Category Archives: PSP

“Compassionate Communication” – do’s and don’ts

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Recently I attended a workshop on dementia caregiving.  This wonderful handout on compassionate communicate was shared.  Though written by an Alzheimer’s support group leader and addressed to caregivers of those with “memory impairment,” the suggestions of “do’s” and “don’ts” apply to all dementia types, even without memory impairment.

Here’s a link to the handout:

www.ocagingservicescollaborative.org/wp-content/uploads/2013/03/Compassionate-Communication-with-the-Memory-Impaired.pdf

Compassionate Communication with the Memory Impaired
Liz Ayres, Alzheimer’s Support Group Leader, former Caregiver, Orange County, CA
Copyright 2008

[Editor’s Note, 2013: handout link from 2008 no longer working.]

I’ve copied much of the handout below.  Page 2 of the handout contains lots of examples.

Robin

Compassionate Communication with the Memory Impaired
Liz Ayres, Alzheimer’s Support Group Leader, former Caregiver, Orange County, CA
©1995, 2001, 2005, 2007, 2008

DON’T

Don’t reason.
Don’t argue.
Don’t confront.
Don’t remind them they forget.
Don’t question recent memory.
Don’t take it personally.

DO

Give short, one sentence explanations.

Allow plenty of time for comprehension, then triple it.

Repeat instructions or sentences exactly the same way.

Eliminate ‘but’ from your vocabulary; substitute ‘nevertheless.’

Avoid insistence. Try again later.

Agree with them or distract them to a different subject or activity.

Accept the blame when something’s wrong (even if it’s fantasy.)

Leave the room, if necessary, to avoid confrontations.

Respond to the feelings rather than the words.

Be patient and cheerful and reassuring. Do go with the flow.

Practice 100% forgiveness. Memory loss progresses daily.

My appeal to you: Please elevate your level of generosity and graciousness.

REMEMBER

You can’t control memory loss, only your reaction to it. Compassionate communication will significantly heighten quality of life. They are not crazy or lazy. They say normal things, and do normal things, for a memory impaired, dementia individual. If they were deliberately trying to exasperate you, they would have a different diagnosis. Forgive them…always. For example: they don’t hide things; they protect them in safe places…and then forget. Don’t take ‘stealing’ accusations personally.

Their disability is memory loss. Asking them to remember is like asking a blind person to read. (“Did you take your pills?” “What did you do today?”) Don’t ask and don’t test memory! A loss of this magnitude reduces the capacity to reason. Expecting them to be reasonable or to accept your conclusion is unrealistic. (“You need a shower.” “Day care will be fun.” “You can’t live alone.”) Don’t try to reason or convince them. Give a one sentence explanation or search for creative solutions. Memory loss produces unpredictable emotions, thought, and behavior, which you can alleviate by resolving all issues peacefully. Don’t argue, correct, contradict, confront, blame or insist.

Reminders are rarely kind. They tell the patient how disabled they are––over and over again. Reminders of the recent past imply, “I remember, I’m okay; you don’t, you’re not.” Ouch! Refer only to the present or the future. (If they’re hungry, don’t inform them they ate an hour ago, offer a snack or set a time to eat soon.) They may ask the same question repeatedly, believing each time is the first. Graciously respond as if it’s the first time. Some days they seem normal, but they’re not. They live in a different reality. Reminders won’t bring them into yours. Note: For vascular dementia, giving clues may help their recall. If it doesn’t work, be kind…don’t remind.

Ethical dilemmas may occur. If, for instance, the patient thinks a dead spouse is alive, and truthful reminders will create sadness, what should you do? To avoid distress, try these ways of kindness: 1) distract to another topic, or 2) start a fun activity, or 3) reminisce about their spouse, “I was just thinking about ___. How did you meet?” or you might try, “He’s gone for a while. Let’s take our walk now.”

Open ended questions (“Where shall we go?” “What do you want to eat/wear/do?”) are surprisingly complex and create anxiety. Give them a simple choice between two items or direct their choice, “You look great in the red blouse.”

They are scared all the time. Each patient reacts differently to fear. They may become passive, uncooperative, hostile, angry, agitated, verbally abusive, or physically combative. They may even do them all at different times, or alternate between them. Anxiety may compel them to shadow you (follow everywhere). Anxiety compels them to resist changes in routine, even pleasant ones. Your goal is to reduce anxiety whenever possible. Also, they can’t remember your reassurances. Keep saying them.

