Excerpt from PSP/CBD Guide on Sinemet

Several medications, all available only by prescription, can help PSP in some cases.

• Sinemet
This is the brand name for a combination of levodopa and carbidopa. Levodopa is the component that helps the disease symptoms. Carbidopa simply helps prevent the nausea that levodopa alone can cause. When levodopa came along in the late 1960’s, it was a revolutionary advance for Parkinson’s but, unfortunately, it is of only modest benefit in PSP. It can help the slowness, stiffness and balance problems of PSP to a degree, but usually not the mental, speech, visual, or swallowing difficulties. It usually loses its benefit after two or three years, but a few patients with PSP never fully lose their responsiveness to Sinemet.

Some patients with PSP require large dosages, up to 1,500 milligrams (mg.) of levodopa as Sinemet per day to see an improvement, so the dosage should be pushed to at least that level, under the close supervision of a physician, unless a benefit or intolerable side effects occur sooner. The most common side effects of Sinemet in patients with PSP are confusion, hallucinations and dizziness. These generally disappear after the drug is stopped. The most common side effect in patients with Parkinson’s disease, involuntary writhing movements “chorea” or dyskinesias) occur very rarely in PSP, even at high Sinemet dosages.

Patients with PSP should generally receive the standard Sinemet (or generic levodopa/carbidopa) preparation rather than the controlled-release (Sinemet CR or generic levodopa/carbidopa ER) form. The CR form is absorbed from the intestine into the blood slowly and can be useful for people with Parkinson’s disease who respond well to Sinemet but need to prolong the number of hours of benefit from each dose. In PSP, however, such response fluctuations almost never occur. Because Sinemet CR is sometimes absorbed very little or erratically, a poor CR response in a patient with PSP might be incorrectly blamed on the fact that the disease is usually unresponsive to the drug. Such a patient might actually respond to the standard form, which reaches the brain in a more predictable way.

A new formulation of levodopa-carbidopa is Parcopa, which dissolves under the tongue. For people with PSP who cannot swallow medication safely, this could be useful. Another approach for such patients is to crush a regular levodopa-carbidopa tablet into a food or beverage that is easily swallowed. Another new formulation of levodopa-carbidopa (called Stalevo) combines those two drugs with a third drug, entacapone, in the same tablet. The entacapone slows the rate at which dopamine is broken down. It is useful for patients with Parkinson’s whose levodopa-carbidopa works well but only for a few hours per dose. This situation rarely, if ever, occurs in PSP.

Case Report: First Symptoms are Agitation/Anxiety

The “Journal of Clinical Neurology” is published in Seoul by the Korean Neurological Association. Last month a case report was published about a 52-year-old woman with behavioral changes. She was thought to have the behavioral variant of frontotemporal dementia (bvFTD). Some of her symptoms were restlessness, insomnia, agitation, inappropriate behavior (meddling in other people’s affairs), and severe anxiety. Later, she developed a gait disturbance. She had no eye movement problems. The working diagnosis changed to FTD with parkinsonism. The clinicians “requested a genetic study for FTD and parkinsonism linked to chromosome 17 (FTDP-17), but this did not reveal a genetic mutation of microtubule associated protein tau.” Eventually vertical gaze problems appeared, and the woman was diagnosed with PSP.

A brain biopsy was done; the diagnosis was PSP. I’ve heard about brain biopsies being done in very rare instances. I was talking years ago to someone about this and they said: “you’re not going to remove any part of my loved one’s brain while he is still alive!”

The authors note that previous investigations of behavioral symptoms in PSP reveal “that apathy was the most frequent behavioral abnormality; …the presence of high apathy and low agitation scale scores correctly identified the patients with PSP. In contrast, in our case, agitation and disinhibition were the earliest and most prominent behavioral symptoms. … To the best of our knowledge, agitation or disinhibition as an initial symptom of PSP is very rare… Based on previous reports and our case, gait instability and gaze abnormality are not absolute requirements as presenting symptoms for a diagnosis of probable PSP, and the initial clinical diagnosis criteria are not mandatory.”

The article is available at no charge online.

Robin

Journal of Clinical Neurology. 2010 Sep;6(3):148-51. Epub 2010 Sep 30.

Behavioral changes as the earliest clinical manifestation of progressive supranuclear palsy.

Han HJ, Kim H, Park JH, Shin HW, Kim GU, Kim DS, Lee EJ, Oh HE, Park SH, Kim YJ.
Department of Neurology, Myongji Hospital, College of Medicine, Kwandong University, Goyang, Korea.

Abstract
BACKGROUND: The clinical and pathological heterogeneity of progressive supranuclear palsy (PSP) is well established. Even with a well-defined clinical phenotype and a thorough laboratory workup, PSP can be misdiagnosed, especially in its early stages.

CASE REPORT: A 52-year-old woman, who we initially diagnosed with a behavioral variant of frontotemporal dementia developed parkinsonian features, which then progressed to gait instability and gaze abnormality.

CONCLUSIONS: We report herein a pathologically confirmed case of PSP presenting with behavioral changes including agitation and irritability, which eventually led to the cardinal symptoms of progressive supranuclear palsy.

PMID: 20944816

The full article is available here:

XML version –>
http://synapse.koreamed.org/search.php? … vmode=FULL

PDF version –>
http://synapse.koreamed.org/Synapse/Dat … -6-148.pdf

“Nigel Dempster – Life with PSP”

Every once in awhile I check the PSP Association’s website at pspeur.org as they often post great materials for the PSP community. They are based in the UK. Last week, they posted this announcement:

“The PSP Association have released for the first time film footage featuring the late Nigel Dempster talking about his life with Progressive Supranuclar Palsy (PSP). Nigel made the DVD to raise awareness in the hope that it would help people affected by PSP, their families and carers receive better access to care and support, and also highlight the need for more research in to causes, treatments and ultimately a cure.”

Nigel Dempster died in 2007 at age 65 with PSP. He was a well-known newspaper columnist.

You can find the 8-minute video here:
http://www.pspeur.org/news_and_events/video.html

Over several introductory slides, PSP is described as follows:

“Progressive Supranuclear Palsy is a neuro degenerative disease involving the death of neurons (or nerve endings) mainly in the brain stem and basal ganglia. This is the area, above the nuclei (hence supranuclear), which controls balance, movement, vision (particularly upgaze and downgaze), speech and ability to swallow.”

“Average life expectancy is seven years from onset, for the last two of which the patient is often wheelchair or bedbound, tube fed, on ‘around the clock’ care and unable to communicate with the world around although their intellect remains largely intact.”

“Recent research confirms a prevalence of at least 5 per 100,000 of the population, though leading neurologists estimate there could be up to 10,000 people living with PSP in the UK, many misdiagnosed (often as Parkinson’s Disease, which it can mimic in its early stages) and many, particularly amongst the elderly, left undiagnosed.”

“There is today no effective treatment and no cure for this disease, though there is an increasing amount of ongoing research into its cause, treatment and cure, mainly sponsored by the PSP Association.”

Then the story of Nigel Dempster is told. He is shown at the age of 62, with a PSP diagnosis. He had to quit his job at age 61 due to PSP symptoms (backward falls). Some clips of Dempster as a younger man were shown.

It’s quite a powerful video. Dempster’s story is told openly.

Robin

Dr. Litvan Webinar on CBD – Notes

What a shame that this CurePSP webinar with such an expert as Dr. Irene Litvan was held at 6:30am California time with very little advance notice.  I was able to get up in time and noticed one other local support group member on the call.  There was a ten-minute delay in getting started (due to technical problems).  Judging by the few questions that were asked, I don’t think that many people participated in the webinar.

My key takeaway from today’s webinar with Dr. Litvan is that there are three common clinical presentations of CBD:

  • corticobasal syndrome
  • frontotemporal dementia presentation
  • progressive aphasia

Here are the details Dr. Litvan shared on the three presentations:

#1 – corticobasal syndrome:  symptoms are unilateral; parkinsonism (slowness, stiffness); ideomotor apraxia (ie, difficulty using a limb not due to motor of sensory problems); myoclonus (jerky movements); dystonic posture (abnormal posture/contracture); alien limb syndrome (feeling of limb as alien); levitation (uncontrolled elevation of limb); sensory neglect (unaware of sensation only when there is double stimulation).

