Our Mission

* Educate healthcare professionals

* Establish a clinic for FTD sufferers

* Support research

Wednesday, December 31, 2008

Call Anytime

I know sometimes it all gets overwhelming and especially this time of year with all the people and festivities. If I can help in anyway, please let me know...just leave a comment and I will get my number to you.

Sometimes it helps to talk to someone familiar with Frontotemporal Dementia.

I will be meeting with the doctors in Houston in January and look forward to learning what new findings there have been since we last spoke....I hope there is some good news.

Will keep you posted.

Monday, December 29, 2008

Getting Organized for 2009

Spent part of the day gathering up old notes about my brother and sister....notes of their behaviors before we knew what was going on with them. Mother was very good about journaling and I kept all the online emails and research I was doing early on. I had forgotten how much information I had accumulated.....boxes of internet research on the brain.

I just had to have some time away from it all .... seems selfish I know. I packed all this away during our move back to the East Coast. It is definately time to get it out and use it to help other people.

I know it would have made a world of difference if I had someone to talk with about Frontotemporal Dementia and what my sister was going through.

Sunday, December 28, 2008

Changes in Speech and Handwriting

Found 2 birthday cards Michael sent mom one month before his death. One was signed "Have a Nice Day" and the other signed "Merry Birthday". Now I know, both were real clues as to what was going on with him.

He was saying very little at this time and mostly would repeat "Have a Nice Day".

Then for him to say Merry Birthday instead of Happy Birthday.....was out of context for him.

His handwriting was changing as well. Patsy's handwriting changed drastically as well. I noticed her handwriting changed about 7 years before her death. Michael's didn't change until about a year before his death.

Friday, December 26, 2008

Research Hospital for FTD in Houston

Paul E. Schulz, M.D.
Associate Professor of Neurology and NeuroscienceDirector, Neurology Residency ProgramDirector, Cognitive and Behavioral Neurology FellowshipDirector, Neurology FellowshipsDirector, Neurology Grand Rounds CMEDirector, Cognitive Research Group

Clinical Service Area
Neurology - Behavioral Neurology

Behavioral and Cognitive Disorders Neurodegenerative Disorders

Board Certification
American Board of Psychiatry and Neurology, Neurology

Clinic Appointments

Medical School
Boston University School of Medicine, Boston, MA

Neurology, Baylor College of Medicine, Houston, TX

Neurology, Baylor College of Medicine, Houston, TX

Clinical Interests
Dementias, including Alzheimer's disease, vascular dementia, fronto-temporal dementias (FTD), corticobasal ganglionic degeneration, parkinsonism, and manganism. Disorders of thinking, memory, and language. Cognitive impairment in other neurologic disorders such as ALS, Myotonic Dystrophy, and West Nile Virus. Behavioral changes associated with cognitive dysfunction, including anxiety, depressions, mood lability, mania, and apathy. Thought disorders (psychosis) associated with cognitive impairment such as confusion, delusions, hallucinations, misidentifications, misperceptions, and suspiciousness.

Clinic Location
Baylor NeurologySmith Tower6550 Fannin St., Suite 1801Houston, TX 77030

Research Interests
Clinical Research: Frontotemporal dementia (FTD) and FTD associated with amyotrophic lateral sclerosis (ALS), including functional imaging (fMRI) to study social interaction, genetic studies for mutations underlying FTD and ALS, epidemiologic studies of FTD and ALS to ascertain factors that increase or decrease the probability or rate of progression of FTD and ALS, and pathologic approaches of FTD and ALS to ascertain diagnostic accuracy and processes at the cellular level that underlie the disorders; semantic memory, including changes in FTD-ALS, and epilepsy or epilepsy surgery; epidemiologic investigations of dementia to study the frequency, recognition, and treatment of dementia and aggression and anxiety in dementia, and to ascertain risk factors for dementia, which would give clues as to underlying pathophysiology.
Laboratory Research: The normal cellular mechanisms underlying synaptic plasticity, which are changes in the connections between neurons (nerve cells) that contribute to the cellular basis of learning and memory, language development, visuospatial function, emotional changes, etc.- the forms of synaptic plasticity under study include LTP, LTD, and decremental potentiation; using electrophysiologic (extracellular and patch-clamp recording techniques) and genetic approaches to study the induction and expression of these forms of synaptic plasticity, which includes investigating intracellular molecules, transcriptional regulation, and mechanisms of expression; investigating how normal synaptic plasticity mechanisms are disrupted in mouse models of dementia to gain clues as to the mechanisms underlying these disorders; the cellular mechanisms underlying a more rapid form of neuronal loss, i.e. that involved in global cerebral (brain) ischemia, and neuroprotection mechanisms to reduce neuronal loss—the hypothesis being that similar mechanisms may be involved in acute (stroke) and more chronic (neurodegeneration) cell loss.

