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Early

Onset

Dementia

Alberta

Foundation

Types of Dementia

Mild Cognitive Impairment (MCI)

MCI is a condition with cognitive changes between normal aging and dementia. In MCI, memory problems may be minimal to mild.

Etiology

The etiology of MCI is varied and can be associated with metabolic and endocrine factors (hormones), chronic kidney disease and decreased Testosterone levels. Testosterone substitution is useful if a low testosterone level is present but general screening for testosterone deficiency is not yet recommended. Although an association between vitamin B12 deficiency and MCI is present, substitution of vitamin B12 or folate does not appear to prevent cognitive decline.

Symptoms of MCI

• Difficulty performing more than one task at a time

• Difficulty solving complex problems or making decisions

• Forgetting recent events or conversations

• Taking longer to perform more difficult mental activities

A diagnosis of MCI means you're able to perform your usual daily activities with minimal change and little difficulty. Writing reminders and practicing specific strategies can help a person compensate for these mild changes. When this is no longer the case, dementia may be the appropriate term to describe the condition.

Memory loss generally occurs in dementia, but memory loss alone doesn't mean dementia. Dementia implies there are problems with other brain functions as well, and that more than one dementia symptom is present.

Symptoms of dementia

Dementia symptoms can be due to many things such as an underactive thyroid, low cortisol’s, vitamin deficiency, brain tumors and depression. Even certain medications can cause dementia symptoms however, once treated the symptoms can be reversed. If during a medical evaluation these reversible causes of dementia are ruled out, the probable cause of dementia symptoms may be due to Alzheimer's. Some forms of MCI are regarded as potential preclinical forms of dementia. MCI may increase your risk of later progressing to dementia, caused by Alzheimer's disease or other neurological conditions. But some people with mild cognitive impairment never get worse, and a few eventually get better.

The causes of dementia can vary, depending on the types of brain changes that may be taking place

References:

Mayo Clinic.Org

http://www.ncbi.nlm.nih.gov/pubmed/20109582

Prepared by: Jody McCoppen - October 2016

The Seven A's of dementia

One way of understanding how dementia affects the brain is to look at the seven A's of dementia. Each A represents damage to a particular part of the brain. Please keep in mind that someone with dementia may not experience all of the A's.

Anosognosia: means that you can no longer recognize that something has changed and that there is something wrong. You might not understand why you have cognitive problems or that you are experiencing any problems at all. Because the part of your brain that helps you reason is damaged, you do not see the changes in your abilities that others may see.

Agnosia: means you can no longer recognize things through your senses: sight, sound, taste, touch, and smell. You might not be able to sort out what you see or hear. You might have trouble recognizing familiar people. Your safety may be at risk if this part of the brain is affected because you might confuse objects and what they are used for.

Aphasia: means you lose the ability to use language. This includes the ability to speak, understand, read and write. Although a person may retain the ability to speak for some time, the ability to understand what other people are saying may be affected early in the disease. If you cannot understand what is being said to you, this can lead to misunderstandings between you and those around you. You might find yourself withdrawing from social interactions because you are worried that you will not understand others, or that they may not understand you.

Apraxia: means you have lost the ability to tell your body how to carry out purposeful movement. As well, if you have apraxia, you may also have trouble understanding terms such as back, front, up, down. When this happens, it becomes difficult to do things such as tying shoelaces, doing up buttons and zippers, and any activity involving co-ordination. The ability to move your body according to a certain pattern, such as co-ordinating hand and leg movement, also affects your ability to do specific activities such as driving.

Altered perception: happens when you misinterpret the information your senses are giving you. For some people, this is a bigger problem in the late afternoon or early evening when light changes. Another important change is the loss of depth perception—the ability to see in three dimensions. It becomes harder to judge how high, deep, long, wide, near or far things are. For example, if the floor and furniture are the same colour, it may be difficult to judge when one is close enough to a chair to try to sit.

Amnesia: means loss of memory. This is an important loss because most things we do depend on our ability to remember. For example, a person with short-term memory problems loses the ability to remember what was just said. This explains why you might find yourself asking questions over and over again. Earlier in the disease a person's short-term memory will be affected. As the disease progresses, long-term memories will become harder to retrieve. Apathy is not being able to take initiative. The part of the brain that helps you start to do something, either to carry out an activity or to communicate, is damaged. You might find that you have difficulty beginning activities. You may need someone else to give you cues (hints) to keep you involved in a conversation or a task.

Apathy: is not being able to take initiative. The part of the brain that helps you start to do something, either to carry out an activity or to communicate, is damaged. You might find that you have difficulty beginning activities. You may need someone else to give you cues (hints) to keep you involved in a conversation or a task.

Alzheimer’s disease

There are 3 types of Alzheimer's

Late-onset Alzheimer's

This is the most common cause of dementia among adults over age 65. Symptoms become apparent in their mid-60s.

Early-onset Alzheimer's

This occurs in people under 65 and as early as age 30 to 60. It is a less common form of Alzheimer’s. People with this disorder face different issues, such as dealing with disability at work, raising children, and finding the right support groups.

Familial Alzheimer's disease

(FAD) is a form of Alzheimer's disease that doctors know for certain is linked to genes. In families that are affected, members of at least two generations have had the disease. FAD makes up less than 1% of all cases of Alzheimer's. Most people who have early onset Alzheimer's have FAD.

Etiology

Most people with Alzheimer’s have the apolipoprotein E (APOE) gene which is involved in late-onset Alzheimer’s. This gene has several forms. One of them, APOE ε4, increases a person’s risk of developing the disease and is also associated with Early Onset Alzheimer’s. However, carrying the APOE ε4 form of the gene does not mean that a person will definitely develop Alzheimer’s disease, and some people with no gene will develop Alzheimer’s.

Alzheimer’s disease is the most common cause of dementia among older adults. Alzheimer's is a type of dementia that causes problems with memory, thinking and behavior. The brain has 100 billion nerve cells called neurons. These nerve cells connect with many others to form communication networks. Groups of nerve cells have special jobs. Some are involved in thinking, learning and remembering. Others help us see, hear and smell. These nerve cells process and store information and communicate with other cells. Keeping everything running requires coordination as well as large amounts of fuel and oxygen. As damage spreads, cells lose their ability to do their jobs and eventually die, causing irreversible changes in the brain.