If presentation #1 includes bilateral parkinsonism, rather than unilateral parkinsonism (which is more common), then the survival time is shorter.

#2 – frontotemporal dementia (FTD) presentation:  the primary symptom is frontal dementia (executive, frontal behavioral and language disturbances).  May or may not have bilateral parkinsonism.

Presentation #2 has a shorter survival time.

#3 – progressive aphasia:  language problems.

Unfortunately, Dr. Litvan did not describe the third presentation at all nor did she give the percentage prevalence of these three common forms.  (Or, if she did, I missed it!)  You’ll have to refer to the notes from Dr. Boeve’s presentation for that.  PPA (primary progressive aphasia) was one of the four disorders Dr. Boeve addressed in his webinar.
One other item mentioned briefly was the use of transcranial ultrasound in Europe (but not in the US) for aiding in the diagnosis of CBD.

In some ways, Dr. Litvan’s presentation was an excerpt of Dr. Boeve’s webinar from several months ago.

If you listened to today’s webinar, please let me know your key takeaways and if your understanding of the three clinical presentations is different from mine (described above).

My notes from Dr. Litvan’s presentation and the very short Q&A follow.  [Editor’s Note:  The webinar recording is no longer on the CurePSP website.]

Robin

————————————————————————-

10/18/20 Webinar
Hosted by CurePSP
Challenges in CBD Management
Irene Litvan, MD, Director, Division of Movement Disorders, University of Louisville, Louisville, KY

Topics addressed in today’s presentation
* Diagnostic Challenges
* Pathogenesis (Cause/s)
* Treatment

CBD Diagnostic Challenges:
* Varied clinical presentations
* No markers for the disease (no blood test)
* Clinical presentations do not always correspond to underlying CBD brain lesions

Early and accurate diagnosis is important for:
* appropriate management
* prognosis
* clinical research

CBD Epidemiologic Aspects
* Underdiagnosed (25-48%).  Fewer than half who have the disease are diagnosed as having it during life.
* Diagnosis:  at half course of the disease
* There are no epidemiologic studies
* Togasaki & Tanner estimated that
(a) 4-6% of patients with parkinsonism have CBD
(b) incidence:  0.6 – 0.9 new patients per 100,000 each year
(c) prevalence:  4.9 – 7.3 per 100,000
* Sources:  Litvan et al, 1996; Wenning et al 1998; Litvan et al 2000; Togasaki and Tanner, 2000

CBD usually has a combination of:
* cognitive features (such as language) and/or
* motor features (such as dystonia or parkinsonism)
* underlying brain lesions of aggregated tau protein.  (This protein is a problem in AD and PSP.)

Classically, CBD presents with the corticobasal syndrome unilaterally:
* parkinsonism (slowness, stiffness)
* ideomotor apraxia (ie, difficulty using a limb not due to motor of sensory problems)
* myoclonus (jerky movements)
* dystonic posture (abnormal posture/contracture)
* alien limb syndrome (feeling of limb as alien)
* levitation (uncontrolled elevation of limb)
* sensory neglect (unaware of sensation only when there is double stimulation)

Underlying brain lesions are heterogeneous in the corticobasal syndrome (CBS):
* 36 people presented with CBS while only 18 had CBD.  6 had PSP; 4 had AD; 3 had CJD; 3 had non-specific changes; 1 had Pick, 1 had Pick/AD.
* Source:  Boeve paper

[Robin’s note:  I will try to find this article.  The one I’ve seen on 36 Mayo cases is on the neuropsychiatric features and all 36 had CBD.]

Alzheimer Disease presenting with CBD:
* visuomotor disturbances
* ideomotor apraxia
* intermanual conflict
* action myoclonus
* left dystonia
* left alien limb syndrome
* aphasia (including difficulty naming objects)
* clock drawing shows hemi-neglect on the right side (of the brain).  So nearly all 12 numbers are on the right side of the clock.  Usually hemi-neglect is on the left side.
* brain autopsy showed:  AD with lesions on one side; this is a rare presentation of AD
* Source:  Chand et al 2006

CBS with underlying TDP-43 proteinopathy with progranulin mutations
* Described by:  Guerreiro et al 2008; Le Ber et al 2008; Lopez de Munain et al 2008; Kelley et al 2007; Spina et al 2007

We need to know the underlying disease in order to find treatments

Clinical diagnoses in 32 consecutive autopsied cases at the Mayo Clinic with brain CBD pathology:
* Initial Clinical Diagnosis:  10 CBD (31%); 6 atypical PD; 2 PSP; 4 Primary progressive aphasia; 6 dementia/AD; 1 DLB; 1 Marchiafava-Bignami disease; 1 Multiple sclerosis; 1 Stroke
* Final Clinical Diagnosis:  18 CBD (50%); 7 PSP (22%); 4 Primary progressive aphasia; 1 AD; 1 DLB; 1 Marchiafava-Bignami disease
* Source:  Boeve et al 2000

[Robin’s note:  I will try to find this article.]

CBD with a frontotemporal dementia (FTD) presentation:
* dementia (executive, frontal behavioral and language disturbances)
* may or may not have bilateral parkinsonism
* Sources:  Bergeron et al 1998; Wenning et al 1998; Litvan et al 1999; Grimes et al 1999

Chart from Murray et al, Neurology 2007;6k:1274-1283 (UPenn study):
Only a few had the final clinical diagnosis of CBD yet the chief complaints were classic CBD (unilateral problems)

CBD presentation matters because survival is different for each presentation.  Shorter survival in CBD when presenting with dementia and/or bilateral parkinsonism.

In summary, diagnostic challenges are:
* No diagnostic biological markers.  Diagnosis still relies on clinical features, confirmed by pathology.
* CBD presents with various clinical presentations including CBS, FTD, and primary progressive aphasia
* The underlying brain lesions of the CBS vary and include CBD, PSP, Alzheimer Disease, FTD, progranulin mutations and Creutzfeld-Jakob Disease

Lab tests that can help some with diagnosis:
* brain MRI in CBD:  might be able to see unilateral atrophy
* transcranial ultrasound:  used in Europe; might help with diagnosis

What goes wrong in the brains of those with CBD?
* Tau aggregates in neurons and glia.
* Astrocytic plaque is indicative of CBD.
* Some key terms:  NFT, tufted astrocyte; threads; coiled body; astrocytic plaque.

Tau is important for the brain cell structure.  In CBD, tau collects in insoluble forms.

What triggers tau to change in CBD?
We don’t really know but question whether the following factors may play a role — genetics; mitochondrial abnormalities (oxidative stress); dietary/environmental toxicants; inflammation; gene/environment combination

What do we know about genetics?
* CBD is very weakly hereditary
* No highly penetrant tau mutations are found
* CBD is associated with H1 haplotypes and H1 hyplotype variants.  Also true in PSP.
* ? Genetic predisposition CBD

From looking at H1/H1, H1/H2, and H2/H2 at PSP, CBD, and in controls, we conclude:
* H1/H1 genotype is (nearly) necessary but far from sufficient for CBD or PSP to develop
H1/H1:  88% of PSP; 84% of CBD; 60% of controls
* Predisposition triggered by other factors of relative rare mutation with low penetrance?
* ? H2/H2 is protective
H2/H2:  0% in PSP; CBD of CBD

Defined tau mutations give rise to tauopathies presenting with PSP/CBD clinical and pathological phenotype.  Example:  PPND (FTDP-17 with N279-K mutation).  Seen in Mr. Wszolek, a Mayo patient.

What do we know about mitochondrial abnormalities and oxidative injury?
* Impaired activity of Complex I of the mitochondrial respiratory chain
* Oxidative stress in CBD.  Cells die.

What do we know about inflammation?
* Activated microglia in the brain in CBD.  Can see this in people who have died and in living people.
* Sources:  Ishizawa and Dickson 2001; Gerhard et al 2004; Henkel et al 2004)

Hypothesized pathways to cell death in CBD and PSP:
* H1 haplotype variants:  do these lead to tau dysfunction?
* Inflammation:  does this lead to tau inclusions, which causes cell death, which leads to more inflammation

CBD Treatment Challenges:
* Difficulties in accurately diagnosing patients during life
* Difficulty in slowing disease progression when the cause is unknown
* Symptomatic treatments have limited efficacy.  We can improve the quality of life.

