Multiple System Atrophy and Cognition Webinar – Recording, Resources + Notes

Brain Support Network and Stanford University co-hosted a webinar last Monday, November 12th about Multiple System Atrophy (MSA) and cognition.

———————–

RECORDING

The webinar recording can be found here —

https://youtu.be/hr0TCWqBgUY

———————–

SURVEY

If you listen to the webinar recording, please take LESS THAN TWO MINUTES to answer six questions on our survey.  See:

https://www.surveymonkey.com/r/W23K9RW

———————–

FUTURE WEBINARS ON MSA

To be alerted to future webinars on MSA, join Brain Support Network’s MSA email list –
www.brainsupportnetwork.org/join

———————–

RESOURCES

For additional information on the topics addressed during the webinar, see:

Multiple system atrophy (including our list of Top Resources from around the world) – www.brainsupportnetwork.org/msa

Brain donation to support MSA researchwww.brainsupportnetwork.org/brain-donation

Lewy body dementia – www.brainsupportnetwork.org/lbd

Parkinson’s Disease (PD) – parkinsons.stanford.edu

Orthostatic hypotension –
parkinsons.stanford.edu/orthostatic_hypotension.html

Cognition in PD – parkinsons.stanford.edu/cognition.html

Psychosis in PD – parkinsons.stanford.edu/psychosis.html

Make an appointment with Dr. Poston or other movement disorder specialists at Stanford – Call 650-723-6469

———————–

SUMMARY

Our terrific volunteer, Denise Dagan, wrote a summary of the webinar:

Movement disorders specialist Kathleen Poston, MD discussed the diagnostic criteria for MSA with special attention on recent research showing memory and cognition problems can occur in MSA patients.

Here are some points Dr. Poston covered in this talk:

* Symptoms of MSA (now unofficially includes cognitive impairment).

* How to approach treatment of cognitive impairment based on how suddenly symptoms appear.

* How to identify if cognitive symptoms are associated with dopamine medications.

* Outline of six cognitive domains.

* In all variants of MSA executive cognition is affected, typically later in the disease process.

* The other five cognitive domains are affected to greater and lesser degrees, depending on the variant of MSA in question.

* Definition of psychosis.

* When sudden onset or worsening of hallucinations may occur, and how to treat them.

* When a diagnosis of MSA, Parkinson’s or Lewy body dementia (LBD) should be made — based on what symptoms present and the timing of those symptoms.

———————–

NOTES

Our terrific volunteer, Denise Dagan, took notes from the webinar.

Webinar
Multiple System Atrophy and Cognition

Speaker:  Kathleen Poston, MD, movement disorders specialist, Stanford University
Host: Candy Welch, former MSA caregiver, Brain Support Network

November 12, 2018

This webinar will address these topics:
• What are normal cognitive problems in multiple system atrophy (MSA)?
• Can “cognitive impairment” be part of MSA?
• Can “dementia” be part of MSA?
• Are there MSA symptoms that can mimic dementia?
• When should a diagnosis of “Lewy body dementia” be given?  Does it make a difference?
• What’s the treatment?

DR. POSTON’S PRESENTATION

Diagnostic Criteria for Multiple System Atrophy (MSA)
From: the Second Consensus Statement on the Diagnosis of MSA (Gilman, et. al., Neurology 2008)
Every person has early, significant autonomic dysfunction (blood pressure fluctuations, urinary frequency or incontinence, constipation, sweating, sexual dysfunction, etc.) PLUS
* Parkinsonism (slowness, stiffness, muscle rigidity) = MSA-P
OR
* Ataxia (poor coordination) = MSA-C

Autonomic Systems (reiterated) =
• Blood pressure and heart rate fluctuations (orthostatic hypotension)
• Urinary difficulty and incontinence
• Obstructive Sleep Apnea and/or RBD
• Difficulty with sexual functioning

Parkinsonism =
• Bradykinesia (slow movement)
• Rigidity (stiffness in the muscles)
• Tremor (rhythmic shaking)
• Balance and walking problems

Cerebellar Symptoms =
• Ataxia (poor coordination)
• Can be either with limb movement or with the trunk and neck movements
• Speech problems
• Swallowing problems

Traditionally the diagnosis of ‘dementia’ has been considered a red flag that the diagnosis is not MSA.  However, it has recently been recognized that memory and cognition problems can happen in patients with MSA
2008 currently being considered for revision.

Motor versus Non-Motor Symptoms:
Classic Motor Symptoms =
• Ataxia
• Bradykinesia, Tremor, Rigidity

Mixed Motor/Non-Motor Symptoms =
• Hypophonia (soft speech) and Dysarthria (difficult or unclear articulation of speech)
• Gait and Balance Problems

Non-Motor Symptoms =
• Mild Cognitive Impairment and Dementia (rarely dementia)
• Psychosis (hallucinations and delusions)
• Sleep disorders (RBD and OSA)
• Depression/Apathy, Anxiety, Fatigue
• Constipation, Orthostatic hypotension, Urinary frequency, urgency, incontinence

Types of Cognitive Impairment:
1. Sudden
2. Intermittent (off and on)
3. Slowly progressive

1. Sudden Change in Cognition
* Most common reason for a sudden onset or change in symptoms, including cognition, is general medical illness or other medications
– Infection (such as a UTI or a cold/flu – elderly & those with neurodegenerative disorder may not run a fever or feel pain urinating)
– Medications (for pain, urinary frequency)
– Physical stress (constipation, poor sleep, travel, new physical environment)
– Emotional stress (anxiety)
– Being in the hospital (all of the above)

How to treat a Sudden Change
* Deal with the underlying cause (prior slide)
* Reduce or stop certain medications:
– Artane (any other anticholinergics)
– Amantadine
– Dopamine agonists
– Sinemet CR (carbidopa/levodopa CR)
– Comtan (entacapone)

2. Intermittent Cognitive Impairment
* Sometimes called fluctuations in cognition
* Can be associated with level of alertness throughout the day
– Determine whether cognitive impairment is related to dopamine medication by keeping a daily diary of medication dosages and times, symptom timing and duration, and meals.