Saturday, December 20, 2008

Artistic and Musical Abilities

Howard S Kirshner, MD, Professor of Neurology, the doctor that helped me find the wonderful doctors in Houston, wrote Nov 2, 2006 in emedicine, a great article - "Frontal and Temporal Lobe Dementia"

This was one aspect I find very interesting, since both my brother and sister had artistic abilities surface with this disease. Another clue for families. Dr. Kirshner states:

"Some North American authors have included under the FTD category cases in which artistic and musical abilities have actually emerged after the onset of the illness, usually in association with progressive language impairment. "

Friday, December 19, 2008

Thankful for Friends, Family, & Great Doctor's

I heard from a wonderful doctor today, Dr. Paul Schulz, Dept. of Neurology, Baylor College of Medicine, Houston Texas. He was my sister's last doctor there. He was so kind and caring, while helping us understand what was going on with her. He had a challenge as we were over 400 miles away. He and his staff helped this family put a face on the disease of Frontotemporal Dementia.

Since hearing from him today, I can't stop crying....he has meant so much to this family. As much as I have told him all this, he probably doesn't realize it. I am sure he is an angel for many many families.

I am also thankful for friends and family, that have helped me work on getting this information out there and help me plan out 2009.

I am a very blessed individual.

Short List Clues to Identifying FTD or Pick’s Disease

If you wonder things like “Did they have a stroke?” “Are they depressed?” “Are they having problems due to alcohol consumption?” “Why are they acting so strange?” “Are they Schizophrenic?” “Have they lost their mind?” “They would never act like this in their right mind”

If we had seen a published list of behavior changes related to Pick’s or FTD on Good Morning America or Oprah, we might have realized sooner what was going on with them. When I look at the list, I say to myself, why didn‘t I see it, why didn‘t I recognize it sooner? It is like those mind bender puzzles, once you hear the answer, you go OH of course that is the answer.

Here is my list, but my brother and sister were poster children for FTD, so you will see these behaviors on many lists:

Early Stage

More flirtatious with opposite sex
Depressed; felt the world was crashing in on occasion
Compulsive behaviors; my brother used a women’s bathroom in a fine dining restaurant (he would never do that normally). Behaviors out of the norm for them
Would drink alcohol in group situations, makes them feel more confident
Mood changes are slighter in this stage; can get angry, but you generally can reason with them
Start drawing or showing artistic (could be music) abilities

Middle Stage

More reclusive, paranoid behaviors
Talk less
Smiled less
More conscious about spending money
Read less and watch more TV
Stop favorite activities; golf, because not able to make a put anymore
Speech is harder to understand; suspect they may have had a minor stroke
More aggressive behaviors; loose temper easily and can be physically violent
Problems keeping a job
You notice fewer friends around
Inappropriate behaviors; walk in on you in the bathroom/bedroom, change the TV station while you are watching it, pass gas in a restaurant, eat with mouth open, laugh at sad things, just RUDE
Rigid in behaviors. Taking a vacation, you get to airport and they say they have to go home to do their nails.

