24 Mayo Patients with CBS Diagnoses

This is a newly-published Mayo Rochester study of 24 patients with a clinical diagnosis of CBS (corticobasal syndrome) who had undergone MRI during life and donated their brains upon death. These 24 patients had these pathologic diagnoses:

7 had CBD
6 had PSP
6 had AD (Alzheimer’s Disease)
5 had FTLD with TDP-43

29% were diagnosed accurately during life. 54% were diagnosed accurately during life if you include the PSP diagnoses. (As PSP and CBS/CBD are treated similarly and are both tauopathies, I’m willing to consider PSP a correct diagnosis. Many of you will not agree.)

Indeed, in our local support group, several diagnosed while alive with CBS/CBD turned out to have had PSP. And there are many on the CBD-related Yahoo!Group who report that their family members were diagnosed during life with CBS/CBD but upon death with AD.

The researchers then looked at the previous MRI scans to “determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS.” They found:

“Widespread atrophy points toward a pathologic diagnosis of FTLD-TDP or AD, with frontotemporal loss suggesting FTLD-TDP and temporoparietal loss suggesting AD. On the contrary, more focal atrophy predominantly involving the premotor and supplemental motor area suggests CBD or PSP pathology.”

It’s a nice study. Wish it could’ve been with more than 24 patients.


Neurology. 2010 Nov 23;75(21):1879-87.

Imaging correlates of pathology in corticobasal syndrome.

Whitwell JL, Jack CR Jr, Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, Senjem ML, Knopman DS, Petersen RC, Dickson DW, Josephs KA.
Department of Radiology, 200 1st Street SW, Rochester, MN.

BACKGROUND: Corticobasal syndrome (CBS) can be associated with different underlying pathologies that are difficult to predict based on clinical presentation. The aim of this study was to determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS.

METHODS: This was a case-control study of 24 patients with CBS who had undergone MRI during life and came to autopsy. Pathologic diagnoses included frontotemporal lobar degeneration (FTLD) with TDP-43 immunoreactivity in 5 (CBS-TDP), Alzheimer disease (AD) in 6 (CBS-AD), corticobasal degeneration in 7 (CBS-CBD), and progressive supranuclear palsy in 6 (CBS-PSP). Voxel-based morphometry and atlas-based parcellation were used to assess atrophy across the CBS groups and compared to 24 age- and gender-matched controls.

RESULTS: All CBS pathologic groups showed gray matter loss in premotor cortices, supplemental motor area, and insula on imaging. However, CBS-TDP and CBS-AD showed more widespread patterns of loss, with frontotemporal loss observed in CBS-TDP and temporoparietal loss observed in CBS-AD. CBS-TDP showed significantly greater loss in prefrontal cortex than the other groups, whereas CBS-AD showed significantly greater loss in parietal lobe than the other groups. The focus of loss was similar in CBS-CBD and CBS-PSP, although more severe in CBS-CBD.

CONCLUSIONS: Imaging patterns of atrophy in CBS vary according to pathologic diagnosis. Widespread atrophy points toward a pathologic diagnosis of FTLD-TDP or AD, with frontotemporal loss suggesting FTLD-TDP and temporoparietal loss suggesting AD. On the contrary, more focal atrophy predominantly involving the premotor and supplemental motor area suggests CBD or PSP pathology.

PubMed ID#: 21098403

“8 Life Issues You’re Bound to Face When Caring…”

This article on “8 Life Issues You’re Bound to Face When Caring for an Aging Parent” is written by Caring.com and published in “Parade” magazine.  The eight issues are:

1. Understanding your parents’ life stage
2. Talking about tough issues — from assisted living to adult diapers
3. Family disagreements
4. Having the “car talk”
5. Sex, drugs, and alcohol
6. Work-life balance
7. Death and dying
8. After the funeral

Here’s a link to the full article:


8 Life Issues You’re Bound to Face When Caring for an Aging Parent
by Connie Matthiessen, Caring.com Senior Editor
Parade Magazine

The article includes these Caring.com webpages that are related to some of the eight issues:

Demystifying Your Aging Parents’ New Stage of Life

How to Talk to the Elderly

Caring for Elderly Relatives: How to Handle Family Conflicts

Difficult Conversations: How to Approach Older Adults With Concerns About Their Driving

Talking to a Loved One About Death

How to Avoid Strained Sibling Relationships

How to Help an Older Adult Create a Lasting Legacy.