Scenario 1: Non-motor symptom improves with dopamine
7:00a – Take 1, 25/100 Sinemet (carbidopa/levodopa)
9:00a – Symptom A starts
10:00a – Take 1, 25/100 Sinemet : Symptom A continues
10:30a – Symptom A ends
12:00p – Symptom A starts again
1:00p – Take 1, 25/100 Sinemet ; Symptom A continues
1:30p – Symptom A ends

Scenario 2: Non-motor symptom worsens with dopamine
7:00a – Take 1, 25/100 Sinemet
7:30a – Symptom A starts
8:00a – Symptom A ends
10:00a – Take 1, 25/100 Sinemet
10:30a – Symptom A starts
11:00a – Symptom A ends
1:00p – Take 1, 25/100 Sinemet
1:30p – Symptom A starts
2:00p – Symptom A ends

Scenario 3: Non-motor symptom is independent of dopamine, but related to meals.  This happens especially in orthostatic hypotension (fluctuating blood pressure).  Cognition can be reduced by low blood pressure.  A home blood pressure monitor can help determine if this is the situation.
7:00a – Take 1, 25/100 Sinemet
7:30a – Symptom A starts (Breakfast)
8:30a – Symptom A ends
10:00a – Take 1, 25/100 Sinemet
12:00p – Symptom A starts (Lunch)
1:00p – Take 1, 25/100 Sinemet
2:00p – Symptom A ends

3. Slowly progressive change in Cognition
* More likely to be part of the disease process

Cognitive Domains:
Each controlled by different parts of the brain.  Different parts of the brain are affected by pathology of different neurodegenerative disorders.
* Memory
* Processing speed
* Attention and working memory
* Executive function
* Visuospatial function
* Language

Memory:
* Example: On your way out the door your spouse asks you:  “Can you pick up some milk while you are at the store?”
* The process of memory includes:
– Encoding = storing information properly
– Maintenance = keeping it in storage long enough to be useful
– Retrieval = recalling the information when it is needed

Processing Speed:
Time it takes to absorb new information and come up with a response.

Attention and Working Memory
Example: A friend tells you a 10 digit phone number – you keep it in your mind for about 30 seconds while you look for a pen and paper to write it down or enter it into contacts on your phone.

Executive Function:
* The cognitive processes that dictate flexible and dynamic adjustment of performance in response to a changing environment.
* Examples:
– Planning/organizing
– Complex or novel problem-solving
– Shifting attention, keeping track of, or alternating from 1 task to another
– Not being susceptible to distractions
– Generating fluent sequence of thought or words
– Learning the rules of a new task (example: programming new DVR) without direction or explicit cueing

Visuospatial Function:
[Slide showing 4 complex geometric drawings and various trapezoids, rectangles and parallelograms in the center of each.]
Given an isolated parallelogram, an impaired subject would have difficulty finding a parallelogram of the same shape within the complex geometric drawings.
* Causes problems with parking the car or clipping corners when driving
* Causes problems with directions somewhere that is not familiar and getting turned around

Language:
* Names of objects and people
* Problems can be simple:  ‘Tip of the tongue’
* Problems can be more severe:  You can’t remember the name of something you use regularly.

What is dementia?
* Comment: Alzheimer’s is the most commonly diagnosed type of dementia, but not the only one – by far.
* A person who can no longer do an activity of daily living because of a cognitive impairment has dementia.
* Examples:
– Can no longer pay the bills correctly
– Gets lost when driving
– Cannot shop for groceries

[Slide showing study results, “Cognitive Impairment in MSA: A Position statement by the Neuropsychology Task Force of the MDS Multiple System Atrophy (MODIMSA) Study Group,” Stankovic, et. al., Movement Disorders, vol. 29, no. 7, 2014.]

Autopsy verified diagnosis.
Table 1. Impaired cognitive functions in:
MSA (multiple system atrophy)
MSA-P (multiple system atrophy, parkinsonian variant)
MSA-C (multiple system atrophy, cerebellar variant)

MSA P+C
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous recall (immediate & delayed), recognition, visuospatial functions

MSA-P
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous immediate recall, visuospatial functions

MSA-C
Often impaired:  Executive cognition
Sometimes impaired: Attention and working memory, spontaneous delayed recall, recognition, visuospatial functions]

[Slide results from same study:]
* Prevalence of rates of cognitive impairment in autopsy confirmed MSA:
– 22% Mild Cognitive Impairment,
– 2% Moderate Cognitive Impairment,
– 0.5% Severe Cognitive Impairment
* 50% of those MSA patients with more than 8 years after diagnosis had some Cognitive Impairment
– Cognitive impairment seems to happen most frequently late in the disease process
* 14% of MSA patients had dementia in the last year before death
* 9% had well-formed visual hallucinations (one study)

[Slide showing study results, “Profile of Cognitive Impairment and Underlying Pathology in MSA,” Koga, et. al., Movement Disorders, vol. 32, no. 3, 2017.  Autopsy verified diagnosis.]

Table 2. Comparison of demographic and clinical features between 102 patients:
MSA-CI (multiple system atrophy with cognitive impairment) 33 patients
MSA-NC (multiple system atrophy without cognitive impairment) 69 patients

Data collected:
– Females with and without cognitive impairment
– Age at onset, in years
– Age at death, in years
– Disease duration
– Family history of dementia
– Family history of parkinsonism
– Having clinical diagnosis of MSA.  Only 70% accurately diagnosed.
– Clinical phenotype (both Parkinson’s and Cerebellar)
– Clinical symptoms of depression]

[Slide showing Characteristics of cognitive impairment in same 33 patients with MSA and cognitive impairment.  11 of the 33 had other pathologies that may have contributed to cognitive impairment, in addition to MSA pathology.]