Later Stage

Repeat familiar phrases; i.e. Have a Nice Day, It’s a Great Day, eventually mute
Flip words around; Merry Birthday
Rarely smile
Crave sweets
Stop cooking, become afraid of using appliances or electronics
Paranoid behavior; shutting the blinds, locking the doors
Inability to function or interact in social or personal situations
Problems with personal hygiene
Repetitive behavior; go to the same store, buy the same candy bar
Rapid mood change, violent, angry
Failure to show concern, empathy, sympathy, compassion
Gain weight, physical changes, they walk differently or slump shoulders
Stop bathing, cleaning house, or doing laundry
Reckless driving
Less writing and signature may change drastically
Urinary incontinence

Wednesday, December 17, 2008

Clinical Trial for FTD

Clinical Trial of Memantine for Frontotemporal Dementia and Semantic Dementia


Pick's not generally passed down in familys!!!

This was interesting...found on National Library of Medicine/National Institutes of Health

Causes od Pick's Disease
People with Pick's disease have abnormal substances (called Pick bodies and Pick cells) inside nerve cells in the damaged areas of the brain. Pick bodies and Pick cells contain an abnormal form of a protein called tau. This protein is found in all nerve cells. But some people with Pick's disease have an abnormal amount or type of this protein. The exact cause of the abnormal form of the protein is unknown. A gene for the disease has not yet been found. Most cases of Pick's disease are not passed down through families.

Pick's disease is rare. It is more common in women than men. It can occur in people as young as 20, but usually begins between ages 40 and 60. The average age at which it begins is 54.

Great website UCSF

I think University of California San Francisco does an excellent job of describing FTD. Twice we sent of Patsy's blood work for confirmation of one of the strains of PICKS. She tested negative. It is the only place in the US that tests for this.

Disease Progression
FTD usually first appears when someone is in their mid-40s to early-60s and causes a steady, gradual decline in the ability to complete the daily activities of life. The disease can last anywhere from three to 17 years from the first symptom until death, with an average duration of eight years after diagnosis.

Behavioral variant FTD

Mild bvFTD

In the first several years, a person with bvFTD (often called Pick's disease or just FTD) tends to exhibit marked behavioral changes such as disinhibition, apathy, loss of sympathy or empathy for others, or overeating. Problems with planning organization and sometimes memory are evident, but the individual is still capable of managing household tasks and self-care with minimal help. However, impairment in judgment can lead to financial indiscretions with potentially catastrophic consequences. Social withdrawal, apathy and less interest in family, friends and hobbies may be evident. At times, they may behave inappropriately with strangers, lose their social manners, act impulsively and even break laws. But at this stage, the behaviors can often be managed with lifestyle and environmental changes (read our practical tips for ideas). A MRI image at this point will show mild atrophy in particular areas of the frontal lobes. [Michael got his 1 ticket for speeding and my sister had an accident; both had previously been very careful drivers. Both slowly withdrew from social situations, friends and family. Both gave up hobbies, sports, and cooking...gradual at first.}

Moderate bvFTD
Over the course of a few years, the symptoms seen in the mild stage will become more pronounced and disabling. You might also notice compulsive behaviors like repetitive urination, hoarding or collecting objects, compulsive cleaning or silly repetitive movements (like stomping on ants). Binge eating may create weight problems and other health issues. The cognitive problems associated with dementia become more pronounced, with mental rigidity, forgetfulness and severe deficits in planning and attention. The MRI image at this point will show that the shrinking of the brain tissue has expanded to larger areas of the frontal lobes, as well as the tips of the temporal lobes and basal ganglia, deeper brain structures involved in motor coordination, cognition, emotions and learning. [My sister would go to the bathroom every 5 minutes (generally not doing anything, just going through the motions), Michael would suck on his teeth, both would repeat short phrases ie "Have a nice day" "Life is good", both craved sweets and would eat as much as you would put in front of them - neither had a weight problem before the disease, but gained weight during this stage. There was no reasoning with them if they wanted to stay or go, would be very rigid about certain things...this varied. Didn't seem to care if they saw their children or grandchildren, didn't want to hold the babies. Patsy would collect straws, sugar packets, plastic cups and hide them in her closet.]