 

“Clinical outcomes” paper – PSP and MSA

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This is a very interesting article written by some of Europe’s top PSP researchers (and presumably top MSA researchers too).  The first author is O’Sullivan.  This is also a very important paper because it includes analysis of brains donated to the University College of London brain bank.

110 pathologically-confirmed PSP cases and 83 pathologically-confirmed MSA cases were examined for early clinical features and survival.  PSP cases were divided according to the D. Williams criteria of Richardson’s syndrome (RS) and PSP-parkinsonism.  MSA cases were divided according to the presence of early autonomic failure.

The PSP findings confirms what D. Williams has told us before:

“In PSP an RS phenotype, male gender, older age of onset and a short interval from disease onset to reaching the first clinical milestone were all independent predictors of shorter disease duration to death. Patients with RS also reached clinical milestones after a shorter interval from disease onset, compared to patients with PSP-P.”

The MSA findings are new to me (though maybe not to many of you):

“In MSA early autonomic failure, female gender, older age of onset, a short interval from disease onset to reaching the first clinical milestone and not being admitted to residential care were independent factors predicting shorter disease duration until death. The time to the first clinical milestone is a useful prognostic predictor for survival.”

Interesting (and scary?) that “not being admitted to residential care” predicts *shorter* disease duration in MSA.

I’ve copied below the abstract.

Update:  the full paper is now available at no charge.  See our post here with links.

Robin

—————-

Brain. 2008 Apr 2 [Epub ahead of print]

Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.

O’Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ.

Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK, Faculty of Medicine (Neuroscience), Monash University (Alfred Hospital Campus) and Department of Neurosciences, Monash Medical Centre, Melbourne, Australia.

Prognostic predictors have not been defined for progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). Subtypes of both disorders have been proposed on the basis of early clinical features. We performed a retrospective chart review to investigate the natural history of pathologically confirmed cases of PSP and MSA.

Survival data and several clinically relevant milestones, namely: frequent falling, cognitive disability, unintelligible speech, severe dysphagia, dependence on wheelchair for mobility, the use of urinary catheters and placement in residential care were determined.

On the basis of early symptoms, we subdivided cases with PSP into ‘Richardson’s syndrome’ (RS) and ‘PSP-parkinsonism’ (PSP-P).

Cases of MSA were subdivided according to the presence or absence of early autonomic failure.

Sixty-nine (62.7%) of the 110 PSP cases were classified as RS and 29 (26.4%) as PSP-P.

Of the 83 cases of MSA, 42 (53.2%) had autonomic failure within 2 years of disease onset.

Patients with PSP had an older age of onset (P < 0.001), but similar disease duration to those with MSA. Patients with PSP reached their first clinical milestone earlier than patients with MSA (P < 0.001). Regular falls (P < 0.001), unintelligible speech (P = 0.04) and cognitive impairment (P = 0.03) also occurred earlier in PSP than in MSA.

In PSP an RS phenotype, male gender, older age of onset and a short interval from disease onset to reaching the first clinical milestone were all independent predictors of shorter disease duration to death. Patients with RS also reached clinical milestones after a shorter interval from disease onset, compared to patients with PSP-P.

In MSA early autonomic failure, female gender, older age of onset, a short interval from disease onset to reaching the first clinical milestone and not being admitted to residential care were independent factors predicting shorter disease duration until death. The time to the first clinical milestone is a useful prognostic predictor for survival.

We confirm that RS had a less favourable course than PSP-P, and that early autonomic failure in MSA is associated with shorter survival.

PMID: 18385183

“Frontal-subcortical dementias” (PSP, CBD, LBD, and MSA)

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This newly-published abstract reviews the clinical presentation of frontal-subcortical dementias, lists them, and suggests how they relate to cortical dementias. The classic “cortical dementia” is Alzheimer’s Disease. Three dementias in our atypical parkinsonism group are mentioned as frontal-subcortical dementias — Parkinson disease dementia (also called Lewy body dementia), progressive supranuclear palsy, and corticobasal degeneration.

Interestingly, multiple system atrophy is listed as a frontal-subcortical dementia though dementia is exclusionary for MSA.

Robin

The Neurologist. 2008 Mar;14(2):100-107.

Frontal-Subcortical Dementias.

Bonelli RM, Cummings JL.
>From the *Department of Psychiatry, Graz Medical University, Graz, Austria; and the †Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, CA.