What is meant by ‘psychosis’?
* Illusions – Misinterpretations of real stimuli. Can be unformed, such as a sense of presence or a fleeting false impression.  Example: see a jacket on a chair and misinterpret that it is a cat.
* Hallucinations – Spontaneous, fully formed. Most often visual, but can be auditory, tactile, olfactory (smell), or gustatory (taste).  Example: seeing there is a cat when there is not a cat.  With insight the person knows the hallucinations are not real.  Without insight they cannot know the hallucinations are not real; at this point, consider medication intervention.
* Delusions – False thinking. Can be paranoia.  Also, consider medication intervention.

Motor Symptoms – Sometimes Improved by dopamine medications
BUT
Hallucinations – Can be worsened by dopamine medications

Sudden Onset or Worsening of Hallucinations (Same as any worsening symptoms, including cognition.)
* Most common reason for a sudden onset or change in symptoms is general medical illness or other medications
– Infection (such as a UTI or a cold/flu)
– Medications (for pain, urinary frequency)
– Physical stress (constipation, poor sleep, travel, new physical environment)
– Emotional stress (anxiety)
– Being in the hospital (all of the above)

How to treat a Sudden Change
1. Deal with the underlying cause (prior slide)
2. Reduce or stop certain medications:
* Artane (any other anticholinergics)
* Amantadine
* Dopamine agonists
* Sinemet CR (carbidopa/levodopa CR)
* Comtan (entacapone)

What is the difference between MSA, Parkinson’s disease and Lewy body Dementia (LBD)?
Clinically:
Cognition = Memory and Hallucinations AND
Motor = Ataxia, Bradykinesia, Rigidity and Tremor WORSEN OVER TIME

Pathologically (only visible at autopsy):
[Slide showing Lewy body Pathology (image) in Both PD & LBD
Glial Cytoplasmic Inclusion Pathology (image) in MSA
Both Lewy Bodies and GCIs are make of up of the protein a-synuclein]

As is currently defined, all 3 would have autonomic dysfunction:
* The Diagnosis of PD with cognitive impairment or Dementia should be made when memory problems develops within the context of established PD.
* The Diagnosis of LBD is appropriate when the diagnosis of Dementia precedes or coincides within 1 year of the development of motor symptoms (parkinsonism).
* The Diagnosis of MSA is appropriate when the there is primary Autonomic Dysfunction, along with either parkinsonism or ataxia (with, or without memory problems).

Please take a 20 question online survey about MSA diagnosis and symptoms!!!
http://med.stanford.edu/poston-lab/msa-survey.html

QUESTION AND ANSWER
Lots of questions were received in advance.

Q. When will the diagnostic criteria be updated?
A. It is in the works.  So, probably the next year and a half.

Q. Why aren’t MDs able to determine if someone has MSA or LBD?
A. Even though in pathology the Lewy Bodies look different from the GCIs, there is no way to image them through an MRI, etc.

Q. Are there any specific assessments or screenings that you recommend for evaluating and monitoring an MSA patient’s cognitive status?
A. The screening a doctor would do during an office visit.  The Montreal Cognitive Assessment covers all the cognitive domains.  The problem is that is it fast (30 questions) so not detailed.  A neuropsychological battery, 2-3 hours of testing requires a PhD, clinical psychologist, to administer.

Q. Connection between NPH (normal pressure hydrocephalus) and MSA.
A. NPH can cause spaces in the brain to enlarge and press against other areas of the brain affecting urinary, cognition and gait.  The symptoms manifest differently from MSA and can be seen in an MRI.  That is one way to identify those with NPH and exclude MSA.

Q. What is sleep’s role in cognition?
A. A bad night sleep affects processing speed, executive function so address sleep apnea, have a nap, monitor blood pressure and minimize BP fluctuation to maximize cognitive function.

Q. How are executive functions affected in MSA?
A. This can come across as difficulty with multi-tasking because that requires a very high level of cognitive function.  Figuring out what to do in the right order is required.  When someone has trouble making a to-do list, there are some executive function issues.

Q. What is fatigue’s role in cognition?
A. Fatigue is very involved in blood pressure.  There is a lot of fatigue fundamental to each of these disorders.  Fatigue gets some improvement with napping or resting where blood pressure and dementia does not.

Q. How common are hallucinations in MSA?  Can there be hallucinations without Lewy body dementia?
A. Yes, recent studies have convinced clinicians.  That wasn’t known 10 years ago and Dr. Postonhas seen it in her clinic.

Q. How does dementia in MSA differ from Lewy body dementia?  Parkinson’s disease dementia?
A. It can be pretty darned similar.  The dementia, particularly the executive function issues, which is why the neuropsychological testing doesn’t differentiate between these.

Q. How does the cognitive impairment in MSA differ from that seen in Progressive supranuclear palsy (PSP)?
A. Patients with PSP can have a lot of executive function difficulty as well, but they often have poor judgement, even though they know their balance is terrible.  This can happen in other neurodegenerative disorders, but is more common in PSP,

Q. My husband is now very susceptible to TV ads and asks me to order products we do not need.  Is this executive function impairment?
A. Impulsivity can be part of the underlying disorder but can be made worse with dopamine agonists (Requip, Mirapex, Nupro patch).

Q. My husband is slow to respond, slow to make decisions, and has “on-and-off” periods.  Is this dementia?
A. It depends on the degree to which the on/off periods and indecision interfere with ability to complete activities of daily living.  On/off could be from medications or blood pressure fluctuations, so its hard to tell.

Q. Could MSA-related cognitive impairment be mistaken for vascular-related mild cognitive impairment?
A. The only study addressing that is the one showed where 4-5 had vascular disease found at autopsy that could have contributed to cognitive impairment.  It is impossible to tease out how much each contributes how much to cognitive impairment.

