Parkinsonian-Pyramidal syndromes: A systematic review

We propose a pragmatic presentation based on several established classifications and we will distinguish inherited PPS found in complex hereditary spastic paraplegia, young onset parkinsonism, neurodegeneration with brain iron accumulation, primary familial brain calcifications, inborn errors of metabolism, and few rare others inherited neurodegenerative diseases, then non-inherited neurodegenerative PPS. We therefore suggest guidelines (based on age at onset, family history, associated clinical signs, brain MRI findings as well as certain laboratory investigations), for the diagnosis and the management of PPS. Many pathophysiological pathways may underlie PPS but the most frequent are those usually involved in both inherited Parkinson's disease and spastic paraplegia, i.e. mitochondrial pathway, vesicular trafficking including endosomal and lysosomal pathways as well as autophagy.