Document Detail


Autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy.
MedLine Citation:
PMID:  22357716     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: Autonomic deficits in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have not been adequately quantitated. The Composite Autonomic Severity Score (CASS) is a validated instrument for laboratory quantitation of autonomic failure derived from standard autonomic reflex tests. We characterized dysautonomia in CIDP using CASS.
METHODS: Autonomic function was retrospectively analyzed in 47 patients meeting CIDP criteria. CASS ranges from 0 (normal) to 10 (pandysautonomia), reflecting summation of sudomotor (0-3), cardiovagal (0-3), and adrenergic (0-4) subscores. Severity of neurologic deficits was measured with Neuropathy Impairment Score (NIS). Degree of small fiber involvement was assessed with quantitative sensation testing. Thermoregulatory sweat test (TST) was available in 8 patients.
RESULTS: Patients (25 men) were middle-aged (45.0 ± 14.9 years) with longstanding CIDP (3.5 ± 4.3 years) of moderate severity (NIS, 46.5 ± 32.7). Autonomic symptoms were uncommon, mainly gastrointestinal (9/47; 19%) and genitourinary (8/47; 17%). Autonomic deficits (CASS ≥1) were frequent (22/47; 47%) but very mild (CASS, 0.8 ± 0.9; CASS ≤3, all cases). Deficits were predominantly sudomotor (16/47; 34%) and cardiovagal (10/47; 21%) with relative adrenergic sparing (4/47; 9%). TST was abnormal in 5 of 8 patients (anhidrosis range, 2%-59%). Sudomotor impairment was predominantly distal and postganglionic. Somatic deficits (disease duration, severity, small fiber deficits) did not predict presence of autonomic deficits.
CONCLUSION: Our data characterize the autonomic involvement in classic CIDP as mild, cholinergic, and predominantly sudomotor mainly as a result of lesions at the distal postganglionic axon. Extensive or severe autonomic involvement (CASS ≥4) in suspected CIDP should raise concern for an alternative diagnosis.
Authors:
J J Figueroa; P J B Dyck; R S Laughlin; J A Mercado; R Massie; P Sandroni; P J Dyck; P A Low
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-02-22
Journal Detail:
Title:  Neurology     Volume:  78     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-03-06     Completed Date:  2012-07-06     Revised Date:  2013-06-26    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  702-8     Citation Subset:  AIM; IM    
Affiliation:
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Autonomic Nervous System / physiopathology*
Autonomic Nervous System Diseases / physiopathology*
Body Temperature Regulation / physiology
Female
Heart Rate / physiology
Humans
Male
Middle Aged
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology*
Severity of Illness Index
Sweating / physiology
Grant Support
ID/Acronym/Agency:
NS 32352/NS/NINDS NIH HHS; NS 44233/NS/NINDS NIH HHS; T32 HD07447/HD/NICHD NIH HHS; U54 NS065736/NS/NINDS NIH HHS; UL1 RR24150/RR/NCRR NIH HHS
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