Q. How can you tell the difference between orthostatic hypotension and cognitive impairment?
A. The most common place you see this is when blood pressure stops and processing speed slows so much the person cannot answer questions. Blood pressure should be done during these episodes.  The tilt table test during cognitive testing can reveal whether the person has cognitive changes associated with blood pressure changes.

Q. In our local support group, people with MSA are taking Alzheimer’s medications such as Aricept and Exelon.  Does this mean they have dementia?  Lewy body dementia?
A. As far as medications to treat cognition, Alzheimer’s is the only disease FDA approved to treat.  Aricept, Exelon and Namenda are the most common.  These are not FDA approved for any other cognitive impairments.  The best data is behind Aricept and Exelon for Alzheimer’s and LBD.  They do help a little bit, but they can help for other cognitive and memory issues.

Q. How is the care of a person with MSA different from someone with dementia?  Or different from someone with other
neurological diseases (such as PSP or CBD)?
A. Longitudinal studies show the care of any two patients with MSA is completely different.  Care must be specifically tailored to each person.  One may have severe urinary issues and another not.  This is true of all the neurodegenerative disorders.

Q. If someone with MSA has cognitive issues, is his/her prognosis (survival time) any different?
A. Chance of having cognitive problems is most likely if they live 8+ years after diagnosis.  Once someone has a diagnosis of Parkinson’s disease with dementia their prognosis is shortened.

Q. How can medications affect cognition?
Q. If opiates can affect cognition and cause hallucinations, what kind of pain reliever can I give to my mother with pain?
A. Anything that treats pain dulls all neurons in the brain, blocking the sensation of pain.  Therefore simultaneously slows cognition, worsens hallucinations, etc.  Start with over the counter pain killers.  If you have to go to prescriptions discuss with doctor about what to try and start with lowest dose possible.

Q. Can cognitive problems be prevented in MSA?
A. We don’t understand what causes cognitive problems so we don’t have a way of preventing them.

Q. How can caregivers respond to someone with MSA and cognitive problems?
A. It is difficult because patients can get frustrated.  Patients do improve with a certain amount of cueing, reminding, etc.  Unfortunately, spouses call this nagging.  Try to know what the problem is by getting neuropsychologist testing and refer back to the test or have the doctor tell him/her what they have difficulty with.

Q.Do people with MSA not live long enough for dementia to show up?
A. No.

Q. Can people with MSA *also* have Alzheimer’s disease?
A. Yes.

Q. Can people with MSA *also* have Lewy body dementia?
A. Yes, but very rare.

Q. When do we determine when the patient is unable to work because decisions are cloudy or cognition is impaired?
A. Depends on the job and flexibility the employer has to move employee into a different role and relationship between the two.  Most of the time once executive functioning is impacted, it is difficult to be employed because decisions are too hard.

Q. Once the patient is non verbal and unable to move on his/her own accord, is this considered a disability automatically?
A. In filling out disability paperwork people qualify long before someone is nonverbal or unable to move.

Driving
Q. When do we determine when the patient is unable to drive because cognition is impaired?
A. This varies by state.  In CA if someone has dementia the physician is required by law to report them to the DMV for evaluation.  Occupational therapists can do an assessment, even with a driving simulator, to see what concerns might be, especially visuospatial changes.

Q. Importance of brain donation to MSA research.
A. Critical.  Without autopsy diagnosis confirmation we would still be guessing.  Before research based on brain donations we used to think MSA was 3 different disorders.  We can only continue research into MSA and other neurodegenerative disorders with continued access to brain tissue donated for research purposes.

“Caring for Someone with Lewy Body Dementia” – Live Chat Notes

Recently, Home Instead Senior Care hosted a conversation with Angela Taylor, Director of Programs for the Lewy Body Dementia Association (lbda.org). She provided a brief summary of what distinguishes Lewy Body Dementia (LBD) from some other dementias, followed by a lengthy question and answer period.

Lewy Body Dementia (LBD) includes both:
Dementia with Lewy Bodies (DLB) and
Parkinson’s Disease Dementia (PDD)

Treatment and behavior management is the same for both.

According to the research criteria, someone is diagnosed with DLB when both cognitive changes (hallucinations and fluctuating cognition) and parkinsonian symptoms (gait and balance difficulty and/or tremor) appear at nearly the same time.

And someone is diagnosed with PDD the cognitive changes appear more than a year after parkinsonian symptoms or a Parkinson’s diagnosis.

Brain Support Network uber-volunteer Denise Dagan recently listened to the webinar, and shares her notes below.

For additional resources on LBD, check out Brain Support Network’s list of Top Resources for LBD.

Robin

================================

Caring for Someone with Lewy Body Dementia
Home Instead Senior Care
June 7, 2018 Webinar

Live Chat with Angela Taylor of the Lewy Body Dementia Association

Lewy Body Dementia (LBD) is most common type of dementia after Alzheimer’s.  Each type of dementia has different caregiving challenges.

Dementia is an umbrella term which is a change in a person’s cognitive skills that interferes with daily functioning. There are over 100 causes of dementia. Some are treatable, like hydrocephalus. Most are progressive.

LBD is the most misdiagnosed form of dementia and affects 1.4 million Americans. It is a disease of older age (50+).  LBD affects more than just cognitive skills. Biologically, there are the same changes as those with Parkinson’s Disease.

Changes that affect movement. Those changes are called parkinsonism because they resemble Parkinson’s Disease.

Cognitive changes are not so much memory initially, but in planning, paying attention, understanding how things relate in 3D, fluctuations in their cognition even from hour to hour.

Changes in mood and behavior commonly seen in mental health disorders, like hallucinations (commonly people, animals or children) and delusions (when you think something is true, even with no facts to support the belief), depression and anxiety.

REM sleep behavior disorder – physically acting out dreams (hit, shout, kick, fall out of bed), or insomnia, restless leg, sleep apnea

Changes in autonomic nervous system, including digestion, constipation, low blood pressure.

