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Neurol Clin. 2015 Nov;33(4):749-60. doi: 10.1016/j.ncl.2015.07.007. Epub 2015 Sep 8.

Primary Lateral Sclerosis.

Author information

1
Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA. Electronic address: jstatland@kumc.edu.
2
Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA.
3
Human Spinal Physiology Unit, National Institute of Neurological Disorders and Stroke, Building 10, Room 7-5680, 10 Center Drive, Bethesda, MD 20892, USA.
4
Department of Neurology, Columbia University Medical Center, 710 West 168th Street, New York City, NY 10032, USA.

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.

KEYWORDS:

Motor neuron disease; Neuroimaging; Primary lateral sclerosis; Pseudobulbar affect; Spastic quadriparesis; Upper motor neuron disease

PMID:
26515619
PMCID:
PMC4628724
DOI:
10.1016/j.ncl.2015.07.007
[Indexed for MEDLINE]
Free PMC Article

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