Content, instructions, and scoring of the final version of the Parkinson’s

Disease Cognitive Rating Scale (PD-CRS)

Items are administered to the subjects in the same order as presented below.

1. Immediate free recall verbal memory.

Instruction: The subject is asked to read aloud the written words shown on 12

consecutive cards. Three trials are performed, and the subject is asked to recall as many

words as possible after each trial.

Content:

Words
LIGHT
SILK
SAND
EYELASH
RICE
TIE
BLACKBOARD
BICYCLE
STAR
LION
RING
FRAGRANCE

Score: 1 point for each word recalled. The highest number of words recalled in any one

trial is the score. (0-12)

The subject is asked to name the line drawings shown on 20 consecutive cards. There is

no time limit for response, and only one trial is given. No semantic or phonemic cues are

provided. When objects are included in their context (bib, buckle, mane, hook, jingle bell,

and hoof), the examiner is allowed to indicate the part of the line drawing to be named.

Content:

Images (see line drawings at the end of

the appendix)

BIB

CANDLE

CHERRY

STOOL

ANCHOR

TURTLE

KITE

FISHBOWL

BULB

GUITAR

BUCKLE

MANE

HOOK

SCREWDRIVER
PANEL SCREEN

SAFETY PIN

JINGLE BELL

HOOF

EXTINGUISHER

DOOR BOLT

Score: 1 point for each line drawing correctly named. (0-20)

3. Sustained attention.

Instruction: An ascending series of letters and numbers are read to the subjects. The

subject is asked to report the number of letters in the sequence. Ten series of letters and

numbers are presented, divided into five levels of ascending complexity. Two training

series are provided at the beginning of the test.

Content:

Correct answer

2LT 2 letters
Example
8A9 1 letter

2P654 1 letter
1
3A6KL 3 letters

B904LT 3 letters
2
3CP573 2 letters
 395L4ZA 3 letters
3
I1ASQ41 4 letters

75DA4TB2 4 letters
4
968437LC 2 letters

Z49ATD384 4 letters
5
95MD4SC3E 5 letters

Score: 1 point for each correct series. (0-10)

4. Working memory.

Instructions: The examiner reads aloud a randomized list of numbers and letters ranging

in length from 2 to 6 letters and numbers. After each series the subject is asked to repeat

the numbers first, and then the letters. This test ends when the subject is unable to give

the correct answer in two consecutive series. Two training series are provided at the

beginning of the test.

Content:

Correct answer

L2T 2LT
Example
8A9 89A

M3 3M
1
7P 7P

2 G8M 8GM
 9I6 96I

T04A 04TA
3
7V6J 76VJ

M64NI 64MNI
4
35SGC 35SCG

1R9VB3 193RVB
5
M274Z9 2749MZ

Score: 1 point for each correct series. (0-10)

Clock drawing task.

Instructions:

5. Umprompted drawing of a clock: The subject is asked to draw a clock face on a

blank sheet of paper, and to set the hands at “twenty-five minutes past ten”. (0-10)

6. Copy drawing of a clock: The patient is asked to copy the presented clock. (0-10)

12
11 1

10 2

9 • 3

8 4

7 5
6
 Unprompted Copy

Yes No Yes No

The figure looks like a clock.

The clock is not divided by lines or sectors.

There is a symmetric disposition of numbers.

Only 1 to 12 numbers are drawn.

Hour numbers are correctly sequenced.

Only two hands are drawn.

Clock hands are represented as arrows.

Hour hand is shorter than minute hand.

No words have been written.

The number ‘25’ has not been drawn.

Score: 1 point for each correct item. (0-10 for each task)

7. Delayed free recall verbal memory.

Instructions: The subject is asked to recall as many words as possible from the list of

words presented at the beginning of the scale.

Content:

Words
LIGHT
SILK
SAND
EYELASH
RICE
TIE
BLACKBOARD
BICYCLE
STAR
LION
RING
FRAGRANCE

Score: 1 point for each word recalled. (0-12)

8. Alternating verbal fluency.

Instructions: The subject is asked to alternately generate as many different words as

possible beginning with the letter ‘S’ and words describing articles of clothing during 60

seconds. Participants are instructed not to use proper nouns or to repeat the same word

with a different ending (e.g., swim, swimming, swimsuit).