Two abnormal structures called plaques and tangles are prime suspects in damaging and killing nerve cells. These plaques and tangles in the brain are considered some of the main features of Alzheimer’s disease. Another feature is the loss of connections between nerve cells (neurons) in the brain. Neurons transmit messages between different parts of the brain, and from the brain to muscles and organs in the body.

Plaques are deposits of a protein fragment called beta-amyloid that builds up in the spaces between nerve cells.

Tangles are twisted fibers of another protein called tau. Tau builds up inside the cells.

It is unknown exactly what role plaques and tangles play in Alzheimer's disease. Most experts believe they somehow play a critical role in blocking communication among nerve cells and disrupting processes that cells need to survive. Advances in brain imaging techniques allow researchers to see the development and spread of abnormal amyloid and tau proteins in the living brain, as well as changes in brain structure and function. Scientists are also exploring the very earliest steps in the disease process by studying changes in the brain and body fluids that can be detected years before Alzheimer’s symptoms appear. Findings from these studies will help in understanding the causes of Alzheimer's and make diagnosis easier.

Though most people develop some plaques and tangles as they age, those with Alzheimer's tend to develop far more. They also tend to develop them in a predictable pattern, beginning in areas important for memory, before spreading to other regions. It's the destruction and death of nerve cells that causes memory failure, personality changes, problems carrying out daily activities and other symptoms of Alzheimer's disease.

The damage to the brain starts a decade or more before memory and other cognitive problems appear. During this stage, people seem to be symptom-free, but toxic changes are taking place in the brain as abnormal deposits of proteins form amyloid plaques and tau tangles throughout the brain. The once-healthy neurons stop functioning, lose connections with other neurons, and die. Symptoms usually develop slowly and get worse over time, becoming severe enough to interfere with daily tasks. The damage initially appears to take place in the area of the brain essentially where memories are formed. As more neurons die, additional parts of the brain are affected, and they begin to shrink. By the final stage of Alzheimer’s, damage is widespread, and brain tissue has shrunk significantly.

Dementia ranges in severity from the mildest stage, when it is just beginning to affect a person’s functioning, to the most severe stage, when the person must depend completely on others for basic activities of daily living.

Signs and Symptoms

Memory problems are typically one of the first signs of cognitive impairment related to Alzheimer’s disease. Some people with memory problems have a condition called Mild Cognitive Impairment (MCI). In MCI, people have more memory problems than normal for their age, but their symptoms do not interfere with their everyday lives. Movement difficulties and problems with the sense of smell have also been linked to MCI. Older people with MCI are at greater risk for developing Alzheimer's, but not all of them do. Some may even go back to normal cognition.

The first symptoms of Alzheimer's vary from person to person. For many, decline in non-memory aspects of cognition, such as word-finding, vision/spatial issues, and impaired reasoning or judgment, may signal the very early stages of Alzheimer’s disease.

Researchers are studying biological signs of disease found in brain images, cerebrospinal fluid, and blood to see if they can detect early changes in the brains of people with MCI and in cognitively normal people who may be at greater risk for Alzheimer’s. Studies indicate that such early detection may be possible, but more research is needed before these techniques can be relied upon to diagnose Alzheimer's disease in everyday medical practice.

Scientists are conducting studies to learn more about plaques, tangles, and other biological features of Alzheimer’s disease. Advances in brain imaging techniques allow researchers to see the development and spread of abnormal amyloid and tau proteins in the living brain, as well as changes in brain structure and function. Scientists are also exploring the very earliest steps in the disease process by studying changes in the brain and body fluids that can be detected years before Alzheimer’s symptoms appear. Findings from these studies will help in understanding the causes of Alzheimer's and make diagnosis easier.

Stages of Alzheimer’s disease

Stage 1: No Impairment

During this stage, Alzheimer’s disease is not detectable and no memory problems or other symptoms of dementia are evident. A person may function independently and still drive, work and be part of social activities.

Stage 2: Very Mild Decline

A person may notice minor memory problems or lose things around the house, although not to the point where the memory loss can easily be distinguished from normal age related memory loss. The person will still do well on memory tests and the disease is unlikely to be detected by physicians or loved ones.

Stage 3: Mild Decline

At this stage, the friends and family members may begin to notice memory and cognitive problems. Performance on memory and cognitive tests are affected and physicians will be able to detect impaired cognitive function.

Stage 4: Moderate Decline

In stage four clear cut symptoms of Alzheimer’s disease are apparent. Patients with stage four Alzheimer’s disease:

Moderate Alzheimer's is typically the longest stage and can last for many years. As the disease progresses, the person with Alzheimer's will require a greater level of care.

Stage 5: Moderately Severe Decline

During the fifth stage of Alzheimer’s, patients begin to need help with many day to day activities.

People in stage five of the disease may experience:

Stage 6: Severe Decline

A person needs constant supervision and frequently requires professional care.

Symptoms include:

Stages 7: Very Severe Decline

Stage seven is the final stage of Alzheimer’s disease. Because Alzheimer’s disease is a terminal illness, patients in stage seven are nearing death.

References:

www.alz.org

www.Alzheimer.ca

http://www.webmd.com/

NIH National institute on aging

Medifocus guide

Prepared by: Jody McCoppen - October 2016

Vascular Dementia

Vascular Dementia is caused when vessels that supply blood to the brain become blocked or narrowed. Blood is delivered to the brain through a network of vessels called the vascular system. If the vascular system within the brain becomes damaged - so that the blood vessels leak or become blocked, then blood is unable to supply the brain cells with the amounts of nutrition and oxygen it needs. These brain cells will eventually die and the brain will not be able to perform thought processes effectively. The death of brain cells can cause problems with memory, thinking or reasoning, planning and judgement. Together these elements are known as cognition. When these cognitive problems are bad enough to have a significant impact on daily life, it is referred to a vascular dementia.

Vascular Dementia is the second most common type of dementia.

Etiology

Common conditions that may lead to vascular dementia

Stroke (infarction). If the blood supply in the artery of the brain is blocked for more than a few minutes, the stroke will lead to the death of a small area of tissue in the brain. This area is known as an infarct. Strokes that block a brain artery, usually cause a range of symptoms that may include vascular dementia.Whether a stroke affects your thinking and reasoning, depends on your stroke's severity and location. Some strokes don't cause any noticeable symptoms. These "silent brain infarctions" still increase dementia risk.With both silent and apparent strokes, the risk of vascular dementia increases with the number of infarctions that occur over time. One type of vascular dementia involving many strokes is called multi-infarct dementia.

