Expert: Mary Gordon - 7/17/2010

Question
QUESTION: My mother just got her ct scan results back in which they were seeking to identify why she was having memory loss and seizures.  Unfortunately, the results came back showing rapid shrinkage of the frontal lobe and was therefore diagnosed with rapid-onset alzheimers disease.  She is only 49 years old.  I would love to hear any information about this type of alzheimers.  We were told this only occurs in about 3% of diagnosed patients and that her life expectancy is only about 5 to 6 years at best.  Please help any way you can.  Thank you.

ANSWER: Hi Sam,

Has your mom  been seen by a neurologist or psychiatrist with a specialty in early onset dementias? I ask only because shrinkage of the frontal lobe alone suggests a frontal lobe dementia rather than Alzheimer's. Usually what you see with AD is a general atrophy of the brain, widened indentations in the tissues, and enlargement of the fluid-filled chambers called cerebral ventricles. Generally CT scans are not used to diagnose Alzheimer’s disease. They are used to confirm or rule out other causes of dementia in people who show signs of memory loss.
At any rate, assuming she has been assessed by a specialist, her age and the seizures alone suggest early onset AD which often has a strong genetic component.

http://www.cchs.net/health/health-info/docs/2400/2498.asp?index=9592%20

Here is an article about the general issues associated with early onset.
http://www.alz.org/national/documents/report_earlyonset_summary.pdf

It will follow the path typical for all the progressive dementias. The most commonly used "staging" tool is the FAST scale created by Dr. Reisberg. It defines stages by functional abilities. Although different dementias do have some unique characteristics, almost all of the progressive dementias, including early onset AD, will erode abilities as brain damage progresses, and will lead the person through the stages in a relatively predictable fashion - abilities tend to be lost in specific order. Not everyone will fit neatly into a specific stage, but they are a useful tool since they give us common language to describe where a person is in their illness, and a way of anticipating what is likely to happen next as their cognitive and neurological status deteriorates.

I've pasted a description of these stages below my signature, so you can read them and get an idea where your mom is.

I know this is an absolutely devastating diagnosis. My best advice is to see a lawyer immediatly and get her affairs in order. This includes wills and powers of attorney for both financial and personal care decisions. I would also discuss some painful subjects with her if she is still well enough for the conversation on a living will, end of life preferences (such as a DNR order, hospice etc.) and funeral preferences. I know this is awful, but if you don't do it now, the opportunity to know her wishes may be lost, and believe me, it's comforting to know you are doing what a person wanted.

I would also consult the lawyer about estate planning and how to arrange her finances to preserve funds for her future care and how to set up ways to manage them on her behalf. Insurance rules can vary greatly from place to place and it really pays to get expert advice on what the rules are where you live, and what she may qualify for, including a good understanding of any private insurance she may have. This is absolutely crucial in the US - particularly since the rules around medicaid and medicare are so different from state to state.

The more you know about the various programs and supports that might be available to her, the less stressful this will be. Here is an example.

http://www.news-medical.net/news/20100212/SSA-to-add-early-onset-Alzheimers-dise

She is going to need full time care - she will need supervision and support 24/7, and she will decline physically as well as mentally, meaning that her care becomes heavier and heavier as time goes on. Your family will need help. This is one illness where it pays to be a pessimist. If you expect the worst, you will plan for it. You will do your research and homework on local supports, programs, facilities, and you will be ready for anything. Then if things get bad slower than you feared, you can be pleasantly relieved you don't need to put your plans into effect just yet. If you don't have a plan, you have the double whammy of being in distress over her decline and having to scramble in a crisis. It is off the scale stressful. So, even if you are not ready to think about all these things because you are still digesting the diagnosis, I would get my sneakers on and start finding out everything you can about dementia resources where you live.

Here is an excellent article to help you understand what your mother is going through.
http://www.alzheimer.guelph.org/downloads/12%20pt%20Understanding%20the%20Dement

Hope this helps

Mary  

In 1982 Dr. Barry Reisberg published what was to become the best and most widely accepted description of the stages of Alzheimer's disease. Even today, years later, when experts refer to a person being in stage 5 or stage 6, they are referring to Dr. Reisberg's scale of seven stages.

