Case report- PSP-P patient recvd DBS

This case report was published last year. It’s about an Italian patient diagnosed clinically with PSP-P (the parkinsonism form of PSP, not the dementia form of PSP) who received deep brain surgery, with an implant placed in an area of the brain called the nucleus tegmenti pedunculopontini (PPTg). This letter reports that the procedure was safe and the patient saw “modest benefits.”

“Gait was improved only in terms of a consistent amelioration of the primary gait ignition failure. On the other hand, the impact on postural stability and direction changes was absent. When evaluated with a specific questionnaire as the Giladi for FOG [freezing of gait] DBS-mediated effect were modest. DBS-related effect could be detected in non-motor domains (such as dysphagia and swallowing impairment). Neuropsychological tests did not reveal major cognitive changes apart from minimal improvement in verbal fluency.

The researchers considered this surgery based upon published data showing “some degree of gait impairment amelioration and cognitive benefits” to PD patients where an implant was placed in a similar part of the brain. In this PSP-P patient, the researchers saw no cognitive benefits and gait improvement only in terms of “consistent amelioration of the primary gait ignition failure.”

The researchers conclude: “Although this single case seems not to support the PPTg implantation as a critical therapeutic option for PSP-P patients, at least in advanced ones, it is possible that larger trials, centred either on PSP-P recruited at the disease onset or on PSP Richardson type, will verify further the potential of DBS-mediated stimulation to activate directly surviving fibres or even delay the disease evolution.”

I’ve copied the citation below.

Robin

Movement Disorders
Volume 24 Issue 13, Pages 2020 – 2022
Published Online: 11 Aug 2009

Letter to the Editor
Implantation of the Nucleus Tegmenti Pedunculopontini in a PSP-P Patient: Safe Procedure, Modest Benefits

Livia Brusa, MD, PhD 1, Cesare Iani, MD 1, Roberto Ceravolo, MD 2, Salvatore Galati, MD 3 4, Vincenzo Moschella, MD 2 4, Francesco Marzetti, MD 3 4, Paolo Stanzione, MD 3 4, Alessandro Stefani, MD 3 4

1 Ospedale S.Eugenio UOC Neurologia, Rome, Italy
2 Dipartimento di Neuroscienze, Clinica Neurologica, Università di Pisa, Pisa, Italy
3 Clinica Neurologica, Universita’ di Roma Tor Vergata, Rome, Italy
4 IRCCS Fondazione S. Lucia, Rome, Italy

Note: no abstract is available on PubMed. The PubMed ID# for this letter is #19672983.

FDG-PET for differentiating PD, PSP, and MSA

This article on the use of FDG-PET in differentiating PD, MSA, and PSP was published today in The Lancet Neurology. I haven’t had a chance to wade through all of the article yet but it seems to be one of the better articles we’ve seen lately on PSP and MSA. 167 patients in the NY area participated in the study. Only 9 of these patients have died and donated brain tissue thus far. So I’m not sure how “real” the diagnostic accuracy percentages are. And the diagnosis of CBD was not part of the study.

Below, I’ve copied a HealthImaging.com article on this research as well as the abstract.

Robin


The Lancet Neurology, Early Online Publication, 11 January 2010.

Differential diagnosis of parkinsonism: a metabolic imaging study using pattern analysis

Chris C Tang MD, Kathleen L Poston MD, Thomas Eckert MD, Andrew Feigin MD, Steven Frucht MD, Mark Gudesblatt MD, Vijay Dhawan PhD, Martin Lesser PhD, Jean-Paul Vonsattel MD, Stanley Fahn MD, David Eidelberg MD

Summary

Background
Idiopathic Parkinson’s disease can present with symptoms similar to those of multiple system atrophy or progressive supranuclear palsy. We aimed to assess whether metabolic brain imaging combined with spatial covariance analysis could accurately discriminate patients with parkinsonism who had different underlying disorders.

Methods
Between January, 1998, and December, 2006, patients from the New York area who had parkinsonian features but uncertain clinical diagnosis had fluorine-18-labelled-fluorodeoxyglucose-PET at The Feinstein Institute for Medical Research. We developed an automated image-based classification procedure to differentiate individual patients with idiopathic Parkinson’s disease, multiple system atrophy, and progressive supranuclear palsy. For each patient, the likelihood of having each of the three diseases was calculated by use of multiple disease-related patterns with logistic regression and leave-one-out cross-validation. Each patient was classified according to criteria defined by receiver-operating-characteristic analysis. After imaging, patients were assessed by blinded movement disorders specialists for a mean of 2·6 years before a final clinical diagnosis was made. The accuracy of the initial image-based classification was assessed by comparison with the final clinical diagnosis.

