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Case Study of Possible PSP with Severe Functional Impairment in a 59-Year-Old Woman

Extract

Objective: Progressive Supranuclear Palsy (PSP) is one of a group of less common Parkinson’s-related diseases that is clinically described and neuropathologically determined. Primary features are postural instability, akinesia, visual motor disturbance, and cognitive changes in executive functions, language and memory. A complex, atypical PSP case with considerable functional impairment is presented, with consideration of diagnostic criteria. Challenges to differentiation between PSP and other dementias, and among PSP subtypes in this patient are presented. Method: Patient with three years of progressive functional decline presenting for initial assessment: difficulty with IADLs, gait and movement changes, multiple falls, vision changes, apathy, dream enactment, and delusion are described. Cognitive complaints include confusion, disorientation, memory and language difficulty. Observations: stimulus bound behavior, perseverative and tangential thought, visual and motor impairment. Neurological evaluation demonstrates ocular motor apraxia, ocular ataxia and simultagnosia. MRI revealed mild white matter changes and mild atrophy. Results: Neuropsychological evaluation revealed significant impairment in multiple domains, including motor and executive functions, memory, and language. There was dramatic impairment in visual processing, incorporating impaired perception, visual motor integration, organization, and spatial processing. In contrast, she was able to visually identify objects from the Fuld Object Memory Task and could read single words, which aided diagnostic conclusions. Judgment and insight were poor. Conclusions: Patient presents with a complex array of symptoms that may be suggestive of multiple neuropathologies. Neuropsychological evaluation favors PSP, though differentiation of subtype is limited. Consideration of atypical patient presentation in light of recently published criteria is presented.