Category Archives: LBD

Dementia with Lewy Bodies in Today’s Caregiver

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Dementia with Lewy Bodies in Today’s Caregiver

Caregiver.com’s enewsletter, Today’s Caregiver, has a feature today on Dementia with Lewy Bodies. I’ve copied parts of the article below.  Check out the link if you’d like to read the full article.

Robin

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Dementia with Lewy Bodies
Caregiver Thought Leader Interview: Scott Losk, PhD
Principal Investigator, Summit Research Network
by Gary Barg, Editor-in-Chief
Today’s Caregiver eNewsletter
December 2, 2016

Gary Barg: Let’s start at the beginning. What is dementia with Lewy bodies?

Dr. Scott Losk: Dementia with Lewy bodies happens to be the second most common cause of dementia affecting somewhere between a million and a million and a half people in the United States.

The symptoms are very different from Alzheimer’s disease though. I know that people get them confused, but the symptoms are different, especially the symptoms that we see early on in the course of the disease. Symptoms include visual hallucinations or seeing things that are not there. A large percentage of afflicted individuals experience REM sleep behavior disorder, so sleep is disrupted by thrashing around and sometimes really bad dreams. It is an impairment in the actual cycle of sleep.

Some of the other symptoms include variability in the level of alertness or arousal that a person may have. Cognitive symptoms include visual-spatial dysfunction, maybe some memory dysfunction and then what we call executive functioning impairment is often seen. This is a difficulty with mental tracking and organization and planning and those kinds of things.

The pathology in Lewy Body Disease are alpha-synuclein bodies that affect the brain in different places than the plaques and tangles we see in Alzheimer’s disease. Which is why we get the different symptom presentation.

Gary Barg: And it is harder than most dementias to diagnose from what I understand.

Dr. Scott Losk: If you are not aware of some of the early kinds of symptoms, you might miss it because you are looking for well, how bad the memory is or, how bad is one’s ability to learn new information? And frankly, a lot of my Lewy body patients are still able to learn fairly well. On memory testing they might actually perform at something close to the normal range and that is obviously very different than what we see in Alzheimer’s disease. So, if we see some of these symptoms I have described earlier, early in the course of the dementia, the evaluator (neurologist, geriatric psychiatrist, neuropsychologist, even primary care physician) needs to be thinking dementia with Lewy bodies (DLB). Then if we see any Parkinsonian symptoms in the individual, it is highly likely that DLB is going to be the more accurate diagnosis.

Gary Barg: We just had a conference in Tampa and a lot of the morning was taken up talking about Alzheimer’s and at lunch a lady said, “Please talk about Lewy body. It’s so much more difficult than just having a loved one live with Alzheimer’s because I’ve had both.”

Dr. Scott Losk:  I’ve got a couple of situations where mom has dementia and the caregiver’s sixty-five-year-old husband also has dementia. So both cases are unrelated. There is no heredity, there, but this person happens to be a caregiver for two people with Alzheimer’s or dementia. A horrible situation, but certainly, I think for caregivers, it is important to help the physician understand that the symptoms in each person are not the same. Because frankly, the patient with dementia with Lewy bodies may walk into the primary care doctor’s office and take that brief Mini Mental State Exam, or that MOCA, and perform nearly perfectly. Then the primary care doctor may say, “Oh, there is nothing wrong with you. You do fine on this brief memory test.” Well, the problem is that particular cognitive test does not measure what we need to be measuring in early onset dementia with Lewy bodies. We need a robust assessment of executive function, of visual special function and then a robust history that tells us more about the sleep disturbance and the hallucinations and about Parkinson’s type symptoms. So it can be difficult to diagnose, but in the hands of a skilled evaluator, this differential can be made relatively easily.

Gary Barg: And Lewy bodies came into the public eye two years ago when Robin Williams had experience with the disease. Tell me some of your thoughts about his story.

Dr. Scott Losk: Yes, it is a horribly sad story. I’m convinced that one of the cognitive attributes that makes a person brilliantly funny is to be able to tell a story, or to tell a joke, and then at the very end, the path where your brain is going, is not where his brain goes, and it ends up being this disconnect that happens to be hilarious. And he had an incredible gift for being able to do that. He had what I would call extremely advanced frontal lobes. The frontal lobes are responsible for being able to reflect on one’s own condition, being able to plan for the future and being able to empathize with other people. It is also that gut level intuition that comes from the frontal lobes. So he was acutely aware of his brain changing at the hands of dementia with Lewy bodies.

