“Actually, let’s not be in the moment” (NYT)

Someone in the local support group sent this to me, sort of tongue in cheek.  She said that I’m always promoting “mindfulness-based stress reduction” at support group meetings and asked that I give equal time to this article about NOT being in the moment.  So here’s a link to last Sunday’s New York Times article for equal time!


SundayReview | Opinion
The New York Times
Actually, Let’s Not Be in the Moment
By Ruth Whippman
November 26, 2016


Is Alzheimer’s Disease a Uniquely Human Disorder? (webinar)

Alzforum is hosting a webinar on Wednesday, December 7th at noon eastern time on the topic of “Are we humans alone in developing Alzheimer’s disease?”

Other primates and even dogs accumulate amyloid plaques as they age, but seem surprisingly resistant to neurofibrillary tangles, neurodegeneration, and full-blown dementia. What sets us apart from other animals, and what can we learn from their resilience?

Join Lary Walker, Marc Dhenain, Elizabeth Head, Patrick Hof, Cynthia Lemere, and Peter Nelson for an Alzforum Webinar.  For more info and a link to the registration page, check out:



Hospital Discharge Planning (Notes from caregiver conference)

Brain Support Network volunteer Denise Dagan attended the Avenidas Caregiver Conference in late October 2016.  She took some notes from the various talks.  Here are Denise’s notes from the talk on “Patient Rights and the Discharge Planning Process” presented by Paula Wolfson, the Avenidas senior center social worker.

The main resource mentioned by Ms. Wolfson was the Medicare website and its explanation of the discharge appeal process:


The remainder of Denise’s notes are copied below.


Notes from Brain Support Network volunteer Denise Dagan:

The last information session I attended at the Avenidas Caregiver Conference was titled, “Patient Rights and the Discharge Planning Process.”  It was presented by Paula Wolfson, a social worker with Avenidas.  We learned to “…effectively mediate and negotiate [hospital] discharge plans.”  Ms. Wolfson gave us so much related information I could not possibly summarize it all here.

Through several hospitalizations for my parents, myself, and my daughter, I’ve never heard of, nor been invited to, a discharge planning ‘meeting’.  Whether an actual meeting happens, all of these people are involved in the discharge process.  Any one of them can question the readiness of a patient for discharge and slow, or stop the process until all requirements are in place for a safe discharge that meets all the patient’s recovery needs.

– The physician, who recommends and writes the discharge orders

– The physical, occupational and speech therapist team, to determine if the patient is at risk for falling or not able to perform the activities of daily living independently, and make recommendations for in-home or skilled care upon discharge

– The social worker, who reviews the patient’s social supports, housing, medical follow up resources, transportation, financial and legal concerns

– The nurse case manager, to assists with skilled care discharge placements and insurance coverage

– The patient and his/her designated caregiver, who should evaluate the discharge plan to determine if it accommodates all personal care, safety, medical follow up, transportation, and financial concerns for the patients recovery.

If you feel discharge is premature or not safe you have the right to appeal.  Consult with the nurse case manager or social worker.  They can arrange for you to have a conversation with the physician to extend the discharge until your concerns are addressed. This often works well because the Medicare appeals process is a major hassle for the hospital.  If the doctor is dismissive of your concerns, let him/her know you are prepared to file an appeal with Medicare.  Upon hearing that news, he or she should postpone the discharge for a day, or two.  Start working with the discharge team immediately to resolve your concerns.

If you feel you must appeal, during the admissions process and a few days prior to discharge you receive information about how to file a Medicare appeal.  If you can’t find it, request the “Important Message from Medicare” from the hospital admissions or business office.  This is an instructional guideline on appealing your hospital discharge and will explain the appeal process.  It gives you a phone number to call Medicare and advocate for the appeal which will trigger an independent review of the discharge decision and delay in the discharge date.  Medicare will pay until the appeal is resolved.  Complete information is available at:


This website also has information about filing a complaint (grievance) about the quality of care or other services you get from a Medicare provider, and how to file an appeal if you have issue with a plan’s refusal to cover a service, supply, or prescription.

