Case report of 71 year-old woman with CBD

This is an abstract of a recently published article giving a case report of one woman with a clinical diagnosis of CBD. The symptoms and signs all point towards CBD — 71 years old, dysarthria, clumsiness, progressive asymmetric bradykinesia, rigidity in left arm, postural instability with falls, blepharospasm, dysphagia, dystonia in right arm, pyramidal signs (Babinski and Hoffmann), asymmetric cortical atrophy on MRI, visuospatial impairment, frontal-executive dysfunction, and hemineglect. The title of the article refers to “an unusual case of dementia,” but we aren’t told in the abstract why this case is unusual or given any details of the dementia (except we are told about the frontal-executive dysfunction).

Presumably the woman is still alive or she has died and didn’t donate her brain because there doesn’t seem to be pathologic confirmation of the diagnosis.

Robin

International Journal of Alzheimers Disease. 2011;2011:536141. Epub 2011 Jul 12.

An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature.

Mastrolilli F, Benvenga A, Di Biase L, Giambattistelli F, Trotta L, Salomone G, Quintiliani L, Landi D, Melgari JM, Vernieri F.
Department of Neurology, “Campus Biomedico” University, Rome, Italy.

Abstract
Corticobasal degeneration (CBD) is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition.

A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson’s Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs (Babinski and Hoffmann).

Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect.

This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition.

There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography (EEG) and electromyography (EMG) provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.

PubMedID#: 21785700 (see pubmed.gov for this abstract only)

Time to redefine the clinical diagnosis of corticobasal degeneration” (UCSF researchers)

This is news out of UCSF’s Memory & Aging Center. (UCSF = University of California San Francisco) The center has a special focus on CBD.

This short article about CBD in the latest issue of the UCSF MAC “Mind Matters” newsletter. Highlights include:

* “Much to the disappointment of the neurology community, it was recently discovered that nearly two-thirds of the patients diagnosed with CBD during life turned out to have another condition at autopsy.”

* “The results [of a recent review of clinical and MRI data in 58 CBD patients] suggested that the symptoms that best predicted CBD were progressive non-fluent aphasia, apathy, disinhibition with poor judgment or problems with leg movement. The MRI finding most suggestive of CBD was tissue loss in the frontal lobes and basal ganglia, not in the parietal lobe as was previously suspected.”

If you view the article online, you’ll see some brain images. And you can select “subscribe to list” online as well to get a subscription to future issues of this newsletter.

Robin

http://us1.campaign-archive1.com/?u=d90 … 742c689de9

UCSF Memory & Aging Center
“Mind Matters” Newsletter
Summer 2011

Seeing the Data with New Eyes
The data suggest that it is time to redefine the clinical diagnosis of corticobasal degeneration

Corticobasal degeneration (CBD) is a progressive neurological disorder first described in 1968 by Rebeiz, Richardson and Kolodny. Yet, only recently has there been a systematic effort to reliably diagnose this condition. Nothing can be more discouraging than to have a loved one misdiagnosed during life. Furthermore, it is now known that CBD is caused by abnormal accumulations of the protein tau and, as disease-specific therapies are emerging, getting the right diagnosis has become critically important.

Much to the disappointment of the neurology community, it was recently discovered that nearly two-thirds of the patients diagnosed with CBD during life turned out to have another condition at autopsy. Surprisingly, many of the patients suspected of suffering from CBD had Alzheimer’s disease as the underlying cause for their illness. Even more disturbing, patients who truly suffered from CBD were often misdiagnosed with other conditions during including progressive aphasia, frontotemporal dementia, Parkinson’s disease or progressive supranuclear palsy.

To address this problem, UCSF Memory and Aging Center researchers reviewed clinical and MRI data in 58 patients who met current clinical criteria for the diagnosis of CBD or suffered pathological criteria for CBD in order to identify the full spectrum of clinical and anatomical features that defined CBD. The results suggested that the symptoms that best predicted CBD were progressive non-fluent aphasia, apathy, disinhibition with poor judgment or problems with leg movement. The MRI finding most suggestive of CBD was tissue loss in the frontal lobes and basal ganglia, not in the parietal lobe as was previously suspected.