Lots to read!

“The Role of Caregivers in Parkinson’s” – lecture notes

Though this lecture focuses on the role of caregivers in Parkinson’s Disease, the content applies to all caregivers, regardless of disorder.

The 30-minute lecture by Elaine Lanier, RN, of UCSF’s Parkinson’s Center, presents an caregiver job description, lists the stresses of caregiving, and outlines practical ways to manage that stress. Here’s a link to the lecture, given at the UCSF Parkinson’s Conference in November 2010:


Brain Support Network volunteer Denise Dagan listened to the lecture.  Denise’s takeaways from Ms. Lanier’s talk included:

  1. Assess your situation periodically.  Something that has worked for you might just not, anymore.  Employ the 3 R’s:

– Reassess!

– Recognize when you need to adjust to new circumstances.

– Request assistance from friends, family or services.  It’s the best way to care for both yourself and your loved one.

  1. Take get-aways and mini-breaks.

– Get-aways are scheduled times for your hobbies, church, personal grooming, friends, exercise, etc. (with or without your caree).

– Mini-breaks are anything to get your mind off the responsibilities of caregiving for 20 minutes, or so.  Some suggestions are, take a walk outside and a deep breath, grab some chocolate, or read.  De-stress, and then go back to it.

  1. Letting someone else help increases your creativity and skill set.  When you see how someone else is doing a task, you may wonder why you didn’t think to do it that way.
  1. Make a list of things to do and another list of people who can help.  Match the tasks with the most appropriate person to do it (by proximity, skill set, transportation, etc.) and ask the person to do that specific task.  Start with small things and ask them to do more if it goes well.  Some examples are:

– The neighbor kid who just got his driver’s license would probably love to pick up your prescription or some groceries.

– People who are far away can do your taxes, make your travel arrangements, buy Xmas gifts and ship them in your name, etc.

Think outside the box a bit for your own best health.

Denise’s extensive notes are copied below.



Denise’s Notes from


“The Role of Caregivers in Parkinson’s Disease”
Presented at the UCSF Conference on Parkinson’s Disease, November 13, 2010
Speaker, Elaine Lanier, RN, MS

Caregiver Job Description: A challenging “…job that takes place in an unstructured environment without coworkers…, no formal job description or training, and supervision by a boss that is by definition… ‘out sick.’”  (Fay Mikiska, the Caregiving Tool)

The Value of Family Caregivers

* Economic value of services family caregivers provide for “free” is approx. $375 billion per year.  This is almost double what is spent on home care and nursing home services combined (National Alliance for Caregiving and Evercare, 2009)

* Help to MD, nurse, clinicians is significant

* Service to loved one is beyond measure in terms of love, depth of care, and concern

* Your care is the person with Parkinson’s most valuable asset

What Do PD Caregivers Do?

* Help with Activities of Daily Living (ADLs), medication management & Administration, household chores, financial management, transportation, emotional support, medical plan management

* Cope with all PD symptoms – both motor (stiffness, tremor, rigidity, freezing, etc.) and non-motor (depression, anxiety, sleep disturbance, behavior and thinking changes, etc.)

* Role is a demanding,  24-hour job 7 days per week & duties increase with advancement of disease

Stresses of PD Caregiving

* Caregivers are often ill prepared to manage the emotional & physical demands of caregiving

* Face increasing demands due to PD progression

* Have their own aging processes and physical decline

* Caregiving can place constraints on social & family life, employment and finances

Story about the speaker having to give up visiting with her mother in the nursing home during meals because visiting at that time was just too stressful for both of them.  There are similar adjustments you will have to make as a Parkinson’s caregiver by being aware of circumstances as they change and being flexible.  Parkinson’s affects the entire family.  If you are still working as a PD caregiver, you’re juggling a lot – maybe too much.  Reducing your hours or quitting will affect your finances, but our work often contributes to our self esteem.  You have stress just in deciding whether to give that up, too.