Symptomatic RX:
Dystonia (limbs) — botox
Dystonia (neck) — botox (avoid when antecollis)
Speech problems — speech therapy; communication aids
Myoclonus — clonazepam, piracetam, valproate
Belpharospasm — botox
Walking — PT; weighted walker
Patient and family support — social services; laymen associations; support RX

In summary:
* CBD has various clinical presentations:  CBS, progressive aphasia (language problem), frontal dementia
* The underlying brain lesions corresponding to these presentations are not always CBD
* Telltale signs (such as focal signs) are present in mid-phase of the disease
* More research is needed to identify:
(a) better ways to diagnose CBD during life;
(b) causes contributing to development;
(c) specific ways to slow the progression of the disease

Questions & Answers:  (all questions were answered by Dr. Litvan)

BRAIN DONATION

In response to someone noting that they’ve made arrangements for brain donation and to someone else to donated her mother’s brain upon death, Dr. Litvan said:  “Thank you for donating your brain!”

RESEARCH

Q:  What are some centers that are studying this disease?

A:  Mayo, UPenn, UCSF

Q:  Are there any known causes?

A:  There is a genetic susceptibility for those with the H1 genotype.  There is a study going on in PSP as to the causes, but not in CBD.  We aren’t studying this in CBD due to the diagnostic challenges.

Q:  Any hope on the horizon for developing biomarkers?

A:  There are multiple groups trying to search for this.  One group is trying to label the protein tau with a PET scan.  This would help with the diagnosis and at least to differentiate from other disorders.  There are a lot of researchers involved.  Of course we want more researchers to be involved and more money.

ABOUT THE THREE PRESENTATIONS AND SYMPTOMS

Q:  Are there typical symptoms to expect as disease progresses?

A:  It depends on the form of the disease.  If the disease presents in a unilateral way, the disease may affect the other side of the body.  If the disease presents with dementia, the disease may affect both sides of the body.

Usually memory is not a major issue in this disease.

Q:  Will dementia appear?

A:  It can happen that dementia never appears.

Q:  Alien limb.

A:  Usually the arms are more effected than the legs.

Q:  My sister has language problems.  What guess can you make about her survival time?

A:  Forms that have speech problems progress slowly.  Those with CBS have a survival time of 5-8 years.

Q:  In PSP, there are two common forms.  Do you guess that the same two forms appear in CBD?

A:  Yes, this is exactly what I said.  There are three forms in CBD.

Q:  Are itching and constipation part of CBD?

A:  Itching is not part of CBD.  Constipation is part of many neurodegenerative diseases.

ABOUT TREATMENTS

Q:  If CBD presents as AD, can AD drugs help?

A:  We don’t have AD drugs that can help with CBD symptoms.

Q:  Would CBD patients benefit from DBS (deep brain stimulation)?

A:  No.  People with PD who get DBS do improve because they respond to dopamine medication.  If you don’t respond to levodopa/carbidopa, then you won’t respond to DBS.

New 6-Page Pamphlet on MSA (Parkinson Society Canada)

Pam Bower, one of the moderators of the ShyDrager Yahoo!Group, posted about this pamphlet today.  I think it’s one of the best documents I’ve seen for laypeople.  It’s very similar to the brochure available from the Multiple System Atrophy Trust (UK).

You can find this MSA pamphlet on the Parkinson Society Canada website:

http://www.parkinson.ca/wp-content/uploads/Multiple-System-Atrophy-English.pdf

Pam indicates “This was created with much input from MSA patient Paul Walsh in Toronto. He has lobbied Parkinson Society Canada vehemently to get more resources available for MSA patients and families.”

I’ve copied some of the text below.  I encourage you to check-out the link because the layout is nice and there’s a diagram of the brain.

Robin

————————————————————————-

Excerpts from:

Multiple System Atrophy
Parkinson Society Canada
First Published 2010 (?)

Multiple System Atrophy falls into the category of Atypical Parkinsonism, or Parkinson plus syndrome, a group of diseases linked to a lack of dopamine in the brain. Dopamine controls movement. While Parkinson’s is the most common Parkinsonism, approximately 20 percent of people will be diagnosed with another Parkinson’s-like condition.
What is Multiple System Atrophy?
Multiple System Atrophy (MSA)* is a progressive brain disorder caused by loss of nerve cells in specific areas of the brain. This loss causes problems with movement, balance and autonomic functions of the body. (Autonomic functions are body functions that occur automatically, such as bladder control.)

*Terminology
MSA includes conditions that were previously known individually as Shy-Drager syndrome, striatonigral degeneration and sporadic olivopontocerebellar atrophy. Researchers have learned that there is a common underlying cause in all three disorders, so they are now referred to as MSA.

Who gets MSA?
MSA usually starts between the ages of 50-60 years, although it can affect people younger and older than this. The average age of onset is 54. MSA is a disorder that occurs randomly in the general population and is considered rare (4 – 5 in 100,000). There is a slightly higher incidence in men.

Which parts of the brain are affected in Multiple System Atrophy?
In MSA, cells are damaged in different areas of the brain which control a variety of body functions. The three areas affected are the basal ganglia, cerebellum and brain stem.

In MSA, brain cells in the affected areas shrink (atrophy). This can sometimes be seen on MRI scans. When brain tissue is examined under a microscope, structures called glial inclusion bodies can be seen; they contain a protein called alpha-synuclein. It is the presence of these inclusion bodies in the movement, balance and autonomic control centres of the brain that confirms a diagnosis of MSA.

What are the symptoms of MSA?
Parkinson symptoms – relating to slowness and stiffness of movement
• feeling slow and stiff when moving
• difficulty turning in bed
• difficulty starting to move
• difficulty fastening buttons on a shirt or blouse

Cerebellar symptoms – relating to co-ordinating movement and balance
• feeling clumsy, dropping things
• feeling unsteady in crowds
• inability to balance without support
• difficulty writing
• slurred speech

Autonomic symptoms – relating to automatic body functions
• bladder problems
• erectile dysfunction
• dizziness or fainting (blood pressure problems)
• constipation
• cold hands and feet
• inability to sweat

Other symptoms may include:
• weakness in arms and legs
• heightened emotional response (laughing or crying disproportionate to the situation)
• restless sleep
• nightmares
• noisy breathing during the day, snoring at night
• unintentional sighing
• swallowing problems, difficulty chewing, choking episodes
• weak, quiet voice
• cognitive problems, slowness in thinking, difficulty with multi-tasking

How is Multiple System Atrophy treated?
Currently, there is no treatment to slow the progression of the disease. The complex nature of MSA means you should see a neurologist who specializes in movement disorders. It is important to look at and treat each symptom separately in order to maintain daily activities and quality of life. Often a multidisciplinary team, including a neurologist, social
worker, speech language pathologist, physiotherapist, urologist, clinical nurse and family doctor will be involved.

Suggestions that may be useful for managing symptoms of Multiple System Atrophy

Movement: Drugs to help stiffness and slowness are the same drugs used in Parkinson’s disease. Medications in MSA may be effective for some people and not effective for others.

A drop in blood pressure (orthostatic hypotension): A drop in blood pressure when standing can cause dizziness, lightheadedness, fainting, or blurred vision. It may be treated with drugs that raise blood pressure. Some people find getting up slowly from a chair can reduce fainting spells. Raising the head of the bed can make it easier to get out of bed. Increasing salt in the diet and wearing pressure stockings can also help. It is important to have regular blood pressure checks.

Erectile dysfunction: Medications or penile implants may help with impotence.

Breathing: If you have problems with snoring or sleep apnea (you stop breathing while asleep), you should report this to your doctor. A breathing tube may need to be inserted.

Spasms: Medications may help treat spasms.

Constipation: Increased dietary fiber and plenty of fluids on a daily basis. Taking laxatives may relieve constipation.

Swallowing: To improve swallowing and reduce risk of choking, food may need to be pureed. If swallowing becomes difficult, a tube may be surgically inserted into the stomach.

Muscle coordination: A routine exercise program with stretching, range of movement and strength building is very important. It helps keep muscles strong and allows for safer mobility.

Urinary retention: Medications can reduce urgency and frequency problems, improve tone of bladder muscles or reduce production of urine overnight. You should take care to avoid infection and seek treatment. Consulting a urologist may help.

