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 LBD + PSP: a case study 
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Joined: Sat Jul 19, 2008 10:29 am
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Location: Italy and Toronto (Canada)
Post LBD + PSP: a case study
Here is an interesting article which discusses a case study of a patient presenting both LBD symptoms and those of PSP (progressive supranuclear palsy).
It's worth reading because I personally have often found the symptoms of these two diseases to be very similar with much overlap.

Here is the link to the full article:

http://www.springerlink.com/index/F5415520P1G27541.pdf

The following are some brief excerpts which I have copied:

Abstract: Description of a case of probable dementia with
Lewy bodies featuring parkinsonism, dementia and
supranuclear gaze palsy. This is the first patient to our
knowledge affected with vertical gaze palsy receiving clinical
diagnosis of DLB when alive and to be treated with
cholinesterase inhibitors.

Key words: Dementia with Lewy bodies • Progressive
supranuclear palsy • Steele-Richardson-Olszewski syndrome •
Supranuclear gaze palsy • Parkinsonism • Dementia

Introduction:Alzheimer’s disease (AD), dementia with Lewy bodies
(DLB) and Parkinson’s disease (PD) represent a clinicopathological
continuum in neurodegenerative disorders,
affecting predominantly cognition (AD), motor functioning
(PD) or both (DLB). Along this continuum, pure AD
pathology and pure Lewy body pathology (that is PD and
‘pure’ DLB), as well as mixed pathology (Lewy body variant
of AD) are described [1]. Moreover, intracerebral distribution
of pathological findings may vary. Particularly,
Lewy body pathology is classified into brainstem predominant,
limbic and neocortical subtypes, according to distribution
of Lewy bodies [1].
Current diagnostic criteria [1], including cognitive
impairment (with prominent attention and visuospatial
dysfunction), recurrent visual hallucinations and parkinsonism,
allow differentiation of DLB from AD. On the
other hand, distinguishing DLB from PD and other
parkinsonism represents a harder task to clinicians, as no
operational criteria have been borne out so far to our
knowledge.
In particular, parkinsonism presenting with vertical
gaze defect tends to be identified with progressive supranuclear
palsy (PSP) [2] in daily practice. Nevertheless, a few
autoptic cases of DLB with supranuclear gaze palsy have
been described so far [2–8]. We describe hereby a case of
parkinsonism, dementia and supranuclear gaze palsy for
whom we made a clinical diagnosis of DLB, according to
McKeith’s criteria [1].


Tue Dec 14, 2010 5:25 am
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Post Re: LBD + PSP: a case study
Shomy,

From what you've excerpted, this appears to be a clinical case report, not a pathological case report. A clinical case report is of very limited value.

I'm only aware of one pathological case where PSP and DLBD co-occurred. Someone on the PSP Forum posted about this many years ago.

I forget the exact number but something like 30% of people with path-confirmed PSP have Lewy bodies in the brain. Having Lewy bodies in the brain does *not* mean these people had Lewy body dementia.

The only way to know the confirmed diagnosis is to have the brain autopsied. Do you and your sister intend to donate your father's brain?

All of the atypical parkinsonism disorders are easily confused. Those with dementia are easily confused with AD. And those without dementia are easily confused with PD. One person in our local SG had an LBD diagnosis during life and a PSP diagnosis upon brain autopsy. And I know of others who have had a PSP diagnosis during life and an LBD diagnosis upon death.

Robin


Tue Dec 14, 2010 11:27 am
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Joined: Fri Jan 15, 2010 9:33 pm
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Location: Vermont
Post Re: LBD + PSP: a case study
There are a number of neurological diseases which seem so similar - lots of overlap in symptoms. Since my dad was diagnosed with "POSSIBLE" LBD, CBD, PSP, AD, ALS, spastic paraperisis, ventricular assymetry, (along with a few other things), I've read about them all. His symptoms also seemed to fit the symptoms of Creutzfeld-Jakob (mad cow disease). He ate LOTS of hamburger his whole life....
At any rate, the family did not want a brain autopsy, so we will never know, but I wonder, if he had 2 or more of these diseases, would anyone really have been able to identify which ones he did have? Lynn