Help for the PD Caregiver

* Be/get prepared

* Take care of yourself

* Get help – don’t try to do it all yourself, its too much.

* Keep good relationship/communication with the person who has Parkinson’s.  Its important for both of you.

Be/Get Prepared

* Education: internet, PD organizations, libraries, ask questions of doctors and nurses

* Evaluate present situation:  Whether you’re new to caregiving or not, assess your situation.

– Loved one’s needs.

– Home environment  Rearranging furniture to prevent falls, are there stairs in your home that will eventually be problematic?

– Your health, emotional state, commitments  (job, kids, parents, etc.)

– What you can and can’t do yourself  (Learn how to say, “No,” so you don’t take on too much.)

– What outside support needed


– Finance issues: health insurance (is PD covered, Is there a high deductible? Can you keep your coverage?), employment, paying bills, power of attorney (Don’t wait until there are cognitive issues before doing the paperwork for this.  Pick someone whom the person with PD trusts)

Have a Family Meeting

* Reasons/Benefits

– Parkinson’s affects the whole family

– Opens communication lines

– Helps everyone understand present & future situation

– Have early in decease, but never too late

– Include the person with PD whenever possible

Take Care of Yourself

* “Your own good health is the best present you can give your loved one” (Suzanne Mintz, caregiver of MS, president, Co-founder of the National Family Caregiver Alliance)  Call NFCA to find resources to help you.  Resources at the end of this talk.

* Maintain mental and physical health.  Make and keep medical and dental appointments.

* Keep your job whenever possible

* Join a support group for caregivers

* Get your sleep (even if PD person can’t)  Move to your own room/space if your sleep partner is keeping you awake.

* Take breaks – get-a-ways and mini-breaks. Walk outside and take a deep breath, grab some chocolate, or anything to get your mind off the responsibilities of caregiving for 20 minutes to de-stress, and then go back to it.

* Make and keep your social activities (even if the person with PD can no longer join you) and spiritual activities (church, yoga, or whatever)

* Keep (or develop) sense of humor.  It’s hard to have a sense of humor when you’re watching someone you love struggle and decline, but try.  Ms. Lanier shared a story about writing things her mom with Alzheimer’s said.  Sometimes there are funny things.  Recognize them.

Get Help – Don’t do it alone

* Benefits

– Lessens feelings of isolation and feeling like you’re the only one shouldering this responsibility.  You don’t have to be!

– Encourages independence of the person with PD.  If you’re not there to do everything, sometimes they might do it without you.  It may not be the way you want it done (messy, take longer, etc.) but its done!

– Gives you more confidence in your ability to caregive.

– Increases your creativity  (when you see how someone else is doing something, you wonder why you didn’t think to do it that way)

– Helps you be more peaceful & effective

How to Define and Get Help

* Asking for help is a sign of strength, not weakness

* Caregiving is a Job with individual tasks

* Make a list of caregiver tasks in a typical week

* Organize that list into categories

– Caregiving needs and how people can help

– Decide what to let go and what to keep

* Start with something small and specific like,pick up medicine at drug store, buy loaf of bread.  Match up what needs doing with who may be able to help and ask them to do something specific:

Make a list of tasks: pick up groceries, medication, mow the lawn, calling Kaiser about insurance issues, etc.

Make a list of the people who could help: grandson who just got his driver’s license; sister who lives at the other end of the state, has Kaiser, and great communication skills

Where to get help: Resources

* Local & Community

– Free to low cost: neighbors, friends, churches, synagogues, senior centers, adult day health, Meals on Wheels, door-to-door vans

– Fee based: In-home care (help with cooking, bathing, dressing, meal prep, etc.)

