RESEARCH: Idiopathic autonomic neuropathy (pandysautonomia)

[Guest guest]

Neurol Neurochir Pol 2001 Mar-Apr;35(3):439-52

[idiopathic autonomic neuropathy (pandysautonomia)]

Jankowicz E, Drozdowski W, Pogumirski J.

Kliniki Neurologii AM w Bialymstoku.

On the basis of current literature, clinical and neuropathologic features of

idiopathic autonomic neuropathy is presented. Idiopathic autonomic

neuropathy is

a disease characterized by acute or subacute onset, monophasic course over a

period of several years, it is often preceded by an infection. The spectrum

of

autonomic changes ranges from cholinergic or adrenergic dysfunction to

pandysautonomia, leading to heterogeneity of its clinical features. Possible

sympathetic system abnormalities found in autonomic neuropathy are: poor

pupillary response to light in darkness, orthostatic hypotension leading to

syncope, hypotension without compensatory tachycardia, ejaculation

disturbances

and vasomotor instability. Possible parasympathetic dysfunctions are:

salivation

and lacrimation disturbances, absent pupillary constriction to light and

near

gaze, gastrointestinal tract immobility and impairment of gastrointestinal

function, atonic bladder with large residual volume, erectile impotence.

Pandysautonomia is thought to result from an immune mediated mechanism and

responds well to plasmaferesis and intravenous immunoglobin therapy leading

to

gradual, sometimes not full, recovery. Moreover in this article we pay

attention

to the clinical value of many tests like cardiovascular or pharmacological

studies in the diagnosis of pandysautonomia and in differentiation of pre-

and

postganglionic changes. In order to diagnose idiopathic autonomic neuropathy

one

has to rule out a large number of diseases with autonomic dysfunction e.g.:

diabetes, malignant neoplasms, acute intermittent porphyria, Shy-Drager

syndrome, Riley-Day's dysautonomia, Parkinson's disease, amyloidosis and

others.

PMID: 11732267 [PubMed - indexed for MEDLINE]