Frontal-subcortical dementias are a heterogeneous group of disorders that share primary pathology in subcortical structure and a characteristic pattern of neuropsychologic impairment. Their clinical presentation is characterized by memory disorders, an impaired ability to manipulate acquired knowledge, important changes of personality (apathy, inertia, or depression), and slowed thought processes (or bradyphrenia). It also has marked frontal dysfunction.

Classic frontal-subcortical dementias include Huntington chorea, Parkinson disease dementia, progressive supranuclear palsy, thalamic degeneration, subcortical vascular dementia, multiple sclerosis, the acquired immunodeficiency syndrome dementia complex, depressive pseudodementia, and some other rare dementias like spinocerebellar degenerative syndromes, Hallervorden-Spatz disease, choreoacanthocytosis, idiopathic basal ganglia calcification, Guamanian parkinsonism-dementia complex, corticobasal degeneration, multiple system atrophy, Wilson disease, metachromatic leukodystrophy, adrenoleukodystrophy, hypoparathyroidism, sarcoidosis, and other CNS inflammatory disorders.

Anatomic data suggest that the frontal signs result from a disconnection of the frontal cortex from the basal ganglia. However, most frontal-subcortical dementias show cortical atrophy in later stages, and cortical dementias have subcortical pathology at some point. In fact, the concept might be seen as a continuum, and only the 2 extremes would be represented by pure cortical or subcortical pathology. Anyway, subcortical disorders may still be more similar to one another than they are to AD. Possibly, frontal-subcortical and cortical dementias are the description of the prior main target of the disease process, ending up in both cases in a global dementia. Although the dichotomy cortical versus frontal-subcortical dementia is not strict, the 2 concepts still seem to have advantages.

PubMed ID#: 18332839 (see pubmed.gov for abstract only)

Photophobia, VH, and RBD in PSP+CBD (Mayo Rochester study)

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This is a rather weak study because they looked at 10 patients with the clinical diagnosis of PSP and 11 patients with the clinical diagnosis of CBD. No pathological confirmation was available. Their findings included:

* “Photophobia occurred in all 10 (100%) PSP patients vs 2 (18%) patients with clinically suspected CBD (p=0.0002).” And: “The presence of photophobia is significantly more frequent in clinically diagnosed PSP than CBD and can be used as a feature in differentiating between the two diseases in clinical practice.”

Every PSPer I’ve met has photophobia and some (but not all) of the CBDers I’ve met have photophobia.

* “Visual hallucinations and RBD occurred in patients with PSP and CBD but were rare occurrences (5% for each symptom).” And: “Visual hallucinations and RBD occur infrequently in PSP and CBD and are not useful symptoms in clinical differentiation.”

I usually hear “photophobia” called “photo sensitivity.” Whenever I was in the hospital or skilled nursing facility with my dad, I’d always close the blinds/curtains and, if there was an overhead light on, we’d put sunglasses on dad or a washcloth over his eyes to block out the light. “Photophobia” is extreme sensitivity or aversion to sunlight and any other light.

Robin

Parkinsonism & Related Disorders. 2008 Mar 5 [Epub ahead of print]

Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: A prospective study.

Cooper AD, Josephs KA.
Department of Neurology, Mayo Clinic, Rochester, MN.

Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) have overlapping clinical features that can make clinical distinction between these two entities difficult. The present study compared the frequency of photophobia, visual hallucinations, and REM sleep behavior disorder (RBD) in patients clinically diagnosed with PSP to those clinically suspected to have CBD. Photophobia occurred in all 10 (100%) PSP patients vs 2 (18%) patients with clinically suspected CBD (p=0.0002). Visual hallucinations and RBD occurred in patients with PSP and CBD but were rare occurrences (5% for each symptom). The presence of photophobia is significantly more frequent in clinically diagnosed PSP than CBD and can be used as a feature in differentiating between the two diseases in clinical practice. Visual hallucinations and RBD occur infrequently in PSP and CBD and are not useful symptoms in clinical differentiation.

PubMed ID#: 18328771 (see pubmed.gov for abstract only)

I received a copy of this full article today. In my earlier post, after reading the abstract only, I concluded that no cases in this Mayo Rochester study had been pathologically-confirmed. This is incorrect. The full article notes that “Ten patients had PSP, two with pathologic confirmation, and 11 patients had CBS, one with pathologic confirmation.”

