Category Archives: CBD

Billionaire Richard Rainwater Dies with PSP

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Local support group member Lynn forwarded me a link to this article about the death on Sunday of billionaire Richard Rainwater, who had PSP.  Several years ago Mr. Rainwater began the Tau Consortium, which funds PSP and tau research at UCSF among other places.  Tau is the protein involved in PSP and CBD.  It is also one of two proteins involved in Alzheimer’s Disease.

As part of the obituary in the Forth Worth Star-Telegram, there’s a 3-minute video from January 2015 featuring Mr. Rainwater’s son and Bruce Miller, MD, of UCSF’s Memory & Aging Center.  They note that the PSP research may also benefit Alzheimer’s Disease plus CTE, the disease that football players get.

Here’s a link to the obituary:

www.star-telegram.com/news/local/community/fort-worth/article36766056.html

Fort Worth billionaire Richard Rainwater dies
by Judy Wiley
September 27, 2015
Star-Telegram

Robin

Therapeutic interventions in CBD (Italian paper)

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There was an interesting article published online last week by Italian neurologists about corticobasal degeneration (CBd).  Here are the highlights:

* CBD has low diagnostic accuracy — 25–56% of cases.

* The only way to confirm a diagnosis is through brain donation.  If it’s not CBD, what is it?  Alzheimer’s disease (AD), progressive supranuclear palsy (PSP), Pick’s disease, dementia with Lewy bodies (DLB), Creutzfeldt-Jakob disease (CJD), etc.

Robin’s note:  Of the many supposed CBD brain donation cases I’ve been involved with, we’ve seen all of these — AD, PSP, Pick’s, DLB, and CJD.  Probably we’ve seen more AD than anything else.

* People with confirmed CBD can be grouped into four main “clinical types” —

  • CBS (corticobasal syndrome)
  • frontal behavioral-spatial syndrome
  • nonfluent/agrammatic variant of primary progressive aphasia
  • progressive supranuclear palsy syndrome

* There are few randomized clinical trials of pharmacological treatment of CBS symptoms.  Physicians rely on expert opinion or anecdotal descriptions in prescribing medications.

* “Levodopa is…not particularly helpful in CBS.”

*  “Amantadine has been occasionally reported to improve akinesia and rigidity in patients with AP, but evidence is anecdotal.”  (AP = atypical parkinsonism)

* Botox “may be helpful for CBS-associated limb dystonia and may be used to alleviate abnormal posture, pain and for maintaining hand hygiene.”

* “Deficits in single cognitive domains have been described at disease onset in CBS, but dementia is not a classical presenting feature of CBS, and will occur in only about 25% of cases with advanced disease. Dementia is more frequent in CBD patients who do not present with classical CBS, occurring in the FTLD-tau phenotype in which the central presenting features are cognitive/behavioral symptoms.”

* “It is also important to consider that CBS patients who partially respond to AChEIs/memantine treatment may have underlying AD as well.”

* “For psychosis, aggression and agitation, antipsychotics (preferably atypical agents) are currently employed despite the possible adverse events that include extrapyramidal symptoms, cognitive deficits and also death; particular caution is needed when using neuroleptics in patients with overt parkinsonism.”

* “CBS patients also frequently report depressive symptoms. Selective serotonin reuptake inhibitors (SSRIs) provide effective treatment in these subjects.”

* “[R]ehabilitation therapies are an essential component of the multidisciplinary approach to CBS patients. The rehabilitation team may address mobility, safety, assistive devices, activities of daily living and communication.”

* Transcranial direct current stimulation “may represent a promising tool for cognitive enhancement and neuro-rehabilitation but further studies in a larger cohort are necessary.”

* “[A]dvanced CBS patients ideally require assistance in specialized palliative care units, with the specific aim of alleviating the burden of symptoms, and of preserving patients’ autonomy and supporting their families.”

* “In particular, aspiration pneumonia and urinary infections are the two main causes of death in CBD patients. All these problems are common in advanced stages of neurodegenerative disorders and clinicians need to recognize and discuss their implications with the patient and the family. For example, patients with dysphagia should be immediately referred for swallowing evaluations with fibrolaryngoscopy. As the disease progresses, patients, relatives and caregivers have to be informed about the opportunity to place a percutaneous endoscopic gastrostomy (PEG) tube, especially if all other aspects of quality of life are preserved. Moreover education and support of caregivers and families by the multidisciplinary team members is recommended where these services exist.”

