Neudefinition atypischer Parkinson-Syndrome

The definition of atypical parkinsonian syndromes is changing. The umbrella term of atypical parkinsonian syndrome includes diseases with different underlying pathologies. These are, on the one hand, multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), both characterized by intracellular aggregates of the protein alpha-synuclein and on the other hand, corticobasal degeneration (CBD) and progressive supranuclear gaze visualization (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 clinical pathological studies have shown that the clinical syndrome often does not coincide with the molecular-pathological diagnosis, 2) sometimes in patients with a pathological diagnosis of an typical parkinsonian syndrome, a clinical parkinsonian syndrome is even missing and 3) current therapeutical trials based on the molecular disease mechanisms require not a symptom-oriented, but a pathogenetically oriented diagnosis. The early and correct identification 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.