Expert: Mary Gordon - 1/13/2009

Question
QUESTION: I am perplexed about the symptoms my husband displays. I don't know what stage he is in. He has never not known his surroundings, people in his life, past events. Never has repeated himself or wandered. Have you ever heard of someone having all that ability and being in stage six as I am told he is? It's like his body is breaking down and his speech and motor function to the point that you would say he is in stage six. I would appreciate any information you can give.

ANSWER: Hi Susan, you don't tell me enough about his symptoms to really gauge what's been going on. The stage descriptions are a general guideline only - not everyone will experience the same things, or necessarily fit perfectly into a single stage description.

Many older people have a constellation of health issues that can affect cognition and neurological systems. Its not uncommon to have more than one cause of dementia happening at the same time (i.e. a person may have Alzheimer's and Parkinson's at the same time, or mini-strokes (called multi-infarct or vascular dementia) on top of Lewy Body dementia.)  Sometimes when there is more than one cause happening at the same time, the family will be told the person has a "mixed" dementia.

I don't know what they did to diagnose your husband in the first place - sometimes it can be very complicated to figure out the root causes of progressive dementia, particularly if they have an involved medical situation. He may even have something other than Alzheimer's entirely. Sometimes, it doesn't matter what the underlying causes are, if treatable ailments have been ruled out. When a person is experiencing irreversible brain damage, and there is no reasonable expectation of recovery, the prognosis is never positive. Most of the progressive dementias end up at the same place by the later stages, no matter how winding a path they may take to get there.

Some of these dementias do impact speech and movement more than memory, so they can have a somewhat different course than straight "classic" Alzheimer's.

Frontotemporal dementias often primarily involve language deterioration, while memory may be relatively spared until later stages. Motor problems like stiffness and balance are often seen with FTD.

Lewy Body dementia goes with a physical deterioration that looks like Parkinson's - in fact, falls may be an early sign.

I'm not saying this is true of you, but its often tempting to assume that the person is more "with it" than they are, particularly if their personality is intact far into the illness. We had family and friends who staunchly believed there was nothing wrong with my mother in law until she was almost at the end stage. After all, they cared about her, and she could fake a good social conversation by smiling and nodding, and making encouraging noises to whatever they said.  They never grilled her on facts the way an outsider might (such as saying, if you know who I am, what's my name? Where are we exactly? What did we eat for lunch today?). They wanted to believe that she was still "in there" intact, to give her the benefit of the doubt, so they interpreted her actions and words in that way.  They tended to see the person she had been rather than who she had become.   

You might want to speak to his doctors to find out if they have any ideas about why his memory has stayed relatively intact compared to his motor skills and speech, and if they suspect a dementia other than Alzheimer's.

Not sure if this has really answered the question - the changes in behavior and skills of a person with brain damage can be very hard to anticipate, and everyone is different.

Mary G.
Toronto

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

QUESTION: He is 61 years old. Was diagnosed 5years ago. I noticed changes as long as 10 years ago. He had every known test and scan available. It was suggested it was frontal lobe. I have read up on everything you mentioned above. He fits nothing. He does fake understanding a complete situation but he is still part of and understands everything and answers as best he can. So assuming it is frontal lobe can it be the end stage in your opinion and still have his mental ability? I have gotten mixed answers from doctors. His symptoms are incontinence, can't add or subtract, can't read, needs help showering, dressing, can't figure out how to unroll the toilet paper, has a slight jerking of limbs. walks very slow but has never fallen. Very slight cough is something new. It is just so hard because he doesn't follow the normal  progression.

Answer
Hi Susan,

The average age of onset  for the frontotemporal dementias is younger than that of sporadic Alzheimer disease (vs. familiar Alzheimer's), so this fits with his situation.

Some patients with experience only aphasia for periods exceeding 10 years, while others progress to dementia within a few years. A significant percentage of people with FTD develop severe muscular problems that can cause loss of coordination, widespread muscle atrophy, weakness, twitching, problems with the muscles of the tongue, jaw, face and neck etc.

Here are descriptions of two variants that might fit what you are seeing.

Progressive nonfluent aphasia (PNFA)

PNFA accounts for only about 20% of all people with FTD. Unlike semantic dementia where the person maintains the ability to speak but loses the meaning of the word, people with PNFA have difficulty producing language fluently even though they still know the meaning of the words they are trying to say. The person may talk slowly, having trouble saying the words, and have great trouble with the telephone, talking within groups of people or understanding complex sentences. In recent years it has become apparent that many patients with PNFA go on to develop severe Parkinsonian symptoms that overlap with progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) such as an inability to move the eyes side-to-side, muscle rigidity in the arms and legs, falls, and weakness in the muscles around the throat.

FTD with motor neuron disease

Approximately 15% of patients with FTD also develop motor neuron disease (FTD-MND). Most often, this combination occurs in patients with bvFTD, and only rarely does MND arise in patients with SD or PNFA. MND affects motor nerve cells in the spinal cord, the brain stem (which sits on top of the spinal cord), and the cerebral cortex. Because the brainstem was once referred to as the “bulb”, you may hear some MND symptoms described as "bulbar symptoms". The most common type of MND is amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, which can occur as a purely motor disorder. More often, however, patients with ALS also have behavioral or cognitive problems similar to those seen in FTD. MND symptoms include slurring of speech, difficulty swallowing, choking, limb weakness or muscle wasting. In patients with FTD-MND, there is often (but not always) a family history of the disease, and scientists are getting closer to identifying gene mutations that cause the illness.

Sadly, even if one of these is what is going on, being sure of the cause wouldn't be much help to you since there are no treatments that can stop these illnesses.

Mary G.