CLINICAL UPDATE FROM BARNEY AND KAREN: September 3, 2011

"…plus ça change, plus c'est la même chose…” (…the more things change, the more they stay the same…”) French proverb

"It's déjá vu all over again." Yogi Berra, baseball player and coach

“What has been will be again, what has been done will be done again; there is nothing new under the sun.” Ecclesiastes 1:9, NIV
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“What day is this?”

“What is this place?”

“I am going to give you three items to remember and will ask you to tell them to me in 5 minutes.”

“Spell ‘WORLD’ backward.”

“Draw a clock face with the numbers in it and put the hands on it to show ten minutes until eleven.”

The above are sample questions from a MMSE: the “Mini-Mental Status Examination”, devised years ago by folks at Johns Hopkins. It is not a diagnostic test of a specific disease; it works best to screen for possible cognitive problems, and can be used as a tracking tool in people with known cognitive disorders. Every three months or so when Karen visits a neurologist, someone will administer a MMSE; tracking her scores over time gives a baseline picture of the speed of decline. There are variations on the original test, which has a perfect score of 30; one can score 28 or above and the results be considered normal, to make allowance for the occasional bad day. A score of 23-25 raises suspicion of a problem, and anything less than 23 is consistent with cognitive impairment. Karen scored 24 years ago on initial evaluations, and her scores have trended downward, now fluctuating between lows in the mid-teens, still sometimes as high as 21. She will be able to do something on one visit that she could not do on the previous, and vice versa. There is no practice making perfect here: she never remembers having taken the test before.

“What is today’s date? Day of the week? Month? Year? Season?” Karen does not know. She starts to guess, then admits she has no idea.

“What are the three things that I asked you to remember?” No clue.

“Spell ‘world’ backward.” This one she can do, always has been able to, and can usually repeat up to seven digits in reverse order.

“Draw a clock face, put the numbers in it, and draw the hands to show 10 minutes until 11.” Now we’re in trouble. Karen can still tell time from looking at a clock, but re-creating that picture in her mind from memory is a daunting task. It takes her about 30 seconds but with encouragement she finally draws a circle, then begins to put in the numbers, in correct order but somewhat off kilter; the “12” is at the usual “11” position. She completely draws a blank about how to draw the hands; she finally just writes the numbers “10 to 11” in the middle of the circle.

We returned to the neurologist last week to get reports on the most recent PET scan and any other info he might have. I had written in previous reports that research-level tests have shown that Karen does not have the particular abnormal protein whose deposition in brain tissue is the basic problem in Alzheimer’s Disease, ergo she doesn’t have Alzheimer’s dementia. Pick’s Disease, one of the lesser common FrontoTemporal Dementias, has been the most recent label. On this visit, the neurologist goes over the PET scan and reports that it is not at all consistent with Picks or FTD. He announces we are now back to a diagnosis of Alzheimer’s Disease. I asked him about the issue of the undetectable abnormal protein, and he admits that is a problem, but that they have seen some rare (not an unfamiliar word for us in our tour of the wonderful world of dementia) patients who either have a different form of the protein that doesn’t lend itself to being detected, or perhaps have a different mechanism underlying the damage which is identical to Alzheimer’s.

Since we have come full circle, he started her on another medication for Alzheimer’s, one available for everyone but the only one she had not tried previously. These medications don’t change outcome of disease; the course and duration of the illness is the same, but when they do work they may allow function to be preserved a bit longer than would have been the case without the medication. And, Karen will still be maintained in the Alzheimer’s Disease research study. Welcome back, although we never really left.

So, we proceed, looking for places where being able to spell DLROW may be useful and trusting that our daily activities won’t demand that we draw a clock. The test did not go well, and the only new news we have is old news revisited. The only comfort in knowing that Karen may not know where she is, is that she does not remember having been there in the first place. But, all the test results were not negative. She may not know the day or date or season or time, but there is one thing of which Karen is sure:

“Take this piece of paper and write a complete sentence on it.” Karen takes the paper, writes something, folds it and gives it back to the examiner. The lady glances at it, smiles, and replies, “Yes, he is.” I take a look:

God is in control.

“The last and final word is this: fear God. Do what he tells you. And that’s it.” Eccl. 12:13-14, The Message