What do care partners need to know?
If you are a care partner or family member, MSA will affect your life, too. Learn as much as you can. Care partners often believe they will remain healthy and will always able to provide care. However, care partners often burn out and become ill. Take care of yourself. Maintain a healthy lifestyle, including exercise and good nutrition. Consider using community services. Have an emergency plan in case you become ill. Build a network of support.

CBS and PSP listed in “Frontotemporal Disorders” booklet (NIH)

PSP and CBD folks –

A great new publication, Frontotemporal Disorders: Information for Patients, Families and Caregivers,has just been released by the National Institute on Aging and NIH.  It is free and available by download or in hardcopy via the following link:  [Editor’s note: link modified in 2017]

www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/introduction

Jennifer Merrilees, RN, from UCSF’s MAC, assisted in the creation of this booklet along with people from Northwestern University and the Association for Frontotemporal Dementias.

I’ve copied below some excerpts that are on terminology, CBS, and PSP.  I especially liked the terminology section (“FTD? FTLD? Understanding Terms”) and the section on symptoms (with a breakdown by type of symptom — behavioral, language, emotional, and movement).  Of course not all of the symptoms listed occur in each disorder nor in each person with a given disorder.

Robin

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www.nia.nih.gov/alzheimers/publication/frontotemporal-disorders/introduction

Excerpts from

Frontotemporal Disorders: Information for Patients, Families, and Caregivers
NIA and NIH
Published October 2010

FTD? FTLD? Understanding Terms
One of the challenges shared by patients, families, clinicians, and researchers is confusion about how to classify and label frontotemporal disorders. A diagnosis by one doctor may be called something else by a second, and the same condition or syndrome referred to by another name by a pathologist who examines the brain after death.

For many years, scientists and physicians used the term frontotemporal dementia (FTD) to describe this group of illnesses. After  further research, FTD is now understood to be just one of several possible variations and is more precisely called behavioral variant frontotemporal dementia, or bvFTD.

This booklet uses the term frontotemporal disorders to refer to changes in behavior and thinking that are caused by underlying brain diseases collectively called frontotemporal lobar degeneration (FTLD). FTLD is not a single brain disease but rather a family of neurodegenerative diseases, any one of which can cause a frontotemporal disorder (see “Causes,” page 11). Frontotemporal disorders are diagnosed by physicians and psychologists based on a person’s symptoms. FTLD can be identified definitively only by brain autopsy after death.

Types of Frontotemporal Disorders
Frontotemporal disorders can be grouped into three types, defined by the earliest symptoms physicians identify when they examine patients.

• Progressive behavior/personality decline—characterized by changes in personality, behavior, emotions, and judgment (e.g., behavioral variant frontotemporal dementia).

• Progressive language decline—marked by early changes in language ability, including speaking, understanding, reading, and writing (e.g., primary progressive aphasia).

• Progressive motor decline—characterized by various difficulties with physical movement, including shaking, difficulty walking, frequent falls, and poor coordination.

Types of Frontotemporal Disorders
Diagnostic Terms and Main Early Symptoms
Progressive Motor Decline

Corticobasal syndrome (CBS)
• Muscle rigidity
• Difficulty closing buttons, operating simple appliances
• Language or spatial orientation problems

Progressive supranuclear palsy (PSP)
• Progressive problems with balance and walking
• Slow movement, falling, body stiffness
• Restricted eye movements

FTD with parkinsonism
• Movement problems similar to Parkinson’s disease, such as slowed movement and stiffness
• Changes in behavior or language

FTD with amyotrophic lateral sclerosis (FTD-ALS)
• Combination of FTD and ALS (Lou Gehrig’s disease)
• Changes in behavior and/or language
• Muscle weakness, shrinkage, jerking

Movement Disorders
Two rare neurological disorders associated with FTLD, corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP), occur when the nerves attached to muscles malfunction and cause problems with movement. The disorders may affect thinking and language abilities, too.

CBS is caused by corticobasal degeneration—gradual atrophy and loss of nerve cells in specific parts of the brain. This degeneration causes progressive loss of movement and muscle rigidity, typically beginning around age 60. Symptoms may appear first on one side of the body, but eventually both sides are affected. Occasionally, a person with CBS first has language problems or trouble orienting objects in space and later develops movement symptoms.

PSP causes serious problems with balance and walking. People with the disorder typically move slowly, fall, and have body stiffness, especially in the neck and upper body—symptoms similar to those of Parkinson’s disease. A hallmark sign of PSP is trouble with eye movements, such as involuntary closing of the eyelids, difficulty opening and closing the eyes, trouble looking way up and way down, and limited blinking. These symptoms may give the face a fixed stare. Behavior problems can also develop.

Other movement-related frontotemporal disorders include frontotemporal dementia with parkinsonism and  frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).

Frontotemporal dementia with parkinsonism is an inherited disease caused by a genetic mutation. Symptoms include movement problems similar to those of Parkinson’s disease, such as slowed movement, stiffness, and balance problems, and changes in behavior or language.

FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms include the behavioral and/or language changes seen in bvFTD as well as the muscle weakness, shrinking, and jerking seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time. Mutations in certain genes have been found in some patients with FTD-ALS.

Confusing symptoms
Carol had a tingling sensation and numbness in her upper right arm. Then her arm became stiff. She had to change from cursive handwriting to printing. Carol, 61, told her doctor that she had trouble getting her thoughts out and  described her speech as “stumbling.” She had increasing trouble talking but could still understand others. Eventually, she was diagnosed with CBS.

Trouble with walking
For a year and a half, John had trouble walking and fell several times. He also had trouble concentrating. He couldn’t read because the words merged together on the page. John, 73, also seemed less interested in social activities and projects around the house. His wife noticed that he was more irritable than usual and sometimes said  uncharacteristically inappropriate things. John’s primary care doctor did several tests, then referred him to a  neurologist, who diagnosed PSP.

Common Symptoms
Symptoms of frontotemporal disorders vary from person to person and from one stage of the disease to the next as different parts of the frontal and temporal lobes are affected. In general, changes in the frontal lobe are associated with behavioral symptoms, while changes in the temporal lobe lead to language and emotional disorders.

Symptoms are often misunderstood. Family members and friends may think that a person is misbehaving, leading to anger and conflict. For example, a person with bvFTD may neglect personal hygiene or start shoplifting. It is important to understand that people with these disorders cannot control their behaviors and other symptoms.

Behavioral Symptoms
• Problems with executive functioning—Problems with planning and sequencing (thinking through which steps come first, second, third, and so on), prioritizing (doing more important activities first and less important activities last), multitasking (shifting from one activity to another as needed), and self-monitoring and correcting behavior.

• Perseveration—A tendency to repeat the same activity or to say the same word over and over, even when it no longer makes sense.

• Social disinhibition—Acting impulsively without considering how others perceive the behavior. For example, a person might hum at a business meeting or laugh at a funeral.

• Compulsive eating—Gorging on food, especially starchy foods like bread and cookies. People may take food from other people’s plates.

• Utilization behavior—Difficulty resisting impulses to use or touch objects that one can see and reach. For example, a person picks up the telephone receiver while walking past it when the phone is not ringing and the person does not intend to place a call.

Language Symptoms
• Aphasia—A language disorder in which the ability to use or understand words is impaired but the physical ability to speak properly is normal.

• Dysarthria—A language disorder in which the physical ability to speak properly is impaired (e.g., slurring) but the message is normal.

Emotional Symptoms
• Apathy—A lack of interest, drive, or initiative. Apathy is often confused with depression, but people with apathy may
not be sad. They often have trouble starting activities but can participate if others do the planning.

• Emotional changes—Emotions are flat, exaggerated, or improper. Emotions may seem completely disconnected from a situation or are expressed at the wrong times or in the wrong circumstances. For example, a person may laugh at sad news.

• Social-interpersonal changes—Difficulty “reading” social signals, such as facial expressions, and understanding personal relationships. People may lack empathy—the ability to understand how others are feeling—making them seem indifferent, uncaring, or selfish. For example, the person may show no emotional reaction to illnesses or accidents that occur to family members.

• Changes in insight—An inability to understand changes in one’s condition and how they affect others. This lack of awareness is hard for caregivers to deal with because the person may reject efforts to help.