This work will soon be published in the prestigious journal Annals of Neurology. New research criteria for CBD are now being formulated, integrating observations from this study and others. Hopefully, some day soon, patients with CBD will receive the correct diagnosis and appropriate therapies.

“I was diagnosed with PSP in 2009” – what are the stages?

I received the following email:

“I was diagnossed with PSP only back in 2009. I started with the usual falling (bacwards of course and after reading the various posts, I quikly realised that this was normal as far as PSP goes.) I was very active before PSP. I also realize, that I am at the beginning stages from reading the posts. Where do I find the different stages? Are there such things – as stages? Can you help me?”

You can find some info on stages here at this post.  This document was written by laypeople (mostly caregivers). There has been discussion on the PSP Forum as to whether this document is of any value or not.

The best source of info on the clinical course “on average” of those with the two most common forms of PSP — Richardson’s Syndrome and PSP-Parkinsonism — is the “Clinical Outcomes” article by O’Sullivan, et al. Relevant posts are here.

Robin

 

Cytokines and neuro-inflammation in PSP

This is an abstract about research out of the University of Louisville. Here are the highlights from the abstract: “Although little is known about the etiology of progressive supranuclear palsy (PSP), genetic and epigenetic factors, oxidative injury and inflammation are thought to contribute to its development and/or progression. Evidence for activated glia involvement in PSP has raised the possibility that neuroinflammation may contribute to its pathogenesis. These results…[suggest] that these cytokines may contribute to the pathologic process. If so, the use of cytokine-inhibitors and/or other anti-inflammatory agents may be able to slow disease progression in PSP.”

Sorry but I couldn’t find a list of cytokine inhibitors. From spending a few minutes on the web looking into this, it seems that cytokine research is a rapidly evolving area.

Of course “anti-inflammatory agents” includes NSAIDs (nonsteroidal anti-inflammatory drugs). (Ibuprofen is an example of an NSAID.) The authors refer to a trial of NSAIDs in Alzheimer’s Disease in the final sentence of the article: “Although in the case of AD the evidence from anti-inflammatory clinical trials remains controversial, no such trials have been conducted in PSP patients.”

All of the brain tissue utilized for this study came from Mayo Jax. Tissue samples were used from both frozen brains and formalin-fixed brains. Thanks to all those who donate brain tissue of loved ones to Mayo Jax for making research like this possible.

I’ve copied the abstract below.

Robin


Parkinsonism and Related Disorders. 2011 Jul 6. [Epub ahead of print]

Cytokine expression and microglial activation in progressive supranuclear palsy.

Fernández-Botrán R, Ahmed Z, Crespo FA, Gatenbee C, Gonzalez J, Dickson DW, Litvan I.
Department of Pathology and Laboratory Medicine, School of Medicine, University of Louisville, Louisville, KY.

Abstract
Although little is known about the etiology of progressive supranuclear palsy (PSP), genetic and epigenetic factors, oxidative injury and inflammation are thought to contribute to its development and/or progression.

Evidence for activated glia involvement in PSP has raised the possibility that neuroinflammation may contribute to its pathogenesis.

To investigate the correlation between neuroinflammation and PSP, a comparative study was conducted on the patterns of cytokine expression in different regions of the brains of PSP, Alzheimer’s disease (AD) patients and normal controls.

Our results show different patterns of cytokine expression in each disease, with the expression of IL-1beta transcripts being significantly higher in the substantia nigra of PSP than in AD and controls, while AD brains had significantly higher IL-1beta expression in the parietal cortex compared to PSP and controls.

In addition, expression of TGFbeta was significantly higher in the cortical areas (particularly frontal and parietal lobes) of AD compared to PSP and controls.