I think this is adequate for most of you (and maybe even too much already!).  For those who want to read more, I’ve copied the abstract below.  You can link from the abstract to the full article; there may be a fee to purchase the full article.

Robin

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Parkinsonism & Related Disorders. 2015 Sep 9.  [Epub ahead of print]

Therapeutic interventions in parkinsonism: Corticobasal degeneration.
Marsili L, Suppa A, Berardelli A, Colosimo C.

Abstract
Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder resulting from pathological accumulation of tau protein and is included in the spectrum of Atypical Parkinsonism. The typical clinical phenotype of CBD is characterized by the Corticobasal syndrome (CBS). In recent years it has become clear that the clinical picture of CBS may be caused by different pathological conditions, resulting in frequent misdiagnosis. CBD has high morbidity and poor prognosis with no effective therapies. In this review, we will discuss the symptomatic treatment, the palliative care and the disease modifying strategies currently in use. Symptomatic treatment in patients with CBD may sometimes be useful for improving motor (parkinsonism, dystonia and myoclonus) and non-motor (cognitive-behavioral) symptoms, but the effects are often unsatisfactory. In addition, non-pharmacological strategies and palliative care are useful integrating components of the multidisciplinary therapeutic approach for patients with CBD. Despite many efforts, a disease-modifying treatment is still unavailable for CBD.

Copyright © 2015 Elsevier Ltd.
PubMed ID#:  26382843

Exercise and dementia (research updates from Alzforum)

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This post may be of general interest since many of us are dealing with dementia or will be dealing with dementia at some point in our lives.

At the recent Alzheimer’s Association International Conference in Washington DC (mid-July), a lot of research was presented on exercise.  The Alzforum has two good summaries of the research.

The first summary is here:

alzforum.org/news/conference-coverage/exercise-boosts-cognition-symptomatic-disease

The first summary examines research into whether Alzheimer’s disease can be tempered by aerobic exercise and whether dementia can be avoided through exercise:

“Speakers…presented new evidence that regular aerobic exercise can help people in prodromal disease stages maintain their cognition, while for those with full-blown dementia it relieves neuropsychiatric symptoms. Some studies provided hints that exercise can also hone thinking at the dementia stage, but only if the participants reach moderate intensity heart rates during their workout. Exactly how exercise helps the brain is still not known, but several talks reported better cerebral blood flow and improved structural and functional connectivity in exercisers, and even some signs that six months or more of physical activity can slow pathology.  Researchers agreed that the duration and intensity of an exercise intervention are crucial to determining its effects. For aerobic exercise in particular, the field is standardizing methods and narrowing in on the appropriate dose to prescribe. Some believe supervised exercise classes could become part of the standard of care for people with cognitive problems. … Researchers have few doubts now that exercise protects normal older adults against brain decline.”

(prodromal = before symptoms appear)

The second summary is here:

alzforum.org/news/conference-coverage/can-exercise-slow-progression-alzheimers-pathology

The second summary explores research into whether exercise can slow the progression of a neurodegenerative process:

“Overall, the findings indicated that working out enhances vascular brain health and connectivity, implying a direct benefit to brain structure and function. Data were mixed on whether exercise slows the progression of underlying Alzheimer’s pathology, however. One six-month study of moderate aerobic exercise reported a drop in cerebrospinal fluid tau in cognitively impaired people, but a shorter intervention failed to budge brain amyloid in people with AD. In general, speakers agreed that the cognitive boost from exercise likely comes from diverse benefits on several different aspects of brain function, something that would be hard to match pharmacologically.”

Both summaries are worth reading if exercise research is of interest.

While this may be a good day to go to the gym, this is probably not a good day to exercise outdoors.

Stay cool,
Robin

Tau imaging research and how tau may spread (Alzforum update)

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This post may be of interest to those in the PSP and CBD community who are interested in what’s happening in research, particularly imaging of tau, as well as a hypothesis of how tau spreads in the spread.

