Redefinition of Atypical Parkinsonian Syndromes

Abstract

The definition of atypical parkinsonian syndromes is undergoing a change. The umbrella concept of atypical parkinsonian syndromes includes diseases with different underlying pathologies. These are, on the one hand, multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), both of which are characterized by intracellular aggregates of the protein alpha-synuclein, and on the other hand, corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), which are characterized by aggregates of the protein tau. The current syndrome-based classification of these diseases no longer meets current requirements since 1) numerous clinico-pathological studies have shown that the clinical syndromes do not always coincide with the molecular-pathological diagnosis, 2) a clinical parkinsonian syndrome is sometimes not even present in patients with a pathological diagnosis of an atypical parkinsonian syndrome, and 3) current therapeutic trials that intervene in the molecular disease mechanisms require no symptom-oriented, but a pathogenetically oriented diagnosis. The early and correct prediction of the underlying molecular pathology as a prerequisite for a causative therapy therefore presents a challenge that the old disease concept is no longer capable of meeting.

Key words

parkinsonian syndromes - new definition - diagnosis

• References

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