Primary lateral sclerosis (PLS) is a rare progressive degenerative disease of the corticospinal and corticobulbar tracts (upper motor neurons [UMNs]), which spares the anterior horn ...
PLS is a diagnosis of exclusion.
Clinical diagnosis defined as UMN only involvement for at least 4 years from symptom onset
The most recent set of diagnostic criteria was presented by Singer e...
Mainly supportive care
Nursing home placement or hospice at end stage
Discussion of advance directives and completion of ...
PLS has not been shown to shorten life expectancy.
Median survival after diagnosis is 19 years.
An older age at onset is associated with rapid decline in functional status.
Pringle CE, Hudson AJ, Munoz DG, et al. Primary lateral sclerosis. Clinical features, neuropathology and diagnostic criteria. Brain. 1992;115(Pt 2):495–520.
Tartaglia MC, Laluz...
PLS affects only UMNs.
Onset is usually asymmetric spasticity in the lower extremities. The patient may present complaining of stiffness, slowness, clumsiness, falls, balance issues, an...
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