For use in the assessment of Medical Conditions in patients seeking Immunoglobulin treatment
Guillain-Barré syndrome (GBS) Disability Score
Download PDFThe Guillain-Barré syndrome (GBS) disability score is a widely accepted scoring system to assess the functional status of patients with GBS. It was originally described in Hughes et al. (1978) and since then, various iterations have appeared in the literature. The adaptation best suited for use in the Criteria and BloodSTAR is from van Koningsveld et al (2007). The Criteria requires that the patient’s level of disability be documented using the scale from 0 to 6 as below.
| Score | Description |
|---|---|
| 0 | A healthy state |
| 1 | Minor symptoms and capable of running |
| 2 | Able to walk 10m or more without assistance but unable to run |
| 3 | Able to walk 10m across an open space with help |
| 4 | Bedridden or chairbound |
| 5 | Requiring assisted ventilation for at least part of the day |
| 6 | Dead |
Modified Rankin Scale (MRS)
Download PDFThe Modified Rankin Scale was originally developed by Rankin J (1957) and has been replicated for use in CIDP, Stiff person syndrome and Susac syndrome in the Criteria from Van Swieten et al. (1987). The Criteria requires the patient’s level of disability to be assessed using a scale from 0 to 6 ranging from no symptoms at all to death.
The Modified Rankin Scale from which this scale has been replicated is available on the Opens new window Stroke Engine website at:
Please note for the conditions paediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) or paediatric acute neuropsychiatric disorders (PANS), Sjogren’s syndrome, AMAE and Rasmussen syndrome the description of disabilities in the Modified Rankin Scale has been adapted given that there is no loss of motor function.
Medical Research Council (MRC) Scale for Muscle Strength
Download PDFThe Medical Research Council (MRC) Scale for Muscle Strength is a commonly used scale for assessing muscle strength from Grade 5 (normal) to Grade 0 (no visible contraction). It was originally described by the Medical Research Council in 1943 Opens new window The MRC sum score was first described by Kleyweg et al (1988) for use in the Dutch Guillain-Barré trial. This score was defined as the sum of MRC scores from six muscles in the upper and lower limbs on both sides so that the score ranged from 60 (normal) to 0 (quadriplegic).
The Criteria requires that each of the six muscle groups listed in the table are examined bilaterally, each with a score from 0 to 5 according to the scale in the right hand column.
MRC Sum score
| Muscle | Side | Score 0 - 5 |
|---|---|---|
| Shoulder abductors | Left | |
| Right | ||
| Elbow flexors | Left | |
| Right | ||
| Wrist extensors | Left | |
| Right | ||
| Hip flexors | Left | |
| Right | ||
| Knee extensors | Left | |
| Right | ||
| Foot dorsiflexors | Left | |
| Right | ||
| Total (out of 60) |
MRC scale for muscle strength (0-5)
- Grade 5: Normal
- Grade 4: Movement against gravity and resistance
- Grade 3: Movement against gravity over (almost) the full range
- Grade 2: Movement of the limb but not against gravity
- Grade 1: Visible contraction without movement of the limb (not existent for hip flexion)
- Grade 0: No visible contraction
- MRC grade for each muscle given in full numbers: (4+/4.5 =4) (4- =3) (5- = 4)
Childhood Myositis Assessment Scale – CMAS
Download PDFThe Childhood Myositis Assessment Scale (CMAS) has been shown to be a valid measure of physical function in children with juvenile idiopathic inflammatory myopathies. It is the most commonly used assessment tool and has been recommended as a core set measure. The CMAS was first published in 1999 by Lovell et al, however the standardized method for conducting the scale can be found below and at the International Myositis Assessment and Clinical Studies Group web site.
The Criteria requires submission of a score calculated by assessing each of the 14 manoeuvres in the table and scored according the scale to yield a total maximum score of 52.