– Social Worker from health plan or hospital can connect you to these services

* Resources, State & National

– Family Caregiver Alliance, San Francisco  www.caregiver.org, 415-434-3388

– National Family Caregivers Association  www.nfcacares.org/index.cfm

– National Parkinson’s Foundation  www.parkinson.org/caregivers.aspx

– Michael J. Fox Foundation  www.michaeljfox.org  (Look under “Living with Parkinson’s,” guide for the caregiver)

* Each website gives information and other websites for resources

Relationship/Communication with Person with Parkinson’s

* Can be most difficult and rewarding part of caregiving

* Roles change  (e.g. adult children who must take an authoritative role with their parent)

* Research shows that despite high levels of strain, caregivers with good quality relationships have reduced depression & better physics health.

How PD Person Can Help Caregiver

* Appreciate caregiver and tell them – thank you’s go a long way

* Be an honest communicator

– Don’t minimize the situation

– Don’t avoid communication to “spare” the caregiver.  Caregivers/family want to know

Ms. Lanier shared a story about a support group.  The issue came up that a person with PD worries that if they ask for help now, it won’t be there later, when I really need it.  Caregivers all says they want to know what’s going on.

* Be Cooperative, be a team player

– Work with, not against caregiver

– Don’t expect things to be done exactly your way.  Ms. Lanier shared a story about asking her husband to go to the grocery store.  He didn’t come home with the sizes and/or brands she would have bought.  He saved her at least an hour, so it was good enough.



* Caregivers

– Get prepared with education, assess your situation as you go along, have regular family meetings

– Take care of yourself (take mini breaks, keep your medical appointments, reduce your stress)

– Get help (use the resources listed)

* Person’s with Parkinson’s

– Appreciate your caregiver(s)

– Be open and honest

– Be cooperative, you’re a team!

* To Both of You

– Work on having a positive relationship/communication with each other – for the health benefits.

“Limited case” for recommending ChEIs in PSP

This article reviews the use of ChEIs (Aricept, Exelon, and Razadyne) in PSP and other non-Alzheimer’s disorders. There is “only a limited case for recommending ChEIs in” PSP.

Expert Review of Neurotherapeutics. 2010 Nov;10(11):1699-705.

Cholinesterase inhibitors: beyond Alzheimer’s disease.

Larner AJ.
Walton Centre for Neurology and Neurosurgery, Liverpool, UK.

Cholinesterase inhibitors (ChEIs) are widely licensed for the symptomatic treatment of Alzheimer’s disease, but their use has also been examined in a wide variety of neurological disorders besides Alzheimer’s disease, and this article reviews these uses.

The evidence currently available suggests that ChEIs may possibly have a role in the treatment of some patients with dementia with Lewy bodies and Parkinson’s disease dementia, but at this point in time there would seem to be only a limited case for recommending ChEIs in mild cognitive impairment, Down syndrome, progressive supranuclear palsy, pure vascular dementia, frontotemporal lobar degeneration, Huntington’s disease, multiple sclerosis, epilepsy, delirium, traumatic brain injury, sleep-related disorders or certain psychiatric disorders (e.g., schizophrenia and bipolar disorder).

Clinical practice with respect to non-Alzheimer’s disease indications for ChEIs may vary according to jurisdiction, specifically with regards to whether national guidelines effectively limit off-licence drug use.

PubMed ID#: 21046692

“Study: Brain energy crisis may spark Parkinson’s”

Today there was news that a cause of Parkinson’s Disease may be mitochondrial dysfunction. Mitochondrial dysfunction is also a possible cause of PSP and CBD as well. Two possible treatments are mentioned in the article: a diabetes drug named Actos and a nutrient named Coenzyme Q10 (CoQ10).

A researcher cautions: The average Parkinson’s patient has lost about 70 percent of his or her dopamine-producing neurons by the time of diagnosis. So if blocking a brain energy drain is going to do any good, scientists may have to find ways to spot brewing Parkinson’s much earlier. “I don’t think you can turn back the clock,” he says.

http://www.sfgate.com/cgi-bin/article.c … z146V9bQy7

Study: Brain energy crisis may spark Parkinson’s
Monday, November 1, 2010

(11-01) 10:04 PDT WASHINGTON, (AP) —

Parkinson’s disease may stem from an energy crisis in the brain, years before symptoms appear.

