Isolated Autonomous Insufficiency

The isolated autonomous insufficiency caused by the loss of neurons in autonomic ganglia, causing orthostatic hypotension and other autonomic symptoms.

The isolated autonomous insufficiency formerly called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome. Here is a generalized autonomic failure without CNS involvement. This disorder is different from the multiple system atrophy, because it lacks a central or preganglionic participation. The isolated autonomous insufficiency affects more frequently women manifests itself tends to be in the fourth or fifth decade of life and does not lead to death.

The isolated autonomous insufficiency caused by the loss of neurons in autonomic ganglia, causing orthostatic hypotension and other autonomic symptoms. The isolated autonomous insufficiency formerly called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome. Here is a generalized autonomic failure without CNS involvement. This disorder is different from the multiple system atrophy, because it lacks a central or preganglionic participation. The isolated autonomous insufficiency affects more frequently women manifests itself tends to be in the fourth or fifth decade of life and does not lead to death. The etiology is unknown usually. Some cases are based on a Synukleinopathie; Synuclein may accumulate in patients with Parkinson’s disease, multiple system atrophy and Lewy body dementia. Some patients with isolated autonomous insufficiency eventually develop a multiple system atrophy or Lewy body dementia. Occasionally, the cause is an autoimmune autonomic neuropathy. Symptoms and complaints The main symptom is orthostatic hypotension, other autonomic symptoms such as depressed level of sweating, heat intolerance, urinary retention, bladder spasms (which may cause incontinence), erectile dysfunction, fecal incontinence or constipation and pupillary abnormalities occur. Diagnosis Clinical evaluation Diagnosis is a diagnosis of exclusion. The noradrenaline is usually <100 pg / ml in the beds and does not increase on standing. A postural tachycardia syndrome can be distinguished because it does not result in standing usually hypotension, norepinephrine level rises and the heart rate increased by> 30 beats / min or up to 120 beats / min within 10 min. Symptomatic treatment therapy Treatment is symptomatic: Orthostatic hypotension: vasopressors and support stockings constipation: fiber diet and stool softeners bladder spasms: Blister antispasmodics urinary retention: Possibly Self-catheterization of the bladder abnormalities when sweating: prevention of hot conditions

Health Life Media Team

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