Q&A

Q. How to best deal with hallucinations?

A. Up to 80% of people will have hallucinations. Sometimes, it is the first symptoms families notice. It can be frightening for the family, even if the subject of the hallucination is not frightening to the person with LBD. Hallucinations are a huge indicator that you are dealing with LBD as it is not a symptom that appears early in PD.

Caregiver should listen with your heart before listening with your brain. Respond to what your family member is feeling because you cannot convince them they are having a very real hallucination. First comfort their emotional state. Enter their reality. Engage in ‘therapeutic lies’ to help them deal with what they are experiencing. Try moving them to another location/room to change their visual experience. Turn off TV. Remove mirrors.

Q. How do you convince relatives who only see LBD symptoms on a person’s good days that there really are challenges?

A. People with LBD can really rise to the occasion when they are visiting with someone they are excited to see. The next day they are depleted and their Sx are all worse. If you want a relative to see the aftermath, you really need to ask them to visit for a few days to see the fluctuations in person. Provide the relative with educational material or forward them web resources links explaining LBD symptoms.  Sometimes, the relative is resistant due to fear and grief that they don’t want to believe the person with LBD is declining so dramatically.

Q. How to best deal with delusions?

A. Every situation is unique due to individual experience. Angela’s father became convinced his wallet was stolen. Capgras syndrome = someone believes a person in their household has been replaced by an imposter. Respond that you will fetch the person who has been replaced, leave for a few minutes and return. Often someone with LBD will not see their home as being their home. Therapeutic lies to calm fears or tell them what they believe is going on here is not happening where they live, then relocate them.

For both hallucinations and delusions you may have to try a few different calming comments before finding one they can buy into. As long as you remain calm and not escalate the situation, you will eventually be successful.

Q. Are there treatments for LBD or detrimental treatments for mis-diagnosis of LBD?

A. LBD has a lot of symptoms. There are medications that help many of those Sx. We can’t make dementia go away, but we can improve quality of life for both the patient and family caregiver. You really need a specialist for this because when you treat one area, you may make another worse. Treating autonomic symptoms nearly any doctor can do. Speech therapy can help as those muscles are affected. Physical therapy can help to counter stiffness and rigidity of parkinsonism rather than medications.
Those that treat cognitive skills (Nameda, Exelon) can improve hallucinations, anxiety, etc.  Treating movement symptoms medications can worsen hallucinations in LBD. May not treat minimal movement symptoms.  Treating behavior and mood symptoms (depression and anxiety) can improve quality of life and make them cooperative to care.
Treating hallucinations & delusions medications can worsen parkinsonism symptoms.

5 areas of symptoms in LBD:
Thinking
Movement
Behavior/Mood
Sleep
Autonomic dysfunction

LBDA recently added 24 major medical institutions as centers of excellence for LBD treatment. Increases opportunities for families to participate in research trials.

Q. Importance of early diagnosis, but how?

A. Start with your primary care physician. They should rule out underlying medical conditions. If they can’t figure it out they should refer to a neurologist. 2/3 of accurate diagnosis are done by neurologists, neuropsychologist, geriatricians. To diagnose, they may do a brain scan, cognitive evaluation or other tests to narrow the diagnosis.

Q. Family member with LBD symptoms include jerking of the arms and hands as well as staring unresponsively.

A. Staring into space is a manifestation of fluctuating cognition or orthostatic hypotension (low blood pressure). Parkisonism symptoms include masked face, stiffness, tremor. Jerking can be caused by medications used to treat parkinsonism or may be a symptoms of LBD. You should have a doctor see her.

Q. How quickly do motor skills decline? Do some symptoms present before others?

A. LBD is an umbrella term. There are 2 specific diagnoses.  Patients w/PD that then develop LBD have PDD. They have the same Lewy bodies in their brain but heir earliest problems are motor. DLB initially has cognitive issues, and later develop motor symptoms. Each individual will have varying degrees of intensity of symptoms between the 5 areas of symptoms in LBD.

If something has changed suddenly it is important to take your family member to see a doctor. It could be a medical issue that can be treated, but if it is physical changes it could be transition into a later stage of the disease. Only a doctor can determine this distinction. If so, it could be time to call in hospice to support the family.

Q. What should I look for in final stages of LBD and how to choose a good hospice?

A. There are no formal stages of LBD. Late stage is characteristic of being unable to care for themselves in all areas of daily living. Tell your Dr. if your family member having trouble swallowing so they can help you put services in place to support the patient and family, including hospice, speech therapy, feeding specialist, etc. Tell your Dr. if you think your family member may be in pain. Physical therapy & occupational therapy can bring in a hospital bed, special padding, etc. to make them more comfortable. Engage hospice and in-home care as early as possible to support the family so the family can do less hands-on care and focus more on bringing in experiences your family member will enjoy (music, art, movies, reading aloud, animals, family, etc.)

Q. How to deal w/LBD family member who has lost involvement in life?

A. LBDA has a private Facebook group so you can see what others are dealing with and share creative ways to cope. Talk to your Dr. about your family member’s change of activity and motivation. If it is depression it can be treated. Bring your family member’s passions to them or take them to their passions (Example: golf – can go putting, watch friends/fellow golfers at the driving range or a tournament, even watch a tournament on TV). Keep people socially stimulated, physically active and feeling they contribute value to society in addition to emotional support.

Dementia Resources:

– HelpForAlzheimersFamilies.com

– Facebook.com/RememberForAlzheimers

– HomeInstead.com

– LBDA Research Centers of Excellence: www.lbda.org/rcoe

– An Introduction to LBD Booklet: www.lbda.org/content/intro-to-lbd

– LBD Symptoms Checklist: www.lbda.org/go/comprehensive-lbd-symptoms-checklist

– LBD: State of the Science: www.lbda.org/go/lbd-state-science

 

Australian pop duo The Veronicas – mother with LBD and PSP

Australian pop duo The Veronicas were recently named ambassadors for Dementia Australia, after putting their music on hold last year to spend more time with their mother.  After four years of misdiagnoses, their mother was diagnosed with Lewy Body Dementia (LBD) and progressive supranuclear palsy (PSP). Their goal is to use their platform and their mother’s story to break down the stigma and shame surrounding dementia, and to build a social movement to advance community awareness.