Alzheimer’s is a disorder of two kinds of proteins — tau and amyloid (or beta amyloid, Aβ).  PSP and CBD are disorders of one kind of protein — tau.

At the recent Alzheimer’s Association International Conference in Washington DC (mid-July), scientists reported on data from tau PET imaging.  Three ligands or chemical tracers from La Roche are being studied, with one seeing superior results.  The data also provide clues as to how tau may spread in the brain.

The Alzforum has a good summary of the tau imaging research presented at the conference:

“…tau imaging data bolstered the idea that tau sits tight in the medial temporal lobe until Aβ builds up, then it spreads, wreaking havoc on cognition. New imaging data suggests that once it breaks loose, tau spreads through functional networks and impairs brain metabolism. What’s more, unlike Aβ, which appears diffusely throughout the brain, tau deposits seem to map closely onto regions where atrophy occurs and cognitive deficits originate. ‘Unlike amyloid imaging, tau PET seems to strongly correlate with cognition and clinical states,’ said Gil Rabinovici, University of California, San Francisco. Researchers were also excited by a new tau ligand that appears to have better specificity and kinetics than its predecessors.”

Here’s a bit more on tau building up in certain brain regions, as in corticobasal syndrome (CBS), and the relevance of tau as a target:

“Disaster Strikes Wherever Tau Lands
Once outside the medial temporal lobe, tau seems to cause trouble wherever it lands. At AAIC, Daniel Schonhaut, who works with Rabinovici, expanded on the notion that tau builds up in brain regions thought to underlie clinical symptoms in patients with atypical forms of AD. He compared performance on a variety of cognitive tests with T807/AV1451 scans from 19 patients with typical and atypical forms of AD, including posterior cortical atrophy, primary progressive aphasia, and corticobasal syndrome.  Schonhaut reported that people with typical AD who performed more poorly on memory tasks had more tau in the hippocampus and surrounding medial temporal areas, while patients with worse visual function indicative of posterior cortical atrophy had more tau in occipital regions. A third group — those with language difficulties typical of primary progressive aphasia — had more tau in the left temporoparietal region. ‘The distribution patterns of tau we’re seeing with this PET tracer seem to underlie the clinical phenotype of our patients,’ Schonhaut told Alzforum.  ‘These data show nicely that there’s a relationship between where the tau is clinical symptomatology,’ said Jagust. ‘We haven’t seen that with amyloid.’ Rabinovici noted that these results corroborate findings from classic postmortem studies demonstrating that cognitive state correlates much more strongly with tangle than amyloid pathology. They also support tau as a therapeutic target, particularly in the symptomatic phase of AD, he told Alzforum.”

The entire Alzforum research summary is worth reading, if research is of interest:

www.alzforum.org/news/conference-coverage/new-imaging-data-tells-story-travelling-tau

Stay cool,
Robin

Falls and Dysphagia (new publication co-authored by BSN)

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This post will be of interest to those dealing with falls and/or dysphagia (swallowing difficulty).

Local support group member Phil Myers and I co-authored a case study on falls and dysphagia in the latest issue of the Association of Frontotemporal Degeneration (AFTD) newsletter.  The case study is about “Jackie Riddle” — a composite of my father’s PSP symptoms (confirmed through brain donation) and Phil’s wife Jackie’s PSP symptoms (also autopsy-confirmed).  Though “Jackie Riddle” had PSP, since the focus of the case study is on falls and dysphagia, all of our BSN group members, regardless of diagnosis, will find something of value here.

The newsletter is written for healthcare professionals but I’m fairly certain the language is understandable.  We assumed that healthcare professionals are completely un-knowledgeable about PSP, fall prevention, and treatment of dysphagia.

There are also sections in the newsletter specifically for healthcare professionals working at care facilities.  So if any of you has a loved one in a care facility, those sections may be particularly helpful for staff.

The last two pages of the newsletter are my ideas for practical things that can be done about falls and dysphagia.  Again, though it’s titled “in PSP,” these ideas apply to all four disorders in our BSN group.

Check it out; it’s hot off the presses:

www.theaftd.org/wp-content/uploads/2015/07/PinFTDcare_Newsletter_summer_2015.pdf

Robin