title: Patterns of Weakness, Classification of Motor Neuron Disease, and Clinical Diagnosis of Sporadic Amyotrophic Lateral Sclerosis.
abstract: When approaching a patient with suspected motor neuron disease (MND), the pattern of weakness on examination helps distinguish MND from other diseases of peripheral nerves, the neuromuscular junction, or muscle. MND is a clinical diagnosis supported by findings on electrodiagnostic testing. MNDs exist on a spectrum, from a pure lower motor neuron to mixed upper and lower motor neuron to a pure upper motor neuron variant. Amyotrophic lateral sclerosis (ALS) is a progressive mixed upper and lower motor neuron disorder, most commonly sporadic, which is invariably fatal. This article describes a pattern approach to identifying MND and clinical features of sporadic ALS.
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: April L
authors.initials: AL
authors.lastname: McVey
authors.affiliation: Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA; Department of Neurology, California Pacific Medical Center, 475 Brannan Street, Suite 220, San Francisco, CA 94107, USA.
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Katz
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
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000266:history; D003937:Diagnosis, Differential; D049673:History, 20th Century; D006801:Humans; D016472:Motor Neuron Disease / Q000145:classification* / Q000266:history / Q000503:physiopathology*; D012720:Severity of Illness Index
keywords: Amyotrophic lateral sclerosis; Lou Gehrig disease; Lower motor neuron; Motor neuron disease; Upper motor neuron