What is dementia?

 

Dementia is loss of cognitive functioning more than what may be expected with aging. We all are born with varying amount of intellect or intellectual capacity. Based upon this inherent ability, if groomed and nurtured appropriately, we grow up and undertake different tasks and professions. As we grow older, brain evolves into performing different types of cognitive functions, and it keeps on changing. In middle school years, our brain specializes in remembering literal facts, so is the reason that a middle-schooler may easily outperform adults in spelling competition. We lose that kind of ability to remember in a few passing years. But that is not dementia; it is part of normal aging.

 

In middle age, many of us struggle with names and telephone numbers, but we do not call it dementia either. The term dementia implies that the cognitive problem is more than what one may expect for a certain age and situation. For example, at the end of a busy stressful day, one may forget to fetch milk if told to do so, but if that person cannot find where he parked his car in the parking lot, he may have a problem. 

 

Memory dysfunction is the most common symptom of dementia, and in many cases it’s the first symptom. A way to explain this problem is following: Imagine there is a memory-making machine in our brain. It works very well when we are young but gets cranky as we grow older. Our capacity to make new memories and storing them in different categories continues all our life, unless there is a problem with this apparatus. In patients with Alzheimer type of dementia, the memory-making machine starts to malfunction, and the patient develops a difficulty with making new memories, or short-term memories. Depending upon the type of memory, some memories may still be made while many others may be missed or lost. In later stages of the disease, patient may also start losing already made or old memories, or the long-term memories. Type of symptoms in dementia depends upon the area of brain involved, which may also depend upon the cause of dementia.

 

 

What is the difference between dementia and Alzheimer?

 

This probably is the most common question asked by patients or their families. Dementia is defined above; Alzheimer disease is one of its reasons or types, and there are many other reasons for dementia. It is like asking what is the difference between headache and migraine? Migraine is the most common type of headache but there are many other reasons for having a headache.

 

 

Is there a test for dementia?

 

Dementia is mostly diagnosed by taking relevant history from the patient and the people around the patient (family members, friends, etc.) who may provide appropriate information. Neurological examination with special emphasis to mental functioning is also helpful.  Most patients can be diagnosed with this much information. Further testing may be required to figure the type of dementia.

 

If Alzheimer disease is suspected, further testing may be needed to make a more definitive diagnosis. This is done with help of a blood test, analysis of cerebrospinal fluid, or a special brain scan called PET.

 

 

What are different causes of dementia?

 

I put the concept of “causes” before “types” of dementia. I believe looking at it in this manner helps to understand this issue better. There are a variety of causes of dementia, just about anything that may damage brain or brain cells. Following are some common causes:

 

1. Degeneration of brain cells: It is the most common cause of dementia. Degeneration is a process destroying brain cells from some unknown or not well-defined trigger, which is not an infection, inflammation, trauma, or aging. In future, hopefully, we shall have better understanding of this process and then we shall describe it in more definitive terms. For now, a lot of dementias are lumped together as degenerative in origin. Classical example of dementia from a degenerative process is Alzheimer disease.

 

2. Problem with blood-supply of brain: This indirectly destroys brain cells. This is usually called Vascular Dementia. There is a variety of problem that may occur in blood vessels or vasculature of brain to cause dementia, most commonly ischemic stroke(s).

 

3. Traumatic brain injury: Especially in young people.

 

4. Infections: Such as Herpes, HIV, and syphilis.

 

5. Autoimmune process: Such as lupus or SLE, and brain vasculitis.

 

6. Drugs: Both drug of abuse and some prescription drugs. Probably the most common drug to cause dementia is alcohol.

 

7. Cancer: Both tumors of the brain, and from other areas of the body (metastatic tumors).

 

 

What are different types of dementia?

 

The term “type of dementia” is somewhat confusing, and a better way to look at this issue is to talk about cause of dementia. In any case, because this term of “type of dementia” is commonly used, it is good to know what it implies. Following are common types of dementia in USA:

 

1. Alzheimer dementia, typically involving parietal and temporal lobes of brain, at least initially.
2. Vascular dementia, from stroke(s).
3. Multifactorial dementia (a vague term, the most common combination is Alzheimer with Vascular dementias).
4. Frontal lobe dementia that predominately involve frontal lobes of brain.
5. Lewy-Body dementia.
6. Dementia with behavioral disorder, again a vague term as most dementias may have behavioral symptoms.
7. Dementia and Parkinsonism complex, also a vague term because many dementia types have Parkinson type symptoms.
8. Primary Progressive Aphasia
9. Huntington dementia.
10. Dementia associated with psychiatric illness
11. Traumatic brain injury
12. Secondary dementia from an infection or an inflammatory process

 

 

What are common types of Degenerative Dementia?