Check out the article about The Veronicas here:

www.dailymail.co.uk/tvshowbiz/article-5892653/The-Veronicas-discuss-helping-mother-cope-dementia.html

Cancelled – “What If It’s NOT Parkinson’s?” Webinar on Tuesday, June 12th

Update on 6/11/18:  Unfortunately this webinar is now cancelled.

Brain Support Network group member Steven Russell shared with me today info on an upcoming webinar, which will give an overview of all four of the atypical parkinsonism disorders — MSA, LBD, PSP, and CBD.  Details are:

What If It’s NOT Parkinson’s?

In this webinar, Dr. Mihaela Alexander, Colorado Neurodiagnostics, will discuss atypical parkinsonisms such as multiple system atrophy and Lewy body dementia, among others (PSP, CBD). Dr. Alexander will cover what we know about these conditions and what treatments are available for each condition.

Tuesday, June 12
10-11am CA time

Organizer:
Parkinson Association of the Rockies, Denver, parkinsonrockies.org

Registration:
attendee.gotowebinar.com/register/3367947443153312002

General Information:  (same as what is above)
parkinsonrockies.org/programs-services/event-calendar/276/2018-06-12/what-if-it-s-not-parkinson-s-webinar

 

“Lewy Body Rollercoaster” – Alzheimer’s Weekly

Here’s an excerpt from an Alzheimer’s Weekly article from April 12, 2014 about Lewy body dementia (LBD):

www.alzheimersweekly.com/2014/07/lewy-body-rollercoaster.html

Excerpts from

Lewy Body Rollercoaster
April 12, 2014
Alzheimer’s Weekly

Attention, alertness and cognition have dramatic fluctuations in Lewy Body dementia. Caregivers call these ups and downs “The Roller-Coaster of LBD.”

“I watched my husband experience a decline in cognition followed by a period of what seemed like improved function only to plunge again into confusion with more frequent hallucinations,” says one caregiver newly acquainted with Lewy body dementia (LBD). According to the Lewy Body Dementia Association (LBDA), these ups and downs in function are sometimes refer to by family caregivers as the “roller-coaster effect” of LBD. Fluctuating levels of cognitive ability, attention and alertness are one of the core features of LBD.

Important Diagnosis
“The combination of the motor signs of Parkinson’s disease (slowed mobility, stooped posture and tremor) and mental confusion, especially if the degree of confusion fluctuates day to day, should raise a red flag for suspicion of LBD,” says Howard I. Hurtig, M.D., Chair, Department of Neurology, Pennsylvania Hospital, and Elliott Professor of Neurology, Perelman School of Medicine, University of Pennsylvania. “If those symptoms are accompanied or preceded by REM sleep behavior disorder (vivid dreams, dream enactment, hitting bed partner, falling out of bed) and visual hallucinations (seeing people, animals, etc., that aren’t truly there), then the diagnosis of LBD is almost certain. Even when all symptoms of LBD are present, it is critical for the evaluating doctor to look for underlying, TREATABLE causes of dementia.” A thorough assessment will include an inventory of daily medications (drugs can produce a “chemical” state of pseudodementia), CT or MRI scan of the brain, blood tests for thyroid function and vitamin deficiency and other causes of dementia that can be identified by routine study.

Following Alzheimer’s disease, LBD is the most misdiagnosed form of dementia but the second most common cause of progressive dementia, affecting 1.3 million Americans. LBD is associated with abnormal protein deposits in the brain, called Lewy bodies, that impair thinking, movement, sleep and behavior (causing people to see hallucinations or act out dreams, sometimes violently). Also, it affects autonomic body functions, such as blood pressure control, temperature regulation, and digestion. Recognizing symptoms early can help people with LBD get comprehensive and appropriate treatment and help caregivers get much needed support. It’s difficult to diagnose LBD, because its early symptoms resemble symptoms found in Alzheimer’s and Parkinson’s disease.

Man found joy in completing puzzles that were family photos

This article from last year is about twin daughters who discovered a company that created puzzles from family photographs, and that their father with Lewy body dementia (LBD) really enjoyed putting together these puzzles.

This activity might be of interest to more than those with dementia.

www.brainandlife.org/the-magazine/article/app/13/1/23/puzzle-power-as-lewy-body-dementia-narrowed-their-fathers-world

Congratulations to “Sue’s Story”

Congratulations to “Sue’s Story” — winner of the “Audience Choice Award” for the best documentary at last weekend’s Jasper Poppy International Film Festival in Morgan Hill, California. When Sue Berghoff was diagnosed with Lewy Body Dementia (LBD), her life was turned upside down. Like most people, she had never heard of the disease even though it is the second most prevalent form of dementia after Alzheimer’s. She encountered a society in which the existence of dementia is burdened by misconceptions, fear and shame. What she did next created a ripple effect of hope and change. Sue’s husband Chuck Berghoff is a member of Brain Support Network’s  LBD caregiver support group. Brain Support Network’s CEO Robin Riddle appears in the documentary, speaking about the importance of brain donation for LBD and the value of a caregiver support group. Stay tuned to Brain Support Network for more news about the distribution of “Sue’s Story.”

Capgras syndrome – almost 20% of people with LBD have

This is a sad but helpful article on Capgras syndrome, a specific type of delusion where a person believes that loved ones have been replaced by identical duplicates. They often believe that the loved one has been kidnapped and the “imposter” is a bad person. This is very common within our local Lewy body dementia support group. According to the article, one report showed a prevalence of 16.6 percent of those with LBD have Capgras syndrome.