Gary Barg: Would you have any specific advice for DLB family caregivers?

Dr. Scott Losk: Yeah, I actually do. One, is if there is still a lack of clarity as to what your loved one’s condition is, first and foremost spouses and caregivers, adult children, really need to be assertive about getting the necessary evaluations done, or referrals to specialists. In this particular case it is neurology, geriatric psychiatry or get them into the hands of a competent neuropsychologist who can do a detailed evaluation.

As a family caregiver, it is of critical importance to not allow your loved one’s disease to completely and totally own you. I mean, it is going to have a huge impact, there is no way around that, but maintaining some of the interests, activities, socialization and involvement for the caregiver in their regular routine, is crucial. And hopefully being able to have one or two or three friends and loved ones that they can share some of their burdens with, and some of their experiences with, and so forth.

The other piece is just to continue to get better and better educated. …

Scott Losk, PhD, obtained a doctorate at Fuller Graduate School of Psychology in 1989, and did a post-doctoral fellowship in medical psychology at Oregon Health Sciences University. He has been in private practice since 1990, with an emphasis on clinical psychology, neuropsychology, and geriatric psychology. Dr. Losk joined Summit Research Network, formerly Pacific Northwest Clinical Research Center in 1990. He has been an investigator in over 100 clinical trials evaluating treatments of disorders of the central nervous system, and the principal investigator in over 75 clinical trials evaluating treatments for Alzheimer’s disease. Dr. Losk is currently conducting a clinical trial for dementia with Lewy bodies, which is the second most common cause of dementia after Alzheimer’s disease.

 

Educational video for neurologists – diagnosing (mostly) and treating PSP, CBS, MSA, and DLB

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Educational video for neurologists – diagnosing (mostly) and treating PSP, CBS, MSA, and DLB

This 60-minute video was put together by CurePSP with the main goal to educate general neurologists on differentiating Parkinson’s Disease from the four atypical parkinsonism disorders – PSP, CBD, MSA, and DLB.  A CurePSP letter with a link to the YouTube video was sent out to 14,000 general neurologists in the US in May 2016.  Though the YouTube video was produced for neurologists, I found it understandable and think most of you will as well.

If you are seeing a general neurologist — not a movement disorder specialist and not a dementia specialist — my suggestion and request is that you share this email with the general neurologist.  Encourage him/her to spend 60 minutes to gain some additional info on diagnosis, evaluation, pathology treatment, care pathology, and prognosis of the four atypical parkinsonism disorders.

Be sure to explain to your general neurologist the resources and services of Brain Support Network — we have local support group meetings in Northern California, email lists focused on the atypical parkinsonism disorders, and that we help people nationally with all neurodegenerative diseases with brain donation.

For everyone viewing this post, my suggestion is that you skip to the last ten minutes of the video and listen starting with the medication management section.  Most of you are beyond the diagnosis so reading about the symptoms, how to diagnose, imaging, and the pathology is probably less interesting.

This video is a great service to general neurologists.  And probably primary care physicians and other healthcare professionals as well.  It’s unfortunate that the four atypical parkinsonism disorders are compared to Parkinson’s Disease ONLY but that is the context of this educational video.  It was made by movement disorder specialists.  As a point of reference, no one would’ve thought my father had Parkinson’s Disease.  Alzheimer’s Disease seemed far more likely in his case but a video that compares PSP to Alzheimer’s ONLY wouldn’t be 100% right either.  We need better “blending” of the movement disorder communities and the memory disorder communities!

I’ve copied below some notes on the 60-minute educational video.  This is definitely not a transcript.  In many places, I say “see slide.”  But the notes at least give you the time markers and some idea of what is discussed at certain times.

By the way, I’ve heard great things about Dr. Stephen Reich, the main speaker in the video.  He’s been part of the PSP/CBD community for many years.  One of the other authors of the educational presentation is Dr. Alexander Pantelyat, a movement disorder specialist from Johns Hopkins I’ve had the pleasure of meeting at conferences.  The third author of the presentation is Dr. Shawn Smyth, who shares some helpful videos of specific symptoms as part of this larger video.

If you learn anything, let me know!  Or, if your general neurologist wants to know more about any of these disorders or get involved with Brain Support Network, let me know!

