Primary Dysautonomias (Dysautonomia)

Disorders of the AUTONOMIC NERVOUS SYSTEM occurring as a primary condition. Manifestations can involve any or all body systems but commonly affect the BLOOD PRESSURE and HEART RATE.

Also Known As:

Networked: 234 relevant articles (8 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hypovolemia
2.	Orthostatic Intolerance
3.	Parkinson Disease (Parkinson's Disease)
4.	Brain Injuries (Brain Injury)
5.	Dyskinesias (Dyskinesia)

Experts

1.	Lonsdale, Derrick: 3 articles (01/2015 - 03/2009)
2.	Emborg, Marina E: 3 articles (01/2014 - 01/2012)
3.	Joers, Valerie: 3 articles (01/2014 - 01/2012)
4.	Vernino, Steven: 3 articles (02/2009 - 06/2003)
5.	Low, Phillip A: 3 articles (08/2008 - 06/2003)
6.	Baguley, Ian J: 3 articles (01/2008 - 05/2007)
7.	Rascol, Olivier: 3 articles (01/2006 - 08/2003)
8.	Goldstein, David S: 3 articles (12/2003 - 06/2003)
9.	Musso, Carlos G: 2 articles (08/2015 - 01/2011)
10.	Obrenovich, Mark E: 2 articles (01/2015 - 12/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primary Dysautonomias:

1.	Norepinephrine (Noradrenaline)FDA LinkGeneric 01/01/2003 - "Norepinephrine and heart rate variability: a marker of dysautonomia in chronic Chagas cardiopathy." 12/10/2001 - "Reduced plasma noradrenaline levels and enhanced pressure response to noradrenaline infusion were compatible with a diagnosis of acute pan-dysautonomia. " 04/01/1989 - "Plasma norepinephrine in Chagas' cardioneuromyopathy: a marker of progressive dysautonomia." 04/01/1989 - "In an attempt to elucidate the nature of the dysautonomia in patients with Chagas' disease, we measured plasma norepinephrine levels, blood pressure, and heart rate, both supine and standing in 26 patients, and compared these values of patients classified according to three clinical subsets of cardiovascular manifestations with the values of nine normal volunteers and 16 patients with nonchagasic heart failure. " 11/01/1999 - "Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels."
2.	BaclofenFDA LinkGeneric 01/01/2012 - "To describe the long-term disorders of consciousness in patients with dysautonomia and hypertonia treated with intrathecal baclofen therapy (IBT). " 10/01/2001 - "If a relapse of dysautonomia occurred at the end of the test period, an implantation of a continuous intrathecal infusion pump delivering Baclofen was performed. " 01/01/2012 - "Their dysautonomia and limb hypertonia also significantly improved, contrary to patients with hypoxic brain injury who needed higher doses of baclofen (p < 0.03). " 10/01/2001 - "Within the framework of a prospective study, evaluating the efficacy of continuous intrathecal Baclofen therapy (CIBT) on hypertonia during the initial recovery phase of severe head injury, the authors report on the preliminary results of this treatment on paroxysmal dysautonomia about four patients. " 01/01/2012 - "At long-term follow-up, patients with hypoxic brain injury had a poorer functional outcome than patients with traumatic brain injury with persistent symptoms of dysautonomia associated with uncontrolled hypertonia, despite the use of intrathecal baclofen."
3.	ReninIBA 01/01/1997 - "Recent studies of the hyperadrenergic (elevated plasma norepinephrine) subgroup of orthostatic intolerance is documenting a clinical spectrum including attenuated plasma renin activity and aldosterone, reduced supine blood volume coupled with dynamic orthostatic hypovolemia, elevated plasma norepinephrine and epinephrine, impaired clearance of norepinephrine from the circulation and evidence of partial dysautonomia. "
4.	Epinephrine (Adrenaline)FDA LinkGeneric 01/01/1997 - "Recent studies of the hyperadrenergic (elevated plasma norepinephrine) subgroup of orthostatic intolerance is documenting a clinical spectrum including attenuated plasma renin activity and aldosterone, reduced supine blood volume coupled with dynamic orthostatic hypovolemia, elevated plasma norepinephrine and epinephrine, impaired clearance of norepinephrine from the circulation and evidence of partial dysautonomia. "
5.	AldosteroneIBA 01/01/1997 - "Recent studies of the hyperadrenergic (elevated plasma norepinephrine) subgroup of orthostatic intolerance is documenting a clinical spectrum including attenuated plasma renin activity and aldosterone, reduced supine blood volume coupled with dynamic orthostatic hypovolemia, elevated plasma norepinephrine and epinephrine, impaired clearance of norepinephrine from the circulation and evidence of partial dysautonomia. "
6.	Levodopa (L Dopa)FDA LinkGeneric 02/01/2013 - "Treatment regimen included l-Dopa with a mean daily dose of 621.4±346.8mg/day and symptomatic treatment of dysautonomia. " 12/01/2009 - "PD seen in the clinic has an inexorable course with eventual development of not only levodopa-refractory motor symptoms, but often cognitive dysfunction and prominent dysautonomia. " 10/23/2007 - "Fourth, in the current era, most of the disability of advancing PD is from involvement of nondopaminergic systems, including levodopa-refractory motor symptoms, dementia, and dysautonomia. " 06/01/2012 - "Multiple system atrophy (MSA) is a sporadic adult-onset neurodegenerative disorder clinically characterized by a variable combination of dysautonomia, levodopa-unresponsive parkinsonian and cerebellar symptoms. " 12/01/2009 - "This contrasts with homozygous parkin, PINK1 or DJ1 parkinsonism, characterized by young-onset (usually <40 years), and a comparatively benign course of predominantly levodopa-responsive symptoms without dementia or prominent dysautonomia. "
7.	Immunoglobulins (Immunoglobulin)IBA 05/01/2007 - "Immunoglobulin-responsive dysautonomia in Sjögren's syndrome." 02/08/2000 - "Acute idiopathic dysautonomia: electrophysiology and response to intravenous immunoglobulin." 11/01/2014 - "Despite a treatment with immunoglobulin and chemotherapy, the patient died suddenly, after a raise of dysautonomia symptoms. " 01/01/2011 - "Painful neuropathic symptoms, as well as symptoms related to dysautonomia from SFN responded significantly to treatment with intravenous immunoglobulin (IVIG). " 11/01/1999 - "Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels."
8.	Hydrocortisone (Cortisol)FDA LinkGeneric 05/01/1994 - "The disrupted patterns of cortisol and blood pressure preceded the development of hypertension and severe dysautonomia, which in turn were paralleled by increasing catecholamine and heart rate levels. " 06/22/2002 - "Urinary cortisol and creatinine concentrations, and the cortisol:creatinine ratio were compared between 12 healthy horses (group 1), 13 horses with Cushing's disease (group 2), and eight horses with dysautonomia syndrome (equine grass sickness) (group 3). " 02/01/1985 - "It is suggested that increased levels of catecholamines and cortisol contributed to the development of dysautonomia as these levels were within normal limits in patients of Guillain-Barré syndrome without autonomic dysfunction." 08/01/2009 - "Mean plasma cortisol levels at baseline and 60 mins after corticotrophin test were 22.9 +/- 11.3 ng/mL and 45.4 +/- 16.1 ng/mL. At baseline, the plasma cortisol levels were significantly higher in 17 (18%) patients, who developed respiratory failure at least 24 hrs later (28.5 +/- 12.1 ng/mL vs. 20.4 +/- 9.6 ng/mL; p = .003) and dysautonomia (33.1 +/- 14.3 ng/mL vs. 21.4 +/- 10.2 ng/mL, p = .003). " 01/01/2015 - "Case report of severe Cushing's syndrome in medullary thyroid cancer complicated by functional diabetes insipidus, aortic dissection, jejunal intussusception, and paraneoplastic dysautonomia: remission with sorafenib without reduction in cortisol concentration."
9.	Immunoglobulin M (IgM)IBA 04/01/2005 - "Treatment resulted in a decline in total IgM and improvement in the patient's painful neuropathy and dysautonomia. "
10.	Cholinesterase Inhibitors (Anticholinesterases)IBA 08/01/2008 - "Our aims were to evaluate the efficacy of cholinesterase inhibitors for dysautonomia and chronic constipation and to assess whether acute effects could predict the long term response. "