Here’s a link to the article in today’s Washington Post:

 

www.washingtonpost.com/national/health-science/this-strange-syndrome-causes-people-to-think-their-loved-ones-have-been-replaced-by-identical-impostors/2018/04/06/0091f168-1be6-11e8-9de1-147dd2df3829_story.html

Health & Science
This strange syndrome causes people to think their loved ones have been replaced by identical impostors
Washington Post
By Meeri Kim
April 7, 2018

Robin

 

Anosognosia – lack of awareness (not denial) of one’s own dementia

This short article from Next Avenue (nextavenue.org) is about anosognosia, or the lack of awareness of one’s own dementia.  This is not denial but being unaware.  “This lack of awareness can cause major stress and heartache for caregivers.”

Here’s a short excerpt:

Both of Kathy Kling’s parents, who are divorced, have Alzheimer’s. Kling recently talked with her mother, Karen Kelly, about her father’s disease. “Oh, I hope I never get it,” her mother replied.  She was diagnosed six years ago.

The full article is here:

www.nextavenue.org/parent-doesnt-recognize-dementia/

When Your Parent Doesn’t Know He Has Dementia
It’s a common aspect of the disorder, but tough on caregivers
By Emily Gurnon, Health & Caregiving Editor
Next Avenue
March 28, 2018

Robin

 

“Choose Your Attitude for the Journey” (empowering caregivers for the long journey)

Though this handout is for a dementia caregiving class by a woman whose husband had Lewy body dementia, I think this resource applies to all caregivers. In the few places below where you see “LBD,” replace that term with the disorder with which you are coping (if it’s not LBD).  See what you think….

Online friend Pat Snyder’s husband John died with Lewy body dementia in November 2015. She is the author of a wonderful book for those coping with the early stages of LBD titled “Treasures in the Darkness: Extending the Early Stage of LBD” (available through Amazon.com). Pat generously gave us a copy, which is circulating among local support group members.

Pat is teaching a dementia caregiver class in Wake Forest, NC. Her mission is to teach as many “rubber meets the road” tools and techniques as she can, in a context of preserving personhood for the patient while educating, encouraging, and empowering the caregiver.

Pat recently shared the notes from the first lesson of this caregiving class. She recommends five initial steps that all caregivers take at the beginning of their caregiving journey so that they can be strong for the duration of that journey. The five steps are:

• Be positive
• Be proactive
• Be perceptive
• Be persistent
• Personify the disease

The lesson goes through these five points. Pat gave permission for me to share her lesson notes here.

Robin
————————-

“Choose Your Attitude for the Journey” (Lesson 1**)
from Dementia Caregiver Class in Wake Forest, NC
by Pat Snyder ([email protected])
February 2018

There are five first steps that I recommend for you to take as you begin your dementia caregiving journey. These approaches will strengthen you in that role for the duration:

• Be positive
• Be proactive
• Be perceptive
• Be persistent
• Personify the disease

Be positive.

After your Loved One’s diagnosis of dementia you are likely to be overwhelmed with negative emotions. These emotions are your first hurdle to overcome, and they tend to be ongoing.

Therefore, a commitment to use positive choices to overcome negative feelings is a technique that will serve you well throughout your time as a caregiver.

Using a positive attitude to overcome a negative situation is not denial of it but a conscious effort on your part to override its impact. There are a number of ways to address this choice of being positive. It takes self-discipline and commitment to fight back an enemy that is trying to steal precious moments from your lives. It often involves doing the opposite of what you are feeling in the moment. So first, identify that negative feeling. Then choose to go in another direction. For example, if you feel sad, try to spend time with a friend who makes you laugh. If you feel empty, make a list of three things for which you are grateful.

These kinds of choices may feel simplistic and artificial when you first start to practice them. Over time, you will feel the benefit of not allowing the negative emotions to swamp you, keeping you in a slump of inactivity and helplessness. You will begin to feel empowered and purposeful in your role. Then you will realize that your positive choices have a profound impact on your Loved One’s experience of dementia.

Be proactive.

You need to take action and not just react to what happens. Being proactive is an ongoing requirement. As you begin to assume your role, these actions will empower you:

• Learn about dementia symptoms.
• Learn about treatments for LBD symptoms.
• Learn about non-pharmacological (non-drug) interventions.
• Learn to identify the specific triggers that affect your Loved One’s experience of LBD.
• Find the right doctor who knows how to treat LBD
• Connect with other LBD caregivers to continue strengthening yourself.

When your Loved One received the diagnosis, you likely also received some printed materials that defined LBD and directed you to some helpful resources available in your community. However, you will need to seek out more in-depth knowledge in order to fulfill your role as the caregiver.

Do not overwhelm yourself in the beginning. Learn about the basics and gradually add more knowledge. It is important to get information from reliable sources. Use only trusted print and non-print resources. Look at the websites of government agencies, universities, hospitals, and associations like LBDA. Find medical journals, articles, and books written by experts and online support groups that offer reliable information and social support. Search for the right doctor who specializes in LBD care or who is willing to learn about proper treatment for this challenging and complex disease. Do not settle for one who does not respect you as the key member of the care team.

Be perceptive.

Be sensitive to the emotional and psychological impacts that dementia can have on you and your family. If left unaddressed, these things can have devastating physical consequences for you and your Loved One. They may determine how gentle the journey is for all of you. The diagnosis is likely to magnify any pre-illness emotional and interpersonal issues. Therefore, it is important to clearly identify those and learn how to manage them better. Doing this could provide significantly better outcomes for everyone. Consider including a counselor as a member of your health care team to help you address communication issues that need attention, relationship dynamics, and any pre-illness issues that can affect how you manage the symptoms of LBD, forgiveness of past hurts, and grief issues.

A second element of being perceptive is to be sensitive to your Loved One’s symptoms. You see him every day, unlike doctors who see him only for a short time during office visits. You need to observe and record changes in physical, psychological, and behavioral symptoms. Report these changes to the dementia specialist and work together to find solutions. Dementia patients may practice “Showtime” behaviors at doctor appointments, which make them seem to be much better than they are on a daily basis at home. Your report to the doctor keeps the information balanced, honest, and accurate. Sometimes changes in medicine can bring about an improvement of symptoms. At other times, non-drug choices can address these issues.

