Economic Costs of PSP and MSA in 3 European Countries

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This is a study of the economic costs of PSP and MSA in France, Germany, and the UK, looking at 742 people — 352 with PSP and 390 with MSA — over a 6-month time period. At the time of the study, the average disease duration was about 4 years. Included are “service costs,” which are the costs associated with seeing an MD or receiving tests, medication, treatment, or equipment, and “unpaid care costs,” which is the value of care provided by family and friends.

The authors state: “Measures of costs for a representative sample of patients allow us to determine relationships between care inputs and patient needs, assess the cost-effectiveness of specific treatments and highlight the impact that these conditions have on society as a whole.”

They found:

* “Unpaid care accounted for 68-76%” of the total costs.

* “Formal and unpaid costs were significantly higher the more severe the illness, as indicated by the Parkinson’s Plus Symptom scale.” And: “[Costs] increase with severity in each country for PSP patients. This is also generally the case for MSA patients, although in the UK the patients with the highest severity do not have the highest costs.”

* “This suggests that interventions which successfully modify disease progression may reduce the economic burden of these conditions.”

* “There was a significant inverse relationship between service and unpaid care costs.” I guess this means if someone is seeing an MD or receiving treatment, there’s less of a need for family and friends to provide “unpaid care.”

* “There was no relation between costs, formal or unpaid, and age or disease duration.”

* “The mean six-month service costs of PSP were €24,491 in France, €30,643 in Germany and €25,655 in the UK.”

25,000 Euros is about $34,000 US dollars. So this would mean that the annual costs are about $68K.

* “The costs for MSA were €28,924 [in France], €25,645 [in Germany] and €19,103 [in the UK].”

The authors compare their findings with data on the economic costs of other diseases: “Based on studies using a similar methodology, the six-month service costs of multiple sclerosis are around £8500 in the UK and €10000 in Germany. Likewise, the six-month costs of Alzheimer’s disease in the UK is approximately €13000 per person and schizophrenia €5500. Whilst these figures indicate the high care needs of PSP and MSA relative to other conditions, it should be recognised that the total ‘burden’ will be less given the lower prevalence rates. Of particular interest, and in common with other degenerative conditions, is the fact that unpaid family care accounts for most of the cost.”

I’ve copied the abstract below. The full article is available online at no charge through the PloS One journal’s website:
http://www.plosone.org/article/info%3Ad … ne.0024369

Robin

PLoS One. 2011;6(9):e24369. Epub 2011 Sep 8.

The Economic Costs of Progressive Supranuclear Palsy and Multiple System Atrophy in France, Germany and the United Kingdom.

McCrone P, Payan CA, Knapp M, Ludolph A, Agid Y, Leigh PN, Bensimon G; for the NNIPPS Study Group.
Health Service and Population Research Department, Institute of Psychiatry, King’s College London, London, United Kingdom.

Abstract
Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are progressive disabling neurological conditions usually fatal within 10 years of onset.

Little is known about the economic costs of these conditions. This paper reports service use and costs from France, Germany and the UK and identifies patient characteristics that are associated with cost. 767 patients were recruited, and 760 included in the study, from 44 centres as part of the NNIPPS trial. Service use during the previous six months was measured at entry to the study and costs calculated. Mean six-month costs were calculated for 742 patients.

Data on patient sociodemographic and clinical characteristics were recorded and used in regression models to identify predictors of service costs and unpaid care costs (i.e., care from family and friends).

The mean six-month service costs of PSP were €24,491 in France, €30,643 in Germany and €25,655 in the UK.

The costs for MSA were €28,924, €25,645 and €19,103 respectively.

Unpaid care accounted for 68-76%. Formal and unpaid costs were significantly higher the more severe the illness, as indicated by the Parkinson’s Plus Symptom scale. There was a significant inverse relationship between service and unpaid care costs.

PubMed ID#: 21931694

Biomarker confirmed PSP and symptom explanation

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One sentence in this hard-to-understand abstract caught my eye: “Thus, in a clinically diagnosed and biomarker-confirmed cohort with early PSP, we demonstrate that neostriatal volume and shape are significantly reduced in vivo.”

I wondered what they meant by biomarker-confirmed PSP. There is something called the “penguin” or “hummingbird” sign, which is how the pons and atrophic midbrain appear on a mid-sagittal MRI. It is present in even mild to moderate PSP. I have never heard of anyone in our local support group having an MRI that shows the penguin sign or hummingbird sign. Years ago, I visited the Mayo Jax Brain Bank; one of the researchers there showed me the hummingbird sign on several MRIs.

