Eye movement abnormalities are among the most characteristic and diagnostically valuable features of Progressive Supranuclear Palsy (PSP), a 4R-tauopathy that causes progressive postural instability, vertical supranuclear gaze palsy, and cognitive impairment. Oculomotor dysfunction in PSP results from degeneration of the brainstem nuclei and cortical pathways controlling eye movements, particularly affecting vertical saccades. This page provides a comprehensive guide to eye tracking and saccade testing for PSP diagnosis, covering key abnormalities, testing protocols, diagnostic criteria, and differential diagnosis. [1]
The oculomotor abnormalities in PSP arise from selective vulnerability of specific neuronal populations: [2]
| Structure | Function | PSP Pathology | [3]
|-----------|----------|---------------| [4]
| Midbrain | Vertical gaze control | Tau-positive neuropil threads, neuronal loss |
| Superior colliculus | Saccade generation | Degeneration of intermediate layers |
| Rostral interstitial MLF | Vertical saccade control | Neurofibrillary tangles |
| Pontine omnipause neurons | Saccade gating | Cell loss |
| Substantia nigra | Eye movement modulation | Dopaminergic neuron loss |
The cortical gaze control system includes the frontal eye fields, supplementary eye fields, and parietal cortex. In PSP, these frontal lobe regions show early tau pathology, contributing to saccadic dysfunction.
VSGP is the cardinal eye movement abnormality in PSP and a core diagnostic feature in the MDS-PSP criteria [1].
| Parameter | Normal | PSP | Clinical Significance |
|---|---|---|---|
| Vertical saccade velocity | >400°/s | <200°/s | Key discriminator |
| Vertical saccade latency | <250ms | >350ms | Early finding |
| Saccadic accuracy | >95% | <80% | Hypermetria/hypometria |
| Smooth pursuit gain | >0.9 | <0.6 | Vertical > horizontal |
Eyelid opening apraxia is a distinctive feature of PSP that helps differentiate it from other parkinsonian disorders [2].
Square wave jerks (SWJs) are involuntary saccadic intrusions that interrupt fixation and are highly characteristic of PSP [3].
Vertical Saccade Assessment:
1. Patient fixates on examiner's nose at eye level
2. Examiner holds target (finger or pen) 30cm from patient
3. Target moves rapidly upward (30° amplitude)
4. Patient instructed: "Look at the target as quickly as possible"
5. Repeat 5-10 trials, measure velocity qualitatively
6. Repeat with downward movement
7. Record: velocity, accuracy, latency, corrective saccades
For research and detailed clinical assessment, quantitative measures provide superior sensitivity:
| Measure | Technique | PSP Finding |
|---|---|---|
| Saccade velocity | Infrared eye tracking | Marked reduction in vertical saccades |
| Saccade latency | Video-oculography | Prolonged latency, especially vertical |
| Antisaccade task | Interactive paradigm | High error rate (>50%) |
| Memory-guided saccades | Delayed target paradigm | Impaired accuracy |
The Movement Disorder Society-PSP criteria (MDS-PSP 2017) incorporate oculomotor findings as core diagnostic features [1]:
| Feature | Points | Description |
|---|---|---|
| VSGP | 4 | Vertical supranuclear gaze palsy, downward > upward |
| VSGP or slow vertical saccades | 2 | Either VSGP or reduced vertical saccade velocity |
| Feature | Points | Description |
|---|---|---|
| Eyelid opening apraxia | 1 | Inability to open eyes voluntarily |
| Excessive square wave jerks | 1 | Frequent SWJs during fixation |
The original NINDS-SPSP (National Institute of Neurological Disorders and Stroke-PSP Scientific) criteria from 1994 established the first standardized diagnostic framework for PSP [4]. While largely superseded by MDS-PSP criteria, the NINDS-SPSP remains important for historical comparison and certain research contexts:
| Criterion | Description |
|---|---|
| Possible PSP | Vertical supranuclear gaze palsy OR vertical saccade slowing + postural instability |
| Probable PSP | Vertical supranuclear gaze palsy + postural instability within 1 year |
The MDS-PSP criteria improved on NINDS-SPSP by adding suggestive features, biomarker support, and variant-specific criteria.