These results show a disease-specific topographical relationship among the expression of certain cytokines (IL-1beta and TGFbeta), microglial activation and neurodegenerative changes, suggesting that these cytokines may contribute to the pathologic process. If so, the use of cytokine-inhibitors and/or other anti-inflammatory agents may be able to slow disease progression in PSP.

Copyright © 2011 Elsevier Ltd. All rights reserved.

PubMed ID#: 21741294 (see pubmed.gov for this abstract only)

“Support groups a lifesaver for caregivers”

This is a good article from the Herald-Tribune (heraldtribune.com) about support groups being a “lifesaver” for caregivers.  Brain Support Network coordinates a caregiver-only support group in the San Francisco Bay Area for Lewy Body Dementia, Progressive Supranuclear Palsy, Multiple System Atrophy, and Corticobasal Degeneration.  Here’s the Herald-Tribune article as to why you should join!

Robin

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www.heraldtribune.com/news/20110712/support-groups-a-lifesaver-for-caregivers

Support groups a lifesaver for caregivers
by Paula Falk, correspondent
Herald-Tribune
Posted Tuesday, Jul 12, 2011 at 12:01 AM

In the sometimes crazy, often exhausting and overwhelming world of caregiving, finding a safe place to share, occasionally vent and figure out what to do next isn’t always easy.
A support group can help hold life together when everything around you seems to be unraveling.

Without support, the impact of caregiving on one’s health can be devastating. It has been estimated that more than 60 percent of caregivers predecease their loved ones. As one long-term attendee of our support group at Senior Friendship Centers observed:

“Do you notice that individuals in our group are still here? I think one of the reasons is because we come to this support group. The support we receive here, the strategies we learn, the strength we gain to move forward and make decisions, all have an impact on our health and well being.”

What to expect: There are two rules in most support groups: personal information is confidential and one person talks at a time. People are given a chance to talk about their situation, and are never pressured to share anything if they feel uncomfortable doing so. Typically, new people tell something about themselves and their caregiving situation, and members of the group ask questions about the challenges they’re facing, what they need and what they are doing to get through it.

You’re not alone: You learn that you’re not the only one having trouble coping. Other members of the group listen and offer solutions. You are encouraged to take bits and pieces from the “experts” around the room who have been through many of the same experiences you’re facing, and to use what works for you. No one judges. When somebody is telling their story for the first time, you will see others nodding to indicate they have had a similar experience.

Your feelings count: “When people say “how are you?” they genuinely want to know how you are, instead of focusing only on your loved one,” as one participant said. All too often it can be easy for the caregiver’s needs and identity to get lost in the process of caring for another.

Taking action: As trust grows, people in the group become accountable to each other and often encourage one another to move forward to find solutions. Together, they help identify options, provide support, build confidence to make decisions, and take action.

For example, one couple attending a group hadn’t had a vacation or time to themselves for over a year. Their mother was in a facility being cared for, but they couldn’t imagine going away for even a day, let alone taking a trip, because they felt they needed to be there for her all the time. The group pointed out that she was well cared for, and it was OK to give themselves permission to care for themselves. It was a tremendous relief to them.

Healthy choices: Sometimes communication can be especially tough for men and for people who internalize stress. Finding a safe place to share feelings is important. We’re finding that more and more men are reaching out and joining support groups to ease the emotional and physical toll caregiving can take on their lives.

Note: Support groups specific to the disease your loved one has been diagnosed with are especially beneficial. There are support groups for Alzheimer’s, cancer, Parkinson’s, stroke and other diseases. Information is available through local organizations serving these diseases as well as through Senior Friendship Centers Caregiver Resource Center.

Paula Falk, the director of the Caregiver Resource Center (CRC) and Adult Day Service Program at The Living Room at Senior Friendship Centers’ Sarasota campus, writes a monthly column for Health + Fitness. The Caregiver Resource Center is a community collaboration bringing together agencies and businesses offering services and products to help caregivers. For more information, call 556-3270, email pf[email protected], or visit www.friendshipcenters.org.