Adapted from Reisberg, B., Ferris, S.H., Leon, J.J. & Crook, T. The global deterioration scale for the assessment of primary degenerative dementia. American Journal of Psychiatry, 1982

Level 1

No cognitive decline - (or Normal Adult). No subjective complaints of memory deficit. No memory deficit evident on clinical interviews.

Level 2
Very mild cognitive decline (forgetfulness or normal older adult). Subjective complaints of memory deficit, most frequently in the following area:
(a) forgetting where one has placed familiar objects;
(b) forgetting names on formerly knew well.
No objective evidence of memory deficit on clinical interview. No objective deficits in employment or social situations. Appropriate concern regarding symptoms.

Level 3
Mild cognitive decline (early confusional or Early AD). Earliest clear-cut deficits. Manifestations in more than one of the following areas:
(a) patient may have gotten lost when traveling to an unfamiliar location;
(b) co-workers become aware of patient's relatively low performance;
(c) word and name finding deficit becomes evident to intimates;
(d) patient may read a passage of a book and retain relatively little material;
(e) patient may demonstrate decreased facility in remembering names upon introduction to new people;
(f) patient may have lost or misplaced an object of value;
(g) concentration deficit may be evident on clinical testing.
Objective evidence of memory deficit obtained only with an intensive interview. Denial begins to become manifest in patient. Mild to moderate anxiety accompanies symptoms. Deficits noticed in demanding employment situations.

Level 4
Moderate cognitive decline (Late Confusional or Mild AD). Clear-cut deficit on careful clinical interview. Deficit manifest in following areas:
(a) decreased knowledge of current and recent events;
(b) may exhibit some deficit in memory of one's personal history;
(c) concentration deficit elicited on serial subtractions;
(d) decreased ability to travel, handle finances, etc.

Frequently no deficit in the following areas:
(a) orientation to time and person;
(b) recognition of familiar persons and faces;
(c) ability to travel to familiar locations.
Inability to perform complex tasks. Denial is dominant defense mechanism. Flattening of affect and withdrawal from challenging situations occur.

Level 5
Moderately severe cognitive decline (Early Dementia or moderate AD). Patient can no longer survive without some assistance. Patient is unable during interview to recall a major relevant aspect of their current lives, e.g., an address or telephone number of many years, the names of close family members (such as grandchildren), the name of the high school or college from which they graduated. Frequently some disorientation to time (date, day of week, season, etc.) or to place. An educated person may have difficulty counting back from 40 by 4s or from 20 by 2s. Persons at this stage retain knowledge of many major facts regarding themselves and others. They invariably know their own names and generally know their spouse's and children's names. They require no assistance with toileting and eating, but may have some difficulty choosing the proper clothing to wear.

Level 6
Severe cognitive decline (Middle Dementia or Moderately Severe AD). May occasionally forget the name of the spouse upon whom they are entirely dependent for survival. Will be largely unaware of all recent events and experiences in their lives. Retain some knowledge of their past lives but this is very sketchy. Generally unaware of their surroundings, the year, the season, etc. May have difficulty counting from 10, both backward and sometimes forward. Will require some assistance with activities of daily living, e.g., may become incontinent, will require travel assistance but occasionally will display ability to orient in familiar locations. Diurnal rhythm frequently disturbed. Almost always recall their own name. Frequently continue to be able to distinguish familiar from unfamiliar persons in their environment. Personality and emotional changes occur. These are quite variable and include
(a) delusional behavior, e.g., patients may accuse their spouse of being an impostor, may talk to imaginary figures in the environment, or to their own reflection in the mirror;
(b) obsessive symptoms, e.g., person may continually repeat simple cleaning activities;
(c) anxiety symptoms, agitation, and even previously nonexistent violent behavior may occur;
(d) cognitive abulla, i.e., loss of willpower because an individual cannot carry a thought long enough to determine a purposeful course of action.

6a - Requires Assistance dressing
6b - Requires Assistance bathing properly
6c - Requires Assistance with mechanics of toileting
6d - Urinary incontinence
6e - Fecal incontinence

Level 7
Very severe cognitive decline (Late Dementia or Severe AD). All verbal abilities are lost. Frequently there is no speech at all - only grunting. Incontinent of urine, requires assistance toileting and feeding. Lose basic psychomotor skills, e.g., ability to walk, sitting and head control. The brain appears to no longer be able to tell the body what to do. Generalized and cortical neurologic signs and symptoms are frequently present.