Narrowed or chronically damaged brain blood vessels. Conditions that narrow or inflict long-term damage on your brains blood vessels also can lead to vascular dementia. The risk factors for vascular dementia are the same as those for heart disease and stroke.

Subcortical dementia Subcortical Vascular dementia is caused by diseases of the very small blood vessels that lie deep in the brain. These small vessels develop thick walls and become stiff and twisted, reducing the flow of blood through them. Small vessel disease often damages the bundles of nerve fibers that carry signals around the brain, known as white matter. It can also cause small infarcts near the base of the brain. Small vessel disease develops much deeper in the brain than the damage caused by many strokes. This means many of the symptoms of subcortical vascular dementia are different from those of stroke-related dementia.

Subcortical dementia is thought to be the most common type of vascular dementia.

These conditions include the wear and tear associated with aging:

Prevention

Early detection and accurate diagnosis are important, as vascular dementia is at least partially preventable. Ischemic changes in the brain are irreversible, however the patient with vascular dementia can demonstrate periods of stability or even mild improvement. Since stroke is an essential part of vascular dementia, the goal is to prevent new strokes.

Symptoms

Vascular dementia symptoms vary, depending on the part of the brain where blood flow is impaired. Symptoms often overlap with those of other types of dementia, especially Alzheimer's disease. Some people with vascular dementia have the presence of brain-related changes such as tau or plaque buildup which can affect the speed of cognitive decline. Vascular dementia can also develop very gradually, just like Alzheimer's disease. Memory loss is common in the early stages of Alzheimer's, but is not usually the main early symptom of vascular dementia. If vascular dementia and Alzheimer's occur together it is called Mixed Dementia.

Vascular dementia symptoms sometimes occur after a series of strokes or mini strokes. If this occurs, the thought processes occur in noticeable steps downward from previously. If Vascular Dementia is suspected, the doctor will often order scans of the brain in order to check for blockages or narrowing of blood vessels in the brain.

A scan such as CT (computerized tomography) or MRI (magnetic resonance imaging) may rule out a tumor or build-up of fluid inside the brain. These can have symptoms similar to those of vascular dementia. A CT scan may also show a stroke or an MRI scan may show changes such as infarcts or damage to the white matter. If this is the case, the brain scan will be very helpful in diagnosing the dementia type, rather than simply ruling out other causes.

Vascular dementia symptoms include:

Prognosis

Unlike Alzheimer's disease which weakens the patient causing them to succumb to bacterial infections like pneumonia, vascular dementia can be a direct cause of death due to the possibility of a fatal interruption in the brain's blood supply.

It does not always have a typical progression that might be classified into stages, although its symptoms can generally be classified as fitting in the early stages, middle stages or late stages of dementia.

Progression depends on the location and extent of the damage in the brain's blood flow. Some people with vascular dementia progress gradually- mimicking Alzheimer's. Others will experience a decline in cognitive abilities, followed by a period of stability, and then there will be another step down in abilities and then stability for a time, etc. This is often referred to as a "step-like progression" or "stepwise" pattern of progression.

Sometimes, the sudden step-like declines are related to clear vascular events, such as the occurrence of a stroke. In other cases, there's a less clear trigger for the decline.

Researchers think there are some genetic factors behind the common types of vascular dementia, and that these are linked to the underlying cardiovascular diseases. Someone with a family history of stroke, heart disease or diabetes has an increased risk of developing these conditions. Overall, however, the role of genes in the common types of vascular dementia is small.

People from certain ethnic groups are more likely to develop cardiovascular disease and vascular dementia than others. Those from an Indian, Bangladeshi, Pakistani or Sri Lankan background living in the UK have significantly higher rates of stroke, diabetes and heart disease than white Europeans. Among people of African-Caribbean descent, the risk of diabetes and stroke - but not heart disease - is also higher. These differences are thought to be partly inherited but mainly due to lifestyle factors such as diet, smoking and exercise.

There is currently no cure for vascular dementia: the brain damage that causes it cannot be reversed. However, there is a lot that can be done to enable someone to live well with the condition. This will involve drug and non-drug treatment, support and activities. If the underlying cardiovascular diseases that have caused vascular dementia can be controlled, it may be possible to slow down the progression of the dementia.

Stages of Vascular Dementia

Stage 1: No Cognitive Impairment

During this stage, Vascular Dementia is not detectable and no memory problems or other symptoms of dementia are evident.

Stage 2: Very Mild Cognitive Impairment

A person may notice minor memory problems or lose things around the house, although not to the point where the memory loss can easily be distinguished from normal age related memory loss. The person will still do well on memory tests and the disease is unlikely to be detected by physicians or loved ones.

Stage 3: Mild Cognitive Decline ( Mild Cognitive Impairment)

During this stage one might see clear-cut deficits

Stage 4: Moderate Cognitive Decline (Mild Dementia)

Clear –cut deficit on careful clinical interview

Stage 5: Moderately Severe Cognitive Decline (Moderate dementia)

Stage 6: Severe Cognitive Decline (Moderately Severe Dementia)

Stage 7: VERY SEVERE COGNITIVE DECLINE (Severe Dementia)

The last stage of vascular dementia

Although dementia is usually progressive, such as in cases of Huntingon's disease and Alzheimer's disease, there are some cases in which it is reversible. Additionally, the signs of dementia sometimes result from nutritional deficiencies, brain bleeding, infections and certain medications. In these cases, addressing the underlying cause may prevent the dementia from worsening.

References:

www.alz.org

http://www.7dementiastages.com/

Prepared by: Jody McCoppen - October 2016

Mixed Cell Dementia

Mixed dementia is a condition in which abnormal characteristics of more than one type of dementia occurs simultaneously in the brain.

Symptoms

Mixed dementia symptoms may vary, depending on the types of brain changes involved and the brain regions affected. In many cases, symptoms may follow a pattern similar to or even indistinguishable from those of Alzheimer's or vascular dementia or a combination of the two. In other cases, a person's symptoms may suggest that more than one type of dementia is present.

Some experts recommend suspecting mixed dementia whenever a person has evidence of both cardiovascular disease and dementia symptoms that get worse slowly.

Diagnosis

A diagnosis of mixed dementia infrequently comes during life. It usually comes after an autopsy of the brain has been performed. Most individuals whose autopsies show they had mixed dementia were diagnosed with one specific type of dementia during life, most commonly with Alzheimer's disease.

