PSP, CBD, MSA, + LBD are approved for Compassionate Allowance

Date
Posted by

I received this email today from Richard Zyne of CurePSP about PSP, CBD, and MSA being added to the list of disorders approved for Compassionate Allowance by the Social Security Administration.  In the attachment that accompanied the email (which I haven’t provided here), all four of the disorders in our support group — PSP (progressive supranuclear palsy), CBD (corticobasal degeneration), MSA (multiple system atrophy), and LBD (Lewy body dementia) — are on the Compassionate Allowance list, effective 12/10/11.  This means that those diagnosed with any of these four disorders will have an easier time to be approved for Social Security Disability.

————————————-

To All Board Members and Staff:

Good News—all of our Atypical Parkinsonisms have just been approved by the Social Security Administration for Compassionate Allowance.  Commissioner Astrue made the announcement today and I just got off the phone with Art Spencer, Associate Commissioner, Office of Disability Programs.

The four disorders include PSP, CBD, MSA, and ALS/PDC [ALS/Parkinsonism Dementia Complex].

Social Security has an obligation to provide benefits quickly to applicants whose medical conditions are so serious that their conditions obviously meet disability standards.

Compassionate Allowances (CAL) are a way of quickly identifying diseases and other medical conditions that invariably qualify under the Listing of Impairments based on minimal objective medical information. Compassionate Allowances allow Social Security to quickly target the most obviously disabled individuals for allowances based on objective medical information that we can obtain quickly.

CAL conditions are developed as a result of information received at public outreach hearings, comments received from the Social Security and Disability Determination Service communities, counsel of medical and scientific experts, and our research with the National Institutes of Health (NIH). Also, SSA considered which conditions are most likely to meet current definitions of disability.

Commissioner Astrue has held seven Compassionate Allowances public outreach hearings. The hearings were on rare diseases, cancers, traumatic brain injury (TBI) and stroke, early-onset Alzheimer’s disease and related dementias, schizophrenia, cardiovascular disease and multiple organ transplants and autoimmune diseases.

The decision to include our neurodegenerative disorders was based on the clinical information which we have provided over the past couple of years (Thank you Drs. Golbe and Steele) and it became clearly obvious to the Commissioner that they qualified for this program.  I have attached the new list of disorders, which was sent to me by Art Spencer.  See the last four on the list.

I am very pleased that CurePSP has been able to advocate on behalf of our patients for this important benefit.

Richard

Richard Gordon Zyne, DMin
President-CEO
 

Treatment of DLB and PDD – A Literature Review

Date
Posted by

This set of authors writes a lot of medical journal articles and materials for laypeople on dementia.  This recently-published article is a review of what is known about pharmacological treatment of Dementia with Lewy Bodies and Parkinson’s Disease Dementia.  It’s relatively short and understandable.  I’ve copied the abstract below.
Robin

—————————

Drugs & Aging. 2011 Oct 1;28(10):769-77.

Treatment of dementia with lewy bodies and Parkinson’s disease dementia.

Ballard C, Kahn Z, Corbett A.
Kings College London, London, UK.

Abstract
Parkinson’s disease dementia (PDD) and dementia with Lewy bodies (DLB) account for 10-15% of late onset dementias. Key treatment targets include cognitive and functional impairments, neuropsychiatric symptoms including intense and persistent visual hallucinations, and parkinsonism.

Six-month, placebo-controlled randomized controlled trials (RCTs) of the cholinesterase inhibitor rivastigmine have indicated modest but significant benefits in cognition, function, global outcome and neuropsychiatric symptoms in both PDD and DLB. The evidence base for other cholinesterase inhibitors from RCTs is inconclusive.

More recent RCTs with memantine in PDD/DLB patients indicate a benefit with regard to global outcome, with some suggestion of a specific benefit with respect to sleep disturbance.

Given the risk of severe antipsychotic sensitivity reactions, antipsychotics should be avoided.