Robin


www.youtube.com/watch?v=BtEiNlivgeI

Atypical Parkinsonian Disorders
60-minute tutorial for general neurologists
Produced by CurePSP, May 2016

Slide set put together by:
Stephen Reich, MD, University of Maryland
Shawn Smyth, MD, Parkinson’s and Movement Disorders Center of Maryland and Johns Hopkins University
Alexander Pantelyat, MD, Johns Hopkins

DIAGNOSIS

The importance of a correct diagnosis:
* avoiding repeated consultations, testing, and/or hospitalizations
* avoiding unnecessary diagnostic testing
* providing accurate prognostic information
* directing patients and families to appropriate resources/networking/clinical trials
* trying treatment strategies/care that may provide helpful

Parkinsonism is NOT Parkinson disease
* ParkinsonISM is a general term to describe movement (motor) problems that commonly appear together in certain illnesses
* The term parkinsonism comes from Parkinson’s disease, but these problems are also seen in other disordres
* The four main motor symptoms of parkinsonism: bradykinesia (slowness of movement), rigidity, tremor (rest), postural instability and gait dysfunction.
* Not everyone with parkinsonism has Parkinson’s disease though the majority do.

Differential diagnosis of parkinsonism in a flow diagram:
* 80% have primary parkinsonism or degenerative disease.  This breaks down as Parkinson’s disease (majority), atypical parkinsonian disorders, and heredo-degenerative parkinsonian disorders.
* 20% have secondary parkinsonism.  This breaks down as other brain conditions — vascular, hydroencephalic, infectious, traumatic, etc — or systemic etiologies — hypothyroidism, meds, toxins, etc.

(4:38) Degenerative causes of parkinsonism

More common presentations:
* Parkinson disease
* Atypical parkinsonian disorders
– progressive supranuclear palsy
– multiple system atrophy
– corticobasal syndrome
– dementia with Lewy bodies/diffuse Lewy body disease

Rarer presentations:  (this isn’t a complete list)
* frontotemporal dementia with parkinsonism (FTD-P)
* Alzheimer disease
* spinocerebellar ataxias (SCAs, often types 2, 3, 17)
* basal ganglia calcification (sporadic and inherited)
* Huntington’s disease (juvenile presentation)+
* Wilson disease (<50 years old)+
* acquired hepatolenticular degeneration

+ often have parkinsonism, dystonia, and tremor

Up to 25% of those who were thought to have Parkinson’s disease upon autopsy were found to have an alternative diagnosis.  The most common alternative diagnosis is another form of parkinsonism.  The most common of these are PSP, MSA, CBS, or DLB.

Degenerative parkinsonism and accumulating intracellular proteins
Synuclein:
* In Lewy bodies: PD and DLBD
* In glial cytoplasmic inclusions (GCIs): MSA
Tau:
* PSP, CBD, FTDP-17, Parkinson dementia complex of Guam

Secondary causes of parkinsonism
* Vascular: lower-half (waist down) parkinsonism; multi-infarct state; TIA history; step-wise progression; MRI scan indicative
* Hydrocephalus
* Space-occupying mass/lesion
* Endocrine (hypothryoid slowness)
* Toxic: manganese, carbon monoxide, cyanide, MPTP, carbon disulfide
* Drug-induced:  dopamine-receptor blockers (first and second generation antipsychotics; antiemetics, including Reglan); anticonvulsants/mood stabilizers (valproic acid, lithium); antiarrhythmics (amiodarone).  Most important category here.  Physicians are not very good at recognizing drug-induced parkinsonism.  This can last for a number of months, even a year, after the drug has been stopped.  It’s important to take a good drug history.
* Post-encephalitic
* Post-traumatic

(8:15) Steps to making a diagnosis
(See the slide)

Usually on PD and CBS are asymmetric.

Are there any symptoms that don’t fit with Parkinson’s?

Very important to take a careful drug history.

Atypical parkinsonian syndromes don’t respond at all to levodopa or have a short-lived response.  In PD, we are reassured that there’s a sustained response to levodopa over five years and when the patient develops dyskinesia.

(10:18) Dementia is typically a late symptom in PD.  If the symptom is early, this would suggest it’s not PD.

(10:36) PD diagnostic criteria – UK PD Society Brain Bank Criteria (Hughes et al, JNNP; 55: 181-184)
(See the slide)
Must have bradykinesia (slowness of movement)
Must have one or more of rigidity, tremor at rest, or postural instability but not at onset

In PD, postural instability usually begins 5-7 years in.

Patients with PD almost always survive ten years and often many more years than that.

