Man with double vision and dementia; PSP upon autopsy

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PSP folks –

This is an interesting case report of a 49-year-old man who was found to have the dementia form of PSP upon brain autopsy. That form of PSP is called “Richardson syndrome (RS).”

I don’t believe this man was diagnosed during life with PSP because he presented with atypical features for PSP: “young age at onset, absence of falls, and the presenting complaint of horizontal diplopia (due to vergence abnormalities). His cognitive impairment was suggestive of frontotemporal dementia. However, vertical saccades were slow at presentation.”

I note that the patient clapped exactly three times. This must refer to the “clap test,” which has now been greatly discounted as a neurological test for PSP.

The patient received extensive testing, including neuro-ophthalmological testing. The clinicians have gone back through the patient’s clinical records after death to try to solve the mystery of how they missed this patient’s PSP.
The researchers conclude “that careful examination of the speed (more than amplitude) of vertical saccades in patients with undiagnosed parkinsonian disorders remains the cornerstone for recognition of PSP and differentiation from other parkinsonian disorders.”

The citation is below.

The text refers to images of brain tissue available as a result of the brain autopsy. You can find those images at the Neurology journal’s website here:
http://neurology.org/cgi/content/full/73/24/2122/DC1 (6 images available for free)

Robin

Neurology. 2009 Dec 15;73(24):2122-4.

Evolution of oculomotor and clinical findings in autopsy-proven Richardson syndrome.

Hardwick A, Rucker JC, Cohen ML, Friedland RP, Gustaw-Rothenberg K, Riley DE, Leigh RJ.
Department of Neurology, Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH, USA.

From the Departments of Neurology (A.H., K.G.-R., D.E.R., R.J.L.) and Neuropathology (M.L.C.), University Hospital, and Daroff-Dell’Osso Laboratory (R.J.L.), Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, OH; Department of Neurology (J.C.R.), University of Louisville School of Medicine, Louisville KY; and Mount Sinai Medical Center (R.P.F.), New York, NY.

PubMed ID#: 20018641 (there’s nothing viewable at pubmed.gov on this article)

Gizmo, Care Status System, and New Caregiver Website

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Here’s a recent blog post in the “New Old Age” blog in the New York Times (nytimes.com).
The article describes:

* one tech gizmo: the Wellcore fall detection and emergency response device;
* a system to stay up to date on your loved one (whom you are not living with): Connect for Healthcare care recipient status system; and
* a new website for caregivers: ecarediary.com that supplies a variety of tools for caregivers.

Here’s a link:

Old Age, New Gizmos
By Paula Span
New York Times
January 6, 2010, 9:56 am

Robin

Dementia and hallucinations in PD related to age of patient

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LBD folks –

I didn’t get a huge amount out of this abstract. These two sentences were interesting:

“Progression of PD, including the development of dementia, and hallucinations is related to the age of the patient rather than the age of disease onset.” I guess this isn’t surprising since PD is an age-related disease.

“The formation of Lewy bodies may represent a marker for protective mechanisms against age-related dysfunction and degeneration of the nervous system.” So the brain may produce Lewy bodies to protect itself.

I’ve copied the abstract below.

Robin

Age and Ageing. 2010 Jan 5.

Ageing, neurodegeneration and Parkinson’s disease.

Hindle JV.
Llandudno Hospital, Care of the Elderly, Hospital Road, Llandudno, UK.

Age is the largest risk factor for the development and progression of Parkinson’s disease (PD).

Ageing affects many cellular processes that predispose to neurodegeneration, and age-related changes in cellular function predispose to the pathogenesis of PD.

The accumulation of age-related somatic damage combined with a failure of compensatory mechanisms may lead to an acceleration of PD with age.

The formation of Lewy bodies may represent a marker for protective mechanisms against age-related dysfunction and degeneration of the nervous system.

Mild parkinsonian signs may be present in older people, which are associated with reduced function. These may be due to age-related decline in dopaminergic activity, incidental Lewy body disease, degenerative pathologies (early PD and Alzheimer’s disease) or vascular pathology.

Ageing may affect the clinical presentation of PD with altered drug side effects, increased risk of developing dementia and an increased likelihood of admission to a nursing home.

Progression of PD, including the development of dementia, and hallucinations is related to the age of the patient rather than the age of disease onset.

PD may reflect a failure of the normal cellular compensatory mechanisms in vulnerable brain regions, and this vulnerability is increased by ageing. PD is one of the best examples of an age-related disease.

PubMed ID#: 20051606 (see pubmed.gov for the abstract only)

“Overlooking the Frail Years” with Binary Thinking

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This post to the “New Old Age” blog of The New York Times (nytimes.com) mentions the case of one healthy 75-year-old who seems willing to consider his death but not willing to consider being sick. Here’s an excerpt:

“This kind of binary thinking — either I’m healthy and fine, or I’m outta here — and the reluctance to look at the frailty likely to occur in between seem to me quite common. Yet most elderly Americans – more than two-thirds of current 65-year-olds, according to a detailed 2005 projection by a team of health policy analysts — at some point will need assistance to cope with daily living, either paid help or unpaid, at home or in a facility. … But this unwillingness to contemplate that possibility can have unhappy consequences, Dr. Gillick pointed out. It can lead fragile older people to undergo aggressive medical treatments they may later regret, for instance, especially when their physicians also engage in binary thinking, or at least binary explanations.”

Here’s a link to the full article:

Overlooking the Frail Years
By Paula Span
The New York Times
January 1, 2010, 3:27 pm

LBD preceded by burning mouth syndrome

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LBD folks –
Someone posted about the problem of a “burning mouth” over a year ago on the LBDA Forum. Now we have a published case report about it.
Robin

Revue Neurologique (Paris). 2009 Dec 30.

[Atypical Lewy body disease revealed by burning mouth syndrome and a pseudo-psychiatric syndrome.] [Article in French]

Varvat J, Thomas-Anterion C, Decousus M, Perret-Liaudet A, Laurent B.
Service de neurologie, CHU Nord, Saint-Etienne cedex, France.

INTRODUCTION: Among the degenerative diseases of the nervous system, Lewy body disease has the most psychiatric symptoms especially hallucinations, delusion and identification disorders.

CASE REPORT: We report a case of Lewy body disease that started with a burning mouth syndrome for three year as the only symptom before the development of a pseudo-psychiatric syndrome (melancholy and Capgras). None of the usual cardinal criteria were present. MRI, cerebrospinal fluid, and DAT scan((R)) findings enabled the diagnosis.

CONCLUSION: The dopaminergic hypothesis put forward in some cases of burning mouth syndrome might explain this symptom in Lewy body disease.

PMID: 20045161