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However, because of the common parkinsonian features, the disorders have been collectively named Parkinson-plus syndromes.
Parkinson-plus syndromes respond poorly to the standard treatments for Parkinson disease (PD).
Glial cytoplasmic inclusions (GCIs) and a neuronal multisystem degeneration are the pathologic hallmarks of this clinically variable disorder (see the image below).
Dejerine and Thomas first used the term olivopontocerebellar atrophy (OPCA) in 1900 when they described 2 patients with a degenerative disorder leading to progressive cerebellar dysfunction and parkinsonism.
Patients with SND usually display parkinsonism and pyramidal signs. Not uncommonly, it is very difficult to distinguish SND from PD.
SDS is identified by parkinsonism, which rarely responds to dopaminergic therapy, and autonomic dysfunction.Though the main features of OPCA are cerebellar manifestations, mild parkinsonism and pyramidal signs are also typically present.