(13:30) Atypical parkinsonian disorders
* Often confused with PD, AD, and other dementias
* Clinical diagnostic criteria for PD, AD, and the atypical parkinsonian disorders have imperfect sensitivity and specificity
* Diagnosis is made clinically as all diagnostic studies only support clinical suspicion
* ONLY WITH FOLLOW-UP can many “red flag” features be identified to improve diagnostic accuracy.  Ask about these at each visit.

(15:15) PD vs. atypical parkinsonian disorders (from Quinn, JNNP suppl: 78-89)

Clues for PD: asymmetric onset of movement dysfunction; significant and sustained benefits to dopaminergic medications (though tremor may not respond); classic resting tremor (or unilateral pill-rolling tremor)

Note: those with MSA may have a low-amplitude somewhat jerky postural tremor of the fingers or hand.

Clues for atypical parkinsonian disorders (PSP, CBS, MSA, DLB): fairly symmetric onset of movement dysfunction (except CBS which is highly asymmetric); poor, transient or no benefit to dopaminergic medications

Red flags to atypical parkinsonian disorders: few are absolute; may not be present at presentation so re-evaluate for these at every visit

(17:20) ALERT for Atypical Parkinsonian Disorders

ALERT is a helpful acronym for approaching these syndromes.

A= atypical for Parkinson disease
* apraxia and/or myoclonus (CBS or AD with parkinsonism)
* saccade changes (slow saccades or vertical ophthalmoplegia is PSP, delayed/apraxic in CBS, and hypermetric in MSA)
* parietal/sensory dysfunction (extinction, cortical sensory loss, alien limb in CBS)
* cerebellar or upper/lower motor neuron signs (MSA)
* certain types of dystonia or dyskinesia:
– retrocollis, blepharospam/eyelid opening “apraxia” or trunk dystonia in PSP
– anterocollis or levodopa-induced facial/oral dyskinesias in MSA
– limb dystonia in CBS or MSA
* faster progression, including “wheelchair sign” (early use of wheelchair – within 3-5 years)

L= lack of response to medications (poor, transient or no benefit to an adequate trial of levodopa – 1000mg/day).  One small exception, if tremor-predominant PD, a trial of 1000mg/day may not be warranted.

E= early (compared to PD):
* falls/postural instability (PSP)
* dysphagia, dysarthria, bulbar dysfunction (PSP, MSA)
* dementia, executive dysfunction, impulsivity, apathy, personality change, or pseudobulbar affective lability (DLB, CBS, PSP)
* hallucinations/delusions (DLB)
* autonomic dysfunction such as constipation and orthostatic hypotension (MSA)

R= refer to resources (second opinion to movement disorder specialists, physical/occupational/speech and swallow therapy – ancillary services become mainstay of treatment; CurePSP)

T= treat symptomatically (even when unsure of the diagnosis)

(23:13) Autonomic dysfunction
(See slide for a list of symptoms/features)

Patients with orthostatic hypotension may not have lightheadedness but may have fatigue, confusion, visual blurring, and the “coat hanger sign.”

Those with erectile dysfunction rarely voice this to the physician.

These are problems that can often be treated and are disabling.

PROGRESSIVE SUPRANUCLEAR PALSY – symptoms, variants, imaging, pathology

(25:01) Progressive Supranuclear Palsy
(see slide)

Bradykinesia tends to be axial.  Difficulty getting up from a chair.  Or getting back into a chair.  Tremor is uncommon.

Rigidity is axial.  Rigidity is often at neck.

Supranuclear palsy – both up and down.  Key diagnostic feature.  Slow vertical saccades – this can be found before supranuclear palsy.  (26:00 – video and audio out of synch for several seconds.)  Frequent saccadic instrusions.

Retrocollis.

Prominent bulbar dysfunction early on.

Frontal dysfunction.  Prominent apathy.  Executive dysfunction.  Impulsivity.  Applause sign – shows perseveration (from frontal lobe dysfunction); not specific to PSP; rarely seen in PD.