Therapies and Procedures

1.	Plasmapheresis 01/01/2009 - "Dysautonomia in AASMN is intractable even with corticosteroid therapy or plasmapheresis. " 09/01/1997 - "The objective of this study was to determine associations between early variables (requirement for ventilator support, anemia, indicators of abnormal peripheral nerve function (proprioception, vibratory, fine touch/pinprick, deep-tendon reflexes, cranial nerve involvement, dysautonomia, electrodiagnostic findings), plasmapheresis, age, and gender) and outcome variables (length of acute hospitalization, length of inpatient rehabilitation, Functional Independence Measure (FIM) Rasch converted scores, and acute and rehabilitation charges) in Guillain Barré Syndrome (GBS). "
2.	Sympathectomy (Sympathectomies) 09/01/2007 - "Recent pathological and clinical studies have suggested greater prevalence of clinical dysautonomia and cardiac sympathetic denervation in PD dementia and dementia with Lewy bodies as compared with PD without dementia. "
3.	Mechanical Ventilators (Ventilator) 09/01/1997 - "Patients requiring ventilator support were more likely to have dysautonomia (P = 0.0009). " 09/01/1997 - "The objective of this study was to determine associations between early variables (requirement for ventilator support, anemia, indicators of abnormal peripheral nerve function (proprioception, vibratory, fine touch/pinprick, deep-tendon reflexes, cranial nerve involvement, dysautonomia, electrodiagnostic findings), plasmapheresis, age, and gender) and outcome variables (length of acute hospitalization, length of inpatient rehabilitation, Functional Independence Measure (FIM) Rasch converted scores, and acute and rehabilitation charges) in Guillain Barré Syndrome (GBS). "
4.	Artificial Respiration (Mechanical Ventilation) 05/15/2004 - "Mortality from GBS most often is associated with dysautonomia and mechanical ventilation. " 09/01/2010 - "Patients with dysautonomia showed significant longer periods of coma (24.78 vs. 7.99 days) and mechanical ventilation (22.67 vs. 7.21 days). " 06/01/2001 - "Progression to mechanical ventilation was highly likely to occur in those patients with rapid disease progression, bulbar dysfunction, bilateral facial weakness, or dysautonomia. " 12/01/2011 - "The guidelines discuss the management of tetanus patients in the intensive care unit, including the use of immunoglobulin therapy, antibiotic therapy, management of analgesics, sedation and neuromuscular blockade, management of dysautonomia and specific issues related to mechanical ventilation and physiotherapy in this population. "
5.	Drug Therapy (Chemotherapy) 01/01/2004 - "Successful pharmacotherapy for an adult case with prolonged symptoms of postganglionic cholinergic dysautonomia." 05/01/1988 - "In one male patient, chemotherapy for SCLC resulted in an early improvement of autonomic dysfunction and the electrophysiological defect, documenting simultaneous regression of dysautonomia and LEMS. " 07/01/2011 - "Anesthetic consideration is focused mainly on complications related to leprosy like cardiac or respiratory dysautonomia, autonomic dysfunctions and side effects which are related to drug therapy and are challenging. " 11/01/2014 - "Despite a treatment with immunoglobulin and chemotherapy, the patient died suddenly, after a raise of dysautonomia symptoms. "