A significant proportion of PDD/DLB patients are responsive to levodopa, but care needs to be taken with anti-parkinsonian treatments because of their potential propensity to exacerbate neuropsychiatric symptoms, particularly hallucinations.

PubMed ID#: 21970305  (see pubmed.gov for this abstract only)

Mayo Clinic research on forms of Alzheimer’s, including hippocampal sparing

Date
Posted by

One of Brain Support Network’s missions is to assist families around the United States with brain donation.  We have helped over 100 families donate a loved one’s brain.  Half of the confirmed diagnoses are different from the clinical diagnoses! We often see one diagnosis “wrong” more often than others – corticobasal degeneration. Often, those with supposed CBD have an atypical form of Alzheimer’s confirmed through brain donation.  And we sometimes see the same pattern in frontotemporal dementia (FTD).

Recently, the Mayo Clinic published research on the various types of Alzheimer’s Disease (AD).  Two atypical forms of AD were specifically highlighted — hippocampal sparing (HpSp) and limbic-predominant (LP).  We’ve seen this HpSp type on several neuropathology reports for people who were thought to have CBD and a few for FTD.

Here’s a link to the Mayo research:

ncbi.nlm.nih.gov/pmc/articles/PMC3175379/

Neuropathologically defined subtypes of Alzheimer’s disease with distinct clinical characteristics: A retrospective study

by Melissa E. Murray, PhD, Neill R. Graff-Radford, MBBCh, FRCP (London), Owen A. Ross, PhD, Ronald C. Petersen, MD, Ranjan Duara, MD, and Dennis W. Dickson, MD

Lancet Neurol. 2011 Sep; 10(9): 785–796.
Published online 2011 Jul 27. 

Well worth reading…

Robin

 

“Rapidly Progressive” (3 Mos) Corticobasal Syndrome (CBS) Turns out to be Creutzfeldt-Jakob disease (CJD)

Date
Posted by

“Case Reports in Neurology” is a new open access journal. The articles are available online only and at no charge.

A report was published recently on a case of “rapidly progressive” corticobasal syndrome. “A 74-year-old woman presented with language impairment, difficulty in walking and poor attentiveness that had begun 10 days before. Other symptoms, such as asymmetrical extra-pyramidal dysfunction, limb dystonia and ‘alien limb’ phenomena, were established over the next 2 months, with rapid progression.”

After three months, the woman died. Upon brain autopsy, it was determined that she had CJD (Creutzfeldt-Jakob disease), sometimes called the human version of “mad cow disease.” (In the US, special precautions are taken when someone with a rapidly progressive dementia donates his/her brain. Presumably the same is true in Portugal, where this woman lived.)

A few people here have reported this same outcome — CJD is found upon brain autopsy. But I think all the people here whose loved ones had a confirmed CJD diagnosis had received clinical diagnoses of PSP rather than CBD.

The authors seem to imply that they used MRI scan to diagnose this woman while alive with CJD. They describe how an MRI is helpful in diagnosing CBD: “On MRI, CBD patients may present asymmetric frontoparietal and midcallosal atrophy. Additionally, on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images, they may also show putaminal hypointensity and subtle subcortical periventricular hyperintensity. On DWI, extensive hyperintensity in the frontoparietal white matter, especially on the predominant side of cortical atrophy, has been described. These features are especially evident in the early course of CBD.”

I’ve copied the abstract below.

The link to get to the article (PDF) online is very long:

http://content.karger.com/ProdukteDB/pr … elNr=32982\
0&Ausgabe=255291&ProduktNr=238704&filename=329820.pdf

It might be easier to go first to the journal’s home page here:
http://content.karger.com/ProdukteDB/pr … roduktNr=2\
38704

Then click on the “May – August” issue. Then click on the article that begins
on page 185.

Robin

Case Reports in Neurology. 2011 May;3(2):185-190. Epub 2011 Aug 23.

Rapidly Progressive Corticobasal Degeneration Syndrome.

