Expert: Mary Gordon - 12/11/2009

Question
My father has experienced an incredibly fast onset of neurological problems.  A year ago, he was fine, except for some basel cell skin cancer on his temple, and a general slowing down.  We had noticed in the past few years he was becoming more ornery and set in his ways but since he's always been rather autocratic, this was not surprising.  In April, he began struggling with word usage: he couldn't remember the word he wanted to use, only it was to a worse degree than just normal forgetfulness.  He continued to get worse, eventually not saying much of anything.  He was diagnosed with Alzheimers in October.  However, according to the Mayo Clinic's stages of Alzheimers, he has progressed from Stage 1 to Stage 5 in 11 months.  I just heard this morning that he is now suffering incontinence.  Is there anything else this could be?  Has anyone ever heard of Alzheimers progressing that quickly?  

Answer
Hi Angela

You don't mention how old he is, or how his general health is - or what process was gone through to arrive at the diagnosis. As you probably know, there are quite a constellation of chronic health issues that can gang up and attack a person's cognitive abilities in their senior years. The brain is a very delicate thing.

When they were diagnosing him, did they put him through a full physical and work up to rule out other conditions? Did they review all his medications and did they do a full cognitive assessment? Did they do a brain scan to look for signs of small strokes, tumors, shrinkage patterns in his brain etc. ?

There are a number of causes of progressive dementias - and not all of them follow the exact same path in terms of areas they affect, and speed of progress. Below my signature I've pasted the FAST scale, which you refer to so you can reference where he is functionally.  

Alzheimer's is often like an ice burg - there can be much more damage than is apparent in a social interaction.  A very intelligent and self confident person can often hide their deficits in social situations far into the illness. They find ways to cover their errors and to compensate.  If you had met my mother in law in at a dinner party even quite far into her illness, you likely would have been like most of our relatives and family friends, and thought she was perfectly charming lady and had not a darned thing wrong with her.  All we were aware of was some relatively minor forgetfulness, and an increased reluctance to attend social functions or try new experiences - and these things were not apparent at all to even her sisters and close friends, and even less so to those who saw her only occasionally. She could also carry on an absolutely perfect conversation on the telephone to the point many of her friends thought we were being cruel and ridiculous wanting her to see a doctor.

My husband went with her to her cognitive assessment, which was done by a psychometrist and was similar to what you would put a child through looking for learning disabilities. It was completely shocking to him - because even though she appeared so intact, she was profoundly impaired on many fronts we would never have suspected, or given a moments thought through. She was just that good at concealing it - and after all, we rarely give our loved one a real grilling about what they tell us, or put them through detailed paces about what they know or don't know, or what they can do. It would be insulting and disrespectful - especially for an authority figure like your father, who is used to being in charge and probably would get annoyed with you if you tried!

Everyone has redundancy in their brains - many pathways the same message can take. In Alzheimer's, as the brain damage progresses, it can be going on with no real outward signs - until that last neural connection involved in a particular ability winks out, and the pathway is broken. Suddenly, a major ability can seem to vanish overnight. My mother in law's speech was like this - although she had word finding problems and had difficulty formulating a coherent answer to questions, she could talk - but suddenly over a two week period, her speech went. After that point, almost everything that she said was garble and nonsense syllables. It was startling.

Keep in mind also, that your father was very likely in Stage 3 even back in April he was actually diagnosed, even if it wasn't readily apparent to family. It is also possible to have more than one cause of progressive dementia going on at the same time, such as multi-infarct dementia or Lewy Body dementia.  

Multi-infarct dementia is very common - its from tiny strokes or blockages, and are common in people who may have cardiovascular risk factors. These may have no symptoms at the time that you can see from the outside and can be happening all the time. Each tiny blockage causes a tiny bit of brain damage and over time the damage accumulates to the point it becomes apparent. People with multi-infarct can have sudden lurches in terms of losses, or rapid decline followed by periods of relative plateauing.

Other things that can make cognitive decline be rapid when layered on top of Alzheimer's include encephalopathy associated with autoimmune thyroiditis, conditions related to autoimmune diseases or to cancer, infections, side effect of certain medications, such as anticholinergic drugs and narcotic pain relievers, Vitamin B-12 deficiency (pernicious/Addison's anemia), depression, and hypothyroidism.

Lewy Body dementia can cause language problems very quickly and people with this type of disease are also very sensitive to some of the drugs used to treat Alzheimers, and as I mentioned it is entirely possible to have more than one cause of dementia happening at the same time. It gets called  a "mixed" dementia.

If you think something unusual is going on, take him back to the specialist who made the diagnosis and have a discussion with him or her about the rapid progress to see if there is anything treatable that might be going on, or if there may be more than one type of dementia at work.  It's worth asking - you need peace of mind - but be prepared that there may be little help for his condition.

There is nothing more heartbreaking than cognitive decline - it is much harder for families to deal with than any other type of illness or disability. I'm thinking of you. Speak to his doctor.

Mary G.
Toronto

Stages of Dementia

In 1982 Dr. Barry Reisberg published what was to become the best and most widely accepted description of the stages of dementia progression - they are referred to as the FAST scale -it is a functional assessment scale based on abilities. When experts referto 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