FTD Disorders Registry is now live

The FTD Disorders Registry is an online database to collect information from those affected by all types of Frontotemporal Degeneration: behavioral variant FTD (bvFTD), any one of the primary progressive aphasias (PPA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or FTD with motor neuron disease (also called FTD-ALS). Persons diagnosed, caregivers (current/former), family, and friends can join and tell your story.

Brain Support Network encourages all those affected by PSP and CBD to join the registry.

www.ftdregistry.org

Even if your family member has passed away, you can still join the registry and tell your story.

Robin

Excerpts on PSP and CBD in “The Dementias” (NIH online-only booklet)

This email may be of interest to those dealing with the dementia forms of PSP and CBD.  (Not everyone with these diseases has dementia.  To read about the types of PSP and CBD, look under the “PSP Education” and “CBD Education” pages of the Brain Support Network website.)

The National Institutes of Health (nih.gov) has several publications on neurological diseases.  I recently came across their booklet on “The Dementias,” which includes a section on tauopathies as types of dementia.  Tauopathies are caused by the abnormal accumulation of the protein tau.  Both corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are covered.  Other tauopathies addressed include frontotemporal disorders (such as Pick’s) and argyrophilic grain disease (AGD).  (In brain donations we’ve helped with, AGD co-occurs in about 20% of all PSP cases.)

“The Dementias” booklet links to the NIH pages on CBD and PSP.  I think the PSP detail page is quite good (and it’s listed as one of our “Top Resources for PSP”).  I don’t think the CBD detail page is nearly as good.

Here are excerpts from the tauopathies section.  Look at the booklet online for other chapters — risk factors, diagnosis and treatment, etc. — and other types of dementia.

Robin

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nia.nih.gov/alzheimers/publication/dementias/types-dementia

Excerpts from

The Dementias
NIH Online Booklet
Published September 2013 (Last Updated July 2016)

Types of Dementia

Various disorders and factors contribute to the development of dementia. Neurodegenerative disorders such as AD, frontotemporal disorders, and Lewy body dementia result in a progressive and irreversible loss of neurons and brain functions. Currently, there are no cures for these progressive neurodegenerative disorders.

Some types of dementia disorders are described below.

Tauopathies

In some dementias, a protein called tau clumps together inside nerve cells in the brain, causing the cells to stop functioning properly and die. Disorders that are associated with an accumulation of tau are called tauopathies.

In AD, the tau protein becomes twisted and aggregates to form bundles, called neurofibrillary tangles, inside the neurons. Abnormal clumps (plaques) of another protein, called amyloid, are prominent in spaces between brain cells and are a hallmark of the disease. Both plaques and tangles are thought to contribute to reduced function and nerve-cell death in AD, but scientists do not fully understand this relationship. It is not clear, for example, if the plaques and tangles cause the disorder, or if their presence flags some other process that leads to neuronal death in AD.

Other types of tauopathies include the following disorders:

Corticobasal degeneration (CBD) is a progressive neurological disorder characterized by nerve-cell loss and atrophy (shrinkage) of specific areas of the brain, including the cerebral cortex and the basal ganglia. The disorder tends to progress gradually, with the onset of early symptoms around age 60. At first, one side of the body is affected more than the other side, but as the disease progresses both sides become impaired. An individual may have difficulty using one hand, or one’s hand may develop an abnormal position.

Other signs and symptoms may include memory loss; trouble making familiar, focused movements (apraxia) such as brushing one’s teeth; involuntary muscular jerks (myoclonus) and involuntary muscle contractions (dystonia); alien limb, in which the person feels as though a limb is being controlled by a force other than oneself; muscle rigidity (resistance to imposed movement); postural instability; and difficulty swallowing (dysphagia). People with CBD also may have visual-spatial problems that make it difficult to interpret visual information, such as the distance between objects.

There is no cure for CBD. Supportive therapies are available to reduce the burden of certain symptoms. For example, botulinum toxin can help control muscle contractions. Speech therapy and physical therapy may help one learn how to cope with daily activities.

Frontotemporal disorders (FTD) are caused by a family of brain diseases that primarily affect the frontal and temporal lobes of the brain; they account for up to 10 percent of all dementia cases. Some, but not all, forms of FTD are considered tauopathies. In some cases, FTD is associated with mutations in the gene for tau (MAPT), and tau aggregates are present. However, other forms of FTD are associated with aggregates of the protein TDP-43, a mutated protein found among people with a type of ALS that is inherited. Mutations in a protein called progranulin may also play a role in some TDP43-opathies.