The third element of being perceptive is to identify the specific triggers that affect your Loved One’s experience of LBD in a negative way. This is where many non-drug interventions can have huge positive impacts on living daily with dementia.

Examples of simple changes with big impacts may be:
• Softening the light in the room
• Using oils like lavender to calm agitation
• Playing favorite music
• Having only one person speak at a time while in the room
• Explaining what you are about to do before you begin to do it

These may seem like small things, but they can make a big difference in the person’s disease experience. You must be alert and perceptive to see what things trigger agitation or frustration. Then you can communicate with key individuals how those things must change in order to make the best care choices.

Be persistent.

Being an dementia caregiver is a long-term commitment. It is a marathon, not a sprint.

The term Lewy roller coaster for LBD folks has been coined to describe the experience due to the ups and downs in cognitive, physical, behavioral, sleep, and psychological symptoms. Your choice to be positive, proactive, and perceptive must be of a continuing, ongoing nature. You need to persist through these fluctuations as your Loved One progressively declines. It is a daily choice.

As new symptoms emerge over time, you will need to learn more and stay up to date about research and treatments that could be helpful. As your care continues, you will monitor disease symptoms daily. At times, you may question the efficacy of an intervention. Is the intervention working, or is this symptom simply a manifestation of the fluctuations of the disease? This is a typical question for you to ask in your role as care partner. Sometimes you will use your intuition correctly and change course. Other times you may not discover the answer. That is also typical, so do not blame yourself at these times.

You will also monitor those who are involved in your Loved One’s treatment and support. Part of your role is to teach any new person on the health care team about your Loved One’s expression of dementia. You may also need to teach them how to best approach the situations that arise in his care. You will deal with significant family members and friends who encounter him. All these things involve a consistent approach on your part. If your Loved One moves into a nursing home or similar facility, your role as advocate and educator will escalate.

Your steadfast persistence will pay off in a gentler LBD journey for everyone. This is one of those places in life where you clearly will make a difference. It is not easy, but it is doable — and it is worth it.

Personify the Disease

One of the most helpful decisions I made early in my Lewy Body Dementia journey with my husband was to personify “Lewy”. It had a huge impact on my grandsons as well as on John and me.

John’s neurologist, Dr. Daniel Kaufer, told us on our first appointment with him that preserving personhood was our key goal for John in all decisions about his care. That resonated with me. It became my touchstone when I had to decide what to do as various symptoms and events presented themselves in our journey.

In my mind, I separated John from his disease. I gave the disease a personal kind of name—Lewy. It gave me someone to blame, who clearly was at fault for whatever was happening. It gave me the power to separate John from actions or words he might do or say that were hurtful. “That’s Lewy talking right now. Just ignore it.” I could say that to myself and know that I was correct. John was not at fault. Truth was not being spoken. A clear enemy was in the room with us, and my job became how to outsmart that enemy. It gave me emotional distance so I could think clearly, problem solve better, and respond kindly to John.

I saw it as a kind of psychological and emotional warfare for a good purpose. Although my enemy was formidable, I still won significant battles along the way that made our overall experience of LBD less damaging to both of our lives. There was victory and empowerment in winning those battles. I was preserving personhood for John and for myself by personifying Lewy.

It also worked beautifully with our grandsons, who were young when John had LBD. When the oldest, Michael, was about five years old, he was chatting happily in the backseat of our car as we drove along a lovely country road. All of a sudden, John barked at Michael harshly and told him he needed to be quiet. Before I had time to think it through, I spoke up and said, “Michael, that is just that mean old Lewy talking to you right now. Your Pops would never talk to you like that. Pops loves you, Michael.” Fortunately, John responded with silence. This caused me to begin using the “mean old Lewy” explanation with all the grandsons from that point on. I would tell them if they entered the room and Pops looked angry or spoke harshly to just leave the room right away. “That was Lewy, not your Pops. Wait a bit and go back to see Pops later.” The boys all seemed to accept that explanation as making complete sense to them. It likely worked because it matched what happened to them. They would reenter the room later and find their sweet Pops happy to see them as usual.. Sometimes I would speak to John and remind him to be especially kind to the boys. We also tried to have only one child at a time in the room with John if he was agitated or tired.

Another example shows how personifying Lewy played out with John. One day John began to have trouble speaking. He babbled a kind of gibberish. I touched his head tenderly and said, “Sweetheart, I can see that you know exactly what you want to say. Right now Lewy is messing with the connection between your thoughts and your ability to speak. Close your eyes, take a nap, and when you wake up you will be able to say whatever you want.” John closed his eyes immediately and went to sleep. When he awakened, he was able to speak normally. That level of trust had been established over a period of years along with the habit of blaming Lewy when a bad symptom presented itself. John had learned that Lewy came and went, and he trusted me to point that out for him. It seemed to keep his anxiety lower.

Personifying Lewy gave the boys more of a sense of empowerment. They clearly stopped taking it personally when John’s disease made him appear mean to them. It also gave them someone to blame for what was happening. It made everything make more sense somehow for all of us while it protected John from the blame he did not deserve. Personifying Lewy enabled me to do a better job of separating the disease from my husband, thereby preserving his personhood while protecting my own heart from being bruised by some of Lewy’s antics.

These first steps lay a foundation for a more gentle journey. Each of them helps you to stay ahead of the disease so you have more control over your daily life. Each of them will make you a better caregiver.

 

**A draft form of this lesson first appeared in a free online booklet, “Prepare to Be an Engaged LBD Care Partner,” by Rosemary Dawson, Jeff Maruna, and Pat Snyder. The booklet may be found here:

www.lbdtools.com/files/CPGuide%20Dawson.pdf

**A briefer form was published by LBDA.org in Resources section.