Types of Mixed Dementia

Alzheimer's/Vascular Dementia - In the most common form of mixed dementia, the abnormal protein that consists of plaque and tangles seen in Alzheimer's disease coexist with previously undetected blood clots or other evidence of vascular disease.

Alzheimer’s/Lewy Body Dementia - In the second most common form of mixed dementia, Alzheimer brain changes also often coexist with the abnormal protein deposits characteristic of Lewy Bodies seen in Lewy body and Parkinson’s disease dementia.

Dementia – Multifactorial - In some cases, a person may have brain changes linked to Alzheimer’s disease, vascular dementia and dementia with Lewy Body.

Treatment and Outcomes

Because most people with mixed dementia are diagnosed with a single type of dementia, physicians often base their prescribing decisions on the type of dementia that's been diagnosed. Physicians who think that Alzheimer's disease is among the conditions contributing to a person's dementia may consider prescribing the drugs that are approved for Alzheimer's.

Many researchers are convinced that growing understanding of mixed dementia, coupled with recognition that vascular changes are the most common coexisting brain change, may create an opportunity to reduce the number of people who develop dementia. Controlling overall risk factors for diseases of the heart and blood vessels may also protect the brain from vascular changes.

References

www.alz.org

www.alzheimer.ca/Mixed-dementia

www.WebMD.com

www.Mayoclinic.org

Prepared by: Jody McCoppen - October 2016

Frontotemporal Dementia

FTD is also commonly referred to as frontotemporal dementia, frontotemporal lobar degeneration (FTLD), or Picks disease.

FTD is an umbrella term for a group of uncommon disorders caused by progressive nerve cell loss in the brain's frontal lobes (the areas behind the forehead) or its temporal lobes (the regions behind the ears).

The nerve cell damage causes the brain to shrink and degenerate leading to loss of function in these brain regions. This causes deterioration in behavior and personality, language disturbances, or alterations in muscle or motor functions.

Frontotemporal dementia was once known as Pick's disease, but now that terminology is reserved for the subtype that actually features distinct symptoms affecting a person’s ability to use and understand spoken, written, and even signed language. Picks disease is only one of several types of frontotemporal dementia.

Etiology

In FTD, the frontal and temporal lobes of the brain shrink. A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia.

The two most prominent diseases that cause frontotemporal degenerations are:

1) A group of brain disorders involving the accumulation of microscopic abnormal tau, (protein- filled structures) that develops within brain cells (Pick bodies).

2) A group of brain disorders involving the protein called TDP43. For reasons that are not yet known, these two groups have a preference for the frontal and temporal lobes that cause dementia. These dementias are inherited in about a third of all cases, however more than half the people who develop frontotemporal dementia have no family history of dementia.

PartsBrain

Signs, symptoms and progression vary, depending upon the portion of the brain affected. Different types of symptoms develop, depending on which part of the frontal or the temporal lobe is affected. It affects the areas generally associated with personality, behaviour and language. Some people with FTD undergo dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use language.

Symptoms

FTD is categorized according to their symptoms. There are several types of frontotemporal dementia. Identifying precisely which diseases fall into the category of frontotemporal dementia presents a particular challenge to scientists. The signs and symptoms may vary greatly from one individual to the next and progressively worsen with time, almost always over years, eventually requiring 24-hour care. People may have more than one type of symptom, particularly as the dementia progresses.

There are 3 Subtypes of FTD:

Behavior Variant Frontotemporal Dementia (bvFTD)

This condition being the major form of FTD, is characterized by extreme changes in behaviour, personality, and conduct. It often occurs in people in their 50s and 60s, but can develop as early as their 20s or as late as their 80s. Individuals typically lack insight into such changes.

In bvFTD, the nerve cell loss is most prominent in areas that control:

Behavior may become impulsive and compulsive. They may repeat the same action over and over. They may walk to the same location every day. They may compulsively pick up and manipulate random objects and put objects in their mouth. They may suck or smack their lips. They may overeat or eat only one type of food.

Primary progressive aphasia (PPA)

This is the second major form of FTD and is marked by the impairment or loss of speech and language difficulties, writing and comprehension. PPA normally comes on in midlife, before age 65, but can occur in late life also.

The two most distinctive forms of PPA have somewhat different symptoms

1) Semantic Variant of PPA - is characterized by an increasing difficulty in using and understanding written and spoken language. For example, people may have trouble finding the right word to use in speech or naming objects. As dementia progresses, people speak less and less or repeat what they or others say. Eventually, they stop speaking.

This group of FTDs includes the well-known Pick’s disease also referred to as Primary Progressive Aphasia, Semantic Dementia.

Pick disease is a brain disorder that causes slowly worsening decline of mental abilities. It gradually damages brain cells and impairs their function, disturbing cognitive processes, such as reasoning, problem solving, and memory.

The disease often affects a person’s ability to use and understand spoken, written, and even signed language. It also affects personality, emotions, and social behavior.

When the decline in mental abilities is severe enough to interfere with a person’s ability to carry out everyday activities, it is called dementia.

Pick's disease is only one of several types of frontotemporal dementia.

2)Nonfluent/agrammatic variant of PPA, a person’s speaking is very hesitant, labored or ungrammatical.

They may have

For some people, language problems are the only symptom for 10 or more years. For other people, other symptoms appear within a few years.

Disturbances of motor (movement or muscle) function

In Rarer subtypes of frontotemporal dementia, some people’s muscles are affected. They may become weak and waste away (atrophy). This is characterized by problems with movement, similar to those associated with Parkinson's disease or amyotrophic lateral sclerosis.

Movement-related signs and symptoms may include:

There are three disorders that are a part of the frontotemporal degeneration spectrum that produce changes in muscle or motor functions with or without behavior (bvFTD) or language (PPA) problems.

Amyotrophic lateral sclerosis (ALS) - causes muscle weakness or wasting. ALS is a motor neuron disease also known as Lou Gehrig’s disease.

Corticobasal syndrome - causes arms and legs to become uncoordinated or stiff.

Progressive supranuclear palsy (PSP) - causes muscle stiffness, difficulty walking and changes in posture. It also affects eye movements.

Diagnosis of dementia

Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease.

A Diagnosis of Dementia is based on the following:

Symptoms and history acquired by asking the person, family members or other caregivers.

