Document Detail


Alpha-synuclein pathology and axonal degeneration of the peripheral motor nerves innervating pharyngeal muscles in Parkinson disease.
MedLine Citation:
PMID:  23334595     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Parkinson disease (PD) is a neurodegenerative disease primarily characterized by cardinal motor manifestations and CNS pathology. Current drug therapies can often stabilize these cardinal motor symptoms, and attention has shifted to the other motor and nonmotor symptoms of PD that are resistant to drug therapy. Dysphagia in PD is perhaps the most important drug-resistant symptom because it leads to aspiration and pneumonia, the leading cause of death. Here, we present direct evidence for degeneration of the pharyngeal motor nerves in PD. We examined the cervical vagal nerve (cranial nerve X), pharyngeal branch of nerve X, and pharyngeal plexus innervating the pharyngeal muscles in 14 postmortem specimens, that is, from 10 patients with PD and 4 age-matched control subjects. Synucleinopathy in the pharyngeal nerves was detected using an immunohistochemical method for phosphorylated α-synuclein. Alpha-synuclein aggregates were revealed in nerve X and the pharyngeal branch of nerve X, and immunoreactive intramuscular nerve twigs and axon terminals within the neuromuscular junctions were identified in all of the PD patients but in none of the controls. These findings indicate that the motor nervous system of the pharynx is involved in the pathologic process of PD. Notably, PD patients who have had dysphagia had a higher density of α-synuclein aggregates in the pharyngeal nerves than those without dysphagia. These findings indicate that motor involvement of the pharynx in PD is one of the factors leading to oropharyngeal dysphagia commonly seen in PD patients.
Authors:
Liancai Mu; Stanislaw Sobotka; Jingming Chen; Hungxi Su; Ira Sanders; Charles H Adler; Holly A Shill; John N Caviness; Johan E Samanta; Thomas G Beach;
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neuropathology and experimental neurology     Volume:  72     ISSN:  1554-6578     ISO Abbreviation:  J. Neuropathol. Exp. Neurol.     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-01-21     Completed Date:  2013-03-11     Revised Date:  2014-02-04    
Medline Journal Info:
Nlm Unique ID:  2985192R     Medline TA:  J Neuropathol Exp Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  119-29     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Acetylcholinesterase / metabolism
Aged
Aged, 80 and over
Axons / pathology*,  ultrastructure
Case-Control Studies
Female
Humans
Male
Nerve Degeneration / etiology,  pathology*
Neurologic Examination
Neuromuscular Junction / pathology,  ultrastructure
Parkinson Disease / complications,  pathology*
Pharyngeal Muscles / innervation,  pathology*
Silver Staining
Vagus Nerve / pathology*,  physiology
alpha-Synuclein / metabolism*
Grant Support
ID/Acronym/Agency:
5 R01 DC004728/DC/NIDCD NIH HHS; P30 AG19610/AG/NIA NIH HHS; R01 DC004728/DC/NIDCD NIH HHS; U24 NS072026/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/alpha-Synuclein; EC 3.1.1.7/Acetylcholinesterase
Comments/Corrections

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


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