Valverde AH, Costa S, Timoteo A, Ginestal R, Pimentel J.
Department of Neurology, Hospital Prof. Dr. Fernando Fonseca, EPE, Amadora, EPE/Hospital de Santa Maria, Lisbon, Portugal.

Abstract
INTRODUCTION:
Corticobasal syndrome (CBS) has a heterogeneous clinical presentation with no specific pathologic substratum. Its accurate diagnosis is a challenge for neurologists; in order to establish CBS definitively, postmortem confirmation is required. Some clinical and radiological features can help to distinguish it from other neurodegenerative conditions, such as Creutzfeldt-Jakob disease (CJD).

CLINICAL CASE:
A 74-year-old woman presented with language impairment, difficulty in walking and poor attentiveness that had begun 10 days before. Other symptoms, such as asymmetrical extra-pyramidal dysfunction, limb dystonia and ‘alien limb’ phenomena, were established over the next 2 months, with rapid progression. Death occurred 3 months after symptom onset. Laboratory results were normal. Initially, imaging only showed restricted diffusion with bilateral parieto-occipital gyri involvement on DWI-MRI, with unspecific EEG changes. An autopsy was performed. Brain neuropathology confirmed sporadic CJD (sCJD).

CONCLUSIONS:
CBS is a heterogeneous clinical syndrome whose differential diagnosis is extensive. CJD can occasionally present with clinical characteristics resembling CBS. MRI detection of abnormalities in some sequences (FLAIR, DWI), as previously reported, has high diagnostic utility for sCJD diagnosis – especially in early stages – when other tests can still appear normal. Abnormalities on DWI sequencing may not correlate with neuropathological findings, suggesting a functional basis to explain the changes found.

PubMed ID: #21941496 (see pubmed.gov for this abstract only)

Features of Corticobasal Degeneration Presenting as Corticobasal Syndrome or PSP-Richardson’s Syndrome

Date
Posted by

Here’s another important paper on autopsy-confirmed cases of CBD (corticobasal degeneration) from Mayo researchers.

As we’ve learned many times of the last couple of years, those with autopsy-confirmed CBD can have many different clinical presentations including corticobasal syndrome (CBS), progressive non-fluent aphasia, behavioural variant frontotemporal dementia, and the Richardson syndrome form of PSP (progressive supranuclear palsy). “Due to the rarity of autopsy-confirmed corticobasal degeneration, the exact proportion of the various clinical presentations is unknown.”

In this study, 26 cases of autopsy-confirmed CBD are analyzed. Eleven presented with corticobasal syndrome (CBD-CBS), “characterized by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus.” Fifteen presented by the Richardson syndrome type of PSP (CBD-RS), “characterized by postural instability, early unexplained falls, vertical supranuclear gaze palsy, symmetric motor disability and dysphagia.”

(Note that the Williams and colleagues criteria for Richardson syndrome is: “gradual onset of postural instability and falls within the first 2 years of disease, along with vertical supranuclear gaze palsy, a frontal dysexecutive syndrome, and rigidity and bradykinesia that is unresponsive to levodopa.”)

“The aim of this study was to identify differences in corticobasal degeneration presenting with corticobasal syndrome or Richardson syndrome with respect to demographic, clinical and neuropathological features.” The researchers found differences in the distribution of tau in the brain.

These 26 autopsy-confirmed CBD cases were compared with 15 autopsy-confirmed PSP/Richardson syndrome cases (PSP-RS). Clinically, the CBD cases “had more cognitive impairment and frontal behavioural dysfunction.” The behavioral problems seen “included disinhibition, social withdrawal, apathy, agitation and impatience.”

How do you tell while someone is alive that they have CBD or PSP, when they present with Richardson syndrome? The researchers say: “Atrophy of anterior corpus callosum may be a potential neuroimaging marker to differentiate corticobasal degeneration from progressive supranuclear palsy in patients with Richardson syndrome.” Cases with CBD-CBS and CBD-RS “had marked atrophy of the anterior corpus callosum compared with patients with PSP-RS.”