Neuropsychological Testing

Sometimes doctors request a more extensive assessment of reasoning and memory skills. This type of testing, which can take several hours to complete, is especially helpful in trying to distinguish dementia from other conditions that can cause similar symptoms, such as age-associated memory impairment, mild cognitive impairment, and depression. If the testing proves to be dementia it can be useful to differentiate between the different types of dementia at an early stage.

Brain scans

By looking at images of the brain, doctors may be able to pinpoint any visible abnormalities such as: clots, bleeding or tumors that may be causing signs and symptoms. Brain imaging often shows damage to the anterior temporal and frontal lobes. If present, this atrophy may be diagnostic.

Three Types of Imaging of the brain:

1) Magnetic resonance imaging (MRI) An MRI machine uses radio waves and a strong magnetic field to produce detailed images of your brain.

2) Computerized tomography (CT) For a brain CT a computer uses the information to create cross-sectional images, or slices, of your brain. The test is painless and takes about 20 minutes.

3) Positron emission tomography (PET) PET scans use a small amount of low-dose radioactive material that's injected into a vein to help visualize brain metabolism, which can help identify abnormalities.

Distinguishing FTD from Alzheimer’s is the only indication for which healthcare may cover a PET scan for evaluation of dementia.

Treatment

There are no specific treatments for any of the frontotemporal subtypes. There are medications that aim to manage symptoms such as reduce agitation, irritability and/or depression, or compulsive behavioural problems. These treatments should be used to help improve quality of life.

Prognosis

The length of progression varies, from 2 to over 20 years with a mean course of 7-13 years from the onset of symptoms. FTD itself is not life-threatening. It does, however, predispose patients to serious complications such as pneumonia, infection, or injury from a fall. The most common cause of death is pneumonia.

FTD inevitably gets worse over time and the speed of decline differs from person to person. A person with FTD may show muscle weakness and coordination problems for many years, leaving them wheelchair or bedbound. These muscle issues can cause problems swallowing, chewing, moving and controlling bladder and/or lung infections.

Key Differences between FTD and Alzheimer's

Both bvFTD and PPA are far less common than Alzheimer’s disease in those over age 65 years. However, in the 45 to 65 age range, bvFTD and PPA are nearly as common as young onset Alzheimer's.

References

www.mayoclinic.org

www.Alzheimer's.org

http://www.theaftd.org/

Prepared by: Jody McCoppen - October 2016

Lewy Body Dementia (LBD)

LBD is a progressive cognitive deterioration characterized by cellular inclusions called Lewy bodies in the neurons. It is the second most common cause of progressive dementia, after Alzheimer’s disease.

Etiology

In the healthy brain, alpha-synuclein protein plays a number of important roles in neurons (nerve cells) in the brain, especially where brain cells communicate with each other. Lewy bodies are characterized by the abnormal deposits of this protein forming clumps or masses inside neurons, interrupting the brain's messages. This process causes neurons to work less effectively and, eventually, to die. The activities of brain chemicals important to brain function are also affected. The result is widespread damage to certain parts of the brain and a decline in the areas of the brain that involve thinking and movement. Why or how Lewy bodies form is unknown.

PartsBrain

Symptoms

Lewy body dementia signs and symptoms may include:

Diagnosis

Diagnosing LBD can be challenging for a number of reasons. Overlap of symptoms in Lewy body dementia and Parkinson disease dementia may complicate diagnosis. When motor deficits (ie: tremors, bradykinesia, rigidity) precede and are more severe than cognitive impairment, Parkinson disease dementia is usually diagnosed. When early cognitive impairment (particularly executive dysfunction) and behavioral disturbances predominate, Lewy body dementia is usually diagnosed.

With Early LBD there is a decline in thinking ability often confused with similar symptoms of Alzheimer’s disease. But over time they also develop movement and other distinctive symptoms that suggest dementia with Lewy bodies. LBD can occur by itself, or together with Alzheimer's disease or Parkinson's. It may have symptoms much like those of both Parkinson's disease and Alzheimer's disease.

People who have Lewy bodies in their brains also have the plaques and tangles associated with Alzheimer's disease. This dementia usually progresses quickly. Problems with memory may not be an early symptom, but can come up as Lewy body dementia progresses. Sensitivity to medication, especially some sedatives, may make these symptoms worse.

Types of Lewy Body Dementia

Lewy body dementia is used to describe all dementias whose primary cause is abnormal deposits of Lewy bodies in the brain.

This includes two types of dementia. The difference between them lies largely in the timing of cognitive (thinking) and movement symptoms.

Lewy body dementia often starts with movement changes such as tremors or muscle stiffness, seen in Parkinson’s disease. Cognitive symptoms are noted within a year of movement changes. An individual will start to have thinking and memory problems that are similar to Alzheimer’s disease, along with changes in behavior. A person also might have hallucinations.

Parkinson’s disease dementia - movement symptoms are pronounced in the early stages, with cognitive symptoms developing later.

Prognosis

LBD is a progressive disease, meaning symptoms start slowly and worsen over time. Some LBD symptoms may respond to treatment for a period of time. Currently, there is no cure for the disease. The disease lasts an average of 5 to 7 years from the time of diagnosis to death, but the time span can range from 2 to 20 years. How quickly symptoms develop and change varies greatly from person to person, depending on overall health, age, and severity of symptoms.

In the early stages of LBD, usually before a diagnosis is made, symptoms can be mild, and people can function fairly normally. As the disease advances, people with LBD require more and more help due to a decline in thinking and movement abilities.

References

Mayo Clinic - Lewy Body

National Institute on Aging

WebMD.com

merckmanuals.com/

Prepared by: Jody McCoppen - October 2016

Parkinson's Disease

PD is a progressive neurodegenerative disorder that devastates the nervous system, causing tremors and making it hard to move, walk and balance. The disease eventually affects people's voices, leaving them with only a whisper. Even swallowing becomes difficult. Parkinson’s changes a person's moods, thinking and concentration, and makes it hard to sleep. It is part of a group of conditions known as motor systems. It is the most common movement disorder and the second most common neurodegenerative disorder, the most common being Alzheimer's disease.

Etiology

Movement is normally controlled by dopamine, a chemical released by nerve cells to send signals to other nerve cells in the brain. This allows nerve impulses to travel smoothly from one cell to the other. When cells that normally produce dopamine die, dopamine is reduced. The messages from the brain to the nerve cells aren't properly transmitted, and the symptoms of Parkinson's appear resulting in the recognizable tremors at rest, rigidity and slowness of movement. The emotional, physical and social impact of Parkinson disease leaves people disconnected from the world, and often leads to depression.