Severe bvFTD
By this point the patient is experiencing profound behavioral symptoms (apathy, loss of empathy, disinhibition) in association with language difficulty and memory loss. They may have trouble coordinating their muscles at this point and may require a wheelchair. Usually 24-hour care is required, whether at home or in an institution. The physical decline and changes that occur throughout the disease course become more and more obvious at this stage. Eventually, the person with FTD may have great difficulty swallowing and moving and they may have urine and/or bowel incontinence. Death from bvFTD is usually caused by the consequences of these physical changes, most commonly infections in the lungs, skin or urinary tract. Although it can vary widely, the time from the first symptom to the end is typically about eight years, whereas the time from diagnosis is, on average, about five years. [Michael died as he was approaching this stage. Patsy progress just as outlined here. The last few months before she passed away, her muscles seemed to not respond at all. She couldn't use her hands and they were swollen.]

RGH E-Bulletin Dementia Therapies

Interesting article about drug therapies for Dementia. I was not familiar with it.

FTD affects the frontal and temporal lobes of the brain

Frontotemporal dementia affects the frontal and temporal lobes of the brain.
Credit: Mayo Foundation for Medical Education and Research

FTD Patients Cannot Detect Sarcasm

Researchers at the University of New South Wales are using sarcasm to determine whether patients have frontotemporal dementia (FTD), otherwise known as Pick's disease:

Researchers at the University of New South Wales found that patients under the age of 65 suffering from frontotemporal dementia (FTD), the second most common form of dementia, cannot detect when someone is being sarcastic.

The study, described by its authors as groundbreaking, helps explain why patients with the condition behave the way they do and why, for example, they are unable to pick up their caregivers' moods, the research showed.

"This is significant because if care-givers are angry, sad or depressed, the patient won't pick this up. It is often very upsetting for family members," said John Hodges, the senior author of the paper published in "Brain".

"(FTD) patients present changes in personality and behaviour. They find it difficult to interact with people, they don't pick up on social cues, they lack empathy, they make bad judgements," he told AFP.
The research, conducted in 2006-07, put 26 sufferers of FTD and 19 Alzheimer's patients through a test in which actors acted out different scenarios using exactly the same words.
While in one scenario, the actors would deliver the lines sincerely, in others they would introduce a thick layer of sarcasm. Patients were then asked if they got the joke, Hodges said.
For example, said Hodges, if a couple were discussing a weekend away and the wife suggested bringing her mother, the husband might say: "Well, that's great, you know how much I like your mother, that will really make it a great weekend."

When the same words were delivered sarcastically and then in a neutral tone, the joke was lost on FTD patients, while the Alzheimer's patients got it.

I wasn't able to find the article in Brain yet because I don't think it is out yet, but here is a similar article on the subject.

A couple comments: Pick's disease is really rare. If you have a relative with dementia odds of overwhelmingly that it is Alzheimer's and not FTD. But it is an interesting finding that patients with FTD fail to understand sarcastic statements while patients with Alzheimer's do.
I guess my question would be, what is the mechanism of perception of sarcasm? It must be the perception of some disconnect between the speaker's perceived intention and their actual statements. We know that mirror neurons -- neurons that are active when you perceive someone else performing a task, neurons that we think are involved in empathy -- are in the frontal lobes, so the loss of these neurons may explain the phenotype. However, at end stage Alzheimer's you also see frontal lobe issues. Maybe there is a stage in Alzheimer's where you would also see loss of sarcasm, although likely by that point they aren't doing much talking anyway.

The most bizarre part about all of this is the thought of putting into practice. Could you visualize going into a patient's bedroom and being sarcastic to see if they had FTD? What if they didn't? You would look like such a jackass.

Furthermore, I know a bunch of healthy people who don't understand sarcasm either. What do you make of them?

Tuesday, December 16, 2008

Crazy Behaviors

In 2001 when we brought my sister to South Texas so we (her son and his wife, mom, my husband, and me) could help care for her and try to figure out what was going on with her. Initially she was living with her son and his wife.

