Progressive Supranuclear Palsy

Abstract

Richardson s syndrome (PSP-RS): prominent postural instability, supranuclear vertical gaze palsy and frontal dysfunction. PSP-Parkinsonism (PSP-P): asymmetrical onset, tremor and moderate initial therapeutic response to levodopa. Pure akinesia with gait freezing is now referred to as PSP with progressive gait freezing (PSP-PGF): it initially presents with an isolated gait disorder years before developing other features of PSP-RS. PSP-corticobasal syndrome (CBS) is characterised by progressive asymmetric limb rigidity, apraxia, cortical sensory loss, alien limb, dystonia and bradykinesia unresponsive to levodopa. Clinically and genetically there is considerable overlap with corticobasal degeneration. PSP-speech language (PSP-SL): this is characterised by halting speech or distortions of language sometimes known as apraxia of speech (AOS). PSP with frontal presentation (PSP-F): this presents with early and progressive deterioration of personality, social comportment, behaviour and cognition. PSP with predominant cerebellar ataxia (PSP-C): this presents with cerebellar ataxia as the initial symptom, eventually accompanied by cardinal features of RS. PSP with mixed pathology: a subset of patients with PSP develop other pathologies such as Alzheimer s disease, Parkinson s disease and other rarer conditions.