Progressive Supranuclear Palsy Rating Scale (PSP-RS)

Original Editor - Rucha Gadgil
Top Contributors - Rucha Gadgil

Introduction

The Progressive Supranuclear Palsy Rating Scale (PSPRS) is a disease specific measure of severity in patients with progressive supranuclear palsy (PSP). It measures disability across 28 items in six domains: daily activities (by history), behaviour, bulbar, ocular motor, limb motor and gait/midline. It was devised by Golbe and Strickland.

It is a quantitative measure of disability and attempts to include all of the important areas of clinical impairment in PSP.

Intended Population

This scale is targeted toward measuring the severity of clinical impairments in patients with Progressive supranuclear Palsy (PSP).

Method Of Use

Equipment:

Scale, Pen, Paper.

Cup of water

Training Required:

some examination item require prior clinical training

Time Required:

10 min

Instrument and Scoring

It is a Clinician reported measuring disability across six domains giving a maximum score of 100. It comprises of 28 items in six areas. Six items are rated on a 3-point scale (0–2) and 22 are rated on a 5-point scale (0–4).

1. The History/Daily Activities area: seven items with a total maximum score of 24,
2. The Mentation area: four items with a maximum score of 16,
3. The Bulbar area: two items with a maximum score of 8,
4. the ocular motor area four items with a maximum score of 16 points,
5. The limb motor area six items with a maximum score of 16.
6. The Gait area: five items with a maximum score of 20.

Item name and score definitions Comments, instructions

I. History

1.Withdrawal

0 None

1 Follows conversation in a group, may respond spontaneously, but rarely if ever initiates exchanges

2 Rarely or never follows conversation in a group

Relative to baseline personality

Consider lack of conversation due to dementia or bradyphrenia as

‘withdrawal’

2. Irritability

0 No increase in irritability

1 Increased, but not interfering with family interactions

2 Interfering with family interactions

Relative to baseline personality

Ask if patient shouts or loses temper easily

3. Dysphagia for solids

0 Normal; no difficulty with full range of food textures

1 Tough foods must be cut up into small pieces

2 Requires soft solid diet

3 Requires pureed or liquid diet

4 Tube feeding required for some or all feeding

Ignore difficulty related to overloading mouth

If certain foods like bread crusts or leafy vegetables must be

avoided, but meats OK, score ‘2’

4.Using knife and fork, buttoning clothes, washing hands and face

0 Normal

1 Somewhat slow but no help required

2 Extremely slow; or occasional help needed

3 Considerable help needed but can do some things alone

4 Requires total assistance

Rate the worst of the 3

If difficulty is related to downgaze, score as if it were purely

motor

5. Falls

0 None in the past year

151 per month; gait may otherwise be normal

2 1^ 4 per month

3 5^30 per month

4430 per month (or chairbound)

Average frequency if patient attempted to walk unaided

Assume no access to walking aids

Ignore near-falls

6. Urinary incontinence

0 None or a few drops less than daily

1 A few drops staining clothes daily

2 Large amounts, but only when asleep; no pad required

during day

3 Occasional large amounts in daytime; pad required

4 Consistent, requiring diaper or catheter awake and asleep

If daytime pad used as precaution but no recent wetting, score ‘3’

7. Sleep difficulty

0 Neither 1� nor 2� insomnia

1 Either 1� or 2� insomnia; averages 55 h sleep nightly

2 Both 1� and 2� insomnia; averages 55 h sleep nightly

3 Either 1� or 2� insomnia; averages55 h sleep nightly

4 Both 1� and 2� insomnia; averages55 h sleep nightly

1� insomnia is difficulty falling asleep

2� is difficulty remaining asleep

Ignore trips to bathroom after which pt. returns to sleep easily

II. Mentation

Items 8 ^11 use this scale:

0 Clearly absent

1 Equivocal or minimal

2 Clearly present, but does not interfere with activities of

daily living (ADL)

3 Interferes mildly with ADL

4 Interferes markedly with ADL

Estimate the degree to which each deficit would interfere with

performance of daily cognitive tasks

8. Disorientation  Use MMSE items 1^10 or history to estimate interference in ADLs

9. Bradyphrenia  If delayed responses prompt the caregiver to answer for the

patient or limit your ability to interview patient, rate at least a ‘3’

10. Emotional incontinence  If there is a history of inappropriate laughing or crying but none

at the time of the examination, rate a ‘1’ or ‘2’, depending on its

frequency

(continued )

PSP rating scale Brain (2007), 130, 1552^1565 1555

Table 2 Continued

Item name and score definitions Comments, instructions

11. Grasping/imitatative/utilizing behaviour  If none is displayed spontaneously (e.g. grabbing your coat or arm,

or the wheelchair arm), ask patient to rest hands on thighs,

palms up. Hold your hands 5^10 cm above his and say nothing.

