Pallido-pyramidal syndromes (PPS)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Pallido-pyramidal syndromes (PPS). Dystonia, Spasticity, Progressive parkinsonism. Fully referenced neurology checklist.

Clinical features - Davison’s triad

  • Dystonia
  • Spasticity
  • Progressive parkinsonism

Types

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Infantile PPS types (onset <2years)

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Juvenile PPS types

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Adult PPS types

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Other PPS types

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Arguments against the existence of PPS

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Synonym

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Acronym

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References

  1. Kruer MC, Salih MA, Mooney C, et al. C19orf12 mutation leads to a pallido-pyramidal syndrome. Gene 2014; 537:352-356. 
  2. Kara E, Hardy J, Houlden H. The pallidopyramidal syndromes: nosology, aetiology and pathogenesis. Curr Opin Neurol 2013; 26:381-394.
  3. Paisan-Ruiz C, Bhatia KP, Li A, et al. Characterization of PLA2G6 as a locus for dystonia-parkinsonism. Ann Neurol 2009; 65:19-23. 
  4. Di Fonzo A, Dekker MC, Montagna P, et al. FBXO7 mutations cause autosomal recessive, early-onset parkinsonian-pyramidal syndrome. Neurology 2009; 72:240-245. 
  5. Tranchant C, Koob M, Anheim M. Parkinsonian-pyramidal syndromes: a systematic review. Parkinsonism Relat Disord 2017; 39:4-16. 
  6. And 2 more. Subscribe to see the full list

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