In FTD, changes to nerve cells in the brain’s frontal lobes affect the ability to reason and make decisions, prioritize and multitask, act appropriately, and control movement. Some people decline rapidly over 2 to 3 years, while others show only minimal changes for many years. People can live with frontotemporal disorders for 2 to 10 years, sometimes longer, but it is difficult to predict the time course for an affected individual. In some cases, FTD is associated with progressive neuromuscular weakness otherwise known as amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease). The signs and symptoms may vary greatly among individuals as different parts of the brain are affected. No treatment that can cure or reverse FTD is currently available.

Clinically, FTD is classified into two main types of syndromes:

* Behavioral variant frontotemporal dementia causes a person to undergo behavior and personality changes. People with this disorder may do impulsive things that are out of character, such as steal or be rude to others. They may engage in repetitive behavior (such as singing, clapping, or echoing another person’s speech). They may overeat compulsively; lose inhibitions, causing them to say or do inappropriate things (sometimes sexual in nature); or become apathetic and experience excessive sleepiness. While they may be cognitively impaired, their memory may stay relatively intact.

* Primary progressive aphasia (PPA) causes a person to have trouble with expressive and receptive speaking—finding and/or expressing thoughts and/or words. Sometimes a person with PPA cannot name common objects. Problems with memory, reasoning, and judgment are not apparent at first but can develop and progress over time. PPA is a language disorder not to be confused with the aphasia that can result from a stroke. Many people with PPA, though not all, develop symptoms of dementia. In one form of PPA, called semantic PPA or semantic dementia, a person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

Other types of FTDs include:

* Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), a rare form of dementia that is believed to be inherited from one parent and is linked to a defect in the gene that makes the tau protein. The three core features are behavioral and personality changes, cognitive impairment, and motor symptoms. People with this type of FTD often have delusions, hallucinations, and slowness of movement and tremor as seen in Parkinson’s disease. Typical behavioral/personality characteristics include apathy, defective judgment, and compulsive and abusive behavior. Diagnosis of the disorder requires the confirmed presence of clinical features and genetic analysis. Palliative and symptomatic treatments such as physical therapy are the mainstays of management.

* Pick’s disease, a tauopathy subtype of FTD characterized by hallmark Pick bodies—masses comprised of tau protein that accumulate inside nerve cells, causing them to appear enlarged or balloon-like. Some of the symptoms of this rare neurodegenerative disorder are similar to those of AD, including loss of speech, inappropriate behavior, and trouble with thinking. However, while inappropriate behavior characterizes the early stages of Pick’s disease, memory loss is often the first symptom of AD. Antidepressants and antipsychotics can control some of the behavioral symptoms of Pick’s disease, but no treatment is available to stop the disease from progressing.

Progressive supranuclear palsy (PSP) is a rare brain disorder that damages the upper brain stem, including the substantia nigra (a movement control center in the midbrain). This region also is affected in Parkinson’s disease, which may explain an overlap in motor symptoms shared by these disorders. Eye movements are especially affected, causing slow and then limited mobility of the eye. The most common early signs and symptoms include loss of balance, unexplained falls, general body stiffness, apathy, and depression. A person with this type of dementia may suddenly laugh or cry very easily (known as pseudobulbar affect). As the disorder progresses, people develop blurred vision and a characteristic vacant stare that involves loss of facial expression. Speech usually becomes slurred, and swallowing solid foods or liquids becomes difficult. PSP gets progressively worse, but people can live a decade or more after the onset of symptoms. Dextromethorphan, a common ingredient in cough medicine, has been approved for the treatment of pseudobulbar affect.

Argyrophilic grain disease is a common, late-onset degenerative disease characterized by tau deposits called argyrophilic grains in brain regions involved in memory and emotion. The disease’s signs and symptoms are indistinguishable from late-onset AD. Confirmation of the diagnosis can be made only at autopsy.

Webinar for healthcare professionals on 3 dementias, including DLB – April 6th

This 90-minute webinar on April 6th is designed for healthcare professionals.  (Geriatricians and social workers can receive education credits.)  Titled “Beyond Alzheimer’s Disease,” the webinar will address the three most common dementias after Alzheimer’s — vascular dementia, dementia with Lewy bodies (DLB), and frontotemporal dementia.

Here’s information from the webinar’s host, Resources for Integrated Care, about the event.

Robin



resourcesforintegratedcare.com/GeriatricCompetentCare/2017_GCC_Webinar_Series/Beyond_Alzheimers

Beyond Alzheimer’s Disease – Other Causes Of Progressive Dementia In The Older Adult
Date/Time:  Thursday, April 6, 201712:00-1:30 pm ET

Registration Link:
secure.confertel.net/tsRegisterD.asp?course=7408411

Description:
Several atypical dementia syndromes may be confused with the more common diagnosis of Alzheimer’s disease (AD). It is important for providers to distinguish among these diagnoses because the management strategies that are effective in the care of adults with AD are often not effective with individuals with these atypical dementias. Three of the most common of these syndromes are:

* Vascular dementia – cognitive deficits most often associated with vascular damage in the brain, either micro or macro in nature.

