To cure sometimes.
To help often.
To console always.

Definition: Neurodegenerative dementia is a syndrome of brain disintegration, which does not specify cause. It is an acquired and substantial decline in thinking (cognition), comportment, behavior, personality, or activities of daily living that has significantly affected functioning. The most common dementia, Alzheimer’s disease:

Alzheimer’s disease represents 60-70% of the dementias diagnosed in the United States, mixed Alzheimer’s disease and vascular ischemic cerebral injury dementia represent 20-30%, dementia with Lewy bodies and/or mixed Lewy body Alzheimer’s disease can represent up to 20%, frontotemporal lobar dementias are less than 5%, and normal pressure hydrocephalus represents less than 1%.

Prevalence in the United States:

It is estimated that at the current time there are about 5.3 million people with Alzheimer’s disease. In 2030, it is estimated that 7.7 million will have Alzheimer’s disease, and in 2050 16 million people. The cost of caring for Alzheimer’s disease in 2007 was 91 billion dollars. If you have a person with Alzheimer’s disease whom you need to place in a nursing home in a metropolitan area, you can now figure on paying between $7,000 and $12,000 a month for their care, and another $2000 to $3000 a year for their medications.

The chance of having Alzheimer’s disease if you are sixty is 1.2%; if you are seventy, 10-15%; if you are eighty, 20-30%; if you are ninety, greater than 50%.

Alzheimer’s disease:

In the Western world, this disease was first described clinically in 1901. It was thought to be divided into 3 stages – mild, moderate and severe.

The cause of this disease is still not known. The leading theory is that there is a toxic buildup of proteins which clump together causing brain damage. This is thought to come from an imbalance between production and clearing of misfolded and toxic forms of amyloid beta protein which causes plaques and tangles in the brain, which interfere with normal brain function.

The disease is divided into a presymptomatic Alzheimer’s disease stage, which is early, with mild brain changes but no symptoms. Next appears mild cognitive impairment with mild changes noted in the brain and mild symptoms. Then comes the true Alzheimer’s disease, which goes through a mild, moderate and severe stage over 7-15 years before death.

Normal age-related cognitive changes are mild decreases in mental perception, processing, and response. There is a somewhat lower rate of learning, there is somewhat slower retrieval, there is diminished fluid intelligence, but there is no increase in forgetting; there is improved vocabulary, there is greater experience and wisdom (crystallized intelligence), and more sophisticated practical and effective life strategies and performance in the real world, and many people lose no obvious function well into their 90s.

Brain plasticity is a concept which describes the capacity of adaptive reorganization, a capacity for physical and functional change. This enables experience to induce learning throughout life. This is the old concept that it takes longer to teach the old dog a new trick, but the dog can still learn. This is not the case with Alzheimer’s disease.

Non-modifiable risk factors in Alzheimer’s disease are age, genetics, specific mutations 1, 14 and 21, family history of dementia in a primary relative, ApoE4 genotype, and Down’s syndrome.

In the workup of dementia, in the history one looks at the instrumental activities of daily living, which are finding belongings, use of the telephone, driving abilities, ability to keep appointments, ability to maintain a checkbook balance, finances, shopping, the ability to calculate tips, the ability to continue your hobbies, the ability to travel—especially to travel alone, the ability to do laundry appropriately, the ability to cook without leaving on the burners or burning things, the use of home appliances, the ability to perform cleaning in a routine way, the ability to do household repairs, and the ability to manage medications appropriately. The basic activities of daily living are dressing, eating, bathing, grooming, feeding, mobility (i.e., getting in and out of bed), toileting, and continence.

If any of these change suddenly in a patient with Alzheimer’s disease, look for infection, especially in the urine and in the lungs; look for silent strokes or TIAs; look for occult heart disease, such as a silent heart attack or the onset of atrial fibrillation or congestive failure. The workup of Alzheimer’s disease includes a complete blood count; a metabolic panel which looks at blood sugar, kidney and liver functions, calcium, etc.; B12 levels; folic acid levels; thyroid functions; ESR and CRP, which are measures for inflammation, infection and malignancy; serum lipids; and a brain imaging study, such as a CT or an MRI. These effectively rule in or out treatable and/or curable causes of memory loss.

Drugs used to treat the disease are Aricept (donepezil), Exelon (rivastigmine), Razadyne (galantamine) now generic, Namenda (memantine). The purpose of giving medications early is to try to slow the progression of functional decline. These drugs keep working in the brain; however, the disease progresses and eventually they no longer slow progression.

It is well known that the combination therapy (for example, Aricept with Namenda) delays nursing home placement.

The best drug is consistent love given freely and liberally with larges doses of patience and understanding.

