pure autonomic failure


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pure autonomic failure

a degenerative, sporadic neurologic disorder of adult onset, manifested principally as orthostatic hypotension and syncope, with no neurologic defects other than autonomic nervous system dysfunction evident; probably caused by selective degeneration of neurons in the sympathetic ganglia, with denervation of smooth muscle vasculature and the adrenal glands.

pure autonomic failure

Neurology A sporadic, idiopathic cause of persistent orthostatic hypotensio in and other manifestations of autonomic failure, unaccompanied by other neurologic features

pure au·to·nom·ic fail·ure

(pyūr aw'tō-nom'ik fāl'yŭr)
A degenerative, sporadic neurologic disorder of adult onset, manifested principally as orthostatic hypotension and syncope, with no neurologic defects other than autonomic nervous system dysfunction evident; probably caused by selective degeneration of neurons in the sympathetic ganglia, with denervation of smooth muscle vasculature and the suprarenal glands.
Synonym(s): Bradbury-Eggleston syndrome.
References in periodicals archive ?
TABLE Conditions associated with autonomic dysfunction (1) * Alcoholism/alcoholic neuropathy * Amyloidosis * Botulism * Cerebral infarcts * Diabetes mellitus * Guillain-Barre syndrome * Huntington's disease * Multiple sclerosis * Multiple systems atrophy * Parkinson's disease * Porphyria * Postural orthostatic tachycardia syndrome * Primary hyperhidrosis * Pure autonomic failure * Reflex sympathetic dystrophy * Spinal cord lesions * Toxic neuropathies * Tumors, paraneoplastic neuropathies
Migraine shares significant diagnostic and clinical features with both pure autonomic failure and multiple system atrophy, yet represents a distinct subtype of chronic sympathetic dysfunction.
Food and Drug Administration (FDA) has granted Orphan Drug designation to its drug candidate Droxidopa for the treatment of symptomatic neurogenic orthostatic hypotension (NOH) in patients with Primary Autonomic Failure, a group of diseases that includes Parkinson's Disease, Pure Autonomic Failure (PAF) and Multiple Systems Atrophy (MSA).
The trial is intended to assess the safety and efficacy of Droxidopa in patients suffering from symptomatic NOH associated with Parkinson's Disease, Pure Autonomic Failure and Multiple Systems Atrophy with the primary efficacy endpoint being defined as improvement in orthostatic blood pressure over time.
29 September 2011 - US Chelsea Therapeutics International Ltd (NASDAQ:CHTP) announced today that it had submitted a New Drug Application (NDA) to the US Food and Drug Administration (FDA) for approval to market Northera (droxidopa), an orally active synthetic precursor of norepinephrine, for the treatment of symptomatic neurogenic orthostatic hypotension (NOH) in patients with Parkinson's disease, multiple system atrophy and pure autonomic failure, dopamine beta hydroxylase deficiency and non-diabetic autonomic neuropathy.