sailor girlAbout Pick's Disease
by Lynn Erstein


I opened the letter from my father and read the following:

"Sit down before your read this, as I must pass on to you some not so good news. Up front, your mother has a condition that relates to a slow deterioration. The neurologist says that it’s treatable but not curable. Science has not figured out yet how this comes about nor can they predict the rate of deterioration. Although it’s not good news, it does explain the problems we have all noted. The fancy medical name is Alzheimer’s disease, named after the German doctor who first described it in 1906".

This was written almost seventeen years ago, and although Alzheimer’s is now a household word, and doctors know much more about its molecular pathology, science has still not figured out how this comes about nor can they predict the rate of deterioration. We now had a reason for the strange things we had been noticing during the last few months:


Speech and Language

One of the first signs we noticed was that my mother would repeat herself, asking the same question over and over and retelling old stories. The sensory aphasia had surprised us, but we had been ignoring this misuse of words for some time. She had been a very articulate woman, so it especially pained us later to have to help her figure out what she wanted to say when she experienced motor aphasia. Her muscles just wouldn’t move to pronounce the words she wanted to say. The slowed and slurred speech was startling because proper enunciation had always been so important to her. There were paraphrasic errors that we later learned were important indicators of aphasic language disorder. She would substitute first just a word and then later strings of words that made understanding what she was trying to say an ever increasing challenge. As she was losing expressive language, her sentences got shorter and shorter. Finally she would give up speaking altogether but continued to communicate by writing for almost another year.


Judgement-both logical and social capacities

She had been a very good, careful driver but we had noticed some disturbing lapses in judgement, such as driving too fast. Her visual agnosia also made driving dangerous as she lost her depth perception and ability to understand what a stop sign even meant. As a diplomat’s wife, she had all the proper social graces, but now she was interrupting people with totally different topics, hanging up the phone mid-conversation, and later would drink from other people’s glasses at the dinner table, not know when to stop eating to the point of vomiting and just have trouble functioning in a social situation. This was especially sad as she wanted so much to continue her old relationships with friends but it became increasingly difficult.


Memory

This was first noticed when travelling. She had trouble remembering new information like where the car was parked. Although at the beginning only short term memory was affected, later we would have to deal with problems like her forgetting how to turn off the bathtub faucet and the stove burners.


Ability to Abstract

Her thoughts as seen in her diminished conversation were becoming more shallow. Her ability and enthusiasm to discuss world events and history was leaving.


Emotional Responsiveness

My mother, a usually outgoing person, was becoming more quiet and aloof. As previously her face and voice would be animated in conversation, she would eventually have a flat affect.

A short time later I read about Alzheimer’s Disease. The article also went on to tell about a related condition, called Pick’s disease or Pick’s convolutional atrophy with similar symptoms plus atrophy of the temporal and frontal lobes of the brain. To arrive at the diagnosis, my parents had made the rounds of doctors, internists, neurologists, and psychologists. Since there were no deductive tests available we had to rely on inductive testing. Her regular internist did the physical exam including a complete blood and urine work-up to rule out vascular, infectious and metabolic disorders, hepatic encephalopathy, certain lung diseases and depression. She was put on Ritalin to combat any depression and help with distractibility. From the mental status testing, the psychiatrist doubted she was suffering from a psychiatric syndrome and specifically doubted that depression was a likely cause of her clinical problems.

My mother was fortunate to be admitted to the Alzheimer’s research program at the National Institutes of Health, in the National Institute of Neurology, Communicative Diseases and Strokes, near Washington DC. Her neurologist there primarily did research and had only 32 patients in his program. The program was free to us. They would identify her problem, monitor her twice a year, perform the latest tests and make recommendations to be carried out by her regular physician. If there were any breakthroughs she would be among the first to have them available. In return, she would be studied to benefit Alzheimer’s research and to expand knowledge of the disease. After her death they would perform a full autopsy and use her brain to further the study of dementia. A thorough work-up during a three week hospital stay at NIH, revealed everything to be within the normal range except the CT scan of the head, which revealed diffuse sulci widening with hydrocephalus ex-vacuo. It showed prominent dilated ventricles, prominent subarachnoid spaces and cortical sulci, most likely representing brain atrophy. The report from the physician in charge stated, "Overall my impression is that the patient is suffering from a disorder which has resulted in a personality change over the course of the past year with some memory, language and apraxic difficulties. There is insufficient evidence to attribute this disorder to a psychiatric problem and it seems entirely possible that the patient is actually suffering from Alzheimer’s disease which is presenting in a very unusual fashion and could, in fact be Pick’s disease".

Personality, behaviour and orientation changes tend to come before memory loss in Pick’s disease, unlike Alzheimer’s disease. From a PET scan, taken less than a year after the first diagnosis, it was apparent that the major involvement was in the right frontal lobe- the area that controls personality, speech, and behaviour. Even my father, with no medical background, could see from the scans, that the right hemisphere of the brain was diminished.

In 1983, one of the leading theories was that Alzheimer’s disease. was due to an imbalance in the chemistry of the spinal cord fluid. A drug was approved and tried based on the fact that the brain works by transmitting small electrical charges through fluid from neuron to neuron. Starting in September 1983, she spent 6 weeks in NIH to monitor the side effects from a new drug, THIP. While there she took part in a seminar on dementias for 50-75 doctors. The drug did not help and later trials were given to patients who were in earlier stages of the disease. During the seven years my mother was in the nursing home, she continued to be monitored semi-annually at NIH. She died of pneumonia on November 21, 1992.


Autopsy Report

A complete autopsy was given at 9:30 AM on November 22, 1992. The part of the report covering the brain-stated that the cerebral hemispheres were symmetrical with marked circumscribed frontotemporal atrophy . Sections of the grossly abnormal areas (frontal and temporal cortex, and to a lesser extent head of caudate), reveal marked cell loss and gliosis in the grey matter. Numerous amphophilic intraneuronal inclusions (Pick bodies) are present. Some ballooned neurons are also seen. The neuropathological findings are typical of Pick s disease, and well explain the clinical findings. It is of interest to note that Pick bodies, which are absent in up to 2/3 of cases of circumscribed atrophy, are numerous here. Although there was no definitive test for Pick’s disease, it turned out that some of the early theories were correct.

The pain and frustration of watching my mother drift away from us was devastating. The only good to come of it is knowing that perhaps she helped scientists understand the disease which will eventually lead to a cure.


The above letter was taken from the newsletter of The Pick's Disease Support Group August 98 Volume 4

You can find more information like this at: http://dementia.ion.ucl.ac.uk/candid/factsheets/


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