title: Primary Lateral Sclerosis.
abstract: Primary lateral sclerosis is characterized by insidious onset of progressive upper motor neuron dysfunction in the absence of clinical signs of lower motor neuron involvement. Patients experience stiffness; decreased balance and coordination; mild weakness; and, if the bulbar region is affected, difficulty speaking and swallowing, and emotional lability. The diagnosis is made based on clinical history, typical examination findings, and diagnostic testing negative for other causes of upper motor neuron dysfunction. Electromyogram is normal, or only shows mild neurogenic findings in a few muscles, not meeting El Escorial criteria. Treatment is largely supportive.
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA. Electronic address: jstatland@kumc.edu.
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Statland
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Barohn
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA.
authors.forename: Mazen M
authors.initials: MM
authors.lastname: Dimachkie
authors.affiliation: Human Spinal Physiology Unit, National Institute of Neurological Disorders and Stroke, Building 10, Room 7-5680, 10 Center Drive, Bethesda, MD 20892, USA.
authors.forename: Mary Kay
authors.initials: MK
authors.lastname: Floeter
authors.affiliation: Department of Neurology, Columbia University Medical Center, 710 West 168th Street, New York City, NY 10032, USA.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mitsumoto
mesh_terms: D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D006801:Humans; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology*; D059906:Neuroimaging; D012720:Severity of Illness Index
keywords: Motor neuron disease; Neuroimaging; Primary lateral sclerosis; Pseudobulbar affect; Spastic quadriparesis; Upper motor neuron disease