(28:18) PSP Variants (from Williams & Lees, Lancet Neurology 2009.  from Dickson et al, Curr Opin Neurol 2010)

PSP-Richardson syndrome: classic presentation; accounts for only approx 25% of PSP pathology; originally described in 1964 by John Steele, who is still living, Richardson, and neuropathologist Olszewski

PSP-Parkinsonism: can have tremor and partial/temporary levodopa response (similarities to Parkinson disease); more benign course than PSP-RS; nearly as common as PSP-RS

PSP-CBS
PSP-Nonfluent Aphasia
PSP-Frontotemporal dementia
PSP-Pure Akinesia with Gait Freezing
Mixed pathologies

(29:50) Imaging features of PSP
(see slide)
Hummingbird/penguin sign in midbrain
Morning glory sign

(30:24) Pathological changes of PSP
(see slide)

CORTICOBASAL SYNDROME – symptoms, variants, imaging, pathology

(31:05) Corticobasal Syndrome
(see slide)

Corticobasal Degeneration now used for pathological confirmation.  During life, we call this corticobasal syndrome.  Only about 50% of those diagnosed with CBS during life are found to have CBD upon autopsy.  30% have Alzheimer’s pathology.  20% have Lewy body pathology.

Often presents unilaterally.

Important to see a saccade.

May present with early frontal dementia.

(33:21) CBS Variants (from Armstrong et al, Neurology 2013)
PSP Syndrome
Frontal-behavioral spatial syndrome
Nonfluent/agrammatic Primary Progressive Aphasia
Mixed Pathologies (with Parkinson Disease, with Dementia with Lewy bodies)

[Robin’s note: Isn’t “corticobasal syndrome” a variant??]

(34:00) Imaging features of CBS
(see slide)

(34:13) CBD Pathology
(see slide)

MULTIPLE SYSTEM ATROPHY – symptoms, imaging, pathology

(34:37) Multiple System Atrophy
(see slide)

MSA subtypes: MSA-P and MSA-C.  All patients have to have dysautonomia (orthostatic hypotension or a combo of urinary incontinence and, with men, erectile dysfunction).

(36:15) Red flags suggesting MSA
(see slide)

Anterocollis

Inspiratory stridor or expiratory sighs

Pseudobulbar affect

(38:00) Imaging features of MSA
(see slide)

Hot cross bun sign

(38:15) MSA Pathology
(see slide)

DEMENTIA WITH LEWY BODIES – symptoms

(38:51) Dementia with Lewy Bodies/Diffuse Lewy Body Disease
(see slide)

Symmetric parkinsonism (often no tremor) with early dementia

Pseudo-delirium

Sensitive to antipsychotic medication

Greater extent of Lewy bodies in brain (especially in cortex)

GENERAL

(40:32) Overlapping symptoms in neurodegenerative disorders
cognitive – emotional – sleep/wake cycle – autonomic – sensory – movement

(41:30) Non-motor symptoms
(see slide)

cognitive – emotional – sleep – fatigue – autonomic – sensory

These can be very disabling.

VIDEOS THAT ILLUSTRATE SYMPTOMS

(43:00) Shawn Smyth, MD presents some videos that illustrate symptoms, which are useful during evaluation and diagnosis.

Woman with anguished look with PSP.

(43:20) Evaluating saccades in woman with PSP.  Evaluating optokinetic nystagmus.

(44:24) Evaluating retropulsion in PSP.

(44:37) Evaluating rigidity in neck and arms in PSP.

(44:50) Evaluating bradykinesia of PSP (unusual).

(45:40) Applause sign in PSP.

(46:04) Clenched-fist dystonia with irregular postural tremor in CBS.

(46:24) Asymmetric bradykinesia and lefthand dystonia in CBS.

(46:36) Apraxia in CBS.

(47:10) Squeaky hypophonia and dysarthria in MSA.

(47:29) Cerebellar signs in MSA.

(48:10) Wide cadence and irregular gait in MSA.

ANCILLARY TESTING

(48:27) Stephen Reich, MD discusses ancillary testing
(see slide)

MRI is worthwhile unless the patient seems to be classic for PD

DaT scan cannot distinguish between any of the PD and atypical parkinsonian disorders

Not challenging: distinguishing between PD and essential tremor.

Routine lab tests: vitamin B12, thyroid

MEDICATION MANAGEMENT

(51:09) Medication management of these syndromes
* Levodopa: test 1000mg/day immediate release (optimally received on an empty stomach during the waking part of the day)
* Cholinesterase inhibitors (rivastigmine) in DLB
* Quetiapine or clozapine for hallucinations, delusions or agitation, used cautiously
* SSRIs or TCAs for depression or anxiety.  Beware of medication interactions with other antidepressant, antipsychotic, or dopaminergic.  Shy away from TCA if constipation or OH is present.
* Levetiracetam or clonazepam for myoclonus, especially in CBS
* Try non-pharmacologic approaches to orthostatic hypotension first.  Review meds that might contribute to low BP.  Adequate salt intake.  Avoid hot baths/showers.  Lots of water.  Fludrocortisone, midodrine, droxidopa, or pyridostigmine for orthostatic hypotension.  Stockings, head of bed elevation, and abdominal binders can be considered.
* Botox injections for sialorrhea, dystonia, and blepharospasm/apraxia of eyelid opening

The atypical parkinsonian syndromes are NOT untreatable.  Medication does play a small role.