“Grieving the Living!” (caregiver grief)

The author of this article, Linda Jordan, manager of Duke Community Bereavement Services, writes beautifully about caregiver grief.  And she rejects the term “anticipatory grief,” stating that the losses are here and now.  (Losses are to independence, physical control, speech, memory, cognition, etc.)  The author is focused on Alzheimer’s caregivers.  I’ve replaced the term “Alzheimer’s” with “neurological disorder” as I believe the concepts apply equally as well.  Here’s an excerpt:

“[As] long as the person with [a neurological disorder] is alive, caregiver grief, though appropriate, is generally not recognized or sanctioned. The community is more likely to associate grief with a physical death. Yet, grieving the living is real; it is constant and complex; and it is important that caregivers receive help in managing it.  When grief is acknowledged… the concept most often used is anticipatory grief. Although this term has value, the losses incurred with [a neurological disorder] are not just future; they are actual and profound in the present. Each pre-death loss in this progressive disease is compounded by all previous losses and introduces new tangible losses, as well as potential losses in the future.”

The remainder of the article encourages healthcare professionals to apply Therese Rando’s six grief processes in working with family caregivers experiencing caregiver grief.  Some interesting stories are shared.  Here’s a link to the full article:


Grieving the Living!
North Carolina Medical Journal
Volume 66, Number I
By Linda E. Jordan, DMin, CT
January/February 2005


“7 Signs It Is Time to Consider a Senior Care Community”

This article is written from the perspective of a caregiver for someone with dementia (especially Alzheimer’s or someone with memory problems) but most of the article applies to a caregiver for any person.  The author is The Pines of Sarasota, which is the educational institute that partners with dementia expert Teepa Snow.

These are the seven signs for someone to consider a long-term care situation:

1. Caregiver Stress.  This is detrimental to your health, and looking after your own health is the best thing you can do for the person in your care.  So, “if your stress levels remain high over an extended period of time, be honest with yourself.”  Consider a change to your living situation, or get more support.

2. Shadowing.  If the person in your care follows you around, this is wearing on the nerves, increasing conflict and adding to caregiver stress.

3. Home Safety & Wandering.

4. Wandering & Elopement.  Wandering is leaving a safe environment.  Elopement is leaving with intention, such as wanting to return to one’s ‘own’ home.

5. Sundowning, which is “increasing confusion and agitation usually occurring around sunset in people with some types of dementia.”  It can be caused by over-stimulation and fatigue.  Senior care facilities can reduce sundowning.

6. Aggression.  Whether verbal, physical, or sexual, aggression may be too much to handle for an in-home caregiver.  [Robin’s note:  This is especially a problem in our Lewy Body Dementia caregiver group.]

7. Changing Care Needs.  Be aware that the needs of the person in your care may eventually surpass your physical capabilities as the disease progresses.  Seek help before you harm your own well being.

Here’s a link to the full article:

7 Signs it is Time to Consider a Senior Care Community
The Pines of Sarasota Education & Training Institute


“Worried about your aging parents? How to have ‘The Talk’”

This post will be of most interest to adult children concerned about aging parents.

This article in last week’s San Jose Mercury News is timely in that the holidays may be one of the few times of the year we see our parents in person.  And we may notice that our parents have declined since the last time we saw them.  This article presents some suggestions for when, where, and how to approach parents on difficult subjects with sensitivity, respect, and the understanding that sometimes change needs to happen slowly.

The approach includes these ideas:

* Don’t put it off
* Timing is key
* Stay positive
* Play the long game
* Start with small changes
* Costs may be an issue
* When professional help is needed
* The talk is definitely worth it


Worried about your aging parents? How to have ‘The Talk’
The Mercury News
By Martha Ross
November 18, 2016 | UPDATED: November 21, 2016



“Fighting Loneliness When Dementia Steals a Spouse”

This is a very personal essay from November 2016 by children’s book author and illustrator Nancy Carlson. She describes the loneliness she feels when dementia has “stolen” her spouse. At this time, her husband Barry, with a frontotemporal dementia (FTD) diagnosis, was living in a care home.

Many spouses in our local support group have expressed similar feelings of loneliness at our support group meetings. One describes it as being widowed yet her husband is still alive.

I’m not sure that dementia is a requirement for the caregiver to feel this kind of loneliness. Having your spouse in a care facility or having a spouse unable to communicate in any way are certainly lonely circumstances.

Here’s a link to the full essay:


Emotional Health
Fighting Loneliness When Dementia Steals a Spouse
By Nancy Carlson
Women’s Voices for Change
November 14, 2016



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!



