Expert: Mary Gordon - 1/6/2011

Question
my grandpa has been diagnosed with lewy body disease and dementia and have been told that he only has between now and a few years left.I would like to know what are signs to watch for when the time is getting closer? And signs and symtoms of it getting worse?

Answer
Hi Angela,

I'm very sorry to hear your grandfather has had this sad diagnosis. You probably know that Lewy Body is the second most common type of degenerative dementia after Alzheimer's disease. It is named after "Lewy Bodies", which are abnormal smooth round lump like structures found in some affected nerve cells in the brain of people with the condition. Lewy Body has many symptoms in common with other dementias - i.e. gradually more and more problems with memory, logic and reasoning, emotional control, personality changes, loss of physical coordination, and perceptual problems. It does have three slightly unusual features that distinguish it from other progressive dementias. These are very vivid visual hallucinations, physical symptoms similar to Parkinson's disease, and fluctuations in ability and alertness.

Your grandfather may see things that aren't there, and they may seem entirely real and vivid to him. They may be very detailed, and he may be upset or he might be amused by them. No matter what, his brain may make him totally sure they are real, even if they make no sense to you, and arguing may just make him upset, rather than convince him he is seeing things. The mother of a friend had Lewy Body, and she would wake up at night thinking there were men in her bedroom. She could even describe what they were wearing in great detail, so my friend learned just to reassure her mom, make up some story to explain what her mom thought she was seeing, and try to distract her and get her back to bed.

Another distinctive symptom of Lewy Body is the Parkinson's like symptoms. The person may develop a shuffling gait, get very stiff, develop "shakes" and have trouble starting movement.

The fluctuations in abilities and alertness can be really startling. He may be able to do something perfectly well at one point and an hour later not be able to do it at all, or he may zone out like he's in a stupor. It may look like he is doing this on purpose, but he can't help it. He may even start to fall or look like he is passing out.

Lewy Body patients usually have sleep disturbances, and may even act out their dreams.

All of the progressive dementias are considered terminal illnesses. That does not mean he is going to keel over out of the blue, unless that is from some other health issue. The Lewy Body will cause him to go relentlessly downhill physically and mentally over the next couple of years. If he has other health problems, they may make his decline faster or more complicated. There is no cure - there are some medications that can slow down the progress in some people, or at least that can make the person more comfortable in terms of how calm they feel. Lewy Body patients have exaggerated reactions to many medications, and not all doctors will understand how dangerous some meds can be for a person with Lewy Body. In specific neuroleptic drugs like tranquilizers used to treat psychiatric, mood and behavioral problems in many dementias are very dangerous for people with Lewy Body and can make them very much worse.

Here is a good web page - the Lewy Body Dementia Association http://www.lbda.org/

Many years ago, a functional abilities scale (FAST) was developed to describe where people are in the progress of many dementias, based on what the person is able to do. No one person will necessarily fit neatly into any one stage perfectly, but in general, the ability to do things tends to be lost in specific orders as brain damage progresses. This makes the FAST scale a handy way to describe how far along in the illness a person is, and helps a family to anticipate what may happen next. I've pasted these below my signature to give you an idea where your grandfather is in the progress.

Hope this helps

Mary

The FAST scale is 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