There are three types of Parkinson's disease and they are grouped by age of onset:

Characteristics of Young Onset

Some of the challenges in Parkinson’s disease are the same regardless of age, but there are a number of issues specific to younger people.

Generally, Parkinson’s proceeds more slowly in younger people. While no two people are the same, a person whose onset age is 40 can expect to work for another 15-20 years on average. For a person with an onset age of 60, the average figure would be half that. These figures are based on the kinds of treatment available today. Future treatment will be even more effective in prolonging the productive life of people with Parkinson’s.

The following characteristics tend to be present in young onset Parkinson’s:

The main types of levodopa-related motor fluctuations include:

Motor fluctuations sometimes can be reduced or delayed by changing the schedule and amount of levodopa. Other medicines may be added to levodopa to help with motor fluctuations, such as dopamine agonists, COMT inhibitors, or MAO-B inhibitors. Increasingly, doctors are using dopamine agonists for initial treatment of Parkinson's disease, especially in younger people, to delay the development of motor fluctuations that eventually occur with long-term levodopa therapy.

The key issues for people with young onset Parkinson’s to consider are when to start using medication for symptom relief and what medication to use at the beginning of treatment. Everyone with Parkinson’s is unique and there is no single strategy that applies to everyone.

The decision to delay taking medication requires close monitoring. Each individual needs to consider whether he/she can do what is required at work and home by making changes to accommodate Parkinson’s, for example, using a computer instead of writing with a pen.

When to start taking medication can be decided in consultation with your neurologist or movement disorder specialist.

Since younger people experience an increased incidence of motor fluctuations, one outcome for treatment, apart from symptom relief, may be to minimize motor fluctuations.

Currently, there are no medications that can slow the progression of Parkinson’s.

It’s important to remember that there are many medications to treat Parkinson’s and more coming every year. A person with this disease can expect the medications to keep them functioning well for a very long time.

Apart from the physical challenges of the disease, people with young onset Parkinson’s face unique issues related to family, career, finances and living long-term with a potentially disabling condition. Talking with other people the same age, with the same condition, can be very helpful.

Employment

It is estimated that 25-35% of people diagnosed with Parkinson’s are still in the workforce. Some continue full or part-time work for many years. While the diagnosis of Parkinson’s doesn’t necessarily call for early retirement, it does require that you look at how you can best do your job and minimize work-related stress.

In looking at how a person does their job, consider the following:

The issue of when and what to tell your employer is very much a personal decision depending upon your condition and personality as well as your employment situation. In many provinces, it is a legal requirement for employers to accommodate a person with a disability.

Here are some things to consider:

Family Issues

A diagnosis of Parkinson’s disease affects other family members as well as the person with the disease - spouse, children, teenagers and older parents, will all have issues to deal with. The care partner of a young or middle-aged person with Parkinson’s faces particular challenges. It is important for couples to keep an open dialogue about the feelings and experiences of living with a person with Parkinson’s. Eventually the care partner may need to assume some of the tasks and roles in the family that the person with Parkinson’s had previously managed. This should be an on-going negotiation and requires constant dialogue around when to hold back help and when to offer assistance. Patience, understanding, stamina and creativity will be required from everyone.

Couples who manage best in the face of a chronic illness begin talking together from day one about how the disease is affecting daily operations in the family and what can be done to make things easier. Care partners who manage best are those who learn early to be flexible, state their own changing needs clearly and protect regular blocks of private time to meet those needs over the years. This is not selfish; it is crucial to the well-being of the person with Parkinson’s and the family that the primary caregiver does whatever it takes to maintain his or her own physical mental and emotional health.

Children and Teenagers

Young families have to deal with the unique issues of communicating the diagnosis and sharing the daily ups and downs of living with Parkinson’s with young children and teenagers.

Children will absorb the reality of Parkinson’s in the family by osmosis and by processing what they are told directly. Kids seem to sense a parent’s frustration with tremors or difficulty walking and are capable of both empathy and sympathetic offers of help. Attempting to hide a diagnosis from children may not be the best decision. They know instinctively when something is not quite right in a family.

Young children may need reassurance that their mom or dad is going to be okay. They may need to hear that both parents will be there for them, that Parkinson’s is not a fatal disease like some cancers, and that it is not contagious like chicken pox. Answers to specific questions on a need-to-know basis can help children cope with a parent who has special needs.

As far as teenagers, it is important to maintain the role as a parent, model or mentor and avoid confiding in your teenage child. Older teens may need reassurance that their lives will go on as planned and that mom or dad does not expect them to be a care partner. A mature teenager can get great satisfaction from helping with chores or chauffeuring tasks that may have once been the responsibility of the parent with Parkinson’s. Sometimes adolescents can benefit from professional counselling to help ease the struggle of having a parent who has physical challenges.

Older Parents

Older parents are often shocked to learn that their middle-aged (or younger) child is diagnosed with a disease that is usually associated with the elderly. Their concern may build if this is someone on whom they’re also dependent for emotional or financial support.

Symptoms

This type of Lewy Body Disease starts as a movement disorder, with symptoms such as:

Not all people with Parkinson’s develop dementia, and it is difficult to predict who will. Being diagnosed with Parkinson’s late in life is a risk factor for Parkinson’s disease dementia.

The major non-motor complications that are often associated with Parkinson's disease, including:

Diagnosis

A diagnosis of Parkinson's can take time. It is important to find a doctor who is knowledgeable about the disease, ideally a neurologist - a specialist who deals with Parkinson's or movement disorder specialist. Together a care plan is developed that will meet the needs of the person with Parkinson’s. There are no x-rays or tests to confirm Parkinson's, so the neurologist will check the medical history, do a careful physical examination and certain tests, and rule out other conditions which may resemble Parkinson's.

Treatment

Because currently there is no known cure for Parkinson's disease, understanding the standard treatments and options is critical so that you can make informed decisions about your treatment for better controlling the symptoms and preserving the patient's overall functional capacity and quality of life.

A person with Parkinson's can live for years.

Some treatment options include:

As symptoms change, medication may need to be taken more frequently or a combination of medications may be necessary to control symptoms.

While medications can alleviate the symptoms, they do not slow the progression of Parkinson’s.