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


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
* In Lewy bodies: PD and DLBD
* In glial cytoplasmic inclusions (GCIs): MSA
* 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.


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-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)


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)


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

Symmetric parkinsonism (often no tremor) with early dementia


Sensitive to antipsychotic medication

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


(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.


(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.


(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


(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.


(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


(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.


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

Try levodopa if significant bradykinesia.

Consider cholinesterase inhibitors for cognitive impairment


(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.


(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.


(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.


(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.


(58:48) Resources

CurePSP, curepsp.org
AFTD, theaftd.org

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

Neurodegenerative Diseases, covered in Nature Insight

The November 2016 issue of Nature Insight is devoted to neurodegenerative diseases, which are increasing in prevalence in part due to extended lifespan.

The collection of articles summarizes our knowledge on six topics:  brain aging and the prospect of stalling the clock; a multipronged treatment approach to Alzheimer’s; ALS, also known as Lou Gehrig’s disease; Parkinson’s; mammalian prions; and the properties of amyloid protein aggregates.  All of the articles are available online at no charge for six months.  See:


In case you are capable of reading/understanding any of these articles, perhaps you can share some tidbits with the rest of us!


Notes from CurePSP Scientific Affairs Update, 11-15-16

Alex Klein, PhD, CurePSP’s VP of Scientific Affairs, hosted a 30-minute Facebook presentation this Tuesday.  It was poorly publicized.  CurePSP didn’t even mention it on its own website or its own online support group!  (Nor did it get the word out to support group leaders.)  And CurePSP had mis-set the privileges on its Facebook page such that you got an error message unless you were already logged in to FB.  Despite all that, many people joined in.

I listened to the excellent talk today and jotted down some notes.  Since I’ve been attending the CurePSP research symposia for a few years (and just attended from the 2016 symposium in late October), there’s nothing new for me here.  But, for most of you, I think these will be the most interesting points about research:

* 97% of all cases of PSP are not familial.

* Researchers can’t yet give a satisfactory answer about the role of the environment in causing PSP.  In Parkinson’s studies, well-water appears to be a cause.  A recent PSP study showed that well-water doesn’t cause PSP.  The only thing found that might increase the risk for PSP is a low educational attainment.  This brings us to secondary factors such as occupation.  For example, if you have a lower educational level, maybe you are more apt to have an industrial job, where you are more exposed to environmental toxins.

* In the US, a “rare” or orphan disease is one where there are fewer than 200K patients.  In the US, there are about 20K patients with PSP.  In 1983, the Orphan Drug Act was passed by Congress, giving tax incentives for pharmaceutical companies.  The procedures of clinical trials are the same.  However, the requirements in terms of statistical power are much less.  Instead of 1K for an Alzheimer’s clinical trial, we only need 100-300 PSP patients to participate.  Reporting requirements are less frequent.  And outcomes or endpoints are closer, or shorter, after starting the treatment.  This means the cost for clinical trials are much lower in PSP.  Since both PSP and Alzheimer’s are tauopathies, if we have a cure for PSP, we may have a cure for Alzheimer’s disease.  That’s why drug companies are funding research into PSP clinical trials.

* The Association for Frontotemporal Degeneration and the Bluefield Project created the FTD Disorders Registry.  The Tau Consortium is now supporting the Registry, and CurePSP will be joining the registry soon.  The registry is a way to link clinical researchers with participants.

Robin’s note:  FTD is an umbrella term that means frontotemporal degeneration; it includes PSP and CBD.  The FTD Disorders Registry has a website – ftdregistry.org – which is marked as “coming soon.”  I heard a talk in May on the registry where the coordinator said it would be launching in July.  Then I heard a talk in October where the coordinator said it would be launching in November or December.  So I’m hoping for early 2017!  Those with PSP and CBD and family members are invited to participate.  Even family members whose loved ones have passed away will be invited to participate.

The “transcript” of sorts is copied below with some of my notes.  And I’ve provided a link to the 30-minute video in case you’d like to watch yourself!



Since CurePSP’s Facebook page requires you to be logged in to FB to view the page, I think it’s best to share a link to the CurePSP video that we’ve posted to the Brain Support Network FB page:


Alex Klein, PhD
VP of Scientific Affairs, CurePSP
Facebook Live Video
November 15, 2016

Notes by Robin Riddle

(Time is counting down.  Clock starts at 32:38)

31:38 Is this disease hereditary? Is there a familial component?

