title: Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population.
abstract: OBJECTIVE
  The goals of this study were to characterize clinical and electrophysiologic findings of subjects with upper motor neuron disease and to explore feasibility of clinical trials in this population.
  
  
  METHODS
  Twenty northeast amyotrophic lateral sclerosis consortium (northeast amyotrophic lateral sclerosis) sites performed chart reviews to identify active clinical pure upper motor neuron disease patients. Patients with hereditary spastic paraplegia or meeting revised El Escorial electrodiagnostic criteria for amyotrophic lateral sclerosis were excluded. Patients were classified into 2 groups according to the presence or absence of minor electromyography (EMG) abnormalities.
  
  
  RESULTS
  Two hundred thirty-three subjects with upper motor neuron disease were identified; 217 had available EMG data. Normal EMGs were seen in 140 subjects, and 77 had minor denervation. Mean disease duration was 84 (±80) months for the entire cohort with no difference seen between the 2 groups. No difference was seen in clinical symptoms, disability, or outcome measures between the 2 groups after correcting for multiple comparisons.
  
  
  CONCLUSIONS
  Minor EMG abnormalities were not associated with phenotypic differences in a clinical upper motor neuron disease population. These findings suggest that subtle EMG abnormalities can not necessarily be used as a prognostic tool in patients with clinical upper motor neuron disease. This study also demonstrates the availability of a large number of patients with upper motor neuron diseases within the northeast amyotrophic lateral sclerosis network and suggests feasibility for conducting clinical trials in this population.
authors.affiliation: Department of Neurology, Emory University, Atlanta, GA.
authors.forename: Christina N
authors.initials: CN
authors.lastname: Fournier
authors.affiliation: Massachusetts General Hospital, Neurology Clinical Research Institute, Boston, MA.
authors.forename: Alyssa
authors.initials: A
authors.lastname: Murphy
authors.affiliation: Massachusetts General Hospital, Neurology Clinical Research Institute, Boston, MA.
authors.forename: Lorena
authors.initials: L
authors.lastname: Loci
authors.affiliation: Columbia Presbyterian Medical Center, New York, NY.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mitsumoto
authors.affiliation: Department of Neurology, University of California, San Francisco, CA.
authors.forename: Catherine
authors.initials: C
authors.lastname: Lomen-Hoerth
authors.affiliation: Department of Neurology, The Ohio State University, Columbus, OH.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kisanuki
authors.affiliation: Department of Neurology, Pennsylvania State University College of Medicine, PA.
authors.forename: Zachary
authors.initials: Z
authors.lastname: Simmons
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, MD.
authors.forename: Nicholas J
authors.initials: NJ
authors.lastname: Maragakis
authors.affiliation: Department of Neurology, University of Kansas Medical Center, Kansas City, KS.
authors.forename: April L
authors.initials: AL
authors.lastname: McVey
authors.affiliation: Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR.
authors.forename: Tawfiq
authors.initials: T
authors.lastname: Al-Lahham
authors.affiliation: Department of Neurology, Drexel University College of Medicine, Philadelphia, PA.
authors.forename: Terry D
authors.initials: TD
authors.lastname: Heiman-Patterson
authors.affiliation: Department of Neurology, University of Connecticut, Farmington, CT.
authors.forename: Jinsy
authors.initials: J
authors.lastname: Andrews
authors.affiliation: Massachusetts General Hospital, Neurology Clinical Research Institute, Boston, MA.
authors.forename: Erin
authors.initials: E
authors.lastname: McDonnell
authors.affiliation: Massachusetts General Hospital, Neurology Clinical Research Institute, Boston, MA.
authors.forename: Merit
authors.initials: M
authors.lastname: Cudkowicz
authors.affiliation: Massachusetts General Hospital, Neurology Clinical Research Institute, Boston, MA.
authors.forename: Nazem
authors.initials: N
authors.lastname: Atassi
mesh_terms: D000368:Aged; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology*; D012720:Severity of Illness Index