Movement Symptoms
• Dystonia—Abnormal postures of the hands or feet. A limb may be bent stiffly or not used when performing activities that are normally done with two hands.

• Gait disorder—Abnormalities in walking, such as walking with a shuffle, sometimes with frequent falls.

• Tremor—Shakiness, usually of the hands.

• Clumsiness—Dropping of small objects or difficulty manipulating small items like buttons or screws.

Managing Movement Problems
No treatment can slow down or stop frontotemporal-related movement disorders, though medications and physical and occupational therapy may provide modest relief.

For people with corticobasal syndrome (CBS), movement difficulties are sometimes treated with medications for depression, Alzheimer’s disease, or Parkinson’s disease. But these medicines do not often lead to much  improvement. Physical and occupational therapy can help a person with CBS move more easily. Speech therapy may delay the worsening of language symptoms.

For people with progressive supranuclear palsy (PSP), sometimes Parkinson’s disease drugs provide temporary relief for slowness, stiffness, and balance problems. Although physical therapy generally does not help, exercises can keep the joints limber, and weighted walking aids—such as a walker with sandbags over the lower front rung—can help maintain balance. Speech, vision, and swallowing difficulties usually do not respond to any drug treatment. Antidepressants have shown modest success. For people with trouble looking up or down, bifocals or special glasses called prisms are sometimes prescribed. …

For any movement disorder caused by FTLD, a team of experts can help patients and their families address difficult medical and caregiving issues. Physicians, nurses, social workers, and physical, occupational, and speech therapists who are familiar with frontotemporal disorders can ensure that people with movement disorders get appropriate medical treatment and that their caregivers can help them live as well as possible.

Caring for a Person with a Frontotemporal Disorder
In addition to managing the medical and day-to-day care of people with frontotemporal disorders, caregivers can face a host of other challenges. These challenges may include changing family relationships, loss of work, poor health, decisions about long-term care, and end-of-life concerns.

Family Issues
People with frontotemporal disorders and their families often must cope with changing relationships, especially as symptoms get worse. …

Work Issues
Frontotemporal disorders disrupt basic work skills, such as organizing, planning, and following through on tasks. Activities that were easy before the illness began might take much longer or become impossible. People lose their jobs because they can no longer perform them. As a result, the caregiver might need to take a second job to make ends meet—or reduce hours or even quit work to provide care and run the household.

Caregiver Health and Support
Caring for someone with a frontotemporal disorder can be very hard, both physically and emotionally. To stay healthy, caregivers can do the following:

• Get regular health care.

• Ask family and friends for help with child care, errands, and other tasks.

• Spend time doing enjoyable activities, away from the demands of caregiving. Arrange for respite care—short-term caregiving services that give the regular caregiver a break—or take the person to an adult day care center, a safe, supervised environment for adults with dementia or other disabilities.

• Join a support group for caregivers of people with frontotemporal disorders. Such groups allow caregivers to learn coping strategies and share feelings with others in the same position.

The organizations listed in the Resources section of this book (starting on page 27) can help with information about caregiver services and support.

Long-Term Care
For many caregivers, there comes a point when they can no longer take care of the person with a frontotemporal disorder without help. The caregiving demands are simply too great, perhaps requiring around-the-clock care. As the disease progresses, caregivers may want to get home health care services or look for a residential care facility, such  as a group home, assisted living facility, or nursing home. The decision to move the person with a frontotemporal disorder to a care facility can be difficult, but it can also give caregivers peace of mind to know that the person is safe
and getting good care.

End-of-Life Concerns
People with frontotemporal disorders typically live 6 to 8 years with their conditions, sometimes longer, sometimes less. Most people die of problems related to advanced disease. For example, as movement skills decline, a person can have trouble swallowing, leading to aspiration pneumonia, in which food or fluid gets into the lungs and causes infection. People with balance problems may fall and seriously injure themselves.

It is difficult, but important, to plan for the end of life. Legal documents, such as a will and durable powers of attorney for health care and finances, should be created or updated as soon as possible after a diagnosis of bvFTD, PPA, or a related disorder. Early on, many people can understand and participate in legal decisions. But as their illness progresses, it becomes harder to make such decisions.

A physician who knows about frontotemporal disorders can help determine the person’s mental capacity. An attorney who specializes in elder law, disabilities, or estate planning can provide legal advice, prepare documents, and make financial arrangements for the caregiving spouse or partner and dependent children. If necessary, the person’s access to finances can be reduced or eliminated.

Resources

Federal Government

National Institute on Aging
www.nia.nih.gov/Frontotemporal

National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov

MedlinePlus
www.medlineplus.gov

Organizations

Association for Frontotemporal Dementias
www.ftd-picks.org

ALS Association
www.alsa.org

CurePSP
www.curepsp.org

National Aphasia Association
www.aphasia.org

WE MOVE
www.wemove.org

Caregiver Services

Eldercare Locator
www.eldercare.gov

Family Caregiver Alliance
www.caregiver.org

National Academy of Elder Law Attorneys, Inc.
www.naela.org

Caregiver Support

FTD Support Forum
www.ftdsupportforum.com

PPA Support Group
www.groups.yahoo.com/group/PPA-support

Well Spouse Association
www.wellspouse.org

Diagnosis, Treatment, and Research Centers  [many are listed, including…]

University of California, San Francisco
Memory and Aging Center
San Francisco, CA
1-415-476-6880
www.memory.ucsf.edu/ftd

Article on Stress and CBD (in new CBD Newsletter)

CBD folks –

The long-time owner of the Washington Wizards NBA basketball franchise was Abe Pollin. He died in late 2009. His clinical diagnosis was CBD, and I heard from someone who is in the PSP community in the Washington, D.C. area that the diagnosis was confirmed upon brain autopsy.

Irene Litvan, MD, is one of this country’s leading experts on CBD. Several years ago she started the Litvan Neurological Research Foundation, named in memory of her father. Abe Pollin donated to LNRF, which led to the creation of the Center of Excellence in Corticobasal Degeneration. This COE is presented located at the University of Louisville in KY, which is where Dr. Litvan currently works.

The Center is working to establish its own website — cbdcenter.org (under construction).

I was digging around on the web tonight and found that the Center has published its inaugural newsletter in Spring 2010. Here’s a link to that newsletter:
http://www.litvanfoundation.com/images/ … 202010.pdf

There are interesting articles on:
* the Center’s creation
* profile of Abe Pollin
* CBD diagnostic criteria being reformulated
* profile of Dr. Irene Litvan
* stress and CBD
* fall prevention

The remainder of this post is a copy of the “Stress and Corticobasal Degeneration” article.

Robin

Stress and Corticobasal Degeneration
CBD Newsletter
Volume 1, Issue 1, Spring 2010
The Center of Excellence in Corticobasal Degeneration

It’s no secret that your mental state can have an effect on your physical well-being. Dealing with the effects of a disease like CBD, either as a patient or as a caregiver, is a very real and perfectly rational cause of stress, but too much stress can have a severe negative effect on your health. This article explores the nature of stress and suggests some methods of reducing its impact on your life.

What is Stress?
Stress is your body’s natural response to a threat. When you are faced with a physical threat, your body releases hormones and adrenaline that help you prepare to either fight the threat, or flee from it. Most commonly, when we refer to “stress” in our daily lives, we are not referring to a physical threat, but rather some perceived psychological or emotional threat. The illness of yourself or a loved one is a typical cause of psychological stress.

In small doses, stress can be beneficial, it can help you to overcome challenges and to excel by increasing your performance capacity. If stress continues for too long, however, it can begin to have a severe toll on your body, in fact chronic stress disrupts nearly every system in your body! Typical effects of chronic stress include pain, heart disease, liver problems, digestive problems, sleep disturbances, and autoimmune diseases, among others.

Stress & CBD
In terms of corticobasal degeneration, increased stress is a normal reaction whether you are the pa-tient diagnosed with the disease or the spouse or loved one caring for a family member with the diagnosis. CBD is a chronic and debilitating disease for which we do not know a cause or an effective course of treatment. You would be very unique if the presence of CBD in your life, in any capacity, didn’t cause you stress.

Because of the lack of knowledge about CBD, its chronic nature, and the level of care needed to maintain patients diagnosed with the disease, CBD-caused stress very typically drifts from the “normal” stress range into the “unhealthy” stress range. So, what can you do to prevent a CBD induced stress overload?