What to do for the patient with Alzheimer’s disease:

They understand that there is no cure, but do not take away hope.

Recall the example of Ronald Reagan, who announced to the country that he had the disease. Recall that he maintained good nutrition, good health habits (swimming every day), a solid family around him, appropriate medication but no inappropriate medication, and he was allowed a graceful death.

Do not defer dreams; enjoy life now. Remove any medications which make the patient feel worse. Avoid antihistamines, narcotics, benzodiazepines, and other medications that may cloud the sensorium.

If you wish to use vitamins and minerals, the only ones that have been studied and have shown minimal or some benefit are vitamin E at 2000 international units twice a day, vitamin C 1000 mg a day, aspirin 81 mg a day, statin drugs, B12, B6, folate, and SSRIs, and omega-3 fish oils at 1000 mg two times a day.

You must understand that the data regarding all of these supplementary medicines are weak in terms of any major improvement, and in fact many people who have Alzheimer’s patients do not prescribe them at all.

Family caregivers must help the patient attend to medical and legal issues while they still have the competency to do this. They must also work on continuing care issues, genetic issues, home safety issues, medication administration, and support for the caregiver, which is critical. Medications for anxiety and depression should be given if these diagnoses can be made.

The enlisting of all social support networks is, again, extremely important.

Support groups have been immensely helpful as have massage, physical therapy, occupational therapy, nutritional guidance, and balanced exercise classes.

Daycare and respite care are valuable in continuing the comforting of people with these dementing illnesses.

It is important to optimize nutrition, sleep, and to decrease stress and anxiety by simplifying life. Eliminate all deleterious medications. Optimize mental activity and physical activity and exercise, social activity and engagement to allow the patient to feel that they still have a place in the community of life.

Other dementias that present:

Dementia with Lewy bodies is 50% coexistent with Alzheimer’s dementia so it can be a mixed spectrum.

This disease is noted for marked fluctuations of alertness, recurrent visual hallucinations, nonvisual hallucinations, delirium, syncope and transient unresponsiveness, unprovoked falls, and Parkinsonian features. These patients are also extremely sensitive to the neuroleptic drugs, such as Haldol, Seroquel, etc. When given these, they often have adverse rather than positive reactions.

The frontotemporal lobar dementias are another group of dementias, which are not uncommon. These cause focal neuronal loss, and they are most commonly associated with behavioral symptoms.

There is a primary progressive dementia which is a language variant.

Corticobasal degeneration, progressive supranuclear palsy, and dementia with motor neuron disease all come in this category.

None of these syndromes present with memory impairment in the early stages. Frontotemporal dementia commonly presents with apathy, detachment, disinhibition and impulsivity, with preservation of memory and language.

Primary progressive frontotemporal dementia shows dearth of language, fluent or nonfluent variety, and language disorder is the principal deficit. Its cause is neurodegenerative. Dementia with Lewy bodies has already been mentioned; subcortical dementias of the basal ganglia, such as Parkinson’s disease, progressive supranuclear palsy, and Huntington’s disease, will be discussed. Progressive supranuclear palsy is characterized by falling backwards, ocular muscle abnormalities, and a frontal lobe syndrome, but the preservation of memory. Parkinson’s disease has a 50% association with a dementia as the disease gets into its later stages.

Vascular dementia is the result of small clinically silent strokes with white matter loss. Its characteristic is that the decline in memory and function occurs in a step-wise rather than a slowly descending fashion, such as that seen in Alzheimer’s disease. This can be seen in a combined form with Alzheimer’s disease.

Nonpharmacologic treatments of dementia:

The provision of rapid relief of any distress in the patient’s life, minimizing functional loss, preventing injury to the patient and to the caregivers, and the institution of pragmatic alliances such as that with multidisciplinary caregiving teams and support groups.

It’s not what you say to the demented patient, it’s how you say it. Simplify their routines, work with them on a regular basis and explain what you’re doing, diminish visual and auditory clutter, protect from injury, and again do not forget interdisciplinary care. Recall that faith heals.

Old age is not an illness waiting to be cured, it is part of the process.

And remember this quote from the Talmud, “We do not see things as they are, we see things as we are.”

I highly recommend that you read the article by Nancy Stearns Bercaw, published January 17, 2001, in the New York Times, “When All Isn’t Enough to Foil Alzheimer’s.” It is a short, 3-page article which is extremely revealing about the course of Alzheimer’s disease and its effect upon the caregivers and the patient.

http://www.nytimes.com/2011/01/18/health/views/18cases.html?_r=1

Thank you for the opportunity to discuss the dementias, illnesses which tug at the heart strings yet give us an incredible opportunity to serve and gain wisdom.

Robert Backus, M.D.