7a - Speech ability limited to about a half-dozen intelligible words
7b - Intelligible vocabulary limited to a single word
7c - Ambulatory ability lost
7d - Ability to sit up lost
7e - Ability to smile lost
7f - Ability to hold up head lost  


---------- FOLLOW-UP ----------

QUESTION: I guess I would like to know roughly how much time we have with my mother untill she is unable to recognize or enjoy her new grand daughter.  And what is approximate life expectancy with a rapid onset.  She was diagnosed by a neurologist and this was one of many ct scans she's had.  From the information you provided she is in the stage of forgetting immediate conversations at times, as well as significant things in the past I thought she would never forget.  She also has flighty conversations and thoughts.  I appreciate your help and thank you.

Answer
Hi Sam,
From what you are saying - she's probably somewhere in Stage 4. I think they gave you a reasonable survival horizon of 5-6 years. In the frail elderly, although they often quote an average survival of 6-8 years, recent studies have shown it is much shorter - more like 3 years. This is not from the dementia itself, but because the dementia contributes to an earlier death when piled on top of other health problems. As you can appreciate, many elderly people have a constellation of complicated health concerns. As the dementia progresses, it gets harder and harder to detect or treat them. The person can't answer questions or report symptoms and you are left with observing behavior or external signs only. Many families will decide that it is not worth putting their loved one through the trauma of tests, procedures, or therapies that can be painful, scary or distressing, when their loved one can't understand the purpose or cooperate - and worse, won't really benefit in terms of better quality of life. If someone is already in Stage 6 or 7, the priority may be to keep them happy, peaceful and comfortable, and let them be, rather than aggressively go after a cancer or heart problem.

At 49, your mother will be robustly healthy compared to an older person, so she is not as likely to experience those problems, and is likely to rub on to the natural end of dementia, rather than be mercifully carried off sooner by some other problem. She is likely to progress to Stage 7 and then survive there for some time - so she may be in that state for the last few years (i.e. unable to walk, talk, or do anything for herself, and not be really aware of where she is or who is with her). It is a relentless, downhill slide. Its very hard to grasp how much of what we think of as personality is dependent on an intact brain. Just keep firmly in mind that she is doing the best she can with what she has left, and if she does strange things, has hallucinations or delusions, makes wild statements or accusations, she is not capable of doing these things on purpose. You can't reason her out of it. Her reality is framed in what her brain will allow. Comfort, cajole, pat, sooth, divert, distract, reassure - with a heavy emphasis on  loving comfort and reassurance. It must be a terrifying experience, so don't get into logic or arguing.If she gets upset, try to deal with the emotions underlying the distress and not the "facts" around her distress. She will have no self insight and will not be able to see what you see.  

So, seize the day, since now is what you have, and what comes next is not happy - and it is also very unpredictable in terms of how fast it may go - sadly, plateaus can happen in the last stage, when it is not a kindness.     

Because this disease generally erodes memories in the reverse order they are laid down, she is very likely to not know a small child is her grandchild after the next year or two. That does not mean she won't enjoy grandchildren - she just may not be able to grasp that they are HER family.  By mid dementia, my mother in law had forgotten her marriage, and asked all the time about her parents and family home where she thought her sisters were waiting for her. She started signing her maiden name, and often thought I was one of her sisters. I think she knew I was a familiar friendly face, but because her memories of her son being a married adult were gone, she couldn't quite figure out who I was. At times, she would think my husband was her husband, probably because they had a physical resemblance.  We had three children born during her decline, and by the time the third was born she was in later Stage 6. I think she recognized our daughter as a cute baby, and would smile and coo at her, and got a lot of pleasure out of seeing her and the other two little boys, but I don't think she knew in specific that they were "hers".  They were just sweet children she liked to see, in the same way holding a puppy or kitten would illicit pleasure from her.

This is heart breaking, I know. I think if you read the article about the experience of Alzheimer's, it will help you understand some of what to expect, and how she experiences what is happening to her.

Hope this helps. I'm thinking of you - this is so unfair for all of you.

Mary