Childhood Myositis Assessment Scale scoring sheet
1. Head elevation (neck flexion):
- 0 = Unable
- 1 = 1 – 9 seconds
- 2 = 10 – 29 seconds
- 3 = 30 – 59 seconds
- 4 = 60 – 119 seconds
- 5 = ≥2 minutes
Score
2. Leg raise/touch object:
- 0 = Unable to lift leg off table
- 1 = Able to clear table, but cannot touch object
- 2 = Able to lift leg high enough to touch object
Score
3. Straight leg lift/duration:
- 0 = Unable
- 1 = 1 – 9 seconds
- 2 = 10 – 29 seconds
- 3 = 30 – 59 seconds
- 4 = 60 – 119 seconds
- 5 = ≥2 minutes
Score
4. Supine to prone:
- 0 = Unable. Has difficulty even turning onto side; able to pull arms under torso only slightly or not at all
- 1 = Turns onto side fairly easily, but cannot fully free arms and is not able to fully assume a prone position
- 2 = Easily turns onto side; has some difficulty freeing arms, but fully frees them and fully assumes a prone position
- 3 = Easily turns over, fully frees right arm with no difficulty
Score
5. Sit-ups:
For each type of sit-up enter either "0" (unable) or "1" (able). Then enter the total sit-up score (0-6).
- Hands on thighs, with counterbalance
- Hands across chest, with counterbalance
- Hands behind head, with counterbalance
- Hands on thighs, without counterbalance
- Hands across chest, without counterbalance
- Hands behind head, without counterbalance
Score
6. Supine to sit:
- 0 = Unable by self
- 1 = Much difficulty. Very slow, struggles greatly, barely makes it. Almost unable
- 2 = Some difficulty. Able, but is somewhat slow, struggles some
- 3 = No difficulty
Score
7. Arm raise/straighten:
- 0 = Cannot raise wrists
- 1 = Can raise wrists at least up to the level of the acromioclavicular joint, but not above top of head
- 2 = Can raise wrists above top of head, but cannot raise arms straight above head so that elbows are in full extension
- 3 = Can raise arms straight above head so that elbows are in full extension
Score
8. Arm raise/duration:
Can maintain wrists above top of head for:
- 0 = Unable
- 1 = 1 – 9 seconds
- 2 = 10 – 29 seconds
- 3 = 30 – 59 seconds
- 4 = ≥60 seconds
Score
9. Floor sit:
Going from a standing position to a sitting position on the floor.
- 0 = Unable. Afraid to even try, even if allowed to use a chair for support. Child fears that he/she will collapse, fall into a sit, or harm self
- 1 = Much difficulty. Able, but needs to hold onto a chair for support during descent. (Unable or unwilling to try if not able to use a chair for support)
- 2 = Some difficulty. Can go from stand to sit without using a chair for support, but has at least some difficulty during descent. Descends somewhat slowly and/or apprehensively; may not have full control or balance as manoeuvres into a sit
- 3 = No difficulty. Requires no compensatory manoeuvring
Score
10. All-fours manoeuvre:
- 0 = Unable to go from a prone to an all-fours position
- 1 = Barely able to assume and maintain an all-fours position
- 2 = Can maintain all-fours position with straight back and head raised (so as to look straight ahead). But, cannot creep (crawl) forward
- 3 = Can maintain all-fours, look straight ahead, and creep (crawl) forward
- 4 = Maintains balance while lifting and extending leg
Score
11. Floor rise:
Going from a kneeling position on the floor to a standing position
- 0 = Unable, even if allowed to use a chair for support
- 1 = Much difficulty. Able, but needs to use a chair for support. Unable if not allowed to use a chair
- 2 = Moderate difficulty. Able to get up without using a chair for support, but needs to place one or both hands on thighs/knees or floor. Unable without using hands
- 3 = Mild difficulty. Does not need to place hands on knees, thighs, or floor, but has at least some difficulty during ascent
- 4 = No difficulty
Score
12. Chair rise:
- 0 = Unable to rise from chair, even if allowed to place hands on sides of chair seat
- 1 = Much difficulty. Able, but needs to place hands on sides of seat. Unable if not allowed to place hands on knees/thighs
- 2 = Moderate difficulty. Able, but needs to place hands on knees/thighs. Does not need to place hands on side of seat
- 3 = Mild difficulty. Able; does not need to use hands at all, but has at least some difficulty
- 4 = No difficulty
Score
13. Stool step:
- 0 = Unable
- 1 = Much difficulty. Able, but needs to place one hand on exam table or examiner's hand
- 2 = Some difficulty. Able; does not need to use exam table for support, but needs to use hand(s) on knee/thigh
- 3 = Able. Does not need to use exam table or hand(s) on knee/thigh
Score
14. Pick up:
- 0 = Unable to bend over and pick up pencil off floor
- 1 = Much difficulty. Able, but relies heavily on support gained by placing hand(s) on knees/thighs
- 2 = Some difficulty. Needs to at least minimally and briefly place hand(s) on knees/thighs for support and is somewhat slow
- 3 = No difficulty. No compensatory manoeuvre necessary
Score
The Myasthenia Gravis Composite score – MGC score
Download PDFThe Myasthenia Gravis Composite score has been validated as an outcome measure of signs and symptoms for patients with myasthenia gravis (MG). The score was first described by Burns et al. (2008) and has been replicated for the use in MGC in the Criteria from Burns et al. (2010). The Criteria requires submission of a score calculated by completing each section in the table after assessment of both physician examination and patient reported signs and symptoms.
