Document Detail


Sympathetic neural outflow directly recorded in patients with primary autonomic failure: clinical observations, microneurography, and histopathology.
MedLine Citation:
PMID:  2356008     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We evaluated 2 patients with primary autonomic failure, without clinical peripheral neuropathy. One had primary autonomic failure alone (PAF), and the other had autonomic failure and multiple system atrophy (MSA). Direct intraneural recordings demonstrated a marked reduction of sympathetic efferent nerve impulse activity in the PAF patient. The patient with MSA had spontaneous bursts of sympathetic nerve impulses that confirmed the functional integrity of post-ganglionic sympathetic efferent neurons. Neurosecretory activity of these neurons correlated with the electrophysiologic findings. The PAF patient had markedly reduced supine norepinephrine (NE) levels that did not rise upon standing. The supine NE level in the MSA patient was normal. Morphometric study of biopsied sural nerve in the MSA patient showed that unmyelinated fibers were normal, whereas the nerve of the PAF patient showed clear evidence of past degeneration. We suggest that the primary preganglionic sympathetic defect in MSA releases viable postganglionic sympathetic efferents from central control. Decentralized postganglionic elements may fire spontaneously, thus activating peripheral effectors and providing potentially useful signs and symptoms for differential diagnosis.
Authors:
R Dotson; J Ochoa; P Marchettini; M Cline
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Neurology     Volume:  40     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1990 Jul 
Date Detail:
Created Date:  1990-07-26     Completed Date:  1990-07-26     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1079-85     Citation Subset:  AIM; IM    
Affiliation:
Department of Neurology, Oregon Health Sciences University, Portland.
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MeSH Terms
Descriptor/Qualifier:
Autonomic Fibers, Postganglionic / physiology,  ultrastructure
Autonomic Nervous System Diseases / blood,  pathology,  physiopathology*
Axons / ultrastructure
Efferent Pathways / physiopathology
Female
Humans
Middle Aged
Neurons, Efferent / physiology,  ultrastructure
Norepinephrine / blood
Plethysmography
Sympathetic Nervous System / pathology,  physiopathology*
Grant Support
ID/Acronym/Agency:
K08 NS01181-02/NS/NINDS NIH HHS; R01 NS 24740/NS/NINDS NIH HHS; R01 NS 24766/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
51-41-2/Norepinephrine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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