Prognosis

PD is a chronic and progressive condition with symptoms worsening over time and new ones appearing. It will require ongoing monitoring and management to maintain one’s quality of life.

Since Parkinson’s is progressive, it is difficult to estimate how quickly or slowly the disease will progress in each person. It may progress more quickly in a person who is older when the symptoms first begin and less quickly when the main symptom is tremor, especially when it starts on one side. In most cases, one’s life is not shortened, however, as a person ages and as the disease progresses, there will be increased risks. For example, impaired balance can lead to falls; swallowing problems, if not managed, can lead to pneumonia.

Progression of Parkinson's disease

Although everyone is different, there are signs that Parkinson’s is progressing:

Cognitive Impairment in Parkinson's disease

Cognitive impairment is a common symptom in the late stages of Parkinson disease (PD). Even early on, patients with PD may have problems with decision making, organizing, planning and multitasking. Dementia with Lewy Bodies shares the same cognitive problems as PD, but begins with cognitive impairment and develops problems with movement later in the course. Similar changes in the brain occur in both PD and Dementia with Lewy Bodies, and these two conditions are therefore managed similarly. Healthy living and medications can help people manage some of their symptom.

References:

National Institute of Neurological disorders and Stroke

merkmanuals.com

WebMD.com

Prepared by: Jody McCoppen - October 2016

Huntington's Disease

Etiology

Huntington's disease is a rare inherited disease that causes the progressive degeneration (breakdown) of the nerve cells called neurons. This occurs in motor regions of the brain as well as other areas. It is caused by an inherited defect in a single gene which is referred to as the HD gene. The defect causes the building blocks of DNA to reproduce more times than is normal, producing a protein called “huntingtin.” In people with HD, this protein gets cut into one short piece and one longer piece. The shorter pieces stick together to form a protein ball. It is not clear how the abnormal HD gene causes the disease, whether it is the breakage of the protein, the formation of protein balls, or some other process that leads to cell death. It is unknown why only certain parts of the brain are affected and die. As the brain cells die, the symptoms of the disease get progressively worse.

With the exception of genes on the sex chromosomes, a person inherits two copies of every gene - one copy from each parent. A parent with a defective Huntington gene could pass along the defective gene or the healthy one. Each child in the family, therefore, has a 50 percent chance of inheriting the gene that causes the genetic disorder. A person may pass on the HD gene to a child before knowing that they themselves have it. If a child does not inherit the HD gene, he or she will not develop the disease and generally cannot pass it to subsequent generations. There is a small risk that a person who has a parent with the HD gene but who did not inherit the gene themselves may pass a possibly harmful genetic sequence to her/his children. A person who inherits the HD gene will eventually develop the disease. Males and females have the same risk of inheriting the disease. Huntington’s occurs in all races.

There are 2 types of Huntington's disease

Symptoms of Huntington's disease

There are significant variations in symptoms, and not everyone will have all the symptoms to the same degree. Symptoms also vary with each stage of the disease. Which symptoms appear first varies greatly among affected people. During the course of the disease, some disorders appear to be more progressive or have a greater effect on functional ability.

Movement Disorders

Impairments in the voluntary movements may have a greater impact on a person's ability to work, perform daily activities, communicate and remain independent.

Cognitive disorders

Cognitive impairments often associated with Huntington's disease include:

Psychiatric disorders

The most common psychiatric disorder associated with Huntington's disease is depression. The depression appears to occur because of injury to the brain and subsequent changes in brain function.

Signs and symptoms of depression may include

Other common psychiatric disorders include

The onset and progression of Huntington's disease in younger people may be slightly different from that in adults

Symptoms of juvenile Huntington's disease

Problems that often present themselves early in the course of the disease include

Physical changes

There are 3 stages of Huntington’s disease - Early, Intermediate and Advanced.

Early Stages

Slight physical changes may also develop at this stage. There can be involuntary movements which may initially consist of “nervous” activity, fidgeting, a twitching of the hands or feet, or excessive restlessness. Individuals may also notice a little awkwardness, changes in handwriting, or difficulty with daily tasks such as driving. At this stage, people with Huntington’s can function quite well at work and at home.

Intermediate Stage

As the disease progresses, the symptoms become worse.

Advanced Stage

Common causes of death include

Diagnosis

If a person notices changes in their movements, emotional state or mental ability they should see a Physician. The signs and symptoms of Huntington's disease can be caused by a number of different conditions. Therefore, it's important to get a prompt, thorough diagnosis.

Huntington’s is usually diagnosed using a genetic test (Lab Test) coupled with a complete family medical history, neurological and psychological tests. Sometimes doctors use brain scans to see whether the specific parts of the brain are working properly, or genetic testing is completed to confirm the diagnosis.

Genetic Testing

Genetic testing for HD is available for a person who is at-risk for Huntington’s or who believes they have the symptoms. Many people at risk choose not to take the test. It is a personal decision and varies from person to person as there is still no treatment to prevent HD from developing if the gene is present. Others make the decision to be tested so they can make arrangements as far as careers, family planning, and other issues.

Brain imaging and function

A doctor may order brain-imaging tests for assessing the structure or function of the brain.

These images may reveal structural changes at particular sites in the brain affected by Huntington's disease, although these changes may not be apparent early in the course of the disease. These tests can also be used to rule out other conditions that may be causing symptoms.

Treatment

Medications are available to help manage the symptoms of Huntington's disease such as depression, anxiety, and involuntary movements, but there are no treatments to prevent the physical, mental and behavioral decline associated with the disease.

These drugs can have side effects, so not everyone with Huntington’s uses them.

Researchers are looking at surgical treatments, such as implanting fetal brain cells into the brains of a person with Huntington’s disease in the hope the cells will grow and take over the functions of the dead cells. They feel they are close to reliable treatments.

Prognosis

There is no known cure, but treatment with medicines may help control the involuntary movements and behavior changes. After the onset of Huntington's disease, a person's functional abilities gradually worsen over time. The rate of disease progression and duration varies. The time from disease onset to death is often about 15 to 25 years. Juvenile onset usually results in death within 10 years.

The clinical depression associated with Huntington's disease may increase the risk of suicide. Some research suggests that the greater risk of suicide occurs before a diagnosis is made and in middle stages of the disease when a person has begun to lose independence.

