A 71-year-old retired accountant is evaluated in the neurology clinic for recurrent, unprovoked backward falls and a progressive slowing of movements over the past year. His family reports that his voice has become increasingly monotonic, and he frequently spills food because he 'cannot look down' at his plate. On mental status examination, he exhibits mild executive dysfunction. The physician suspects progressive supranuclear palsy. Which of the following specific physical examination findings would best confirm this diagnosis and differentiate it from other parkinsonian syndromes? (Select TWO that apply)
- Loss of voluntary downward eye movements with preservation of the vestibulo-ocular reflexAnswer
- Axial rigidity that is disproportionately more severe than appendicular rigidityAnswer
- CA 4- to 6-Hz resting tremor that is temporarily suppressed during voluntary action
- DA paradoxical decrease in systolic blood pressure greater than 20 mm Hg upon standing without compensatory tachycardia
- EMarked asymmetric apraxia accompanied by stimulus-sensitive myoclonic jerks of a single limb
Answer
The correct findings are loss of voluntary downward eye movements with preserved vestibulo-ocular reflex, and disproportionately severe axial rigidity.
Progressive supranuclear palsy (PSP) is an atypical parkinsonian syndrome (tauopathy). The hallmark physical examination finding is a vertical supranuclear gaze palsy. 'Supranuclear' means the neurodegenerative deficit originates above the level of the cranial nerve nuclei; thus, while voluntary downward gaze is impaired, the primitive brainstem reflexes (like the vestibulo-ocular or oculocephalic reflex, tested via the doll's head maneuver) remain intact. Additionally, unlike idiopathic Parkinson disease where rigidity is predominantly appendicular (in the limbs), PSP is characterized by profound axial (neck and trunk) rigidity, which contributes heavily to the classic early backward falls.
Step-by-Step Solution
Key Concept
Physical examination findings differentiating Progressive Supranuclear Palsy (PSP) from other parkinsonian syndromes.