Fighting CBD Stress
Like a gladiator heading to the arena, the first order of business in dealing with CBD stress is to arm yourself appropriately. In the fight against CBD induced stress, the weapon of choice is knowledge.

Yes, its true that very little is known about the causes of CBD or how to treat the disease or even prevent symptoms, but as a patient or as a caregiver, you should still arm yourself with the best knowledge available. Learning all you can about the progression of disease symptoms, while sobering, can help reduce anxiety over what the future will bring. Learning about adaptive devices and techniques that can help CBD patients retain their independence can ease feelings of helplessness and reduce caregiver strain. In general, the more you know, the better prepared you will be to deal with the future, and the better prepared you feel, the less stress you will experience.

Another key way to reduce stress is to build a strong support group. The more friends and family members you know you can count on, the less stressed you will feel. Some of us are blessed with good support networks, others not so much. If you find yourself dealing with your situation on your own, then its time to reach out for help. Old friends, co-workers, family members you haven’t talked to for a while, all can be good general sources of support.

For CBD patients and their caregivers, a number of support groups exist or are being formed that can put you in contact with other patients and caregivers who are dealing with the same issues. Regardless of the form your support group takes, it is a very important tool in reducing your stress load.

Other ways to reduce stress or better prepare your body to deal with the effects of stress include exercise, yoga, meditation, and deep breathing exercises. These activities help our bodies to initiate a relaxation response, the opposite of a stress response.

If you feel that you are slipping into despair or depression, seek out the help of a trained therapist.

For more information on decreasing stress, a number of books have been published on the subject and can be found at your local library, book store or online outlet.

The Center of Excellence in Corticobasal Degeneration is committed to helping patients, their families, and caregivers deal with stress due to CBD. In the near future we will be providing support groups and stress-related work-shops in the Louisville, KY area, as well as coordinating with other providers of similar services throughout the United States to assist individuals in finding re-sources near their location.

Please visit www.cbdcenter.org for more details.

“Bathroom Safety for Older People”

This post will be of interest to those looking to make a bathroom safer.

I ran across a webpage — with great figures — that addresses safety associated with the bath tub, showers, and toilets.

Three short personal notes about safety equipment in or modifications to a bathroom….

When we were remodeling our house ten years ago, we had my husband’s elderly aunt in mind when we installed grab bars in the shower.  We bought the grab bars and the contractor installed them in places he thought they should go.  We were disheartened to learn from the aunt that they were installed in the wrong places to be useful!  It’s important to consult with an occupational therapist or a contractor who specializes in aging-in-place about this sort of equipment!

Second was how we handled toilet safety for my father.  My father was tall and weighed 220 pounds so we needed a grab bar that went into the floor.  We liked the sturdiness of the L-bar (or P-bar) shown in Figure 14 for my father.  We bought a stainless steel grab bar from Adaptive Access (adaptiveaccess.com), and then found a welder to cut the stainless steel bar to the height and length we wanted.  We ended up running two identical bars, parallel to each other, on either side of the toilet.  This really helped my father to sit down on the toilet and stand up from the toilet.

Third is about a portable bidet.  This type of equipment is mentioned below.  We’ve had several local support group members use portable bidets for cleaning their family members’ back sides, and they all recommend the bidet.

So here’s what the experts say:

extension.missouri.edu/publications/DisplayPub.aspx?P=GH7060

Bathroom Safety for Older People
by Anna Cathryn Yost, Department of Consumer and Family Economics, and James Martinr, Physical Therapy Education
Published by the University of Missouri Extension
Reviewed April 1998

Robin

Dementia Coping Skills & Behavior Management

This post will be of interest to those of you dealing with dementia.

I ran across this website today – Mental Help Net (mentalhelp.net). Much of the content is written by two psychologists. This post is about a section called “Dementia Coping Skills & Behavior Management.” Here is a list of the seven topics in this section along with a link directly to a webpage for each topic:

Coping Skills for Those with Dementia
http://www.mentalhelp.net/poc/view_doc. … c&id=15340

Coping Skills for Dementia Caregivers
http://www.mentalhelp.net/poc/view_doc. … c&id=15341

Services for Dementia Caregivers
http://www.mentalhelp.net/poc/view_doc. … c&id=15342

Communication Tips for Dementia Caregivers
http://www.mentalhelp.net/poc/view_doc. … c&id=15343

Behavior Management for Dementia Caregivers
http://www.mentalhelp.net/poc/view_doc. … c&id=15344
This addresses the A-B-C behavior chain — antecedent, behavior, and consequence. These three components are often assessed in classes about dealing with dementia-related behavior.

Behavior Management for Dementia Caregivers Continued
http://www.mentalhelp.net/poc/view_doc. … c&id=15345
This section explores the A-B-C behavior chain further with some examples.

Tips for Dealing with Specific Dementia Problematic Behaviors
http://www.mentalhelp.net/poc/view_doc. … c&id=15346
The nine problematic behaviors addressed include agitation, aggression, repetition (eg, repeating the same question over and over), hallucinations, suspicion, apathy, confusion, sundowning, and wandering.

Copied below is the second topic, Coping Skills for Dementia Caregivers, the fourth topic, Communication Tips for Dementia Caregivers, and the last topic, Tips for Dealing with Specific Dementia Problematic Behaviors.

Note that the Mental Help Net website contains advertisements. The organization supplies content on a variety of topics to various affiliated websites.

Robin

————-

Coping Skills for Dementia Caregivers
Carrie Hill, Ph.D. and Natalie Reiss, Ph.D.
Updated: Jul 3rd 2008
Mental Help Net

Not only is a diagnosis of dementia difficult for the person with the disease; it also poses significant changes and sources of stress for those who care for the person with the diagnosis. Most individuals with dementia have a primary caregiver – usually a spouse, partner, or adult child – and some people have secondary caregivers, such as other relatives or neighbors. The following coping skills are useful to both primary and secondary caregivers, but primary caregivers will find them the most relevant due to the way that their lives have changed.

* Learn as much as you can about the disease as soon as possible. Although you may be reluctant to do so, you will thank yourself down the road. You will be better prepared to handle the variety of challenges associated with dementia if you know what to expect and have some ideas about how other people have handled similar challenges.

* Adjust your expectations by imagining what your loved one is going through. People do not want to have dementia; it is not their fault that they developed the disease. Most of all, try to remember that the person’s brain is not working properly. He or she is not engaging in this behavior on purpose, or to be difficult or annoying. Caregiving is a meditation on patience and understanding.

* Attend to your own physical and mental health. Make time for sleep, exercise, proper nutrition, socializing, and private time. If you are run down, exhausted, socially isolated, etc., you run the risk of becoming physically ill and overwhelmed and/or depressed yourself. You cannot help someone else without helping yourself first.

* Keep the lines of communication open with family and friends. Share your feelings about how you are doing in addition to how your loved one is doing. Those close to you most likely want to know what they can do to help, but they may not ask because they feel uncomfortable doing so, or do not know what to say. Even though it may be difficult, ASK FOR HELP when you need it. If people do not know how to help, think of some specific tasks that they can do. Try to tailor the request to the person offering the help. For instance, if neighbors are uncomfortable being around a person with dementia, ask them for help with getting groceries or running other errands, rather than pressing them to provide respite services (when someone spends time with your loved one so you can have a break). Similarly, if a friend enjoys a more “hands on” role, allow him or her to provide you with respite time to get away for a few hours or days.

* Make sure that legal and financial issues are in order, and include your loved one in the decision-making process as much as possible. Ideally, your loved one will initiate this process in the early stage of the disease while his or her decision-making abilities are still intact.

* Take an active role in your loved one’s health care. Form a working alliance with a physician and other professionals who are experienced in issues related to dementia and other cognitive disorders.

* Plan activities with your loved one that you both enjoy and that can be adapted to the person’s current level of functioning. Good examples of adaptable activities include walking, taking a drive, gardening, doing household chores, looking at pictures or magazines, watching movies or television shows, baking, listening to music, singing, dancing, and playing cards or other games.
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If your loved one can no longer participate in an activity like he or she used to, remember to relax the rules and definitions of what is “appropriate.” For instance, if the person wants to help with household chores, let him fold a basket of laundry. Do not worry about whether things are folded correctly (or that the task is completed quickly). What is important is that he is participating in an activity that makes him feel useful. Likewise, if your relative enjoys playing cards but cannot follow the rules of poker anymore, try sorting a deck of cards into colors and suits instead.

