ALS Functional Rating Scales

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APPENDIX C. FUNCTION TESTS

I. ALS Functional Rating Scales (ALSFRS).

Instructions for Use: The evaluator shall state to the patient (or

spouse or other caregiver if the patient cannot communicate

effectively): " Now I have a few questions I would like to ask to

help me better understand how you (or the patient) are currently

functioning at home. Please answer each question to the best of your

ability. "

The evaluator then asks, " How are you doing at (..)?' " for each

question in the ALSFRS. If the patients is unable to volunteer

satisfactory response, the evaluator may prompt, using one of the

available choices (e.g. when asking about speech, " do you find that

people are having trouble understanding you when speaking? " , etc.)

ALSFRS (Because the ALSFRS has not to date been published, it is

included here, with permission of J.M. Cerdarbaum, M.D., Regeneron

Pharmaceuticals, Inc.):

Speech

4 Normal speech processes

3 Detectable speech disturbance

2 Intelligible with repeating

1 Speech combined with nonvocal communication

0 Loss of useful speech

Salivation

4 Normal

3 Slight but definite excess of saliva in mouth; may have nighttime

drooling

2 Moderately excessive saliva; may have minimal drooling

1 Marked excess of saliva with some drooling

0 Marked drooling; requires constant tissue or handkerchief

Swallowing

4 Normal eating habits

3 Early eating problems; occasional choking

2 Dietary consistency changes

1 Needs supplemental tube feeding

0 NPO (exclusively parenteral or enteral feeding)

Handwriting (with dominant hand prior to ALS onset)

4 Normal

3 Slow or sloppy; all words are legible

2 Not all words are legible

1 Able to grip pen but unable to write

0 Unable to grip pen

a. Cutting Food and Handling Utensils

(patients without gastrostomy)

4 Normal

3 Somewhat slow and clumsy, but no help needed

2 Can cut most foods, although clumsy and slow; some help needed

1 Food must be cut by someone, but can still feed slowly

0 Needs to be fed

5. b. Cutting Food and Handling Utensils

(patients with gastrostomy)

4 Normal

3 Clumsy but able to perform all manipulations independently

2 Some help needed with closures and fasteners

1 Provides minimal assistance to caregiver

0 Unable to perform any aspect of task

Dressing & Hygiene

4 Normal

3 Independent and complete self-care with effort or decreased

efficiency

2 Intermittent assistance or substitute methods

1 Needs attendant for self-care

0 Total dependence

Turning in Bed/ Adjusting Bed Clothes

4 Normal

3 Somewhat slow and clumsy, but no help needed

2 Can turn alone or adjust sheets, but with great difficulty

1 can initiate, but not turn or adjust sheets alone

0 Helpless

Walking

4 Normal

3 Early ambulation difficulties

2 Walks with assistance (any assistive device, including AFOs)

1 Nonambulatory functional movement only

0 No purposeful leg movement

Climbing Stairs

4 Normal

3 Slow

2 Mild unsteadiness or fatigue

1 needs assistance (including handrail)

0 Cannot do

Breathing

4 Normal

3 Shortness of breath with minimal exertion (e.g. walking, talking)

2 Shortness of breath at rest

1 Intermittent (e.g. nocturnal) ventilatory assistance

0 Ventilator dependent

II. Amytrophic Lateral Sclerosis Severity Scale (ALSSS)

The scale and instructions for its use are detailed in:

Hillel-AD, RM, Yorkston K, et al Amyotrophic Lateral

Sclerosis Severity Scale in Progress in Clinical Neurologic Trials.

Vol 1 Amyotrophic Lateral Sclerosis, ed. F. Clifford Rose, Demos,

New York, 1990, 93-97.

III. Timed Walk

Time required for the patient to walk a fixed distance, as fast as

possible without compromising patient safety, is measured, using

digital stopwatch. The fixed distance, e.g. 15 feet, is marked in

the testing room or hallway. The patient is instructed to walk as

fast as possible safely. Use of the patients' normal ambulation aids

or equipment is allowed. Assistive equipment used should be

recorded, as this may affect gait velocity. Sound judgement by the

patient and evaluator must be used to determine the safety risks

involved with this test. If the patient feels uncomfortable with

this test, or if risk of falling is significantly increased, the

test should not be performed. Falls can result in serious injuries

in this patient population, especially in those persons with

inadequate strength and function of upper extremities. The clinical

evaluator should walk behind the patient for safety. No running is

allowed (one foot should always be on the ground). If the effects of

acceleration and deceleration on gait velocity are to be ruled out,

the patient may being walking several feet before the distance

marker, and may walk several feet past the distance marker, before

stopping. Time required to walk the fixed distance is recorded. Gait

velocity can be calculated as follows: velocity = distance/time.

IV. Purdue Pegboard

The number of pegs a patient can place in the pegboard holes (Purdue

pegboard, Lafayette Instrument Co., P.O. Box 5729, Lafayette,

Indiana 47903) in 30 seconds is determined. The patient picks up

pegs, one at a time, from the reservoir, and places them in the

pegboard holes, working from the top of the pegboard to the bottom,

without skipping any holes. The patients should work as quickly as

possible. Any assistance devices or equipment allowed should be

recorded.

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APPENDIX D. BULBAR TEST

Timed speech rates:

A stopwatch is used to time the seconds needed to complete a

predetermined number of syllable repetitions. The patient is told

what syllable to say and how it should be annunciated. Tell patient

to repeat syllable as fast as he/she can, but patient does not have

to complete the test in one breath. Timing begins when the patients

starts speaking and ends with last syllable spoken. Another method

pre-determines the time allowed, and the patient repeats a syllable

as many times as possible in the given time., A tape recorder is

used to track number of syllables spoken..

Instructions:

" I am going to time how fast you can say 'pa' (for example). Go as

fast as you can. It should sound like this 'papapapapapapa...'. If

you need to take a breath, do so. Keep going until I tell you to

stop. " The best of two trials is recorded.