The point of this study of 15 Swedish PSP patients is that there’s an anatomical basis for “the neuropsychiatric manifestations of PSP, such as the dysexecutive syndrome, gaze palsy, obsessive–compulsive phenomena and utilization behavior.” (I’m not sure how gaze palsy is a neuropsychiatric symptom but anyway…) Similarly, there is an anatomical basis for “the movement disorders of PSP, bradykinesia, gait disturbance and rigidity.”

Here’s the abstract, which is loaded with anatomical terms.

Robin

Psychiatry Research. 2011 Sep 5. [Epub ahead of print]

Morphometric analysis of subcortical structures in progressive supranuclear palsy: In vivo evidence of neostriatal and mesencephalic atrophy.

Looi JC, Macfarlane MD, Walterfang M, Styner M, Velakoulis D, Lätt J, van Westen D, Nilsson C.
Research Centre for the Neurosciences of Ageing, Academic Unit of Psychological Medicine, School of Clinical Medicine, Australian National University Medical School, Canberra, Australia.

Abstract
Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by gait and postural disturbance, gaze palsy, apathy, decreased verbal fluency and dysexecutive symptoms, with some of these clinical features potentially having origins in degeneration of frontostriatal circuits and the mesencephalon.

This hypothesis was investigated by manual segmentation of the caudate and putamen on MRI scans, using previously published protocols, in 15 subjects with PSP and 15 healthy age-matched controls.

Midbrain atrophy was assessed by measurement of mid-sagittal area of the midbrain and pons. Shape analysis of the caudate and putamen was performed using spherical harmonics (SPHARM-PDM, University of North Carolina).

The sagittal pons area/midbrain area ratio (P/M ratio) was significantly higher in the PSP group, consistent with previous findings. Significantly smaller striatal volumes were found in the PSP group – putamina were 10% smaller and caudate volumes were 17% smaller than in controls after controlling for age and intracranial volume. Shape analysis revealed significant shape deflation in PSP in the striatum, compared to controls; with regionally significant change relevant to frontostriatal and corticostriatal circuits in the caudate.

Thus, in a clinically diagnosed and biomarker-confirmed cohort with early PSP, we demonstrate that neostriatal volume and shape are significantly reduced in vivo.

The findings suggest a neostriatal and mesencephalic structural basis for the clinical features of PSP leading to frontostriatal and mesocortical-striatal circuit disruption.

Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

PubMed ID#: 21899988 (see pubmed.gov for this abstract only)

AgD (argyrophilic grain disease) – Alzheimer Europe description

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I ran across this short description of AgD (argyrophilic grain disease) recently on the Alzheimer Europe website. I’ve been sharing it with the folks I know whose family members are diagnosed upon brain autopsy with a neurodegenerative disease (such as PSP or CBD) *plus* AgD.

—————————–

http://www.alzheimer-europe.org/Dementi … isease-AGD

Argyrophilic Grain Disease (AGD)
Neuro-Degenerative Diseases

by André Delacourte & Kurt Jellinger

General outline

New disease, which is not fully characterised. A sporadic late-onset form of dementia characterised by a neuro-degenerative process, which mainly affects limbic structures (amygdala, hippocampus and mediobasal temporal/entorhinal cortex).

It is named after silver-staining (argyrophilic) grains or coiled bodies within the cytoplasm of neurons that consist mainly of tau protein isoforms with four microtubule-binding repeates (4-R tau).

Synonym:  Braak’s disease

Symptoms and course

Reduction of short-term memory, disorders of word finding, disorders of reading and writing, disorientation, behavioural disturbances (personality changes, emotional disorders with aggression and ill-temper) may precede or follow memory failure. Clinically it is hard to distinguish from late-onset AD.

The age of onset is around 70 years old. The duration of the disease is between 4 and 8 years.

Causes and risk factors

Neuron degeneration likely associated with dysfunction of tau protein. Grains are composed of abnormally phosphorylated tau protein with 4 repeats. Recent studies indicate that tau protein dysfunction in AGD in contrast to other 4-R-tauopathies (progressive supranuclear palsy, corticobasal degeneration).

Genetics

The disease arises irrespective of the genetic background regarding tau H1 or H2 haplotypes, at the opposite of PSP and CBD (Miserez A. R. et al, 2003). Lack of relationship with apolipoprotein E4.

Frequency

1 to 5% of AD patients (Togo T. et al, 2002).

Diagnostic procedures

It is almost impossible to distinguish from late-onset Alzheimer’s disease. The diagnosis is almost entirely made by post-mortem examination. AGD lesions are found in about 5% of Alzheimer’s disease (Togo T. et al, 2002).