Score: 1 point for each correct answer maintaining the alternation between words

beginning with ‘s’ and articles of clothing. (0-20)

9. Action verbal fluency.

Instructions: We used the instructions listed in Piatt et al. (reference number 45 in the

manuscript) for the action verbal fluency task. The instructions are as follows: ‘‘During

60 seconds, I’d like you to tell me as many different things as you can think of that

people do. I don’t want you to use the same word with different endings, like eat, eating,

eaten. Also, just give me single words such as eat, or smell, rather than a sentence”.

Score: 1 point for each correct answer. (0-30)

ITEM Points

1. Immediate free recall verbal memory

2. Confrontation naming

3. Sustained attention

4. Working memory

5. Umprompted drawing of a clock

6. Copy drawing of a clock

7. Delayed free recall verbal memory

8. Alternating verbal fluency

9. Action verbal fluency

SUBCORTICAL score (0-114)

CORTICAL score (0-20)

TOTAL score (0-134)

Subcortical and cortical PD-CRS scores were obtained by adding the raw scores of the

items within each group. Total scores on the PD-CRS were calculated by adding the

subcortical and cortical PD-CRS scores.

1. BIB

2. CANDLE

3. CHERRY
4. STOOL

5. ANCHOR

6. TURTLE
7. KITE

8. FISHBOWL

9. BULB
10. GUITAR

11. BUCKLE

12. MANE
13. HOOK

14. SCREWDRIVER

15. PANEL SCREEN

17. JINGLE BELL

18. HOOF
19. EXTINGUISHER

20. DOOR BOLT

CRS)

1. ‘Subcortical-type’ cognitive items:

- Attention/Executive functions: Disturbances in both the frontal regulation of

attentional processes and working memory occur early in PD-ND patients,1-3 and

progress throughout the course of the disease.4, 5 They both have been correlated with

prefrontal atrophy, dopaminergic hypometabolism in the dorsolateral prefrontal cortex

(DLPFC),6and underactivation of the caudate nucleus, the ventrolateral and the

DLPFC.7 Participants were asked to say how many letters were presented from among a

series of letters and numbers for assessing attention, and to recall separately a

randomized list of numbers and letters for assessing working memory.

- Resistance to interference: Susceptibility to interference is impaired in PD,8 but

discrepancies about the usefulness and early impairment of this cognitive function in

PD have been reported.2, 3 Resistance to interference relies also upon the DLPFC.9 The

test selected was a computerized version of the Stroop test.

- Verbal fluency (VF) and cognitive flexibility: Phonemic and semantic verbal

fluencies are progressively impaired over the course of the disease10, 11, and their

deterioration is indicative of PDD development. 10 Action verbal fluency (action VF)

appears to be an early indicator of the conversion from PD-ND to PDD.12 Alternating

verbal fluency (alternating VF) requires a mental shift to generate words belonging to

different categories, and is impaired since the earliest stages of the disease.13

Participants were asked to generate as many words as possible in 60 seconds for each

fluency task.
- Verbal memory: Free recall immediate and delayed verbal memory are markedly

impaired in PD-ND2, 14 and their deterioration is indicative of PDD development. 10, 15

Impairment in free recall memory appears to be more related to the defective use of

memory stores due to working memory deficits, than a reduced capacity of

storing/consolidating new information in the temporal lobes.16 The specific task selected

was to recall as many words as possible from a 12-word list.

- Visuoconstructional skills/Clock drawing: PD-ND and PDD17 are associated with

marked visuospatial deficits.17, 18 Visuoconstructional abilities depend on the

functionality of both the prefrontal cortex and the posterior visual cortical areas.19 The

specific task selected for this cognitive function was the unprompted drawing of a clock

set at twenty-five minutes past ten,

2. ‘Cortical-type’ cognitive items:

- Confrontation naming: Naming is normal in PD-ND20, 21 but deteriorates in PDD.20,

mainly correlated with cortical activity in the anteromedial and posteromedial temporal

cortex.23, 24 In this task, participants were asked to name 20 line drawings, with no time

constraint.

- Visuoperceptual skills/Copy of a clock: To assess the functionality of the posterior

visual cortical areas, we assessed the copy of a clock after the unprompted drawing of

such a clock, which has been shown to partially separate the frontal-subcortical from the

posterior cortical component of this cognitive function.25 The task selected was the copy

of a clock set at twenty-five minutes past ten.

The initial PD-CRS included 10 ‘subcortical-type’ items (attention, working memory,

Stroop test, four verbal fluencies, immediate and delayed verbal memory, clock

drawing), and two ‘cortical-type’ items (naming, copy of a clock). Total score ranged
from 0 to 204, subcortical score from 0 to 174, and cortical score from 0 to 30, with

higher scores indicating a better functioning.

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