 

Following are common degenerative dementias:

 

1. Alzheimer
2. LATE (limbic-predominant age-related TDP-43 encephalopathy)
3. Frontal
4. Lewy-Body
5. Primary Progressive Aphasia
6. Huntington
7. Dementia-parkinsonism (not all cases)

 

 

What are common types of Vascular Dementias?

 

Vascular dementia is caused by stroke(s). Even a single relatively small stroke can knock out an area of the brain that manages our language resulting in dementia. Following list helps to understand its types:

 

1. Embolic cerebral infarction(s)
2. Atherosclerotic cerebral infarction(s)
3. Micro vascular ischemic disease
4. CADASIL and CARASIL
5. Intra-cerebral hemorrhage
6. Intracranial hemorrhage

 

 

What are different types of Frontal-lobe Dementias?

 

1. Frontotemporal
2. Picks disease

 

The topic of frontal lobe dementia is much more complicated based upon location of frontal lobe dysfunction. Patients with frontal lobe dementia have more of a behavioral problem (change in personality) than forgetfulness.

 

 

What is Dementia-parkinsonism Complex?

 

Parkinson types of symptoms are common in dementias, but sometimes this term of Dementia-parkinsonism is used to allude to certain dementias, such as Parkinson patients with dementia, Normal Pressure Hydrocephalus, or Lewy Body Dementia. Some patients with vascular dementia may also have Parkinson type features.

 

 

What is Normal Pressure Hydrocephalus (NPH)?

 

This is a condition causing three types of problems: a. Difficulty walking, with somewhat Parkinson type features, b. Loss of control of urination, and c. Dementia. Brain imaging in NPH shows enlargement of central brain cavities (of note, we all have central brain cavity.) Its exact cause is unclear but seems to be a problem with absorption of spinal fluid and degeneration of central areas of brain. This is a type of dementia that is potentially fixable, at least to a certain extent.

 

 

What is Lewy-Body dementia (LBD)?

 

The term Lewy-Body implies certain findings in the brain (the so-called Lewy bodies) seen with a microscope. They are discovered when brain autopsy is performed for some difficult cases of dementia. Nowadays biopsy is rarely done. The diagnosis of LBD, though tentative and based upon set of symptoms, is not that difficult to make. Typically, this dementia presents with mental confusion or forgetfulness, difficulty with balance and walking or falls, and behavioral changes (delusions, paranoia, hallucinations.) Compared to a typical case of Alzheimer, patients with LBD have fluctuating symptoms; they progress faster, and are difficult to manage

 

How is alcohol related dementia different from Alzheimer?

 

Excessive and chronic alcohol drinking is a common cause of dementia. No amount of alcohol is safe for brain. It is a direct toxin to brain cells. Binge alcohol drinking may cause another distinct type of brain injury. Chances of alcohol induced brain injury increase if patient’s diet is not appropriate. Certain areas of brain are more susceptible to alcohol related injury than others, such as cerebellum, temporal, and frontal lobes. Alcohol may also contribute to indirect brain injury from trauma related to falling.

 

 

What is HIV dementia?

 

HIV disease, especially if not appropriately treated, causes destruction of brain cells, which after a certain amount of damage results in symptoms of dementia. The treatment of this dementia is the treatment of HIV disease.

 

 

What is prion disease?

 

Prion is tiny particles of protein and is different from bacteria or viruses. Their excessive accumulation in brain cells leads to their destruction or degeneration, and then dementia. It is a form of rapidly progressive dementia, killing the patient in few months to a year or two. Examples are Creutzfeld-Jacob disease, and the so-called Mad-Cow disease. Nowadays, a brain MRI can diagnose most cases. In doubtful cases, spinal fluid analysis can help. Brain biopsy, which used to be done for its diagnosis, is almost never done. It is not a contagious disease but direct exposure to the nervous tissue of the patient can transmit the disease.

 

 

How is Alzheimer dementia treated?

