Typical Symptoms of Progressive Supranuclear Palsy (PSP)
Progressive Supranuclear Palsy (PSP) is characterized primarily by postural instability with early falls, vertical supranuclear gaze palsy, and axial rigidity, which are the hallmark symptoms of this neurodegenerative disorder. 1
Core Clinical Manifestations
Movement and Postural Symptoms
- Postural instability with early, unexplained falls (typically backward falls within the first year of disease onset) 1
- Axial rigidity more prominent than appendicular rigidity 2, 3
- Parkinsonian features including:
- Bradykinesia/akinesia (slowness of movement)
- Rigidity (particularly in neck and trunk)
- Gait disorders (slow, clumsy walking) 4
- Minimal tremor (distinguishing it from typical Parkinson's disease) 2
Ocular Symptoms
- Vertical supranuclear gaze palsy (especially downward gaze initially, progressing to both upward and downward gaze) 2, 1
- Slow saccades (eye movements) 5
- Decreased velocity of saccades 2
- Absence of optokinetic nystagmus vertically 2
- Fixation instability 6
- Lid retraction, blepharospasm, and apraxia of eyelid opening and closing 6
Cognitive and Behavioral Symptoms
- Frontal dysexecutive syndrome 7
- Apathy 7
- Impulsivity 7
- Visuospatial deficits 7
- Memory impairment 7
- Mood dysfunction 5
- Pseudobulbar affect (inappropriate laughing or crying) 4, 3
Disease Progression
Early Stage (First 4 Years)
- Gait disorders, falls, and general slowness are the most common presenting symptoms 4
- Ocular motility disorders and cognitive impairments may be evident 4
- 75% of patients remain independent in activities of daily living 4
Later Stages
- Progressive dependence in activities of daily living after 4 years 4
- Development of dysphagia (swallowing difficulties) 4
- Worsening of ocular and cognitive symptoms 4
Demographic and Prognostic Features
- Mean age of onset: approximately 63 years 1, 3
- Mean survival from symptom onset: approximately 9 years 3
- More common in men than women 3
Diagnostic Challenges
- Symptoms may be heterogeneous due to variable pathology locations 3
- Supranuclear gaze palsy, though classic, may not appear until late in the disease course 3
- Often misdiagnosed initially, with diagnostic delay averaging 4.39 years 4
It's important to note that PSP must be distinguished from other parkinsonian disorders such as Parkinson's disease, multiple system atrophy, corticobasal degeneration, dementia with Lewy bodies, and vascular parkinsonism 1. The combination of early falls, vertical gaze abnormalities, and poor response to levodopa therapy is particularly suggestive of PSP.