Acute pandysautonomia

Acute pandysautonomia, also known as acute panautonomic neuropathy or pure pandysautonomia, is a rare acquired disorder of the peripheral autonomic nervous system characterized by the rapid onset of widespread failure of both sympathetic and parasympathetic autonomic functions. It is considered a variant of Guillain-Barré syndrome (GBS) that selectively or predominantly affects autonomic nerve fibers rather than motor or sensory nerves. The condition typically develops over days to weeks, often following a viral illness or other infectious trigger, and is believed to involve an immune-mediated attack on autonomic ganglia or nerve fibers. The disease affects multiple body systems controlled by the autonomic nervous system. Key clinical features include severe orthostatic hypotension (a dramatic drop in blood pressure upon standing), gastrointestinal dysfunction (gastroparesis, constipation, diarrhea, or ileus), urinary retention or incontinence, anhidrosis (inability to sweat) or abnormal sweating patterns, dry eyes and dry mouth, pupillary abnormalities with impaired light reflexes, and cardiac dysregulation including fixed heart rate. Patients may experience profound fatigue, blurred vision, and heat intolerance. In some cases, mild sensory symptoms may also be present. Treatment is primarily supportive and symptomatic, as no specific cure exists. Immunomodulatory therapies such as intravenous immunoglobulin (IVIg), plasma exchange (plasmapheresis), and corticosteroids have been used with variable success, particularly when administered early in the disease course. Symptomatic management includes medications for orthostatic hypotension (such as fludrocortisone and midodrine), prokinetic agents for gastrointestinal dysmotility, and catheterization for urinary retention. The prognosis is variable; some patients experience gradual partial or full recovery over months to years, while others may have persistent autonomic deficits. Anti-ganglionic acetylcholine receptor antibodies have been identified in some patients, which may help guide diagnosis and treatment decisions.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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