No.  97% of all cases of PSP are not familial.  Genetic testing is not needed.

Twenty different mutations have been identified.  Main mutation in protein of tau.  If you have the tau mutation, it’s likely you’ll develop PSP.  In most cases, PSP is sporadic.

30:11 Risk factors of developing PSP

STX6 – garbage disposal system of cell
EIF28K3 – protein production
MOBP – mylenation of neurons (insulation of neurons in brain)

Genetics Consortium is a joint effort of CurePSP and the Tau Consortium.  Goals are to Identify more risk factors and find targets for drug development

28:45 How are these diseases related in general?

Protein misfolding is a common characteristic of AD, PSP, CBD, and MSA.

Our whole body and brain consists of proteins.  Systems can go wrong.  Proteins can accumulate.  Proteins can become toxic and cause neuronal death.

Different proteins go wrong.  In Alzheimer’s, it’s A-beta and tau.  In PSP, it’s mostly tau.  PSP is called a pure tauopathy, which isn’t entirely accurate because other proteins go awry.  In PD and MSA, it’s alpha-synuclein.

26:05 Are there environmental causes (chemicals, metals, pesticides) of PSP? Has research been done? Can we do anything about this after exposure?

Can’t give you a satisfactory answer.  There are hints that environmental toxins that cause PSP and related disorders.  We still don’t know which ones [toxins].  How do we find out which toxins cause problems?

In Parkinson’s studies, well-water may cause PD.  A recent PSP study showed that well-water doesn’t cause PSP.

The only thing we found that might increase the risk for PSP development is a lesser educational attainment.  This brings us back to secondary factors such as occupation.  For example, if you were more exposed as an industrial worker to environmental toxins, that gives us a hint for something to follow.

There is a cluster of PSP in northern France where there is a huge increase in PSP incidence.  This used to be a heavily-industrialized area.  There were no environmental laws.  This is a poor area.  Food was grown in people’s back yards.

[Robin’s note:  We heard about the cluster in northern France at the 2015 research symposium.  I had expected we would hear more about this in 2016 but there was no mention of it.  I did hear that the research has been discontinued.  A rumor was going around the conference that perhaps the French government didn’t want research into environmental causes of PSP.]

There is a cluster on the island of Guadeloupe.  Two fruits (sweet sop, sour sop) associated with the development of PSP.  There is a natural decline in the incidence of PSP.  We aren’t sure what was in the fruit that caused PSP.

Eat healthy.  Stay healthy.  Be active.  This is good for the brain.

22:12 How do we make sure all neurology fellow receive PSP training?

We recently emailed 14K neurologists across the US to make them aware of PSP and how to diagnose PSP.  We host webinars.  We are members of the Movement Disorders Society.  We have brochures for healthcare professionals.  Please spread the word to your healthcare professionals, such as PTs.

20:10 What are the very beginning symptoms?

Every PSP case is different.  Many cases start with balance issues.  Backwards falls.  Most PSP patients are misdiagnosed with Parkinson’s.  Many of the symptoms mimic the symptoms of PD.  Be sure to tell your neurologist if your falls are backwards.

Early signs are personality changes.  Not just a movement disorder.  Changes in reasoning.  Can be aggressiveness, irritability, and character changes.

Loss of interest in ordinarily-pleasurable activities is a sign of PSP.  This is different from Parkinson’s.

Less common is gaze palsy (up and down) at the beginning.  Left to right eye sight usually isn’t a problem.

Slurred speech can a problem at the beginning but doesn’t have to be.

CBD is more unilateral.  CBD symptoms are more mild at the beginning.

17:09 How common is PSP?

In the US, a “rare” disease if you have fewer than 200K patients in the US.  In the US, there are about 20K patients with PSP.  There are 5 million AD patients and 1 million PD patients in the US.  5-6 per 100K prevalence for PSP.  20K patients is still quite a lot.

In 1983, Orphan Drug Act was passed by Congress.  Being part of an orphan disease helps PSP research, drug development, and search for a cure.  Pharma needs a market.  Because of this act, there are tax incentives for pharmaceutical companies.  The procedures of clinical trials are the same.  However, the requirements in terms of statistical power are much less.  Instead of 1K for an Alzheimer’s clinical trial, we only need 100-300 patients in PSP.  Reporting requirements are less frequent.  And outcomes and endpoints are closer, or shorter after starting the treatment.  This means the cost for clinical trials are much lower in PSP.