SUPPORTIVE MEASURES

(53:40) Supportive Measures
PT, OT, speech/swallow therapy
Social work/case management consultation
Support groups for patients/families, in-person or online
Palliative care

PSP MANAGEMENT

(54:18) Management of PSP
(see slide)

Trial of levodopa up to 3 months.  Taper or discontinue if unsuccessful or not tolerated.

Weak evidence for amitriptyline and amantadine.

He prefers UStep walker.

CBS MANAGEMENT

(55:22) Management of CBS
(see slide)

Try levodopa if significant bradykinesia.

Consider cholinesterase inhibitors for cognitive impairment

MSA MANAGEMENT

(55:50) Management of MSA
(see slide)

If MSA-P, judicious trial of levodopa.  Watch that orthostatic hypotension isn’t worsened.

See a urologist.

Not many therapies for MSA-C.

DLB MANAGEMENT

(56:42) Management of DLB
(see slide)

Not very responsive to levodopa but worth a trial.  Watch that mental status isn’t worsened and hallucinations aren’t caused.

Cholinesterase inhibitors can be helpful.  Worth trying them plus memantine.

Avoid antipsychotics unless necessary.

PROGNOSIS

(57:32) Prognosis for atypical parkinsonian syndromes

* Quite different from PD, where there’s a normal lifespan.  Cause of death is often unrelated to PD.
* Highly variable
* Most common causes of death due to disease itself — complications of immobility (DVT, infections with spesis), dysphagia (aspiration pneumonia), or injury from falls
* Generally have a lifepsan of <10 years from onset of symptoms, with variability.  Usually 6-10 years.

CONCLUSIONS

(58:15) Conclusions

* Atypical parkinsonian disorders have protean manifestations and can be challenging to diagnose at the front end

* ALERT acronym:
A= atypical for Parkinson disease
L= lack of response to medications
E= early falls, cognitive impairment, personality change, autonomic dysfunction (pay close attention to non-motor symptoms)
R= refer to resources (movement disorder specialists, CurePSP)
T= treat symptoms (even if you don’t have a diagnosis yet)

* It is important to consider these disorders in all middle aged or older patients, as early detection can improve quality of life for the patient and their caregiver/family

* Supportive care and a variety of symptomatic therapies can be offered.  Pay particular attention to the caregiver throughout the disease.

RESOURCES

(58:48) Resources

CurePSP, curepsp.org
AFTD, theaftd.org
ClinicalTrials.gov

[Robin adds: Brain Support Network, brainsupportnetwork.org – educational materials on PSP, CBS/CBD, MSA, DLB]

Diagnostic accuracy is low, even for Alzheimer’s!

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Diagnostic accuracy is low, even for Alzheimer’s!

This email may be of interest to the armchair researchers among us and those following progress on brain imaging for Alzheimer’s and other amyloid-based pathologies.

For the last several years, researchers at major medical centers have had access to amyloid PET scans.  These scans indicate if there’s amyloid in the brain.  Amyloid is one of two proteins involved in Alzheimer’s Disease.  Just because there’s amyloid in the brain doesn’t mean someone has Alzheimer’s but the chances are high given the prevalence of the disease.  And just because there’s amyloid in the brain doesn’t mean that other disorders, such as Lewy Body Dementia, aren’t also present.

Amyloid PET scans are slowly moving into clinical use.  Insurance companies generally don’t want to pay for an amyloid PET scan as having a more accurate diagnosis doesn’t presently lead to any helpful treatment.

An interesting study was recently published about how amyloid imaging can change the clinical diagnosis.  The study is discussed here on the AlzForum:

With Amyloid Scan in Hand, Physicians Manage AD Differently
AlzForum
04 Nov 2016

The most interesting part of the study to me was how poor the diagnosis is without using an amyloid PET scan.  The study reported:  (AD = Alzheimer’s Disease)

“PET scans revealed that about one-third of patients diagnosed with AD were amyloid-negative, while about half of patients with other diagnoses were amyloid-positive.”

So this means that about one-third of all the patients neurologists thought had Alzheimer’s don’t have Alzheimer’s.  And half of the patients neurologists thought didn’t have Alzheimer’s in fact have Alzheimer’s!

