Primary lateral sclerosis (PLS): clinical features
Evidence-based neurology checklist on primary lateral sclerosis (pls): clinical features: Demographic features PLS accounts for 1-3% of MND The onset is usually from the 5th decade There is no family history Onset phenotypes Features of spasticity Cognitive features Other features Diagnostic…
Demographic features
- PLS accounts for 1-3% of MND
- The onset is usually from the 5th decade
- There is no family history
Onset phenotypes
Features of spasticity
Cognitive features
Other features
Diagnostic inclusion criteria
Diagnostic exclusion criteria
Time-sensitive diagnostic labels
Progression and outcome
Predictors of progression to ALS
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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