Parkinsonian Phenotypes – towards New Nosology of Atypical Parkinsonian Syndromes

Summary

Parkinson’s disease is still viewed as a nosological entity (G20), yet it covers a wide range of clinical phenotypes characterised by the presence of parkinsonian syndrome with variable expression of a range of symptoms – motor, cognitive and behavioural – and an uncertain course. It appears that, largely thanks to progress in the recognition of phenotype variability and its clinico-pathological correlations at an ultrastructural level, it will divide into several close but separate nosological entities. In the last 50 years certain atypical parkinsonian syndromes have already split off one by one – Parkinson’s disease dementia (PDD), progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal degeneration (CBGD), diffuse Lewy body disease (DLBD), frontotemporal dementia (FTLD) and the endemic parkinsonian syndromes such as Guam and Guadeloupean parkinsonism. However, the atypical parkinsonian syndromes are still diagnosed on the basis of (mainly obsolete) clinical dia­gnostic criteria which, at the time of their creation, only partially reflected the real pathological nature of the disorder. Their replacement with newly-developed criteria created on the basis of clinico-pathological correlation should be given priority in the field of translational research in the immediate future.

Key words:
Parkinson’s disease – atypical 
parkinsonian syndromes, 
clinico-pathological correlation

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