If the research pans out, it points to a possible new approach for Parkinson’s: Giving a boost to a key power switch inside brain cells in hopes of slowing the disease’s inevitable march instead of just treating symptoms.

“This is an extremely important and interesting observation that opens up new therapeutic targets,” says Dr. Flint Beal of New York’s Weill Cornell Medical College, who wasn’t involved with the new study.

Beal said scientists already are planning first-stage tests to see if a drug now used for diabetes might help Parkinson’s, too, by targeting one of the implicated energy genes.

At issue are little power factories inside cells, called mitochondria. Increasingly, scientists suspect that malfunctioning mitochondria play some role in a list of degenerative brain diseases.

After all, brain cells are energy hogs, making up about 2 percent of body weight yet consuming about 20 percent of the body’s energy. So a power drain could trigger some serious long-term consequences.

“It could be a root cause” of Parkinson’s, says Dr. Clemens Scherzer of Boston’s Brigham and Women’s Hospital and Harvard University.

About 5 million people worldwide, and 1.5 million in the U.S., have Parkinson’s, characterized by increasingly severe tremors and periodically stiff or frozen limbs. Patients gradually lose brain cells that produce dopamine, a chemical key to the circuitry that controls muscle movement. There is no cure, although dopamine-boosting medication and an implanted device called deep brain stimulation can help some symptoms.

No one knows what causes Parkinson’s. To find genetic clues, Scherzer gathered an international team of researchers to comb studies of more than 300 samples of brain tissue – from diagnosed Parkinson’s patients, from symptom-free people whose brains showed early Parkinson’s damage was brewing, and from people whose brains appeared normal. They even used a laser beam to cut out individual dopamine-producing neurons in the most ravaged brain region, the substantia nigra, and examine gene activity.

The team found 10 sets of genes that work at abnormally low levels in Parkinson’s patients, genes that turned out to play various roles in the mitochondria’s energy production, Scherzer recently reported in the journal Science Translational Medicine. Especially compelling, the genes also were sluggish in people with presymptomatic, simmering Parkinson’s.

And all the gene sets are controlled by what Scherzer calls a master regulator gene named PGC-1alpha – responsible for activating many other genes that maintain and repair those mitochondrial power factories.

So might revving up PGC-1alpha in turn boost underperforming mitochondrial genes and protect the brain? To see, the researchers tested dopamine-producing neurons from rats that were treated in ways known to cause Parkinson’s-like damage. Sure enough, boosting the power switch prevented that damage.

This genetic evidence supports years of tantalizing hints that mitochondria are culprits in Parkinson’s, says Dr. Timothy Greenamyre of the University of Pittsburgh Medical Center.

He ticks off the clues: A rare, inherited form of Parkinson’s is caused by a mutated gene involved with mitochondrial function. A pesticide named rotenone that can kill dopamine cells and trigger Parkinson’s symptoms in animals also is toxic to mitochondria. So is another Parkinson’s-triggering chemical named MPTP.

Now with Scherzer’s study, “it’s going to be harder and harder for people to think that mitochondria are just a late player or an incidental player in Parkinson’s disease,” Greenamyre says.

The crux of all that complicated neurogenetics: A diabetes drug named Actos is among the compounds known to activate part of that PGC-1alpha pathway, and Weill Cornell’s Beal says it’s poised for an initial small trial in Parkinson’s.

Separately, a nutrient named Coenzyme Q10 is believed important in mitochondrial energy production, and Beal is leading a study to see if high doses might help Parkinson’s. Results are due in 2012.

But Scherzer issues a caution: The average Parkinson’s patient has lost about 70 percent of his or her dopamine-producing neurons by the time of diagnosis. So if blocking a brain energy drain is going to do any good, scientists may have to find ways to spot brewing Parkinson’s much earlier.

“I don’t think you can turn back the clock,” he says.