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

Primary Lateral Sclerosis

Jeffrey M Statland  1 , Richard J Barohn  2 , Mazen M Dimachkie  2 , Mary Kay Floeter  3 , Hiroshi Mitsumoto  4
Affiliations
Review

Primary Lateral Sclerosis

Jeffrey M Statland et al. Neurol Clin. 2015 Nov.

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.

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References

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