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Measuring Disease Activity and Outcome

Several tools can be used to measure outcome in patients that are being treated for CNS vasculitis. These tools are listed below. 
(Note: these tools will be provided to researchers participating in the BrainWorks study.) 

List of clinical assessment tools:


Quality of Life Measures:


Rehabilitation Outcome Measure:


Stroke Outcome Measure (SOM) (Modified)

  • SOM is a standardized questionnaire to be completed by the doctor for the affected child. The SOM is a short neurologic examination evaluating neurological deficits and function across five domains:
    • Sensorimotor on the right side
    • Sensorimotor on the left side
    • Language production
    • Language comprehension
    • Cognition or behavior
  • Each domain is scored according to the level of functioning and the interference on daily life activities (DLA): 0 (normal), 0.5 (mild deficit, normal function), one(moderate deficit, decreased function), or two (severe deficit, missing function).
  • The doctor can use this tool to assess the patient's neurological outcome at follow-up visits.

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Physician Global Assessment (PGA)

  • Physician Global Assessment evaluates overall disease activity, reversible impairment, and permanent damage of the child.
  • This assessment is a 10 centimeter visual analog scale with 0 being no disease activity and 10 being maximum disease activity.
  • At SickKids, this is part of standard of care and the PGA is completed at every visit.

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Kurtzke's Expanded Disability Status Scale (EDSS)

  • Tool for measuring the disability status of people with multiple sclerosis, but can also be used for patients with inflammatory brain diseases.
  • A total score is calculated which can range from 0 to 10. Zero represents no mental impairment, and higher scores represent increased mental impairment and loss of ambulatory ability.  Ten is death due to the disease.
  • In addition to the total score, 8 subscale measurements called Functional System (FS) scores are determined. These functional systems include:
    1. motor function
    2. cerebellar
    3. brainstem
    4. sensory
    5. bowel and bladder
    6. visual
    7. cerebral or mental
    8. other
  • Each FS (excluding “Other”) is given a score from 0 to 5, as observed clinically.
  • The FS scores are considered, along with ambulatory ability, to determine the total EDSS score.

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Barthel Index (BI)

  • The Barthel Index is an ‘activities of daily living’ (ADL) scale, measuring the ability of stroke patients to perform daily activities including the following:
    Bowel care, bladder care, grooming, toilet use, feeding, mobility, dressing, stairs, and bathing.
  • There are 10 items each testing one of the above activities. The questions can be answered by the individual, a caregiver that is familiar with the patient, or through observation by the interviewer. It does not need to be directly administered to the patient.
  • Some item can be scored 0 or 5, while others can be scored 0, 5, 10, or 15. Zero is allocated to an individual that is fully dependent in a certain daily activity, while a higher score means more independence.  
  • A total score of 100 is possible.
  • This tool is often criticized for having ‘ceiling effects’ due to the fact that it is relatively easy to obtain a high score despite disability. It is not a very sensitive score of disability.

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Modified Rankin Scale (MRS)

  • Measures functional independence (therefore encompassing mental and physical deficits) after stroke.  Also used to assess the efficacy of rehabilitation procedures (improvements in disability).
  • The test consists of questions regarding the patient’s abilities to perform daily living activities. The answer to one question affects the subsequent question that is asked. At the end of the question series, the patient is assigned a score from 0 to 6.
  • 0 signifies no disability, 5 signifies severe disability, and 6 means death due to the disease.
  • It could take between five to fifteen minutes to administer the test.
  • Best suited for adults and elderly adults.

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NIH Stroke Scale (NIHSS)

  • The NIH Stroke Scale is used to quantitatively assess the neurological deficits caused by stroke.
  • The score can also serve as a measure of stroke severity and help to predict the size of the brain region affected.
  • The test can be administered by a professional such as a physician, nurse, or therapist.
  • It includes 15 items and takes approximately 10 minutes to complete. Performance on these items (which include short activities) helps to evaluate the levels of consciousness, eye movement, facial palsy, motor function of arm and leg, limb ataxia, language, extinction and inattention, dysarthria, and sensory loss.
  • Each item can be scored from 0 to 4, with 0 meaning there are no stroke symptoms and 4 meaning there are severe symptoms.
  • The individual item scores can be summed together to give a final score out of 42 points.