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Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Tue Dec 14, 2010 4:09 pm
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Post Re: LBD + PSP: a case study
Lynn,
Typically, the diseases you named don't often co-occur, with the exception of AD (which co-occurs with many disorders). So I think it's unlikely an MD would say "it seems like you have X and Y" and far more likely the MD would say "you might have X or you might have Y."
Robin


Tue Dec 14, 2010 7:32 pm
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Post Re: LBD + PSP: a case study
The most I could pin anyone down with a diagnosis back when I was frantically seeking one, was "your father has at least 2 neurological diseases", and that was from the neurologist. That's what made me wonder if we had done an autopsy, would they have been able to tell what diseases they were, with so many different and overlapping symptoms. Moot point now... Thanks, Lynn

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Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Tue Dec 14, 2010 8:12 pm
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Post Re: LBD + PSP: a case study
Oh, sorry, I misunderstood your question. Absolutely a brain autopsy can determine multiple diagnoses. Nearly all of the neuropathology reports I've seen for anyone over the age of 60 has included at least two diagnoses. The most was seven (maybe it was nine).

Each disease/diagnosis has its own diagnostic criteria, many of which involve staining tissue to search for particular proteins. The proteins involved in the disorders you listed are mostly different: alpha-synuclein for Lewy body disease, tau and amyloid for Alzheimer's, prions for CJD, etc.

Nearly everyone I know who receives a neuropathology report on a loved one is surprised with at least one of the diagnoses.


Wed Dec 15, 2010 1:41 am
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Post Re: LBD + PSP: a case study
robin wrote:
Nearly everyone I know who receives a neuropathology report on a loved one is surprised with at least one of the diagnoses.


Which, of course, is a reflection of how misunderstood these diseases are. We're all working in the dark here. It's amazing the we have any understanding at all. (Maybe we don't!)

I know our neurologist is still 'practicing.'

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Leone Carroll (75); wife of Dale (75) who passed away March 23, 2011


Wed Dec 15, 2010 6:59 am
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Location: Italy and Toronto (Canada)
Post Re: LBD + PSP: a case study
Robin,

The study (as you correctly mentioned) is a clinical case study, and I think it was only intended to point out some of the flaws in the current differential diagnoses criteria.

My sister and I discussed Dad's possible brain dontation with his neurologist. We told her that if it was needed for research, or even for statistical purposes, we would arrange the donation. She told us that for varying reasons, they could not use Dad's brain in any current research and that if we wished, she would arrange to perform an autopsy only to satisfy our curiosity regarding his definitive diagnosis. She is very sure that he has LBD and perhaps some other disorder, and she said that if we biopsied the brain, it would help my sister and myself realize whether Dad has also a disorder which could have a genetic basis and in this way we would know whether we were predisposed to develop the same disorder and/or passing it on to further generations.

My sister and I are not planning to have children and I honestly don't really want to know what possible future brain disorder will be hanging over my head. Obviously, like every other conscientious person, we are preparing our care plans, financial arrangements and living wills, etc... for our old age anyway.

For now, since it cannot be used in research, we have decided not to do the brain donation. But I will keep asking the neurologist (who is by the way an avid researcher) and if at any point, she says they can use his brain for any type of research, they are welcome to it.

Shomy.


Wed Dec 15, 2010 8:07 am
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Joined: Wed Dec 30, 2009 1:46 pm
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Post Re: LBD + PSP: a case study
Shomy, we're in the same position. No one here is accepting brain donations for research right now but we can hire a neuropathologist to perform a brain autopsy. My husband's initial interest in donating his brain for research [he was a research scientist, himself] does not extend to having his brain autopsied just for a diagnosis. Personally, I would like to have it done--and could, without his consent--but I'm still weighing the pros and cons.

_________________
Pat [68] married to Derek [84] for 38 years; husband dx PDD/LBD 2005, probably began 2002 or earlier; late stage and in a SNF as of January 2011. Hospitalized 11/2/2013 and discharged to home Hospice. Passed away at home on 11/9/2013.


