In August 2020, three Parkinson’s organizations hosted a virtual summer symposium. We at Stanford Parkinson’s Community Outreach recently listened to the three talks and are sharing our notes. The notes here are from the “Staging Parkinson’s Disease”talk by neurologist Eleanor Orehek, MD of APDA Minnesota. She spoke of staging Parkinson’s disease (PD) to be able to identify where you are along the progression of the disease.
She mentioned these staging systems:
- Hoehn and Yahr, the most commonly used one which mainly takes into account motor symptoms,
- Braak Staging, which focuses on changes in the brain, and
- Unified Parkinson’s Disease Rating Scale, which is a modified Hoehn and Yahr scale that is more time intensive but takes into account more factors.
Dr. Orehek also offered early-stage disease management advice: exercise and having a comprehensive support system. In later stage management, she emphasized more exercise, as well as fulfilling more care needs and getting extra help. Her parting advice was to live in the moment and give yourself time to feel sad about what you have lost, but then focus on what you can gain.
(Dr. Orehek’s 40-minute session starts at the beginning of the recording.)
See extensive notes on the August 20, 2020 webinar below.
– Joëlle Kuehn
“Staging Parkinson’s Disease” Notes:
Speaker: Eleanor Orehek, MD, Medical Director, Noran Neurological Clinic, and American Parkinson Disease Association (APDA) Minnesota
Symposium Hosts: APDA Minnesota Chapter, Parkinson’s Foundation Minnesota and the Dakotas, Udall Center, University of Minnesota
Date: August 20, 2020
Summary by Joëlle Kuehn, Stanford Parkinson’s Community Outreach
Overview of the Stages of Parkinson’s Disease: This is to help gauge where you are and where you are headed. Tools — Scales/Staging Systems:
Hoehn and Yahr (H&Y)
- Developed by Melvin Yahr and Margaret Hoehn
- Published in 1967
- Most widely used scale due to its simplicity (just 5 stages)
- Interesting drawback is that it mainly takes into account motor symptoms, and doesn’t really take into account non-motor symptoms that often have a big or even bigger impact on someone’s quality of life and function.
- 5 stages: can get messy because it combines both functional disability and more objective physical impairments
- Scale: Increasing disability from 1->5, decreasing independence
- 1. Unilateral involvement only
- One-sided involvement
- It can be a problem if you are Stage 1 but your symptoms are severe (ex. Severe tremors or rigidity), you might be on the scale on an earlier stage but have move disability from that
- Note: It also doesn’t take into consideration that if someone has severe unilateral symptoms they rate lower than someone who has mild symptoms on both side (2.5 or a 3)
- 1.5: Unilateral and axial involvement
- 2: Bilateral involvement without impairment of balance
- Symptoms on both sides but still walking well and not having an impairment on balance
- 2.5: Mild bilateral disease with recovery on pull test
- 3: Mild to moderate bilateral disease; some postural instability; physically independent
- More instability with posture and might start needing to be or cautions when walking
- 4: Severe disability; still able to walk or stand unassisted
- Need a walker or other assisted device to move safely
- 5: Wheelchair bound or bedridden unless aided
- More dependent on 24/7 care
- 1. Unilateral involvement only
- Note: this happens over years into the disease and some people may never reach stage 5 depending on what else happens in life and other health factors
- Braak was a pathologist who published in 2003 and look at actual brains of people with parkinsons and evaluating where the changes in the brain were occurring at different stages of the disease
- Stages 1& 2: Autonomic and olfactory disturbances
- Minimal motor symptoms or no motor symptoms
- Changes in the brain tend to be lower down in the brain stem near the olfactory bulb and via the vagus nerve
- Affecting more areas that are involved in autonomic function (constipation type symptoms, affecting GI tract, loss of sense of smell – why early loss of smell can be a premotor symptom of the disease)
- Stages 3&4: Sleep and motor disturbances
- Where most people with Parkinsons would be diagnosed because that is where motor symptoms start, and non-motor symptoms such as stage REM sleep behavior disorder – act out dreams in sleep
- Changes in brain have spread into those areas involved in motor function
- Stages 5&6: Emotional and cognitive disturbances
- Affects thinking and memory and mood changes
- Had more spread of changes in the brain
Unified Parkinson’s Disease Rating Scale (UPDRS)
- Looks where someone is at with their disease
- Scale from 0-199
- Modified Hoehn and Yahr
- More time intensive but takes into account more aspects (non-motor evaluation and questionnaire that people with Parkinson’s or caregiver would fill out that assess:
- Looks at different disease aspects
- Thinking, memory, mood, behavior changes
- Functioning Activities of daily living like dressing, eating, handwriting
- Motor – tapping fingers and looking at muscle tone
- Complications of therapy – looking at do you get side-effects of medication, bothersome dyskinesias which are wiggly movements that happen from the levodopa
- 0 is no symptoms or disability, highest is 199
- Changes quite a bit dependent on medication – not as reliable when looking at someone in clinic because medication will have different numbers due to medication rather than disease progression; harder to gauge unless done in a controlled more research type setting
Parkinson’s is a really complex disease:
- There are many aspects that are important to take into account: motor symptoms, non-motor symptoms
- Also looking at biomarkers –
- markers that we can look at that give a better way to diagnose it but also track the disease progression
- Measurable markers of biological process or disease
- Spinal fluid, blood, imaging, blood pressure
- Current markets for PD: Validated rating scales, DaTscan
- Parkinson’s biomarkers research – lots of research in this area
- Looking for something that is easier to measure to make tracking less difficult and invasive, and make it more reliable and valid
- Real life impact of the disease:
- Motor symptoms and nonmotor symptoms
- Other medical problem
- Social support
- Other obligations (younger children, older parents you have to care for) external stressors (ex. Financial stressors)
Symptom tracking is important in seeing how they are managing disease and see how best they can help to help a person cope. Not everyone has the same symptoms and they are usually of different severity, so taking symptoms and severity of symptoms into account is important.