* Monitor yourself for signs of caregiver burnout, such as anger, anxiety, irritability, depression (e.g., a loss of interest in normally fun activities, or feelings of worthlessness, guilt, or suicidality), social withdrawal, denial about your loved one’s diagnosis, denial about your role as a caregiver (e.g., you feel that your life has not changed dramatically even though you have left your job or given up most of your previous activities), extreme fatigue, sleep problems, and/or health problems such as ulcers, migraines, or high blood pressure. To prevent or address caregiver burnout, try joining a caregiver support group, which can provide education, emotional support, and connections to local resources that can help you meet your caregiving responsibilities. See the list of web resources at the end of this discussion for more information about support groups.

* If self-coping methods are not working, seek mental health care from a professional who has expertise in addressing caregiver burnout, depression, and grief. If you are having difficulty dealing with some of your loved one’s behaviors (e.g., suspicion, wandering), seek out mental health clinicians with expertise in behavior management techniques for individuals with dementia.

———–

Communication Tips for Dementia Caregivers
Carrie Hill, Ph.D. and Natalie Reiss, Ph.D.
Updated: Jul 3rd 2008
Mental Help Net

As dementia progresses, it becomes more difficult for individuals to express their thoughts and feelings, as well as to understand what is being communicated to them. In order to enhance communication with your loved one, try the following tips:

* Speak slowly, at a normal level (not too loud), using a low-pitched (rather than a “Minnie Mouse”) voice. In addition, try to face the person when you are speaking to him or her (rather than carrying on a conversation out of the person’s line of sight). Speaking rapidly, loudly, or in a high-pitched voice can be overwhelming or upsetting for someone with dementia.

* Use short, familiar words and simple sentences that clearly express what you want to say.

* Allow your loved one sufficient time to respond. If he or she does not respond, it is okay to repeat your question using the same wording as before. If you ask the question in a different way, your relative might think that you are asking a different question and become overwhelmed.

* Ask only one question or give one direction at a time. Although it seems as though this will take longer than combining questions or instructions, it will actually save time because the person with dementia is less likely to become overwhelmed or confused.

* Give positive instructions; avoid saying “don’t” or giving negative commands. For example, instead of saying, “Don’t go in that room,” try saying, “Let’s go over here.”

* Avoid questions that require a lot of thought, memory, and words, or that put the person “on the spot.” For example, asking a person with dementia, “Can you tell your brother about the movie we saw this afternoon?” may be overwhelming, because he or she will either be unable to remember the movie or unable to explain the plot.

* Avoid instructions that require your loved one to remember more than one action at a time. For instance, tooth brushing is made up of many smaller tasks, such as picking up the toothpaste, taking off the cap, picking up the toothbrush, putting toothpaste on the brush, etc. Dementia affects the brain in such a way that a person may not remember every step or forget the correct order of steps in order to complete a task. It will save both of you time and frustration in the long run if you break tasks down into smaller steps that are more manageable for your loved one.

* Avoid arguing or disagreeing with your loved one. In order for two people to “successfully” have an argument, both parties must be able to use reason and logic. Because dementia affects reason and logic, arguing or disagreeing with someone with dementia is futile. For example, a person with dementia often becomes confused about the past and think that someone who has died is still alive. It is not helpful – and could actually be emotionally damaging – to try to convince the person with dementia that someone is actually dead. Instead, use the validation techniques described next.

* Validation techniques involve addressing the feelings of a person with dementia rather than focusing on the facts or accuracy of what the person is saying. For instance, if someone with dementia thinks that the year is 1970, and this is not harmful or hurting anyone else, let it go. Avoid trying to “reorient” the person to the correct year. Instead, try to identify feelings related to 1970. Is the person reminiscing about a pleasant time in his or her life? Tap into this pleasant feeling by asking more about it.

On the other hand, is the person upset, thinking she needs to go “home” because her mother has dinner on the table? Instead of trying to convince her that her mother is dead, try to discern the feeling behind the statement. Does she miss her childhood home? Her mother’s cooking? Ask her what her favorite meal was, or ask her about her old neighborhood. Chances are that she will start talking about old memories and forget about being upset. These are ways of validating the person’s feelings rather than trying to reorient the person to reality.

* If your loved one is upset, and validation or other techniques do not work, remove him or her from the upsetting situation slowly and quietly.

* Redirection techniques can work wonders when other communication techniques are not helpful. For instance, if someone with dementia is upset or preoccupied, try introducing a new activity that you know he or she enjoys, such as listening to music or looking at a photo album. Because of the short-term memory loss associated with dementia, the person will often move on to the new activity and forget why he or she was upset in the first place.

* Communication is often easier if the environment is calm, simple, safe, and quiet. For instance, if you are trying to talk to your loved one and the television is on, the dishwasher is running, and grandchildren are running through the house, your loved one might be distracted or agitated by all of the activity and noise. Try keeping things more peaceful and simple. Along those same lines, try not to have too many people visit your loved one at once.

Keeping a consistent schedule or daily routine will help keep your relative more relaxed; predictability can be calming and soothing. It can also be helpful to add memory cues around the home, such as pictures of key family members and friends with their phone numbers. You can also add picture labels on drawers or closets to identify where different items are kept. Reducing clutter and removing rugs or wires that could be tripping hazards will reduce your loved one’s risk of falling. Finally, keep the home well-lit using soft natural light. Avoid fluorescent lights, which can agitate people with dementia.

———-

Tips for Dealing with Specific Dementia Problematic Behaviors
Carrie Hill, Ph.D. and Natalie Reiss, Ph.D.
Updated: Jul 3rd 2008
Mental Help Net

* Agitation – Individuals with dementia frequently become restless, anxious, or upset (when filling out a behavior chart, it is helpful to use specific, concrete words that clearly describe what your loved one is doing so you can truly track whether your changes work to decrease the behavior). To decrease agitation, listen to the person as he or she expresses her frustration to get a clue about what kind of antecedent is a trigger; try to eliminate and/or decrease triggers as much as possible. Pain, discomfort (being too hot, too cold, hungry, needing to use the bathroom, etc.), frustration, and overstimulation are all common triggers of agitation. It is also very important to examine your behavior in response to the person. If you are not already doing so, reassure the person that you are there to provide assistance and comfort. If it seems like the person needs something to do, try redirection to an enjoyable activity.

* Aggression – Aggressive behavior can include shouting, cornering someone, raising a hand to someone, or actually pushing or hitting. Unfortunately, aggression among people with dementia can happen suddenly and seemingly without warning. Try to identify what triggered the aggression so that the antecedent can be eliminated or modified as soon as possible. As difficult as it may be, react in a calm, reassuring way and focus on the person’s feelings. Reduce environmental distractions as much as possible, such as loud noises or potentially frightening shadows or movements. Redirecting an aggressive person to an enjoyable activity can be remarkably effective.

* Repetition – People with dementia often repeat a word, question, or action over and over again (e.g., saying “What are we doing today?” repeatedly). This behavior is usually harmless, but it can be unnerving and annoying for those who are caring for the person. Repetitive behavior is usually a sign of insecurity, since people with dementia are often looking for something comfortable and familiar – something over which they have some degree of control. To address repetition, look for a specific antecedent or reason for the repetition as well as for the emotion behind it. This can reduce your chances of responding impatiently with the person. If the repetition is an action, try turning it into an activity that makes the person feel useful. For example, if the person is constantly fidgeting with his hands, try giving him some socks to sort or some knick knacks to clean.

* Hallucinations – Hallucinations are sensory experiences that seem real, but are not. The most common hallucinations are visual (i.e., seeing something that is not really there) and auditory (i.e., hearing something that is not really there), but hallucinations can also occur in regard to taste, smell, and touch. Because hallucinations seem real to those with dementia, it is not helpful to try to convince the person that she is imagining things. Instead, recognize the person’s feelings, reassure the person that you are there to help, and redirect her to a pleasant activity. Also consider whether the hallucination is actually bothersome. If it is a “nice” hallucination (e.g., seeing a pretty orchard outside that is not really there), there may be no benefit in trying to discourage the behavior.