Why is that interesting for a pharma company?  These diseases share protein misfolding.  PSP is at the center of prime of life diseases.  If we have a cure for PSP, maybe we have an idea for solving a disease for 5 million Alzheimer’s patients.  That’s a fantastic idea.

13:21 What can we do to help you cure PSP and CBD?

It’s a long list because there’s a lot you can do.

Raise awareness.  Please make others aware of what you are suffering from.  Explain what you have.  Even to your dentist.  This is important.  CurePSP will keep emailing healthcare professionals.

Subscribe to the CurePSP newsletter.  Share the newsletter.

Donate to CurePSP or host fundraisers (bake sales, wine tasting, walks).  Nine employees – 7 in NYC.

Register with registry.

Participate in brain donation.  Researchers need human tissue to test therapies.  After a long period of suffering, it would be wonderful if you and your loved ones would agree on brain donation.  It’s not for everyone.  We respect that.  Brain donation is a wonderful thing to do if you’d like to dedicate your brain to research.  Twelve years ago, started a partnership with the Mayo Clinic brain bank.  140 brains a year.  1000s of brains with PSP and related disorders.  Thousands and thousands of researchers.  Helps the genetics program to have human tissue.

[Robin’s note:  Brain Support Network, brainsupportnetwork.org, can help your family make arrangements for brain donation!  We prefer to make arrangements in advance – and your family prefers this too!  But we can even help you at the last minute.]

[Robin’s note: Mayo accepts on average 80 PSP, CBD, and MSA brains per year so I’m not sure where 140 comes from.  In 2015, they accepted 100 PSP, CBD, and MSA brains.  That’s per the Mayo Clinic presentation at the 2016 research symposium.  I don’t think they’ve ever hit 140 brains in a year with those three diagnoses.] 

8:30 Is there a registry?

Soon.  We will be joining the FTD Disorders Registry.

FTD = frontotemporal dementias.  Umbrella term.  Includes FTD as a disease, PSP, CBD, and other diseases.  We’ve joined this registry.  It was started by the Bluefield Project and the AFTD.  Supported by the Tau Consortium.  Registry will help clinical researchers find patients.  This will let us conduct natural history studies.  This will help us connect patients and science.

[Robin’s note:  The AFTD uses the term FTD to mean “frontotemporal degeneration,” which includes PSP and CBD.  The FTD Disorders Registry has a website – ftdregistry.org – which is marked as “coming soon.”  I heard a talk in May on the registry where the coordinator said it would be launching in July.  Then I heard a talk in October where the coordinator said it would be launching in November/December.  So I’m hoping for early 2017!  Those with PSP and CBD and family members are invited to participate.  Even family members whose loved ones have passed away will be invited to participate.]

6:51 Should I stretch and exercise every day?

Yes, stay as active as you can.

Gait and balance are big problems.  Train how to use a walker.  Consider putting a sandbag at the front of your walker.

Email us at [email protected].  Whatever you do should be in collaboration with your neurologist or physical therapist.  You need a treatment plan monitored by a healthcare professional.

Call us for help finding a local neurologist.  On our website is the MDS (movement disorder specialist) finder with the help of our friends from the Movement Disorder Society.

[Robin’s note:  I have searched and searched and don’t see this “finder” on their website.  I think most local support group leaders keep lists of movement disorder specialists or dementia specialists they would consider as experts on PSP or CBD.  The local American Parkinson Disease Association Information and Referral Center, apdaparkinson.org, can be a good source of referrals as well.]

Caregivers must embrace the disease.  A hazard at home is falling because up-and-down gaze is impaired.  Remove small (low) furniture in a room.  Install handrails.

3:15 What is CurePSP doing to advocate for caregivers?

It takes a strong caregiver to provide good care.  Caregivers must look after themselves.

CurePSP Care partner retreats – two next year, 4/29, in Atlanta and Portland.  Personal matters of caregivers such as nutrition, meditation, mindfulness, and stress management.  Not so much about PSP.

Two family conferences (caregivers and patients) next year – 3/18 in Phoenix and 6/24 in Chicago.  About research, medical trials, therapy management, family and legal matters, support groups, and special topics (art therapy, hospice care).  Check out Events calendar at curepsp.org.

There is a lot of hope in research.  Hopefully we can help you and cure you soon!