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Montreal Cognitive Assessment (MOCA)

  • The MoCA is a test for mild cognitive impairment, including early detection of Alzheimer’s and dementia. It is best suited for individuals 55–85 year olds.
  • The test includes thirteen activities, each assessing one of the following cognitive domains:
    1. Attention and concentration
    2. Executive function
    3. Memory
    4. Visuospatial skills
    5. Language
    6. Conceptual thinking
    7. Calculations
    8. Orientation (in time and space)
  • The overall maximum possible score of these activities is 30 points. A score of 26 and above is considered normal.
  • The test takes approximately 10 minutes to complete. The test results must be assessed by a healthcare professional in the cognitive field (such as a neurologist, neuropsychologist, occupational therapist, and psychiatrists).

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Quality of Life Measure

Rand 36-Item Health Survey (SF36)

  • The Rand Short-form 36 item survey (SF36) is a questionnaire consisting of 36 questions with multiple choice answers ranging from 2 to 6 options
  • The survey can be self-administered by the patient, or administered by an interviewer
  • Results of the survey help to assess a patient’s ability to perform daily functions, and their mental well-being
  • It is a generic measure, and therefore does not focus on testing a specific age group, disease, or treatment. This feature makes it very useful for comparing the test scores of different populations, comparing the relative burden of different diseases, and assessing the effectiveness of different treatments.  
  • The 36 items generate scores for 8 scales, which include frequently represented health concepts:
    1. Physical functioning
    2. Role-Physical
    3. Bodily Pain
    4. General Health
    5. Vitality
    6. Social Functioning
    7. Role-Emotional
    8. Mental Health
  • These eight scales are then clustered into two summary measures including Physical Health and Mental Health. Scores are assigned to each of the eight scales as well as the summary measures.

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Rehabilitation Outcome Measures

There is currently no specific rehabilitation outcome tool validated for use in CNS Vasculitis, mostly in children. Research is currently underway to validate a motor outcome tool for use in CNS Vasculitis patients.  In the meantime, these outcome measures may help clinicians to assess areas of impairment and identify a patient’s rehabilitation needs:

BOT-2: Bruininks-Oseretsky Test of Motor Proficiency, Second Edition[5]
▪ Task-based observational measure which assesses gross and fine motor skills with sex and age-matched normative values for comparison.
▪ Short-form version takes approximately 20 minutes to administer.
▪ More information is available at: http://www.pearsonassessments.com/HAIWEB/Cultures/en-us/Productdetail.htm?Pid=PAa58000 

GMFM: Gross Motor Functional Measure[6]
▪ Designed to assess changes in gross motor function in patients with Cerebral Palsy and regularly used in patients with acquired brain injury[6].
▪ More information is available at: https://canchild.ca/en/resources/44-gross-motor-function-measure-gmfm.
▪ Children’s Orientation and Amnesia Scale (COAT)[7].
▪ Standardized measures  designed to assess cognitive functioning in children and adolescents with an acquired brain injury.
▪ Composed of 16 items, the COAT addressed 3 areas of cognitive functioning:  general orientation, temporal orientation, and memory.

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  11. NIH Stroke Scale (NIHSS). NIH Stroke Scale International, Web. June 23, 2016. <http://www.nihstrokescale.org/>.
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  13. Dawood A. Rehab Measures: Modified Rankin Handicap Scale. Rehabilitation Measure Database, 2010. Web. June 23, 2016 <http://www.rehabmeasures.org/Lists/RehabMeasures/DispForm.aspx?ID=921>.
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  17. Tarver ML. Kurtzke Expanded Disability Status Scale. U.S. Department of Veteran Affairs, 2015. Web. June 23, 2016. < http://www.va.gov/MS/Professionals/Diagnosis/Kurtzke_Expanded_Disability_Status_Scale.asp>

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