Findings
167 patients were assessed. Image-based classification for idiopathic Parkinson’s disease had 84% sensitivity, 97% specificity, 98% positive predictive value (PPV), and 82% negative predictive value (NPV). Imaging classifications were also accurate for multiple system atrophy (85% sensitivity, 96% specificity, 97% PPV, and 83% NPV) and progressive supranuclear palsy (88% sensitivity, 94% specificity, 91% PPV, and 92% NPV).

Interpretation
Automated image-based classification has high specificity in distinguishing between parkinsonian disorders and could help in selecting treatment for early-stage patients and identifying participants for clinical trials.

Funding
National Institutes of Health and General Clinical Research Center at The Feinstein Institute for Medical Research.

http://www.healthimaging.com/index.php? … e&id=20154

TOP STORIES
Lancet: FDG-PET could distinguish between Parkinsonian disorders
Written by Editorial Staff, HealthImaging.com
January 11, 2010

FDG-PET imaging-based classification has high specificity to differentiate individual patients with idiopathic Parkinson’s disease, multiple system atrophy and progressive supranuclear palsy and could help in selecting treatment for early-stage patients and identifying participants for clinical trials, according to research published online Jan. 11 in Lancet Neurology.

David Eidelberg, MD, director of the center for neurosciences at the Feinstein Institute for Medical Research in Manhasset, N.Y., and colleagues assessed whether metabolic brain imaging combined with spatial covariance analysis could accurately differentiate between patients with Parkinsonism who had different underlying disorders.

In the study, 167 patients who had Parkinsonian features but uncertain clinical diagnosis had an 18F-FDG PET scan. The researchers developed an automated image-based classification procedure to differentiate individual patients with Parkinsonian disorders and the accuracy was assessed by comparison with the final clinical diagnosis.

Eidelberg said that out of the 167 patients assessed, image-based classification for idiopathic Parkinson’s disease had 84 percent sensitivity, 97 percent specificity, 98 percent positive predictive value (PPV) and 82 percent negative predictive value (NPV).

Eidelberg and colleagues found that imaging classifications were also accurate for multiple system atrophy (85 percent sensitivity, 96 percent specificity, 97 percent PPV, and 83 percent NPV) and progressive supranuclear palsy (88 percent sensitivity, 94 percent specificity, 91 percent PPV and 92 percent NPV).

In an accompanying commentary, Angelo Antonini, MD, at IRCCS San Camillo, Venice and Parkinson Institute in Milan, Italy, wrote that the “clinical and research relevance of these findings should not be underestimated. Neuroprotective and disease-modifying drug research is intensifying and results mostly rely on accurate early diagnosis.”

“The excellent specificity and PPV of the imaging classification makes this test suitable for diagnostic use rather than as a screening tool,” Eidelberg noted.

“Although imaging might be cost effective for early diagnosis, I expect that these procedures will find their natural application in the identification of suitable candidates for drug trials or complex surgical procedures (example, deep brain stimulation, stem-cell transplantation or fetal tissue transplantation). However, additional blinded, prospective, multicenter studies will first be needed to confirm the accuracy of this pattern-based categorization procedure,” Antonini concluded.

Last updated on January 11, 2010 at 12:48 pm EST

Dementia with Lewy Bodies – “Patient page. Not all dementia is Alzheimer”

I was reading over someone’s brain autopsy report today, and it contained this sentence:

“Considering the degree of Alzheimer type pathology and the distribution of Lewy bodies, the likelihood that this patient would have had clinical features of dementia with Lewy bodies is intermediate.”

The source of the “intermediate” evaluation was the “Third report of the DLB consortium,” which was published in 2005.  That “Third report” is the key diagnostic guide for Dementia with Lewy Bodies.  You can find the criteria on the Brain Support Network website here:

www.brainsupportnetwork.org/education/lewy-body-dementia/

So…I started digging around on PubMed and discovered that you’d have to pay for the “Third report” but there’s an associated “Patient Page” that is free.  I think this “Patient Page” is designed to be given to patients and families by their neurologists.  It’s an understandable two-page description of DLB and other forms of dementia.

Here’s the citation for the “Patient Page.”

Neurology. 2005 Dec 27;65(12):E26-7.
Patient page. Not all dementia is Alzheimer: dementia with Lewy bodies.
Knopman D, Jankowiak J.
PubMed ID#: 16380603

Here are links to the “Patient Page,” available for free online:

www.neurology.org/cgi/content/full/65/12/E26 –> HTML version

www.neurology.org/cgi/reprint/65/12/E26 –> PDF version

This might be a useful document to share with your general neurologist or primary care physician.

Man with double vision and dementia; PSP upon autopsy

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

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