In their conclusion, the authors address future treatments: “If future disease-modifying therapies are discovered that target specifically 4R tau dysfunction, there would be little significance in differentiating CBD-RS from PSP-RS. On the other hand, if the factors that drive cellular and anatomical specificity in corticobasal degeneration differ meaningfully from progressive supranuclear palsy, making this distinction may eventually be of more than academic interest. To that end, additional research is needed to determine if corticobasal degeneration and progressive supranuclear palsy have the same pathogenesis.”

From the article, we learned that between 1999 and 2009, 108 autopsy-confirmed CBD cases had been donated to the Mayo Jax brain bank. Of those 108, 41 had features consistent with Richardson syndrome (CBD-RS), 21 had features consistent with corticobasal degeneration (CBD-CBS), and the others had different presentations. Perhaps some of you donated family members’ brains in that time frame! If there were complete medical records at Mayo Jax, those family members potentially are included in this study.

We also learned that the Mayo Jax brain bank had over 600 autopsy-confirmed PSP cases had been donated to the Mayo Jax brain bank. We aren’t told how many have the Richardson syndrome form of PSP but previous studies show that 60% of PSP cases are Richardson syndrome. At present, neuropathology reports from Mayo Jax do not indicate if the case had a Richardson syndrome clinical presentation. Fifteen of these PSP cases were included in the study. (My father’s brain could be part of the study!)

A total of 41 brains were included in this study. “34% were followed at the Mayo Clinic, while 66% were referral cases to the CurePSP/Society for PSP Brain Bank.”

Thanks to all those who have donated brain tissue to the Mayo Clinic or elsewhere for making research like this possible!

I’ve copied the abstract below.

Robin

Here’s the abstract:

Brain. 2011 Sep 20. [Epub ahead of print]

Neuropathological features of corticobasal degeneration presenting as corticobasal syndrome or Richardson syndrome.

Kouri N, Murray ME, Hassan A, Rademakers R, Uitti RJ, Boeve BF, Graff-Radford NR, Wszolek ZK, Litvan I, Josephs KA, Dickson DW.
Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.

Abstract
Patients with corticobasal degeneration can present with several different clinical syndromes, making ante-mortem diagnosis a challenge.

Corticobasal syndrome is the clinical phenotype originally described for corticobasal degeneration, characterized by asymmetric rigidity and apraxia, cortical sensory deficits, dystonia and myoclonus.

Some patients do not develop these features, but instead have clinical features consistent with the Richardson syndrome presentation of progressive supranuclear palsy, characterized by postural instability, early unexplained falls, vertical supranuclear gaze palsy, symmetric motor disability and dysphagia.

The aim of this study was to identify differences in corticobasal degeneration presenting with corticobasal syndrome (n = 11) or Richardson syndrome (n = 15) with respect to demographic, clinical and neuropathological features. Corticobasal degeneration cases were also compared with patients with pathologically proven progressive supranuclear palsy with Richardson syndrome (n = 15).

Cases with corticobasal degeneration, regardless of presentation, shared histopathological and tau biochemical characteristics, but they had differing densities of tau pathology in neuroanatomical regions that correlated with their clinical presentation. In particular, those with corticobasal syndrome had greater tau pathology in the primary motor and somatosensory cortices and putamen, while those with Richardson syndrome had greater tau pathology in limbic and hindbrain structures.

Compared with progressive supranuclear palsy, patients with corticobasal degeneration and Richardson syndrome had less neuronal loss in the subthalamic nucleus, but more severe neuronal loss in the medial substantia nigra and greater atrophy of the anterior corpus callosum. Clinically, they had more cognitive impairment and frontal behavioural dysfunction.

The results suggest that Richardson syndrome can be a clinicopathological presentation of corticobasal degeneration.

Atrophy of anterior corpus callosum may be a potential neuroimaging marker to differentiate corticobasal degeneration from progressive supranuclear palsy in patients with Richardson syndrome.

PubMed ID#: 21933807 (see pubmed.gov for this abstract only)