonstrated asystole. It is not known whether pacing would be effective for the majority who have neurally mediated syncope without significant bradycardia.
Topics: Adrenergic beta-Antagonists; Autonomic Nervous System Diseases; Cardiac Pacing, Artificial; Child; Child, Preschool; Diagnosis, Differential; Electrocardiography; Fludrocortisone; Humans; Mineralocorticoids; Recurrence; Syncope, Vasovagal; Tilt-Table Test
PubMed: 11224639
DOI: 10.1097/00001573-200103000-00003 -
Acta Medica Portuguesa 1995Autonomic Nervous System (ANS) dysfunctions may be localized or not, and determine either states of hypo or hyperactivity. This article reviews the structure and... (Review)
Review
Autonomic Nervous System (ANS) dysfunctions may be localized or not, and determine either states of hypo or hyperactivity. This article reviews the structure and function of ANS, methods of evaluation, the main clinical features of its dysfunctions, as well as the most common causes involved and current therapeutic possibilities. Localized dysfunctions and states of hyperactivity are not considered.
Topics: Autonomic Nervous System; Autonomic Nervous System Diseases; Humans
PubMed: 7484256
DOI: No ID Found -
Seminars in Veterinary Medicine and... Feb 1990
Review
Topics: Animals; Autonomic Nervous System Diseases; Cat Diseases; Cats
PubMed: 2191396
DOI: No ID Found -
Clinical Neurophysiology : Official... Oct 2022To characterize and quantify autonomic involvement in patients with RFC1-related disorder of adult-onset cerebellar ataxia and idiopathic sensory neuropathy.
OBJECTIVE
To characterize and quantify autonomic involvement in patients with RFC1-related disorder of adult-onset cerebellar ataxia and idiopathic sensory neuropathy.
METHODS
We enrolled 16 subjects with biallelic RFC1 (AAGGG)n expansions and 16 age and sex-matched healthy controls that underwent comprehensive clinical and neurophysiological evaluation. Scales for Outcomes in Parkinson's Disease Autonomic Dysfunction (SCOPA-AUT) score was used to assess autonomic symptoms. Electrophysiological testing included assessment of heart rate variability and quantitative sudomotor axon reflex test (QSART). Between-group comparisons were assessed using non-parametric tests.
RESULTS
In the patient group, there were 9 men/7 women and the median age was 60.5 years. SCOPA-AUT scores were significantly higher in the RFC1 group compared to controls (22 vs 10, p < 0.001). Half of patients had cardiac autonomic neuropathy. In neurophysiology, there was resting tachycardia combined with abnormal responses during Valsalva maneuver and deep breathing among patients. QSART responses were also significantly reduced in the RFC1 group, especially in the lower limbs.
CONCLUSIONS
Autonomic dysfunction is frequent, clinically relevant and involves multiple domains in RFC1-related disorder. Patients have both sympathetic and parasympathetic involvement. From a topographical perspective, this condition is characterized by a small fiber autonomic axonopathy.
SIGNIFICANCE
Dysautonomia is frequent, severe and related to peripheral damage in RFC1-related disorder.
Topics: Adult; Autonomic Nervous System; Autonomic Nervous System Diseases; Female; Heart Rate; Humans; Male; Middle Aged; Neurophysiology; Peripheral Nervous System Diseases; Primary Dysautonomias; Valsalva Maneuver
PubMed: 35970061
DOI: 10.1016/j.clinph.2022.07.501 -
Journal of Neurology May 2011Although extrapyramidal diseases are commonly thought to solely affect the (extrapyramidal) motor system, non-motor symptoms such as behavioural abnormalities,... (Review)
Review
Although extrapyramidal diseases are commonly thought to solely affect the (extrapyramidal) motor system, non-motor symptoms such as behavioural abnormalities, dysautonomia, sleep disturbances and sensory dysfunctions are also frequently observed. Autonomic dysfunction is an important clinical component of extrapyramidal disease, but it is often not formally assessed, and thus frequently misdiagnosed. Symptoms of autonomic dysfunction can impact more on quality of life than motor symptoms. Appropriate symptom-oriented diagnosis and symptomatic treatment as part of an interdisciplinary approach can greatly benefit the patient. This review elaborates a limited overview on the treatment of cardiovascular, gastrointestinal, urogenital and sudomotor autonomic dysfunction in various extrapyramidal syndromes.
Topics: Autonomic Nervous System; Humans; Parkinson Disease; Primary Dysautonomias
PubMed: 21560066
DOI: 10.1007/s00415-011-5946-8