Wed Dec 15, 2010 10:40 am
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Post Re: LBD + PSP: a case study
Shomy,
If you are interested in donating your dad's brain for research, I encourage you to make arrangements to donate the brain to the Mayo Clinic. I think St. Michael's Hospital will do the procurement. It's a shame most neurologists don't know about brain donation.
Robin


Wed Dec 15, 2010 1:28 pm
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Post Re: LBD + PSP: a case study
If there is a genetic component, with no cure, and little that can be done to treat the symptoms, I'd rather not know and neither would my children. That is part of the reason we opted for no brain autopsy. Trying to live a healthy, productive life and planning for old age is about all I feel we can do! Knowing I have some neurological disease to "look forward to" is just too depressing...... Lynn

_________________
Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Wed Dec 15, 2010 9:02 pm
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Post Re: LBD + PSP: a case study
Oh Lynn, I do so understand what your reasoning is about living a healthy life and doing the best you can however there is more, as I have written before, we are the first generation to have Lewy Body even considered by our medical field but even now their hands are tied because of lack of information to help care for our loved ones. So much is trial and error. We receive a Clinical diagnoses and can only hope that it is near enough correct for the proper treatment. I am so thankful that research is being done but the Scientists cannot do their work unless enough of us offer to donate the tissue for them to study. I was surprised when I received the neuropathology report from Mayo Clinic [Jacksonville] at the number of pathologies that were found. I certainly did not suspect that he had Alzheimer's Disease in addition to Lewy Body and I believe that was held as stable as it was because of the Exelon Patch and Namenda [just my feeling, of course].

Twenty years ago my dear friend was brave enough to travel to NIH for study as she battled Alzheimer's Desease. I was so proud of her. Little did I realize at the time that the NIH research program would produce meds that would eventually help me care for Mr Bobby. Help is found one step at a time.

Dorthea

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"See this lady, she's 85, but she's nice"


Thu Dec 16, 2010 1:11 am
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Post Re: LBD + PSP: a case study
I think it is only through brain donation that researchers will find treatments and cures. Every time someone donates a brain, I think the chances are improved that treatments and cures will be found. If my father didn't donate his brain, and I don't donate mine, then how will the discoveries be possible?


Thu Dec 16, 2010 1:29 am
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Post Re: LBD + PSP: a case study
I think if Frank's aunt had donated her brain and had a dx, we might have see a neurologist a lot sooner, it may not have helped but it was years that we just passed it off as nerves, age, lack of confindence??? If they do come up with better ways of treating the dementia diseases it may benefit one if they knew "what" disease their parent died with. Just my thought.

Gerry

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Gerry 67, cared for Frank 71, married 49 yrs; dx 2004, passed away October 26, 2011.


Thu Dec 16, 2010 5:51 am
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Location: Italy and Toronto (Canada)
Post Re: LBD + PSP: a case study
Robin,

Our neurologist did know about brain donation and as I mentioned earlier she runs a lot of research programs in cognitive neurology. However, for various reasons which she briefly explained (and I can't remember right now), they can not use my Dad's brain for pure research at the moment. She did encourage us to have it autopsied (with additional costs both for the autospy as well as the handling and other arrangements) if we needed a definitive diagnosis of LBD and other disorders (for the reasons mentioned in my earlier post).

As far as I know we don't have Mayo Clinics in Canada. But thank you for the lead regarding St. Michael's hospital. I will contact them to see if anything else can be done regarding brain donation. We have an appointment with the neurologist next tuesday and I will bring up the issue of brain donation again.

However, my sister and I both agree with Lynn. We don't need to know his definitive diagnosis after he's gone, even if he might have had another disorder with a genetic basis. Because apart from preparing ourselves for all possible dire conditions in our old age which we are already actively doing, we would rather not have to spend the rest of our lives waiting for the other shoe to drop.

We will donate Dad's brain only if we know for sure that it will be used for research purposes.

Shomy.


Thu Dec 16, 2010 9:09 am
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