 

There are at least two drugs available that could help to slow down progression of Alzheimer disease. Both are biological drugs. These drugs are used in early stages of this disease. As there could be significant side effects, patients are carefully chosen to minimize risk. These drugs on hand can help to slow down the disease, they may rarely cause stroke or bleeding in brain. Other than this type of drugs, may others are available to treat symptoms of dementia.

 

Symptoms of Alzheimer may be divided in three categories:

 

1. Cognitive
2. Behavioral
3. Functional.

 

This type of division is helpful, especially in clinical trials when medicines are tested for dementia. Cognitive symptoms may include problems with memory, language, calculation, space orientation, or drawing. Behavioral problems may include anxiety, depression, paranoia, delusions, hallucinations, anger and agitation. In Functional category are activities a person undertakes to maintain normal healthy life, e.g., personal care and hygiene, space management, day-to-day work, driving, etc.

 

Different types of scales are used for each category to assess severity of symptoms, from mild, moderate to severe. Forgetfulness may be the initial symptom of Alzheimer, in later stages patients usually have problems in all these categories.

 

Before one consider a medicine for dementia, it is important to mitigate any ongoing factors or conditions that may negatively be impacting the patient. This include alcohol or drug use, including marijuana; sleep disorders, including obstructive sleep apnea; medicines that patient is already taking, including over-the-counter medicines, and psycho-social factors, including underlying mood disorder(s). Also, starting from early stage of the disease, the patient and the family are counseled to decide for appropriate proxy documents for health care needs, and Power of Attorney for any legal or fiscal matters.

 

Formal counseling or therapy may provide some help in early stages of dementia, especially in making appropriate decisions about employment, family, and financial matters. It is important to include at least one other person in this process, spouse, or any other person the patient may trust or feels comfortable with. In later stages of dementia, therapy or formal counseling has limited value, if any at all. Frequently, family member(s) needs counseling to help deal with the situation, avoid any conflict between the patient and the family member(s), and sometimes between the family member(s) and the treating physician. I routinely advise families to avoid any conflict-provoking situation within the family, which may have tremendous negative impact on patient’s wellbeing.

 

If needed, a larger group of family members may also meet the treating physician. Depending upon severity of dementia, this meeting can be with or without patient’s presence, but with consent of patient’s Healthcare Proxy, if it applies. This type of meeting may help mitigate any potential conflicts within the family, which may impact the patient. It also helps different family members to get first-hand information from the physician, and directly ask questions. It may not be practical to have this type of meeting on regular basis. I advise families to refer all further inquiries to the patient’s proxy. Finally, physicians do not act as attorneys, but they do have ethical and legal responsibilities to safeguard patient’s interests. In a rare situation, if family is unable to provide appropriate supervision and care, or if no family member or friend is in the picture, the physician may invoke the legal process of an alternate guardianship for the patient. 

 

Unfortunately, there is no organized manner of care for elderly or patients with dementia. This creates another layer of challenge both for the treating physician(s) and the patient’s caretaker(s). Physicians do not have much control over providing needed ancillary services, and the caretaker(s) is left arranging them privately, which a lot of them cannot afford. Some social service groups like Elder Care Services, local support group, or any similar organizations are helpful to fill some of these gaps.

 

 

What is the best place to reside for a patient with dementia?

 

The best place usually is one’s own home, with the spouse or other family members, especially if patient has been living there for a while. Moving a patient with dementia to an unfamiliar environment and away from familiar people may make symptoms worse. On the other hand, every situation is different and there may be some other important issues, which may compel a move. Probably the most common reason for moving to a new dwelling is lack of ground floor bedroom or bathroom. Other reasonable reasons are moving to a smaller easy to manage place, moving closer to children or other family members, or in some cases moving to an assisted living facility to avoid stress of managing a household.

 

 

How to manage anxiety in dementia?

 

Anxiety is a common occurrence in dementia. It does not need to be treated with a medicine. Counseling, therapy, and adjustment in personal life should be tried first, both for the patient and the caretaker. Anxiety provoking situations and meetings should be avoided or minimized. Any other factor contributing to anxiety, if possible, should be eliminated. This sometimes also includes treatment of caretaker’s anxiety disorder. If a medicine is needed, small dose of an SSRI (serotonin reuptake inhibitor), such as sertraline 25-50mg or escitalopram 5mg, may help. Benzodiazepines like lorazepam or alprazolam should be used very carefully, on as needed basis if at all, as they may worsen patient’s cognitive symptoms.

 

 

How to manage depression in dementia?