* Dementia with Lewy Bodies – a dementia that also includes one or more of these core findings: recurrent and detailed visual hallucinations, parkinsonian signs, and fluctuating changes in alertness or attention.

* Frontotemporal dementia – a disease often seen in individuals with onset of cognitive symptoms at a younger age; these individuals present most often with executive and language dysfunction and significant behavioral changes.

This webinar is intended for a wide range of stakeholders – physicians, nurses, social workers, care managers, family caregivers, staff at social service agencies, managed long-term services and supports (MLTSS) and other health plans, consumer organizations, and those who care for people with dementia (MLTSS).

This webinar will:

1) Identify key distinguishing diagnostic features of the more common atypical dementias

2) Demonstrate basic knowledge of key strategies for preventing or reducing difficult behaviors associated with Frontotemporal dementia or Lewy Body Dementia

3) Discuss the impact of these atypical dementias on adults and their families and how to address the resultant care challenges

Webinar Presenters:
* Melinda S. Lantz, MD, Chief of Geriatric Psychiatry, Mount Sinai Beth Israel Medical Center, New York, NY
* Geri Hall, PhD, ARNP, CNS, FAAN, Banner Health, Phoenix, AZ
* Rebekkah Wilson, MSW, Dementia Care Consultant and Trainer
* Sharon Hall, Family Caregiver

Instructions:
After registering, you will receive an email from [email protected] containing event log on information. The email also contains an attachment that, when opened, will save the event log on information to an Outlook calendar.

The log on information is unique to you. Each registration allows for only one connection, so do not share this information with anyone else.

5 common dementias, including Lewy Body Dementia

A post today on a Canadian newspaper website, Castanet (castanet.net), is about five common dementias.  The five include:  Alzheimer’s, vascular dementia, Lewy body dementia, frontotemporal dementia, and Wernicke-Korsakoff’s syndrome (caused by prolonged alcohol consumption).  Here’s a link to the post:

www.castanet.net/news/Dementia-Aware/191198/5-common-dementias

Here’s how Lewy body dementia (LBD) is described:

Lewy body dementia:
Often mistaken for other dementias, e.g. Parkinson’s dementia
* Presence of Lewy bodies: tiny spherical protein deposits that develop inside nerve cells in the areas of thinking, memory and movement
* Fluctuating cognitive impairment: periods of increased confusion & windows of lucidity
* Hallucinations or delusions occur frequently and can be quite detailed
* Spatial disorientation e.g. falls, fainting
* Tremor, rigidity and slowness of movement
* Highly sensitive to neuroleptic drugs: Risperidone

This is OK except for two problems.  First, the author says that LBD is mistaken for other dementias such as Parkinson’s dementia.  Well, by definition Parkinson’s (Disease) Dementia is ONE of the disorders on the Lewy Body Dementia spectrum.

Second, I’m not sure how “fainting” is an example of “spatial disorientation.”  Fainting is an example of autonomic dysfunction.

Definitely not by favorite short LBD overview….

Robin

Multimodal Imaging Ties Tau to Neurodegeneration, and Symptoms

This is an Alzforum (alzforum.org) article about important researcher into tauopathies by researchers at Mass General.  The article was posted last week to Alzforum; the research study was published online in JAMA Neurology a couple of weeks ago.

What the researchers confirmed is that there is a “tight correlation between tau neurofibrillary tangles and neurodegeneration in individual patients in early clinical stages of various forms of Alzheimer’s disease.”  Three patients with typical Alzheimer’s Disease (AD) were studies, and three patients with atypical AD were studied.  One of the “atypical AD” cases was a person with corticobasal syndrome (CBS)

In this study, all patients were given a tau PET scan, an amyloid PET scan, and an MRI.  Researchers found that “tau predicts atrophy [which] predicts symptoms.”  It is not the protein amyloid in the brain that predicts atrophy or predicts symptoms.

In fact, we have known this from brain donation for a long time but now researchers have confirmed this in living patients.

Perhaps one reason that a CBS patient was studied rather than a PSP (progressive supranuclear palsy) patient is that the tau load in CBD is greater than in PSP.

Here’s a link to the article:

www.alzforum.org/news/research-news/multimodal-imaging-ties-tau-neurodegeneration-and-symptoms

Multimodal Imaging Ties Tau to Neurodegeneration, and Symptoms
Alzforum
07 Mar 2017

It is challenging reading.  Check it out online for cool images of the patient with corticobasal syndrome.

Robin