The Myasthenia Gravis Composite score
Ptosis, upward gaze (physician examination)
- 0 = > 45 seconds
- 1 = 11 - 45 seconds
- 2 = 1 - 10 seconds
- 3 = immediate
Score
Double vision on lateral gaze, left or right (physician examination)
- 0 = > 45 seconds
- 1 = 11 - 45 seconds
- 3 = 1 - 10 seconds
- 4 = immediate
Score
Eye closure (physician examination)
- 0 = Normal
- 0 = Mild weakness (can be forced open with effort)
- 1 = Moderate weakness (can be forced open easily)
- 2 = Severe weakness (unable to keep eyes closed)
Score
Talking (patient history)
- 0 = Normal
- 2 = Intermittent slurring or nasal speech
- 4 = Constant slurring or nasal but can be understood
- 6 = Difficult to understand speech
Score
Chewing (patient history)
- 0 = Normal
- 2 = Fatigue with solid food
- 4 = Fatigue with soft food
- 6 = Gastric tube
Score
Swallowing (patient history)
- 0 = Normal
- 2 = Rare episode of choking or trouble swallowing
- 5 = Frequent trouble swallowing, eg. necessitating changes in diet
- 6 = Gastric tube
Score
Breathing (thought to be caused by MG)
- 0 = Normal
- 2 = Shortness of breath with exertion
- 4 = Shortness of breath at rest
- 9 = Ventilator dependence
Score
Neck flexion or extension (weakest, physician examination)
- 0 = Normal
- 1 = Mild weakness
- 3* = Moderate weakness (ie.~50% weak, ±15%)
- 4 = Severe weakness
Score
Shoulder abduction (physician examination)
- 0 = Normal
- 2 = Mild weakness
- 4* = Moderate weakness (ie.~50% weak, ±15%)
- 5 = Severe weakness
Score
Hip flexion (physician examination)
- 0 = Normal
- 2 = Mild weakness
- 4* = Moderate weakness (ie.~50% weak, ±15%)
- 5 = Severe weakness
Score
Expanded Disability Status Scale (EDSS)
Download PDFThe Expanded Disability Status Scale (EDSS) is a commonly used scale for assessing the level of disability in people with multiple sclerosis. The scale was originally described by Kurtze (1983) and has been adapted on the Multiple Sclerosis Trust Opens new window website.
The Criteria requires that each of the functional systems listed below be reviewed. The description in the table below should be referred to and for ambulant patients a score from 0 (no disability) to 5.5 (more severe disability) be indicated. If the patient has impairment such that assistance is required to enable walking (cane, crutches, frame) the score will be from 0 to 6.5, if a wheelchair is required for all but a few steps the score will be 7.0 – 8.5, if confined to bed the score will be 9 and death due to MS is scored 10.