| Oculomotor Feature | PSP | Parkinson's Disease |
|---|---|---|
| Vertical saccade slowing | Present early (core feature) | Present late or absent |
| Square wave jerks | Common, frequent | Uncommon |
| Eyelid opening apraxia | Common | Rare |
| Blink rate | Reduced | Increased (early) |
| VOR | Preserved | Preserved |
| Oculomotor Feature | PSP | CBS |
|---|---|---|
| Vertical saccade slowing | Universal, early | Less common |
| Horizontal saccades | Affected later | Asymmetric impairment |
| Eyelid apraxia | Common | Variable |
| Alien limb | Rare | Common |
| Apraxia | Less prominent | Prominent |
| Oculomotor Feature | PSP | MSA |
|---|---|---|
| VSGP | Core feature | Late or absent |
| Saccadic pursuit | Variable | Prominent |
| Oculomotor palsy | Vertical > horizontal | Variable |
Oculomotor measures correlate with clinical staging and disease progression in PSP:
| Measure | Correlation | Clinical Implication |
|---|---|---|
| Vertical saccade velocity | PSPRS score (r = -0.65) | Lower velocity = worse score |
| Square wave jerk frequency | Midbrain atrophy (r = 0.58) | Higher frequency = more atrophy |
| Eyelid opening latency | Disease duration (r = 0.52) | Longer latency = longer disease |
| Antisaccade error rate | Frontal dysfunction (r = 0.70) | Higher errors = more cognitive impairment |
The Progressive Supranuclear Palsy Rating Scale (PSPRS) incorporates oculomotor items, and vertical saccade velocity decline parallels progression on this scale [7]. The PSPRS includes specific items for:
Serial oculomotor examination provides objective measures for:
The EyeLink series is a widely used video-based eye tracking system in neurological research and clinical trials [6].
| Model | Sampling Rate | Features | Clinical Use |
|---|---|---|---|
| EyeLink 1000 Plus | 2000 Hz | High precision, monocular/binocular | Research-grade saccade measurement |
| EyeLink Portable | 500 Hz | Portable, laptop-based | Clinical screening |
| EyeLink Core | 250 Hz | Cost-effective | Bedside assessment |
| System | Manufacturer | Key Features |
|---|---|---|
| Discovery | Arrington Research | Modular setup, high accuracy |
| iView X | SensoMotoric Instruments (SMI) | Integrated with VR systems |
| Tobii Pro | Tobii Technology | Remote eye tracking, large sample pools |
| Gazepoint | Gazepoint | Consumer-grade, research capable |
| System | Manufacturer | Features | Approximate Cost |
|---|---|---|---|
| EyeLink 1000 Plus | SR Research Ltd. | High-speed infrared eye tracking, 1000Hz sampling | $15,000-25,000 |
| EyeLink Portable Duo | SR Research Ltd. | Portable option, 250Hz | $8,000-12,000 |
| EyeTribe | The EyeTribe | Consumer-grade, 60Hz | $1,000-2,000 |
| Tobii Pro Fusion | Tobii | 500Hz, research-grade | $20,000-35,000 |
| Tobii Pro Spectrum | Tobii | 600Hz, binocular | $30,000-45,000 |
| iScreen | Applied Science Laboratories | Clinical oculography | $10,000-18,000 |
| Component | Approximate Cost (USD) |
|---|---|
| Quantitative oculography | $500-1,500 |
| Neurology consultation | $200-500 |
| Research protocol (academic center) | Often free for study participation |
| Serial monitoring (3 visits/year) | $1,500-4,500 |
Academic movement disorder centers:
Research programs:
Most insurance plans cover oculomotor testing when clinically indicated for differential diagnosis. Pre-authorization may be required. Research protocols may offer free testing as part of clinical trials.
Litvan et al. NINDS-SPSP criteria for PSP (1994). 1994. ↩︎
Golbe et al. PSP Rating Scale validation (2008). 2008. ↩︎