 

Not every dementia patient is depressed, but it is not an uncommon symptom in dementia. With depression, symptoms of dementia get worse, and its treatment may improve patient’s situation. Depression also results in added difficult situation for the caretaker, and with overall care of the patient. Formal counseling and therapy may be tried but has limited success. Some actions on part of the family may also help, such as more socialization, taking the patient out on regular basis, avoiding stress at home, taking care of issues that may be bothering the patient. If a medicine is needed, an SSRI type of medicine can help. This type of medicines is quite safe, not habit forming, and may provide significant relief. Any related issues should also be addressed, e.g., problem with sleeping.

 

 

How to manage sleep issues with dementia?

 

Sleep disturbance is a common symptom of dementia. Probably the most common sleep problem with dementia is disturbed sleep-awake cycle. Other common issues are insomnia, too much sleep, and sleep related behavioral disorders. Obstructive sleep apnea, if present should be appropriately treated.

 

Sleep changes for all of us, as we grow older. It gets more difficult to get into sleep, and we spend less time in deep sleep. Overall amount of sleep also declines with aging. Many of us develop conditions that impact our sleep, e.g., pain from multiple reasons, stress, anxiety and depression, drug addiction, and bladder issues. Some people have sleep disorders like snoring, obstructive sleep apnea, or restless leg syndrome. Let’s look at the sleep issues in dementia:

 

1. Disturbed sleep-awake cycle or Circadian rhythm sleep disorder: A patient with dementia may not have regular or “normal” sleep time. Typically, patient is up when others are trying to sleep. Patient may be sleeping during daytime. In normal elderly, it may be useful to take a scheduled brief nap in afternoon. Patients with dementia may start sleeping in morning hours and not able to relax in afternoon and evening. It is like their internal clock is not working.

 

My first recommendation to manage this condition is to leave it alone and, if it is not too disruptive for rest of the family, let patient sleep whenever desired. Treating it with a medicine may or may not work. One may try behavioral approach and coax the patient to take a brief nap during daytime and fulfill rest of the sleep at night. Exposure to bright light in the morning may also help. Attention should be paid to sleep hygiene and removing any other triggers affecting sleep, including prescription medicines.

 

1. b. Insomnia: It is a complex topic and commenting upon it in a few lines is oversimplification; with that said, any sleep hygiene issues should be adjusted before any medicine is considered for treatment. Small dose of over the counter or prescription melatonin can be tried, though it is seldom effective. Many medicines are available, and it is better to avoid benzodiazepines, especially the ones with long half-life. Small dose of mirtazapine may be tried.

 

1. Sleep behavior disorders: Some patients start shouting and kicking during sleep, like they are trying to defend themselves. This creates a physical risk for the patient, but also for the co-sleeper. One easy way to handle this situation is to let patient sleep alone in a room. But if the situation becomes risky or too bothersome, medicines are available that can help to control this problem.

 

1. Excessive sleep: This usually is a symptom in late-stage dementia. If it is occurring in early stages, a reason should be explored. Possible reasons are depression, a medicine, or a metabolic abnormality. In late-stage dementia, provided there is no other factor such as infection or metabolic abnormality, it does not necessarily need to be treated.

 

1. Extreme sleep rhythm: In this disorder, usually in late stages of dementia, the patient may not sleep for a day or even days, sometimes staying agitated, and then goes into very deep sleep, to the point that caretaker(s) or even nurses and doctors get nervous and try to find a cause for “COMA,” while patient is just in deep sleep. If history is consistent, and all vitals and labs are ok and patient seems to be sleeping, my usual suggestion is to let the patient sleep. Usually, the patient wakes up after 8-24 hours of deep sleep.

 

 

What is the meaning of sundowning in dementia?

 

Many dementia patients seem to do better in morning hours and seem to get more symptoms in afternoon and the evening. This phenomenon is called sundowning. Its exact cause is unclear. It may be a sleep disorder, or a mood disorder. In any case, it is treated with behavioral modifications, such as daylight exposure in afternoon and avoiding darkness, or with some medicines. It may become a significant source of stress for the family.

 

 

What is Capgras syndrome?

 

It is a type of delusion, a false belief. Patient believes that the spouse, who usually is the caretaker, is an imposter. The patient may talk to a third person asking who this person (the spouse taking care of the patient) is? In a somewhat similar delusion, the patient may keep on asking or demanding to go home, while inside that home.

 

What is paranoia in dementia?