These patients (and their significant others) were questioned regarding photophobia. “The question regarding photophobia emphasized discomfort as a result of light exposure as opposed to frequent eye closing or lack of eye opening. This distinction is important since both blepharospasm and apraxia of eye opening can be present in these disorders.”

The findings were: “Photophobia occurred in all 10 (100%) PSP patients vs 2 (18%) CBS patients. The mean time between when the patient first reported photophobia and disease onset was 3.1 years.” Based upon this, the authors argue that “the presence of photophobia may help clinicians to better differentiate between PSP and CBS on a clinical basis…”

Interestingly, of the 2 CBS patients with photophobia, one of these was pathologically-confirmed as CBD. And, “both CBS patients with photophobia were two of only three CBS patients with vertical gaze palsy suggesting pathological involvement of brainstem nuclei in these two subjects.”

The authors admit that they need more pathologically-confirmed cases.

This finding was surprising: “Blepharospasm was noted in only one patient in this series. He was one of the two CBS patients with photophobia and was pathologically confirmed to have CBD.” It seems that in our local support group blepharospasm is more common.

One note to the CBD folks: This article says that the term CBS (corticobasal syndrome) is used for the clinical entity while CBD (corticobasal degeneration) is used for the pathologic entity.

See below for some excerpts to this short article.

Robin

Here are some excerpts that may be of interest:

Introduction
“Progressive supranuclear palsy (PSP) and corticobasal de-
generation (CBD) are neurodegenerative disorders that display
some overlapping clinical features. The core clinical features
of PSP include vertical gaze palsy, axial more than appendic-
ular rigidity, and early postural instability. The core clinical
features of CBD include asymmetric appendicular rigidity and
cortical dysfunction including apraxia of limb. The term corti-
cobasal syndrome (CBS) has been applied to the clinical
entity, while CBD refers to the pathologic entity.”

“When the cardinal features of these two diseases are present
at disease onset, diagnosis may be relatively straightforward.
However, clinical features of these two diseases often overlap
and some of the cardinal features may not occur until later in
the disease course. These atypical disease presentations can
present a diagnostic dilemma, which may make it difficult for
clinicians to predict disease progression. Therefore, additional
clinical features that could distinguish PSP from CBD would be
helpful in clinical practice. Photophobia has been described in
PSP but not CBD to our knowledge. Visual hallucinations and
REM sleep behavior disorder (RBD) have not been emphasized
in either PSP or CBD.We conducted a study to determine
the frequency of photophobia, visual hallucinations, and RBD
in these two disease populations.”

Methods
“One movement disorders specialist (KAJ) evaluated all
patients with features suggestive of PSP and CBD from 2003
to 2006 at a single medical institution.”

“We questioned patients and their significant others regarding
the presence of photophobia, visual hallucinations, and RBD.
The question regarding photophobia emphasized discomfort
as a result of light exposure as opposed to frequent eye
closing or lack of eye opening. This distinction is important
since both blepharospasm and apraxia of eye opening can be
present in these disorders. Only well-formed visual hallucina-
tions that were spontaneous and not associated with medica-
tion use were considered. REM sleep behavior disorder was
considered present if the patient’s bed partner reported
abnormal limb movements during sleep that were disruptive or
injurious to either the bed partner or the patient.”

Results
“Ten patients had PSP, two with pathologic confirmation, and
11 patients had CBS, one with pathologic confirmation. The
median ages at disease onset in PSP and CBS were 66
(range 59-77) and 65 (range 49-91)…”

“Based on clinical criteria for PSP, four patients were clas-
sified as possible, four as probable, and two were definite
(i.e. pathologically confirmed).”

“Photophobia occurred in all 10 (100%) PSP patients vs 2
(18%) CBS patients. The mean time between when the
patient first reported photophobia and disease onset was
3.1 years.”

“Blepharospasm was noted in only one patient in this series.
He was one of the two CBS patients with photophobia and
was pathologically confirmed to have CBD.”

“Visual hallucinations occurred in 1 (5%) PSP patient and
RBD occurred in 1 (5%) CBS patient. The visual hallucina-
tions occurred in a PSP patient taking Levodopa/Carbidopa.
… The hallucinations continued despite reduction of the
Levodopa/Carbidoba and, unfortunately, the patient died
before further dose reductions could be made.”

