Primary lateral sclerosis (PLS): clinical features
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Primary lateral sclerosis (PLS): clinical features. PLS accounts for 1-3% of MND. Fully referenced neurology checklist.
Demographic features
- PLS accounts for 1-3% of MND
- The onset is usually from the 5th decade
- There is no family history
Onset phenotypes
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Features of spasticity
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Cognitive features
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Other features
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Diagnostic inclusion criteria
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Diagnostic exclusion criteria
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Time-sensitive diagnostic labels
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Progression and outcome
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Predictors of progression to ALS
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References
- Pringle CE, Hudson AJ, Munoz DG, et al. Primary lateral sclerosis: clinical features, neuropathology and diagnostic criteria. Brain 1992; 115:495-520.
- Gordon PH, Cheng B, Katz IB, et al. The natural history of primary lateral sclerosis. Neurology 2006; 66:647-653.
- Hassan A, Mittal SO, Hu WT, Josephs KA, Sorenson EJ, Ahlskog JE. Natural history of "pure" primary lateral sclerosis. Neurology 2021; 96:e2231-e2238.
- Calvo A, Moglia C, Canosa A, et al. Early cognitive and behavioral changes in primary lateral sclerosis: a population-based study. Eur J Neurol 2026; 33:e70720.
- Mabuchi N, Watanabe H, Atsuta N, et al. Primary lateral sclerosis presenting parkinsonian symptoms without nigrostriatal involvement. JNNP 2004; 75:1768-1771.
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