Last Updated: Friday 09 October 2009

“Deciding to Die, Then Shown the Door” (NYT)

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This is quite a story in the New York Times about a couple deciding not to eat/drink any more, and then being forced to leave their care facility:

newoldage.blogs.nytimes.com/2011/08/24/deciding-to-die-then-shown-the-door/

The New Old Age: Caring and Coping
Deciding to Die, Then Shown the Door
The New York Times
By Paula Span
August 24, 2011, 1:59 PM

In our local support group, we’ve had many people who have decided to stop eating and drinking.  I only know of one case where this decision was treated as a “problem.”  Recently, a woman who had progressive supranuclear palsy (PSP) for several years wanted to stop eating/drinking.  The woman was on hospice at this time.  Her daughter talked to the hospice agency about her mother’s wishes.  The hospice agency said that they could not discuss this topic with either the mother or the daughter.  So the daughter had to learn about dying in this manner on her own, and had to rely on others for support during this stressful time.  The mother did decide to stop eating/drinking.  This was communicated to hospice after several days.  At that point, the hospice agency returned to being supportive again (mostly).  The daughter kept in touch with me throughout this process (as we made brain donation arrangements), and reported that many friends and family were able to visit during this time.  The mother died peacefully.  Well before her death, she requested that her brain be donated.  (Mayo Jax confirmed the PSP diagnosis.)

The Multiple Phenotypes of CBS/CBD (Boeve article)

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This review article by Dr. Brad Boeve of Mayo Rochester, one of the world’s experts in CBS/CBD, is a good one. It’s a short summary of our current knowledge about CBS and CBD.

Here are a few highlights:

* When thinking of CBS and CBD, “[there] are few other syndromes and diseases in the neurodegenerative disease field that are as complex and frustrating for patients, their relatives, the clinicians involved in their care, the pathologists who analyze tissue, and the scientists devoted to their study.”

* There are primarily six clinical syndromes that are associated with CBD pathology:

#1 behavioral variant frontotemporal dementia
#2 progressive apraxia of speech or progressive aphasia syndrome (PNFA is the most common though SD and LPA can occur) [PNFA = progressive nonfluent aphasia. SD = semantic dementia. LPA = logopenic progressive aphasia] #3 corticobasal syndrome
#4 PSP Richardson syndrome [PSP = progressive supranuclear palsy] #5 dementia syndrome highly consistent with probable Alzheimer’s Disease
#6 posterior cortical atrophy

There are “other rare variants” besides these six.

* The clinical syndrome CBS has many underlying diseases or pathologies associated with it — CBD, PSP, AD, Pick’s Disease, etc. “On one level, this heterogeneity is difficult to explain, yet a unifying pathologic feature of all of these diseases is the maximal involvement of the parietofrontal cortex and their afferent and efferent projections. The topographic distribution of neurodegeneration, therefore, dictates the clinical syndrome.”

I’ve copied the abstract below.

Robin

Here’s the abstract:

Journal of Molecular Neuroscience. 2011 Aug 19. [Epub ahead of print]

The Multiple Phenotypes of Corticobasal Syndrome and Corticobasal Degeneration: Implications for Further Study.

Boeve BF.
Divisions of Behavioral Neurology and Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, MN, USA

Abstract
Corticobasal degeneration (CBD) is a complex neurodegenerative disorder which nomenclature of which its nomenclature and characterization continues to evolve. The core clinical features that have been considered characteristic of the disorder include progressive asymmetric rigidity and apraxia, with other findings suggesting additional cortical (e.g., alien limb phenomena, cortical sensory loss, myoclonus, and mirror movements) and basal ganglionic (e.g., bradykinesia, dystonia, and tremor) dysfunctions.

The characteristic findings at autopsy are asymmetric cortical atrophy that is typically maximal in the frontoparietal regions, as well as basal ganglia and nigral degeneration.

Microscopically, abnormal accumulations of the microtubule-associated tau protein are found in both neurons and glia, and this disorder is now considered one of the “tauopathies.”

CBD was initially thought to represent a distinct clinicopathologic entity. Recent studies have shown considerable clinicopathologic heterogeneity, leading some to use the term “corticobasal syndrome” (CBS) for the constellation of findings initially considered characteristic of the disorder, and the term “corticobasal degeneration” for the histopathologic disorder.

In this review, the multiple phenotypes/syndromes associated with CBD pathology, and multiple diseases associated with the CBS, are presented. The clinicopathologic heterogeneity in CBS/CBD and the implications of this heterogeneity on clinical practice, on understanding the focal/asymmetric cerebral degeneration syndromes, and on future research are all reviewed.

PubMed ID#: 21853287 (see pubmed.gov for this abstract only)