Functional systems
- pyramidal - weakness or difficulty moving limbs
- cerebellar - ataxia, loss of coordination or tremor
- brainstem - problems with speech, swallowing and nystagmus
- sensory - numbness or loss of sensations
- bowel and bladder function
- visual function
- cerebral (or mental) functions
- other
ALL functional system scores must be scored individually and then the EDSS calculated from the table below.
| Score | Description |
|---|---|
| 0.0 | No disability |
| 1.0 | No disability, minimal signs in one FS |
| 1.5 | No disability, minimal signs in more than one FS |
| 2.0 | Minimal disability in one FS |
| 2.5 | Mild disability in one FS or minimal disability in two FS |
| 3.0 | Moderate disability in one FS, or mild disability in three or four FS. No impairment to walking |
| 3.5 | Moderate disability in one FS and more than minimal disability in several others. No impairment to walking |
| 4.0 | Significant disability but self-sufficient and up and about some 12 hours a day. Able to walk without aid or rest for 500m |
| 4.5 | Significant disability but up and about much of the day, able to work a full day, may otherwise have some limitation of full activity or require minimal assistance. Able to walk without aid or rest for 300m |
| 5.0 | Disability severe enough to impair full daily activities and ability to work a full day without special provisions. Able to walk without aid or rest for 200m |
| 5.5 | Disability severe enough to preclude full daily activities. Able to walk without aid or rest for 100m |
| 6.0 | Requires a walking aid - cane, crutch, etc. - to walk about 100m with or without resting |
| 6.5 | Requires two walking aids - pair of canes, crutches, etc. - to walk about 20m without resting |
| 7.0 | Unable to walk beyond approximately 5m even with aid. Essentially restricted to wheelchair; though wheels self in standard wheelchair and transfers alone. Up and about in wheelchair some 12 hours a day |
| 7.5 | Unable to take more than a few steps. Restricted to wheelchair and may need aid in transferring. Can wheel self but cannot carry on in standard wheelchair for a full day and may require a motorised wheelchair |
| 8.0 | Essentially restricted to bed or chair or pushed in wheelchair. May be out of bed itself much of the day. Retains many self-care functions. Generally has effective use of arms |
| 8.5 | Essentially restricted to bed much of day. Has some effective use of arms retains some self-care functions |
| 9.0 | Confined to bed. Can still communicate and eat |
| 9.5 | Confined to bed and totally dependent. Unable to communicate effectively or eat/swallow |
| 10.0 | Death due to MS |
Cerebellar Functional System Score
Download PDFThe Cerebellar Functional System Score has been adapted from the larger Functional Systems Score first described by Kurtske (1983) which was designed for assessing people with multiple sclerosis. However, the Cerebellar Functions section can be used to demonstrate initial disability and response to Ig therapy in children with Opsoclonus-myoclonus ataxia (OMA).
The Criteria requires that the patient’s impairment is indicated using the scale from 0 to 5 as below.
| Score | Criteria |
|---|---|
| 0 | Normal |
| 1 | Abnormal signs without disability |
| 2 | Mild ataxia |
| 3 | Moderate truncal or limb ataxia |
| 4 | Severe ataxia, all limbs |
| 5 | Unable to perform coordinated movements due to ataxia |
| X | Used throughout after each number when weakness (grade 3 or more on pyramidal) interferes with testing |
Overall Neuropathy Limitations Scale
Download PDFThe Overall Disability Sum Score (ODSS) was the first scale designed to assess the limitations of patients with immune-mediated peripheral neuropathies. The ODSS focuses on upper and lower limb functions, and consists of a checklist for interviewing patients. The ODSS has showed reliability, responsiveness and construct validity in people with Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy and paraprotein-associated demyelinating neuropathy. To reduce a possible ceiling effect, the ODSS was modified slightly to include climbing stairs and running. This new measure is called the Overall Neuropathy Limitations Scale (ONLS). The Criteria requires that this scale be completed by adding the total of the Arm scale score (0-5) and Leg scale score (0-7) yielding a total score of 0-12.
To evaluate a change in disability on patient review an Adjusted ONLS is recorded. The adjusted score is identical to the ONLS disability score except for the exclusion of changes in upper limb function from 0 to 1 or from 1 to 0, because these changes have not been judged to be clinically significant in all patients. All other 1-point steps in either the arm or leg scale represent clinically meaningful changes in disability.