Did the scan results change the diagnosis?  The study reported:  (Aβ = beta-amyloid)

“Diagnoses for nearly all the Aβ-negative patients changed to non-AD. Only half the non-AD Aβ-positive patients were given a new diagnosis of AD.”

So, the amyloid-negative scans swayed the post-scan diagnosis.  Why did the amyloid-positive scans not change the diagnosis?  AlzForum says:

“Researchers praised the fact that clinicians did not simply equate an amyloid-positive scan with AD. ‘That’s appropriate. The scan should be just one data piece you use along with other clinical context to make a diagnosis,’ [UCSF neurologist Gil] Rabinovici said. He also liked the fact that clinicians put less weight on a positive scan than a negative one, recognizing that amyloid pathology can occur in other disorders. Nevertheless, the 12 amyloid-negative patients maintained their AD diagnosis because they fit the profile of Alzheimer’s extremely well, Boccardi noted. These patients might have had false negative scans, or they might have suspected non-Alzheimer’s pathology (SNAP), she suggested. Analysis of the collected CSF for disease biomarkers might shed additional light on their pathology.”

Clearly, there’s lots more work to do….

Robin

“The terrorist inside my husband’s brain”

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In the last couple of days, lots of friends (who remember my connection with Lewy Body Dementia) and a very long-time LBD caregiver support group member have emailed me news of Susan Schneider’s editorial.  Ms. Schneider is the widow of Robin Williams.  Robin Williams was diagnosed during life with Parkinson’s Disease.  She suggests he didn’t really believe the diagnosis.  He wanted to know if he had Alzheimer’s, dementia, or schizophrenia.  Upon brain autopsy, it was found he had Lewy Body Dementia.

Note that Mr. Williams’s brain was not donated for research but autopsied.  Of course Brain Support Network encourages brain donation as that way the brain tissue is preserved for future research.

In describing the disorder that Mr. Williams had, Ms. Schneider uses the term “Lewy body disease.”  As she notes in the editorial, technically “Lewy body disease” is a term that includes Parkinson’s Disease.  PD is the most common Lewy body disease.  When she indicates that 1.5 million Americans suffer from Lewy body disease, this number includes the one million people suffering from PD.

Ms. Schneider’s editorial in the journal Neurology notes that Robin Williams suffered from delusions, extreme anxiety, fear, memory problems, and many other non-motor symptoms.  He never reported having hallucinations but she believes he must’ve had them.  And she believes he was keeping other symptoms to himself as well.

She reports that the “loss of memory and inability to control his anxiety was devastating to him.”  It seems that she regretted giving Mr. Williams the antipsychotic prescribed to him for a panic attack.  She learned later that those with LBD can be extremely sensitive to medication; indeed, Mr. Williams’s reactions to medication were unpredictable.

In the editorial, Ms. Schneider says:  “In early May, …he came home from Vancouver—like a 747 airplane coming in with no landing gear. I have since learned that people with LBD who are highly intelligent may appear to be okay for longer initially, but then, it is as though the dam suddenly breaks and they cannot hold it back anymore. … I will never know the true depth of his suffering, nor just how hard he was fighting. … Robin was losing his mind and he was aware of it. Can you imagine the pain he felt as he experienced himself disintegrating? And not from something he would ever know the name of, or understand? Neither he, nor anyone could stop it—no amount of intelligence or love could hold it  back.”

She points out that if Mr. Williams had been given an accurate diagnosis during life, perhaps she would have been comforted with that knowledge:  “How I wish he could have known why he was struggling, that it was not a weakness in his heart, spirit, or character.”

But, then she asks if a diagnosis during life would’ve made a difference.  She says: “But would having a diagnosis while he was alive really have made a difference when there is no cure? We will never know the answer to this. I am not convinced that the knowledge would have done much more than prolong Robin’s agony while he would surely become one of the most famous test subjects of new medicines and ongoing medical trials. Even if we experienced some level of comfort in knowing the name, and fleeting hope from temporary comfort with medications, the terrorist was still going to kill him. There is no cure and Robin’s steep and rapid decline was assured.”