“The CBS patient with RBD had symptoms consisting of
talking and performing exercising movements usually during
the first 1-2 h of sleep.”

Discussion
“The present study demonstrates a significant difference in
the frequency of photophobia in patients with PSP com-
pared to those with CBS. This result suggests that the
presence of photophobia may help clinicians to better dif-
ferentiate between PSP and CBS on a clinical basis, and
may be a helpful feature in predicting underlying pathology.”

“The pathophysiology of photophobia is not entirely under-
stood. Studies have pointed to the trigeminal nerve as one
necessary component for photophobia. … Other studies
have suggested a role of the optic nerve and its connec-
tions with the pretectal nuclei. Indeed, it may be an inter-
action of these two pathways that produce photophobia.
The corresponding subcortical location of the trigeminal
and optic nerve connections and typical subcortical loca-
tion of PSP pathology, such as the superior colliculi, may
explain the high incidence of photophobia found in our PSP
population. The subcortical location of PSP pathology con-
trasts with the more cortical location of pathology found in
CBD and may explain the relatively low frequency of photo-
phobia found in patients with CBS in our study. In fact, both
CBS patients with photophobia were two of only three CBS
patients with vertical gaze palsy suggesting pathological
involvement of brainstem nuclei in these two subjects.”

“Visual hallucinations and RBD were rare occurrences in
each population making them unhelpful in clinical differen-
tiation between PSP and CBS. The low occurrence of RBD
is not surprising since this clinical phenomenon has been
shown to be suggestive of underlying alpha-synuclein path-
ology, and both PSP and CBD are characterized by the
deposition of the microtubule associated protein tau
(MAPT) and not alpha-synuclein pathology.”

“Litvan et al. showed that the presence of gait abnormality,
bilateral bradykinesia, and moderate to severe vertical
supranuclear gaze palsy help to distinguish PSP from
CBS. Their study was based on pathologically confirmed
cases of these two diseases. Since our study is based
on clinical and/or pathologic criteria, we cannot conclude
that the presence of photophobia will definitely predict
pathologic confirmation of PSP. However, 15% of our
cases were pathologically confirmed and prior studies
have shown that more than 75% of clinically diagnosed
PSP patients have PSP pathology.”

“The results of our study suggest that patients suspected
to have PSP or CBD should be questioned regarding the
presence or absence of photophobia as part of routine
questioning. … In this study, we demonstrate for the first
time that the presence of photophobia may be an addi-
tional useful clinical feature to differentiate PSP from
CBD.”

Dr. Paul Donohue on PSP

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Quite a few US newspapers carry a health column written by FL-based Dr. Paul Donohue in which he answers letters. One of today’s letters is about PSP. I got this from SouthCoast Today, a MA newspaper (though many papers carry his column). I read about it on a PSP-related Yahoo!Group.
Robin

http://www.southcoasttoday.com/apps/pbc … /ENTERTAIN

Dr. Paul Donohue: Your health
March 09, 2008

DEAR DR. DONOHUE: Eight years ago my husband was said to have Alzheimer’s disease. A few years later, the diagnosis was changed to Parkinson’s disease. Now the diagnosis is progressive supranuclear palsy. What is that? His speech is hard to understand, and his walking is bad. How does it end up? — B.C.

DEAR BC: Quite a few illnesses can look like Parkinson’s disease or Alzheimer’s disease, especially in their early stages. These illnesses share with each other an attack on brain centers that produce similar signs. However, each also affects brain centers not stricken by the others, and that gives each of these illnesses distinctive features.

An unsteady walk with frequent falls is a sign common to both progressive supranuclear palsy and Parkinson’s disease, because the brain area that governs walking is affected in both illnesses.

Some distinctive signs of PSP are a soft, monotone, barely understandable speech; forgetfulness; irritability; and decreased blinking, which leads to dry eyes. Swallowing can become a formidable task. One of PSP’s most distinguishing features is the inability to turn the eyes downward and, later in the illness, to turn them upward. Loss of eye movement makes reading impossible and adds to the patients’ instability. Many patients eventually have to use a wheelchair.

The Society for Progressive Supranuclear Palsy is ready to help patients and their families with reams of information and with support. You can reach the society at (800) 457-4777 (in Canada, (866) 457-4777) or on the Web at www.psp.org

Write to Dr. Paul Donohue at P.O. Box 536475, Orlando, FL 32853-6475.