Overall Neuropathy Limitations Scale
Instructions: The examiner should question and observe the patient in order to determine the answers to the following questions. Note should be made of any other disorder other than peripheral neuropathy which limits function.
Arms Scale
| Does the patient have any symptoms in their hands or arms, e.g. tingling, numbness or weakness? (if no, go to legs section) | Yes | No |
|---|
| Is the patient affected in their ability to: | Not affected | Affected but not prevented | Prevented |
|---|---|---|---|
| Wash and brush their hair | |||
| Turn a key in a lock | |||
| Use a knife and fork together (or spoon, if knife and fork not used) | |||
| Do or undo buttons or zips | |||
| Dress the upper part of their body excluding buttons or zips |
| If all these functions are prevented can the patient make purposeful movements with their hands or arms? | Yes | No | Not applicable |
|---|
Arms grade score
- 0 = Normal
- 1 = Minor symptoms in one or both arms but not affecting any of the functions listed
- 2 = Disability in one or both arms affecting but not preventing any of the functions listed
- 3 = Disability in one or both arms preventing at least one but not all functions listed
- 4 = Disability in both arms preventing all functions listed but purposeful movement still possible
- 5 = Disability in both arms preventing all purposeful movements
Legs Scale
| Yes | No | Not applicable | |
|---|---|---|---|
| Does the patient have difficulty running or climbing stairs? | |||
| Does the patient have difficulty with walking? | |||
| Does their gait look abnormal? | |||
| How do they mobilise for about 10 metres (i.e. 33 feet)? | |||
| Without aid | |||
| With one stick or crutch or holding to someone's arm | |||
| With two sticks or crutches or one stick or crutch holding onto someone's arm or frame | |||
| With a wheelchair | |||
| If they use a wheelchair, can they stand and walk 1 metre with the help of one person? | |||
| If they cannot walk as above are they able to make some purposeful movements of their legs, e.g. reposition legs in bed? | |||
| Does the patient use ankle foot orthoses/braces? ( If yes, please indicate, Right or Left ) |
Legs grade score
- 0 = Walking/climbing stairs/running not affected
- 1 = Walking/climbing stairs/running is affected, but gait does not look abnormal
- 2 = Walks independently but gait looks abnormal
- 3 = Requires unilateral support to walk 10 metres (stick, single crutch, one arm)
- 4 = Requires bilateral support to walk 10 metres (sticks, crutches, crutch and arm, frame)
- 5 = Requires wheelchair to travel 10 metres but able to stand and walk 1 metre with the help of one person
- 6 = Restricted to wheelchair, unable to stand and walk 1 metre with the help of one person, but able to make some purposeful leg movements
- 7 = Restricted to wheelchair or bed most of the day, unable to make any purposeful movements of the legs
| Is there any disorder, other than peripheral neuropathy, which affects the above functions? If Yes, please describe in the comments section in BloodSTAR. | Yes | No |
|---|
Overall Neuropathy Limitation Scale Score
Range: 0 (no disability) to 12 (maximum disability).
The Six Minute Walk Test – 6MWT
Download PDFThe Six Minute Walk Test (6MWT) is an objective evaluation of functional capacity, which has been demonstrated to be accurate, reproducible, simple to administer and well-tolerated. Relevance to neuromuscular diseases has been established. Guidelines exist to standardise the performance of the 6MWT with adaptations and standard reference values described for use in children.
Instructions: The Criteria requires documentation of the distance walked in 6 minutes using a standardised methodology.
For methodological instructions please see the American Thoracic Society
ATS Statement: Guidelines for the Six-Minute Walk Test
https://www.thoracic.org/statements/resources/pfet/sixminute.pdf Opens new window
Distribution of Stiffness index
Download PDFThe Distribution of Stiffness Index was originally described in Dalakas et al. (2000) Opens new window and replicated for the use in Stiff Person Syndrome in the Criteria from Dalakas et al. (2017). It can be used to provide a baseline and to monitor the response to Ig therapy.