Ms. Schneider’s editorial is written for neurologists and researchers, and was published in the journal Neurology.  You can find her lengthy but wonderful editorial here:

m.neurology.org/content/87/13/1308.full

The terrorist inside my husband’s brain
Neurology
Susan Schneider Williams, BFA
September 27, 2016

You can find a good Huffington Post article here:

www.huffingtonpost.com/entry/inside-robin-williams-devastating-final-year-of-life_us_57eee8ace4b024a52d2f25be

And you can find a good Washington Post article here:

www.washingtonpost.com/news/arts-and-entertainment/wp/2016/10/02/the-hardest-role-of-his-life-widow-describes-disease-that-drove-robin-williams-to-suicide

Robin

Caregiving resources for advanced Parkinson’s (from NPF)

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Caregiving resources for advanced Parkinson’s (from NPF)

We recently stumbled across the National Parkinson Foundation’s CareMAP resources at caremap.parkinson.org.  CareMAP stands for Care in Managing Advanced Parkinson’s.  “Advanced PD” is when a person with Parkinson’s Disease (PD) is no longer physically independent.  Of course all of the Brain Support Network disorders — LBD, PSP, MSA, and CBD — have many similarities with advanced PD.

BSN volunteer Denise Dagan evaluated the CareMAP resources and finds lots to recommend:
* website – caremap.parkinson.org
* worksheets from the website – caremap.parkinson.org/worksheets
* “Caring and Coping” booklet

Take it away Denise…

In 2014 the National Parkinson Foundation (NPF) launched CareMAP [Care in Managing Advanced Parkinson’s] but it didn’t seem to make a big splash at the time.  Maybe that’s because you can’t find it from the NPF website unless you know where to search.  Now that it has come to my attention I want to share with you that it is a well thought out, practical, comprehensive and user friendly resource.

It does a very good job of its intended purpose, which is to provide “practical suggestions for coping with the complex problems that arise as a result of advanced Parkinson’s disease (PD).”  CareMAP describes advanced PD as the point when a person with Parkinson’s is no longer physically independent.  They have “serious problems with mobility and cannot complete activities of daily living by himself or herself. Cognition changes, specifically dementia, are also a hallmark of advanced PD.”

NPF calls the tone of CareMAP a ‘dynamic format,’ but I found the gentle, well paced, matter of fact videos (found only in the Home Care area) to be like inviting a very experienced care specialist into my home for personal instruction.  The video streaming functioned well for me.  I could stop, replay, or skip forward with no waiting for the videos to load.  Videos are available in Spanish and easy to find from the ‘Espanol’ tab on every page.

Here are some excerpts from the original press release describing the site’s purpose and structure:

“Using a dynamic format combining videos and articles, the CareMAP website, caremap.parkinson.org,  explores the key elements of Parkinson’s care. Since the disease progress slowly, with caregivers gradually taking on more responsibilities over time, the site gives caregivers—family members, friends, volunteers or paid professionals—the tools and resources they need to successfully transition from one stage of caregiving to the next.”

“The website is organized into six key areas: Home Care, Outside Help, Caring from Afar, Caring for You, End of Life and Resources. Visitors can explore the topics that are relevant to them at any particular time in their caregiving journey.”

“How-to videos offer practical tips and messages about responding to everyday challenges, such as helping someone get out of bed or get dressed.  Interviews with caregivers showcase their unique stories about how the disease has affected them.”

The more I explored CareMAP, the more useful information I found, not only in the videos and articles in the main body of each page, but in the margins.  Each video and article page has blue boxes along the right side of the screen with, “More in This Category,” and “Related Information.”  Links under these headings take you to more videos and articles, tip sheets, webcasts, worksheets, online brochures, CareZone, Caregivers Forum, etc.  The resources seem endless.

There is even a free comprehensive companion workbook, “Caring and Coping,” available to either order by mail, or download in PDF format at:

https://secure3.convio.net/prkorg/site/Ecommerce/1622547691?VIEW_PRODUCT=true&product_id=1061&store_id=2162

This workbook is designed “to help family caregivers organize all the information they will both receive and generate over the course of their loved one’s Parkinson’s journey. The workbook addresses caregiving questions and concerns from diagnosis through end-of-life care, making it a useful tool for people at any stage of caregiving.”

The workbook includes both ’Tip sheets’ with practical pointers from every day care to travel concerns, and ‘Worksheets’ that “help you keep important information organized and easily located.  There are worksheets to prepare for medical appointments, keep track of medications on a scheduling sheet, a symptoms diary, even worksheets for interviewing and training hired caregivers.”

Worksheets can be printed from:  caremap.parkinson.org/worksheets

CareMAP (or parts of it) is definitely worth bookmarking, and I highly recommend taking a look at the companion workbook, as well.

– Denise