- Rigidity (Stiffness)
- Bradykinesia (slowness)
- Early or Mild stage
- Symptoms are mild, no or mild symptoms and functional impairments
- Still able to do things one enjoys doing
- Moderate symptoms
- Symptoms are more disabling, medications may have more side effects
- Transitioning from working to disability
- Advanced Stage:
- More disability, needing more significant help or care
Changes that happen as disease progresses in response to medication:
- Mild: Dopamine agonists, MAO inhibitors, LD/CT
- Medication can manage disease motor symptoms quite well
- Moderate: Oral LD/CD (frequent dosing), MAO inhibitors, Dopamine agonists
- Medication can cause more motor fluctuation, some symptoms can return when it fluctuates into the off state; other treatment options on top are helpful
- Severe/Advanced: LD/CD intra-duodenal infusion, deep brain stimulation
- Earlier in the disease:
- Anxiety and depression
- Sleep disorders
- Apathy – lack of motivation
- Later in the disease
- Sleep disorders
- Blood pressure issues
- Mood changes
- Other symptoms: some sensory changes
Early Stage Disease Management:
- Exercise! – most frequently recommended
- Fight apathy with a routine – strategies of getting in exercise, social interactions, doing things that are mentally stimulating
- Plan for the worst and hope for the best
- Have backup plans, plan for the future as best you can
- Advanced directive, power of attorneys
- Long term care
- Have a comprehensive support system:
- Care partner/s
- Physical Therapy, Occupational Therapy, Speech Therapy
- Mental health care to help with anxiety, depression and coping with the disease, having a psychologist for coping strategies, independent party that is not your family, friends, or care partner
- Primary Care
- Parkinson’s organizations: American Parkinson Disease Association, Parkinson’s Foundation
Later Stage Management
- More exercise (most important)
- Continue to build support network
- More care needs and help
- Evaluate living situation – home safety evaluation – things are safe around the home
- Live in the moment, unless you feel miserable in the moment
- Give yourself freedom to feel what you’re feeling and don;t feel guilty for feeling what you’re feeling
- It’s ok to grieve what you have lost, but then focus on what you can gain (new friends, hobbies)
- Give yourself time to feel and do different things
- Be flexible and adapt to different situations
Question and Answer:
Question: How often should you see a neurologist? Once a year or only when your symptoms change?
Answer: 2 – 3 times a year depending on how someone is doing; more frequent if there are a lot of medication changes
Question: What do you do if a family member of the person with Parkinson’s asks you to consider telling the patient to make some changes in driving safety – telling them not to drive.
Answer: Driving is a big sign of independence. If a family member is uncomfortable bringing it up to the loved one, you can mention it in an office visit. If there is a discrepancy between what the family member and person with Parkinson’s are saying, she might refer them for a driving assessment. Courage Kenny Rehabilitation Institute has occupational therapists that will do a road test and assess safety that doesn’t go through the DMV so it can still be a decision between family and doctor to decide what is safest.
Question: Recommendations for safe exercise for people in later stages of Parkinson’s?
Answer: If there are concerns about postural instability or fall risk, will want to do more seated exercises, check out “Sit and Be Fit” company (sitandbefit.org) with exercise videos. If you don’t have room for a full stationary exercise bike, they have floor pedallers that can be done sitting in a chair.
Question: What are you recommending for patients who are having breakthrough symptoms?
Answer: Breakthrough symptoms are off times when the symptoms of returning. There are a variety of medications we could try or change the timing or adding a medication, but it would need to be individualized.
Question: How do you handle freezing episodes?
Answer: Freezing episodes don’t often respond to medication, but there are a variety of different strategies that you can find on gait freezing information online or handouts. In particular, see:
- APDA Helpful Tips to Avoid Freezing: apdaparkinson.org/what-is-parkinsons/symptoms/freezing
- Parkinson’s Foundation information on Freezing: parkinson.org/Living-with-Parkinsons/Managing-Parkinsons/Activities-of-Daily-Living/Freezing
These handouts have tips for freezing. There are things like counting steps, marching instead of walking, being aware of which situations tend to cause you to freeze and others.
Question: When should we start finding our support system and getting things lined up?
Answer: The earlier the better. You can’t start too early getting good people in place to help manage things, even if you don’t need them a lot right now or if they say “things look good we don’t need to do much,” you have established that relationship and be aware of what’s out there.
Question: Do some people progress through Parkinson’s faster or are they diagnosed later? Or is it like a different disease such as Lewy Body Dementia specifically?
Answer: There are other diseases that look like Parkinson’s that progress more quickly, and that’s where it goes back to having better diagnostic tools and biomarkers to help differentiate between different types of diseases so that we have a better idea of how someone will progress.
Question: Since apathy is a non motor symptom, what do you recommend for people struggling with apathy?
Answer: We don’t have medication that could help with apathy unless it’s a kind of depression and we could try to treat that. Try on your own to come up with day to day routines and goals and priorities and have someone who can help hold you to that. If you are struggling with that or have a negative thought process, asking a psychologist or counselor to work with you on Cognitive Behavioral Therapy or strategies with a more regular check-in on how things are going and how your plans are going can make a big difference too.
Question: Does the appearance of certain nonmotor symptoms signal a progression to another stage of Parkinson’s?
Answer: Most nonmotor symptoms can occur at any stage, so it doesn’t help that much as far as staging. The main one that would indicate you are moving into a later stage would be cognitive changes to the point of dementia. If you’re having more cognitive changes and it’s impacting your ability to take care of some activities of daily living, that would indicate you are moving into those later stages.