* Suspicion – Memory loss and disorientation can cause individuals with dementia to perceive situations inaccurately. They may become suspicious of others – even those close to them – and accuse them of theft, infidelity, or other offenses. As hurtful as it may be to be accused of something you did not do, try not to become offended. Remember that the behavior is caused by a disease that is affecting the person’s brain. Try to imagine what it would be like to continuously think your possessions are being taken or hidden (because you cannot remember where you put them). Do not try to argue with the person or convince him or her of your innocence. Instead, share a simple response with the person (e.g., “I see that you’re upset that your purse is missing; I’ll do my best to find it for you.”) and avoid giving complicated explanations. Redirection to another activity can also be effective in these situations. Another option is to store “back-ups” of commonly misplaced items (e.g., hats, wallets).

* Apathy – Apathy is a lack of interest in or motivation to engage in activities. While apathy may not seem like a serious behavior problem, it is not healthy for someone with dementia to simply sit around passively. Try to find out what may be triggering the apathy (e.g., being ignored or becoming overwhelmed with a task) as well as what kinds of consequences may be reinforcing it (again, being ignored, not having appropriate choices of pleasant activities). Even though the person is ill, it is important to keep him or her moving and as active as possible in order to maintain physical health and to prevent depression. Try adapting previously pleasurable activities so the person can participate at a level that is comfortable and not overwhelming. Even a small amount of activity is better than none at all.

* Confusion – Dementia often causes confusion about person, place, and time. In other words, the person may still know who he or she is, but may not recognize others and/or the current location, time, date, or year. An individual with dementia may also become confused about the purpose of objects, such as forks or pens. As frustrating as this can be for caregivers, the best way to respond is to stay calm and provide simple, clear, positive answers when the person asks for help. For example, if the person seems confused about the purpose of a spoon, simply say, “Here’s your spoon for eating your soup.” You could also calmly show the person how to use the utensil (e.g., by saying “watch me”). Never scold the person or talk to him or her in a belittling way for becoming confused.

* Sundowning – Sundowning is a term used to describe behaviors that intensify (e.g., increased confusion and agitation) in the late afternoon and early evening, and is most common with Alzheimer’s disease. There are several theories about why sundowning occurs, such as increased fatigue (and as a result, a reduced ability to tolerate stressful situations, such as a chaotic dinnertime or a rushed bedtime routine) or increasing confusion due to darkness and shadows. The best way to approach sundowning is to make late afternoons and evenings as simple and relaxing as possible. Reduce distractions, unscheduled activities, and behaviors that could be done at a different time of the day (e.g., switch to bathing in the morning) and keep rooms well-lit until bedtime.

* Wandering – One of the more dangerous behaviors among individuals with dementia, wandering may be goal-directed (e.g., the person thinks that he or she is going to a job or going “home” to a childhood residence) or non-goal-directed (i.e., the person wanders aimlessly). To reduce the frequency of wandering, make sure the person has plenty of supervised activity to channel his or her energy. Redirecting the person to another activity can also work. Interestingly, dementia sometimes affects perception in such a way that environmental approaches can help reduce wandering. For instance, a black square painted on the floor in front of a doorway – or simply a black doormat – may be perceived as a hole, which can prevent the person from leaving the home.

“When Sleep Apnea Masquerades as Dementia”

This short article is about the fact often sleep apnea goes undiagnosed in the elderly. In particular, the article reports on a case of a woman who had undiagnosed obstructive sleep apnea that was mimicking dementia symptoms. The impression I got from the article is that this is unusual; typically sleep apnea exacerbates dementia symptoms. Here’s the full article and a link to it.

http://newoldage.blogs.nytimes.com/2010 … -dementia/

The New Old Age
Caring and Coping

October 6, 2010, 3:44 PM
When Sleep Apnea Masquerades as Dementia
By Paula Span
The New York Times

The woman who came to see Dr. Ronald Petersen, an Alzheimer’s specialist at the Mayo Clinic, was only in her 60s but complained that she was having trouble concentrating. “Her attention was waning,” Dr. Petersen recalled. “She couldn’t follow a television program or stay focused during a conversation.”

She was probably developing dementia, Dr. Petersen thought as he took her history. But along the way he asked, as he usually does, how she was sleeping. The woman, who lived alone, hadn’t noticed any problems.

Her son, however, had stayed with her the previous night to drive her to the appointment. “She was snoring like a freight train,” he reported.

Aha. Overnight sleep testing determined that the woman had obstructive sleep apnea — nightlong interruptions in breathing that reduce oxygen flow to the brain and prevent deep sleep. The interruptions can happen 10 or more times an hour and are quite common in older adults, exacerbating — or sometimes mimicking — dementia symptoms.

Treated with a C.P.A.P. machine — the acronym stands for continuous positive airway pressure, a therapy that involves wearing a mask over the nose and/or mouth during sleep — the woman rapidly improved. Her scores on neuropsychological tests eventually climbed back into the normal range. A year later, Dr. Petersen said, “I can’t find any abnormalities.”

Most of the time, cognitive problems won’t evaporate when seniors are treated for sleep apnea. But researchers find that with C.P.A.P., many older patients see marked improvement. “They’re not dozing off during the day, they’re not dragging,” said Dr. Bradley Boeve, a neurologist at the Mayo Clinic. “Quality of life improves.”

Life gets easier for their caregivers, too, a key concern in trying to keep people out of nursing homes.

But apnea frequently goes undetected, especially in the elderly, although they are more likely to have it. Estimates of the percentage of older adults with sleep apnea are all over the map, in part because of varying definitions of the condition — but they’re always startlingly high. Sonia Ancoli-Israel, professor of psychiatry at the University of California, San Diego, has studied the disorder for 30 years and reports that almost half of older adults experience apnea to some degree, with even higher rates among those with dementia.

“It’s under-recognized in all age groups,” she told me in an interview. “But in older people, physicians are even less likely to recognize it.”

Why? Apnea in younger people frequently coincides with obesity; in elderly patients, that’s less often true. Loud snoring, often a tip-off, may go unnoticed when seniors live alone. And, as Dr. Ancoli-Israel pointed out, “there’s a belief that old people are supposed to be sleepy during the day.”

They’re not, and one reason she wants them and their caregivers to recognize the problem is the now-established connection between apnea and cognitive decline. “If you’re waking up hundreds of times a night and you’re not getting enough oxygen to the brain, of course you’ll see the effect,” Dr. Ancoli-Israel said.

She and her team, in a study published in 2008 in The Journal of the American Geriatrics Society, randomly assigned patients with Alzheimer’s disease to use real C.P.A.P. machines or fake ones for several weeks. The treatment produced “modest but statistically significant improvements,” particularly in vigilance (the ability to pay attention) and executive function (judgment and decision-making).

“We didn’t cure the dementia,” Dr. Ancoli-Israel cautioned. “But it wasn’t as severe as before.” As the researchers followed up months later, they also found that while all the patients continued to decline cognitively, in those using C.P.A.P. the decline was more gradual.

For years, physicians doubted that dementia patients could or would use these machines. Even younger people with apnea frequently find the C.P.A.P. mask uncomfortable, pulling it off during sleep or just not bothering to use it. But in recent studies, seniors diagnosed with Alzheimer’s disease did use the devices — not for as many hours each night as their doctors might wish, but long enough.

Now Dr. Ancoli-Israel is investigating whether C.P.A.P. therapy might help reduce the cognitive damage from Parkinson’s disease. “This isn’t just Alzheimer’s,” she said. “Any time there are symptoms of dementia, you should think about sleep apnea and discuss it with your doctor.”

Be forewarned: diagnosing sleep apnea can be complicated, requiring an overnight stay in a sleep center. If apnea proves to be the problem, technicians have to calibrate each C.P.A.P. machine’s settings and individually fit the mask. All those processes become harder with someone who’s cognitively impaired. (To find treatment centers, consult the American Academy of Sleep Medicine or the National Sleep Foundation.)

But when someone with apnea does stick with the treatment, “you’ll see the effects within a month or so,” Dr. Boeve said. “Sometimes even within a week.”

Paula Span is the author of “When the Time Comes: Families With Aging Parents Share Their Struggles and Solutions.”