Table of Contents
Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 5A0 Case 5 History
Case 5 History ---- This participant was an 82 yo female with a 2-3 year history of gradual onset of slurred speech, then trouble walking and falls, as well as mild cognitive changes. On examination, she had limited vertical gaze, postural instability, diffuse rigidity without cogwheeling, marked bradykinesia and severe gait impairment. Speech was slow and halting, with rapid fatigue, consistent with apraxia. She was rated CDR of 0.5 with a diagnosis of dementia with Lewy bodies, with dementia of the Alzheimer’s type a likely secondary diagnosis. Progressive supranuclear palsy was also considered. She was started on Aricept and subsequently on Sinemet for bradykinesia. She returned to the MDC after moving to a specialized nursing facility due to increased falls. She was wheelchair-bound and had a G-tube placed because of poor PO intake. Her speech difficulty had worsened and she produced only single words; she communicated by writing on a dry erase board. She had recently begun to keep her head tilted back for hours at a time. On exam she had retrocollis, complete loss of vertical eye movements, bradykinesia and severe postural instability. She was rated CDR 2 with a diagnosis of progressive supranuclear palsy. She returned for her final MDC visit 3/2010 at age 85. On exam she had absent vertical eye movements, bilateral cervical dystonia, slowed fine motor movements with arrest L>R. There was no tremor. She could perform two step commands. Cognitive function was difficult to assess given her speech and motor impairment. She was rated CDR 3 with a diagnosis of PSP. She had likely aspiration pneumonia; G-tube feedings were discontinued. She was in hospice care with comfort measures given and expired 2/2011 of likely aspiration pneumonia. In summary, this lady developed slurred speech, falls and slight cognitive impairment with onset about age 80. She had speech apraxia, impaired vertical gaze, postural instability and diffuse rigidity on exam at age 82. She subsequently developed cervical dystonia and complete loss of vertical gaze, with worsening speech and motor impairment. Progression of cognitive deficits was difficult to assess. She died 5 years after onset; at death she was mute, wheelchair bound and likely had aspiration pneumonia due to severe bulbar impairment. Her terminal CDR was 3,
