Document Detail


Clinical evolution of pure upper motor neuron disease/dysfunction (PUMMD).
MedLine Citation:
PMID:  23169452     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
INTRODUCTION: PLS is defined as pure upper motor neuron disease/dysfunction (PUMND) beyond 48 months after symptom onset. We know little about its early stages, but such knowledge would help to identify the mechanisms underlying PLS and ALS and determine why PLS patients seem to be protected against lower MND (LMND).
METHODS: We reviewed 622 MND cases during a 4-year period and identified 34 patients with PUMND (5.4%).
RESULTS: Among 23 cases with follow-up data/electromyograms (EMGs; 2 had only 1 EMG), 13 (57%) remained classified as PUMND, and 8 (35%) developed LMND (mean, 51.4 months after onset). Of these 8, LMND developed in 3 after 48 months from symptom onset. Patients with PUMND and LMND were more functionally impaired (P = 0.02). Separately, we identified 5 patients with PUMND who developed LMND long after 48 months (range, 50-127 months).
CONCLUSIONS: PLS belongs to the ALS spectrum, and perhaps all cases eventually develop LMND.
Authors:
Emanuele D'Amico; Meredith Pasmantier; Yei-Won Lee; Louis Weimer; Hiroshi Mitsumoto
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-11-21
Journal Detail:
Title:  Muscle & nerve     Volume:  47     ISSN:  1097-4598     ISO Abbreviation:  Muscle Nerve     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2012-12-21     Completed Date:  2013-02-22     Revised Date:  2014-01-09    
Medline Journal Info:
Nlm Unique ID:  7803146     Medline TA:  Muscle Nerve     Country:  United States    
Other Details:
Languages:  eng     Pagination:  28-32     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Wiley Periodicals, Inc.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Disease Progression
Electromyography
Female
Humans
Male
Middle Aged
Motor Neuron Disease / diagnosis*,  pathology,  physiopathology
Retrospective Studies
Grant Support
ID/Acronym/Agency:
1R01 ES016348/ES/NIEHS NIH HHS; R01 ES016348/ES/NIEHS NIH HHS
Comments/Corrections

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