Primary Lateral Sclerosis

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, dif...

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Bibliographic Details
Published inNeurologic clinics Vol. 33; no. 4; p. 749
Main Authors Statland, Jeffrey M, Barohn, Richard J, Dimachkie, Mazen M, Floeter, Mary Kay, Mitsumoto, Hiroshi
Format Journal Article
LanguageEnglish
Published United States 01.11.2015
Subjects
Diagnosis, Differential
Electromyography
Female
Humans
Middle Aged
Motor Neuron Disease - diagnosis
Motor Neuron Disease - physiopathology
Neuroimaging
Severity of Illness Index
Neuroimaging
Primary lateral sclerosis
Pseudobulbar affect
Spastic quadriparesis
Upper motor neuron disease
Motor neuron disease
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Summary: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.
ISSN:1557-9875
DOI:10.1016/j.ncl.2015.07.007