An Update on Terry's Dx

[Guest guest]

Terry's new neurologist has provided additional insight into Terry's

diagnosis. My initial impressions are provided for those on the list with

an interest. There are two aspects that are different from what is

typically described on the list. Five reference links are provided at the

end. I welcome comments and questions.

BACKGROUND Terry's original Dx was PD in 1995. When we moved to

Charlottesville in 1998, we gained access to the medical resources at the

University of Virginia. Terry's first neurologist, N#1, was puzzled by her

case and referred her to N#2 in the Memory Disorders Clinic at UVa. N#2 and

a nuero-psychologist are Co Directors of this Clinic. Based on a series of

clinical observations, psychological testing, several MRIs going back to

1995, and the lack of response to medications for PD, N#2 diagnosed Terry

with MSA/SND.

N#2 left UVa late last year for private research and a search for his

replacement was started. N#2 continued to see patients part time during the

search. He ordered a SPECT scan and an exam by a nuero-ophthalmologist late

last year. N#3, N#2's replacement, has completed his review of Terry's case

and has given us his impressions in two recent visits.

CURRENT VIEW Based on her case history together with the recent test

results, the Dx of MSA/SND is still applicable. In addition, the SPECT scan

revealed " the basal ganglia is defective and several frontal segments are

hypo-metabolic. " Hypo-metabolic suggests the frontal segments are either

not getting a sufficient blood supply or not using the blood it does

receive, or both. As we know from recent posts, the SPECT scan shows which

parts of the brain are functioning and how well.

Many of Terry's symptoms are in two areas: mobility and apathy. The

mobility effects are prominent and the apathetic effects are pervasive. The

low metabolism in the frontal segments can cause dementia among other

things. As I understand it, dementia in this context results in the loss of

sufficient intellectual function and personality so as to interfere with

the person's past emotional characteristics, intellectual capabilities, and

personality. Apathy can result from dementia along with a loss of the

executive function. Many people associate dementia with Alzheimer's

Disease, and AD is, in fact, the major cause of dementia accounting for

over 50% of all dementia cases. However, there are many other causes and

shadings of dementia. [N#2 has assured us that Terry does not have AD and

he has not observed any signs of autonomic system failure.]

In my own research since we met with N#3, it seems to me we may be dealing

frontotemporal dementia, or FTD. Further, it is possible the problems in

the frontal segments are genetic and N#3 is making arrangements for genetic

testing. In this case, I believe it would be FTD with Parkinsonism-17, or

FDTP-17. The number 17 refers to a specific chromosome.

According to N#3, Terry is experiencing freezing not only in her mobility

processes, e.g. freezing of feet to the floor, but also in her mental and

emotional processes. She simply has difficulty " getting over the initial

hump " in the initiation of any of these processes; i.e. getting started, in

physical, mental, and emotional activities. He recommends providing as much

structure in her life as possible. For example, do not ask what she would

like to do, as the answer will be " nothing. " Experience has shown him to be

correct. Instead, provide limited choices on an assertive basis. For

example, ask does she want to do A or B, and expect a choice of A or B.

" Nothing " should not be an alternative. He strongly recommended getting her

involved in activities outside of the home; i.e. get her started in an

activity that she will be able to continue. Arrangements are being made for

her to become involved in volunteer work in the office of the church she

attends.

Terry's outlook on all of this is really quite stoic. She is occasionally

frustrated to tears by the freezing, but otherwise seems content and quite

passive about the whole situation. Apparently, this is an effect of the

apathy. She is not motivated to do much of anything including physical

therapy. She does administer close to ten meds in a fairly reliable way

with only an occasional slip up; she is a type 2 diabetic - treated with

oral meds - and has chronic bursitis, high blood pressure, and a hypoactive

thyroid.

These are my simplistic views of N3's update on Terry's Dx. However, his

explanations appear accurate based on my observations of Terry's behavior.

In hindsight, it is now clear that apathy has been present for 15 or more

years.

BOTTOM LINE: We have a refined and expanded Dx by Terry's new neurologist

who is knowledgeable in these kinds of problems. It is reassuring to me to

have both Co Directors of the Memory Disorders Clinic at UVa available to

help manage Terry's symptoms. As you can see I have a lot to digest. I am

already compiling a list of question for N3 as he supports email contacts

by care givers. As we all know, the trip down the road of neuro

degenerative diseases is loaded with surprises and new challenges that we

could just as well do with out. This update surely demonstrates this aspect

of things. I have already begun sharing verbally some of this information

with the family. However, I do not plan to go into the question of a

possible genetic basis until there are some definitive answers and I know a

great deal more than I do now.

I welcome any comments and questions that folks on the list might have as

you have been a major source for me of intellectual and emotional support.

You have my appreciation and thanks for this help. I hope our experience is

of some help to you.

These are the five links that have been helpful to me:

For basal ganglia, see http://www.nlm.nih.gov/medlineplus/ency/arne/001069.htm.

For dementia, see http://www.parkinson.org/dementiasame.htm.

For frontotemporal dementia, aka Picks Disease, see

http://www.nlm.nih.gov/medlineplus/ency/article/000744.htm#contentDescription,

and

http://www.caregiver.org, then select Fact Sheets under Clearinghouse, and

then select Frontotemporal Dementia.

For FTDP-17, see http://www.geneclinics.org/profiles/ftdp-17/details.html.

Sennewald Charlottesville, Virginia

[Guest guest]

Greetings ,

My sincere condonlences for your current state. It must be overwhelming for

both of you.

Only when I reached extreme fatigue and overload (from external stimuli),

did I encounter a small experience with the mental freezing. And it was

extremely frustrating an unpleasant. I knew I wanted to formulate a

thought, but was simply unable to pull together all the pieces. For me a

nap seems to help. That both Terry, and you, , face this together must

be somewhat overwhelming. As a result, I extend my prayers for Grace and

Peace to both of you.

Regards,

=jbf=

B. Fisher