If he grabs them, rate a 3

If he only imitates your actions during the exam, rate a 2

III. Bulbar

12. Dysarthria

0 None

1 Minimal; all or nearly all words easily comprehensible

2 Definite, moderate; most words comprehensible

3 Severe; may be fluent but most words incomprehensible

4 Mute; or a few poorly comprehensible words

Ignore palilalia and dysphonia

‘Comprehensible’ means to examiner, not caregiver

If generally silent but can be coaxed to speak a few words, rate a

‘4’ no matter how clear those words may be

13. Dysphagia

0 None

1 Single sips, or fluid pools in mouth or pharynx, but no

choking/coughing

2 Occasionally coughs to clear fluid; no frank aspiration

3 Frequently coughs to clear fluid; may aspirate slightly; may

expectorate frequently rather than swallow secretions

4 Requires artificial measures (oral suctioning, tracheostomy

or feeding gastrostomy) to avoid aspiration

Give 30 ^50 cc of water in a cup, if safe

Do not give water if secretions are audible with breathing, if

there is a history of frequent aspiration or if caregiver is apprehensive

1 cough rates ‘2’, multiple coughs ‘3’

IV. Ocular motor

Items 14 ^16 use this scale:

0 Saccades not slow or hypometric; 86 ^100% of normal

excursion

1 Saccades slow or hypometric; 86 - 100% of normal excursion

2 51- 85% of normal excursion

3 16- 50% of normal excursion

4 15% of normal excursion or worse

14.Voluntary upward command movement

15.Voluntary downward command movement

16.Voluntary left and right command movement

17. Eyelid dysfunction

0 None

1 Blink rate decreased (515/min) but no other abnormality

2 Mild inhibition of opening or closing or mild blepharospasm; no visual disability

3 Moderate lid-opening inhibition or blepharospasm causing partial visual disability

4 Functional blindness or near-blindness because of involuntary eyelid closure

V. Limb motor

18. Limb rigidity

0 Absent

1 Slight or detectable only on activation

2 Definitely abnormal, but full range of motion possible

3 Only partial range of motion possible

4 Little or no passive motion possible

19. Limb dystonia

0 Absent

1 Subtle or present only when activated by other movement

2 Obvious but not continuous

3 Continuous but not disabling

4 Continuous and disabling

20. Finger tapping

0 Normal (4-14 taps/5 s with maximal amplitude)

1 Impaired (6 -14 taps/5 s or moderate loss of amplitude

2 Barely able to perform (0 -5 taps/5 s or severe loss of amplitude)

21. Toe tapping

0 Normal (414 taps/5 s with maximal amplitude)

1 Impaired (6 ^14 taps/5 s or moderate loss of amplitude

2 Barely able to perform (0 ^5 taps/5 s or severe loss of

amplitude)

22. Apraxia of hand movement

0 Absent

1 Present, not impairing most functions

2 Impairing most functions

23. Tremor in any part

0 Absent

1 Present, not impairing most functions

2 Impairing most functions

VI.Gait and midline

24. Neck rigidity or dystonia

0 Absent

1 Slight or detectable only when activated by other movement

2 Definitely abnormal, but full range of motion possible

3 Only partial range of motion possible

4 Little or no passive motion possible

25. Arising from chair

0 Normal

1 Slow but arises on first attempt

2 Requires more than one attempt, but arises without using hands

3 Requires use of hands

4 Unable to arise without assistance

26. Gait

0 Normal

1 Slightly wide-based or irregular or slight pulsion on turns

2 Must walk slowly or occasionally use walls or helper to avoid falling, especially on turns

3 Must use assistance all or almost all the time

4 Unable to walk, even with walker; may be able to transfer

27. Postural stability

0 Normal (shifts neither foot or one foot)

1 Must shift each foot at least once but recovers unaided

2 Shifts feet and must be caught by examiner

3 Unable to shift feet; must be caught, but does not require assistance to stand still

4 Tends to fall without a pull; requires assistance to stand still

28. Sitting down

0 Normal

1 Slightly stiff or awkward

2 Easily positions self before chair, but descent into chair is uncontrolled

3 Has difficulty finding chair behind him/her and descent is uncontrolled

4 Unable to test because of severe postural instability

Management / Interventions

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Differential Diagnosis

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Resources

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