As the holidays approached, one doctor put Patsy on Aricept. Soon after, she took a bottle of pills one day when the kids were at work. She called me and told me what she had done. I asked her why she took the pills and she said she didn't know why. She was hysterical, screaming and crying.

The hospital transferred her to a private psychiatric facility for evaluation. This facility was a joke. They didn't want to deal with her because she had no money for treatment. At this stage, Patsy could still talk and was very good at covering many of the symptoms for short periods of time. However, if you spent any length of time with her, you would see the odd behaviors. They called us all in for a meeting, and after telling us all we were sorry examples for human beings because we couldn't care for her...they released her to a Salvation Army store. We of course went over to gather her up and bring her home.

The scary thing here is, if she had no family, she would have become a street person. Makes you wonder about those poor people out there on the streets that may be ill and their families have all been pushed away.

At this point, she would wander aimlessly and leave the house if you weren’t watching every move. She would walk in on us in our bedroom, walk out the front door, yell and throw a fit if we didn’t drive her to the right store or park in the right parking spot….you get the idea.

We actually were a bit afraid. I remember having thoughts that she might grab a knife and come after us. Seems so odd now when I look back on it. She had turned into someone I didn’t know and the doctors were no help.

Friday, December 12, 2008

Elvis Presley and Christmas Music

This time of year I pull out the cassettes my brother made me. He loved Elvis Presley and for years told us to play Elvis songs at his funeral. We did as he requested, which makes it really hard to listen to those songs today without crying like a baby. Crying in the Chapel and Dixie were just a couple of his favorites.

Tuesday, December 9, 2008

New Drug for Alzheimer’s

Dimebon is the new drug available (research purposes) for Alzheimer's...would it be good for FTD patients? I will email them and ask the question. I guess for the memory problems associated with it, it could. My brother and sister really had good memories until the end.

Tuesday, December 2, 2008

What is Frontotemporal Dementia

Taken from NINDS Frontotemporal Dementia Information Page
(National Institute of Neurological Disorders and Stroke)

What is Frontotemporal Dementia ?
Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick’s disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. These designations will continue to be debated. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Spatial skills and memory remain intact. There is a strong genetic component to the disease; FTD often runs in families.

Michael and Patsy had symptoms from both, changes in behavior and problems with language. As the disease progressed, so did the symptoms.

Is there any treatment?
No treatment has been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors. Aggressive, agitated, or dangerous behaviors could require medication. Anti-depressants have been shown to improve some symptoms.

Anti-depressants did help Patsy with the aggressive and angry behaviors. Sleeping pills helped her to rest at night.

Since we didn’t have clue what was going on with Michael, no drugs were administered, so no benefit here. Two weeks before his death, a doctor prescribed Aricept. A doctor later told us, this was the wrong medication, it actually gave him the ability to plan his death. He said it made the disease speed up in his case, allowing him to think more clearing in short periods of time.

What is the prognosis?
The outcome for people with FTD is poor. The disease progresses steadily and often rapidly, ranging from less than 2 years in some individuals to more than 10 years in others. Eventually some individuals with FTD will need 24-hour care and monitoring at home or in an institutionalized care setting.

After putting together a graph of behaviors over the years, I was able to trace Patsy’s odd behaviors to her late 20’s and Michael’s to his 30’s. Both progressed slowly until they weren’t working on a regular basis and at that time, it seems the disease progressed more rapidly.

What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS), and other institutes of the National Institutes of Health (NIH), conduct research related to FTD in laboratories at the NIH, and also support additional research through grants to major medical institutions across the country.

I tried to get Patsy into a clinical trial in NIH, that was the only facility at the time with any trials. We couldn't work it out, since it was so far away and there were no government funds or assistance to help with living expenses/lost wages etc with a location so far away from home (DC Area only at that time). Dr. Paul Schulz, a Neurologist in Houston did want our family to participate in on going testing to keep an eye out. As of this date, we have not done that. There is still very little being done in research compared to other diseases. One doctor told me "Today is like the 1980's were to Alzheimer's, we are just starting to work on FTD Education and Treatment."