 

Paranoia is not an uncommon symptom in later stages of dementia, especially in certain types. It is an irrational fear or anxiety about a situation that may not exist or may not deserve to be fearful about. Patient may see some car flashing outside the window, and may start locking the doors, and covering windows with a worry that other people were using lights to spy on. Or the patient may start calling the police to taker care of the matter. It may take multiple forms and may be a significant source of stress for the caretaker, or a hindrance to the patient’s appropriate care.

 

 

What is the shouting behavior in dementia?

 

Like a child, a patient with severe dementia patient may sometimes start shouting, apparently for no obvious reason. There usually is an emotional or physical trigger. The priority should be making sure there is no physical issue harming the patient. Any emotional situation shall also be diffused. The patient may calm down by simply a compassionate touch or a company.

 

 

What is pacing in dementia?

 

Some dementia patients, in restless fashion, may keep on walking back and forth, known as pacing. They may keep up with this activity until exhausted.

 

 

How to manage psychotic symptoms in dementia?

 

Psychotic symptoms include delusions or false beliefs (e.g., the neighbor is spying on me), visual hallucinations or false visual perception (e.g., seeing people, insects or animals), auditory hallucinations or false hearing perception (e.g., hearing music, noises, or people talking), paranoia (excessive worry and doubt), anger and agitation. Some of these symptoms are common in severe dementia, but in some dementias, they may appear in early stages.

 

If no other plausible reason for these symptoms is found, and the patient is not much affected by them, they do not need to be treated with a medicine. At the same time, it is important to educate the family about the diagnosis and these symptoms. They should avoid an argument with the patient; arguing may create anxiety or distrust and may make the situation worse. I usually tell families to deal with the situation diplomatically; a good diplomat never says no, even when he is saying it. Avoid the urge to negate the patient. If the patient says that there are small animals in the house (visual hallucinations) or there is music next door (auditory hallucinations), and the patient seems not be stressed about it, avoid an argument. Arguing can easily create a stressful situation, both for the patient and the family member.

 

If psychotic symptoms cross a line, by that I mean they are affecting patient’s or the caretaker’s quality of life, or creating an unsafe situation, medicines are available that can help control these symptoms. The first line of treatment is the typical dementia medicines like acetylcholine-esterase inhibitors or memantine, or both together.  One may also try mood stabilizers like valproic acid or gabapentin, depending upon the nature of symptoms. These meds may work for mild symptoms but for overtly psychotic symptoms, none of them make a difference, and the patient requires an anti-psychotic drug.

 

There is a lot of literature about the risk of anti-psychotic medicines, and one should take all commonsense and necessary precautions to avoid any of their complications. With use of anti-psychotic drugs, there is always risk of dizziness, sedation, confusion, agitation, Parkinson-like symptoms, or even death. At the same time, there is significant variety within the group of anti-psychotic medicines, with some quite risky and others relatively safe. Also, the toxic effects depend upon underlying medical conditions, especially heart conditions, and the dose-range; smaller doses have far fewer side effects. Once a decision is made to prescribe an antipsychotic, provided there is no obvious contraindication, a small dose of a relatively safe drug may be tried. It may be prescribed on as needed basis or regularly, depending upon patient’s condition. I usually try small dose of quetiapine or risperidone. Frequently, positive impact of this type of medicine is the difference between patient staying at home with the family or moving to an institution.

 

 

How to manage caretaker’s stress?

 

Dementia makes a human being mentally disable and dependent upon others. The responsibility of care usually falls on the spouse, or one of the siblings. Every patient is different, and some require almost constant care, or the company. This is not an easy task. The situation may get worse if patient’s behavior impacts caretaker’s sleep, mood, and sometimes safety. Many times, it becomes a test of caretaker’s love and commitment for the patient. Other family members should try to provide or arrange some respite. If affordable, help may be arranged to care of patient’s basic needs (cleaning, showering, etc.), and to provide some free personal time to the caretaker. Any conflict within the family should be addressed separately, not in front of the patient. Caretaker should inform his/her medical doctor about this responsibility, so that appropriate support and care be provided to the caretaker, if needed. Many caretakers require treatment for anxiety, depression, and insomnia. Also, family member(s) who are not directly involved in the care should avoid any unnecessary arguments with the caretaker, it may be counter productive if their intention was welfare of the patient.

 

 

Where can I get more information about dementia?

 

American Academy of Neurology, AAN

Alzheimer Association