BSN PSP/CBD Conference – Sharon Sha


Understanding CBS, CBD, and PSP: What Are Taupathies?

Sharon Sha, MD, Stanford

  • Tau – called tauopathy because there’s an abnormal buildup CBD, FTLD, & PSP – Many diseases are taupathies
  • PSP
    • Behavior – apathy obsessive/compulsive behaviors, utilization
    • Cognitive – executive dysfunction
    • Motor – parkinsonism, axial rigidity, postural, instability, bradykinesia, reduced blink
    • Language disorders
    • Supranuclear gaze palsy
  • PSP criteria was updated this year
    • Sporadic occurrence, age 40 or older at onset, gradual progression of PSP related symptoms
    • Core features
      • Oculomotor dysfunction, postural instability, loss of movement, and cognitive dysfunction
    • Many symptoms can have underlying PSP pathology
  • PSP Richardson’s syndrome
    • Classic symptoms – unexplained falls, unsteady gait, slowness in movements
    • Personality changes – apathy or disinhibition
    • Cognitive slowing, executive dysfunction (multitasking)
    • Slow speech, slurred speech, spastic speech, swallowing problems
    • Eye movement problems – slow vertical, difficulty opening eyelids
    • Vertical surpranuclear gaze palsy – onset variable, decreased velocity
  • PSP-Corticobasal Syndrome
    • Variable combo of
      • Progressive limb rigidity, apraxia, cortical sensory loss (not a primary sensory problem), alien limb (floating limb), bradykinesia (slowness), and unresponsive to levodopa
  • PSP Speech Language Component
    • nfvPPA (nonfluent variant)
    • Agrammatism (mainly lacking of function words)
    • Effortful, halting speech with inconsistent speech sound errors and distortions (AOS)
  • PSP Pathology
    • Neuronal loss and gliosis
    • Hyperphosphorylated MAPT
  • Corticobasal Syndrome: Patient 1 (58 year old right handed male, 1 year left sided clumsiness)
    • Difficulty walking down stairs, now has to hold railing
    • 6 months later difficulty with sequence of starting a car
    • Handwriting became worse, tremor
    • Anxiety
  • Exam
    • Praxis worse on left side, tone increased, cogwheel, coordination worse on left, slow
    • Cognitive testing – pretty good overall with minor problems in calculations and copying, but impaired phonemic fluency.
  • Classic for CBD/CBS
  • Typical FDG PET and MRI Patterns
    • Asymmetric, metabolism is diminished in the same area as atrophy
  • CBS/CBD can have multiple pathologies
  • CBS/CBD Degeneration
    • Different presenting syndromes can have CBD pathology
      • Behavioral syndrome (FTD), Non-fluent aphasia syndrome, PSP, CBS
    • Macroscopic Pathology
  • Clinical phenotypes of CBD
    • Probably CBS
      • Asymmetric limb rigidity or akinesia, limb dystonia, limb myoclonus plus 2 of orobuccal or limb apraxia, cortical sensory deficit, alien limb phenomena
    • Possible CBS – may be symmetric
    • FBSS
    • nfvPPA
    • PSPS (PSP Richardson’s syndrome)
  • Patient 2 (70 year old male, 1.5 years progressive motor/cognitive changes)
    • Skiing accident with concussion
    • Right hand coordination problems, cant write or type
    • Speech is slow and slurred
    • Memory problems, difficulty with details, planning

Exam

    • Memory loss recall 1/5, names 4 F words, eye movements with overshoot, saccadic, slurred speech and slow speech, right hand dystonic with increased tone RUE, RLE
  • Amyloid PET is suggestive of AD pathology
  • Looked at ways to predict underlying pathology
  • End notes
    • PSP & CBD can have different clinical presentations
    • PSP-RS is the most common and most predicts
    • CBS usually indicates CBD but can have alternate pathologies