Being at risk of Huntington Disease (HD) can present a number of difficulties, but perhaps the basic one is learning to live with the knowledge realistically. If a person has a parent with HD then that person has a 50% chance of inheriting the disease as well. Some people experience a kind of seesaw effect; on some days they feel sure that they will get HD, and on others they are sure they will not.

Either extreme distorts reality. If a person is too pessimistic, then the person will live in continual dread and are unable to plan a career or rewarding relationship, or cultivate interests. A person may also be constantly looking for symptoms and the smallest incidents take on a different meaning.

Being too optimistic, on the other hand, may lead to denial and irresponsibility, such as not telling a partner and having children.

There are many options now available for a person living at risk of HD.

Become informed: There are many resources available.

References

www.huntingtonsociety.ca

National Institute of Neurological disorders and Stroke

WebMD

Prepared by: Jody McCoppen - October 2016

Multiple Sclerosis

A nerve is made up of hundreds or even thousands of microscopic nerve fibers often all individually wrapped in myelin. Myelin is a substance that surrounds, insulates and protects the nerves in the central nervous system (the optic nerves, brain and spinal cord). Nerves conduct messages to and from the brain by way of electrical impulses. Myelin is necessary for the transmission of electrical impulses through the nerve fibres.

MSPic

Etiology

Multiple Sclerosis (MS) is a chronic disease of the nervous system which affects young and middle‐aged adults. While it is most often diagnosed in young adults aged 15 to 40, younger children and older adults are also diagnosed with the disease. Canada has the highest rate of Multiple Sclerosis (MS) in the world. Repeated damage to parts of the nerves leads to progressive weakness and disability.

MS is an autoimmune disease in which the body's immune system's T cells attacks, damages and destroys its own myelin. By attacking myelin, the immune system causes inflammation and degeneration of the myelin that can lead to demyelination, (a disruption of the myelin) or stripping the myelin covering from the nerve. The immune system mistakenly attacks the myelin as it would a virus or other foreign infectious body. The damaged myelin forms scar tissue (sclerosis), causing a distortion or interruption in nerve impulses. Myelin is necessary for the transmission of nerve impulses through nerve fibres. If damage to myelin is slight, nerve impulses travel with minor interruptions. If damage is substantial and if scar tissue replaces the myelin, nerve impulses may be completely disrupted, and the nerve fibres can be damaged.

The electrical impulses travel more slowly along the nerves resulting in deterioration of vision, speech, loss of muscle coordination and balance.

Myelin Sheath

MSPic2

Although myelin is present in both the central nervous system (CNS) and peripheral nervous system (PNS) only the central nervous system is affected by MS. CNS myelin is produced by special cells called oligodendrocytes. PNS myelin is produced by Schwann cells. The two types of myelin are chemically different, but they both perform the same function, to promote efficient transmission of a nerve impulse along the axon.

The abnormal immune system response produces inflammation in the central nervous system.

This process:

Symptoms

MS affects a person in different ways. Although not everyone will develop cognitive problems, around 65 % of people with MS experience mild cognitive symptoms such as finding it hard to remember information or follow a conversation. Other symptoms such as depression and mood swings are also common. The term “dementia” is not generally used in association with MS, because the decline is not usually as severe as it is in other forms of dementia, such as Alzheimer’s disease. It is more common to describe the person as “experiencing cognitive difficulties”.

If the person with MS has repeated difficulties with memory in ways that did not happen before, it may be due to MS or to other causes, such as depression, infection or side effects of medication. These may cause temporary cognitive difficulties. It is important that the changes are addressed to rule out other causes that may be temporary. Coming to terms with the changes that cognition problems bring is often difficult and frightening. As a result, the person might experience depression and anxiety.

Diagnosis

MS is difficult to diagnose in the beginning. There are no symptoms, physical findings or laboratory tests that can by themselves, determine if a person has MS. The physician performs a variety of tests to evaluate mental, emotional and language functions, movement and coordination balance, vision, and the other four senses. In many instances, the person’s medical history and neurologic exam provide enough evidence to meet the diagnostic criteria.

Because MRI is particularly useful in detecting central nervous system demyelination, it is very helpful to establish the diagnosis of MS. However, a small percentage of people with clinically-definite MS do not initially show lesions on the MRI at the time of diagnosis. If repeat MRIs continue to show no lesions, the diagnosis of MS should be questioned.

MRI is particularly helpful in a person who has had a single demyelinating attack that is suggestive of MS, also called a clinically isolated syndrome (CIS). The number of lesions on an initial MRI of the brain or spinal cord can help the physician clinically diagnose a person with MS. It also helps to assess the person’s risk of developing a second attack in the future. Some of the treatments for MS have been shown to delay the occurrence of a second episode of demyelination in a person who has had only one.

The MRI can also be used to identify a second neurological event in a person who has no additional symptoms, thereby helping to confirm a diagnosis of MS as early as possible. Once a diagnosis of MS has been clearly established, no additional MRI scans are needed for diagnostic purposes. However, subsequent scans are important for tracking the progress of the disease and making treatment decisions. A neurologist may consider disease activity on MRI as well as a person's clinical symptoms and relapses in order to determine whether the current treatment is effective or a change in treatment needs to be considered.

The CSF of a person with MS usually contains:

1) Elevated levels of IgG antibodies, as well as

2) A specific group of proteins called oligoclonal bands.

3) Occasionally there are also certain proteins that are the breakdown products of myelin.

These findings indicate that the body is producing an abnormal immune response against itself within the central nervous system. An abnormal immune response in CSF is found in a number of other diseases, so the test is not specific for MS.

A small percentage of people with MS may not have these CSF abnormalities. Therefore, CSF analysis by itself cannot confirm or rule out a diagnosis of MS. It must be part of the total clinical picture that takes into account the findings of the person’s history and neurologic exam as well other diagnostic procedures.

EP testing is used to identify impaired transmission along the optic nerve pathways. This is a fairly common finding early in the disease of a person with MS, even if the person isn’t aware of any visual symptoms.

Although VEPs are used to help make a diagnosis of MS, other conditions can also produce abnormal results, so this test is not specific for MS. The information the tests provide needs to be considered along with other laboratory and clinical information before a diagnosis of MS can be made.

Prognosis

A physician may diagnose MS soon after the onset of the illness. In others, however, doctors may not be able to readily identify the cause of the symptoms, leading to years of uncertainty and multiple diagnoses. Currently there is no cure for MS. MS can range from relatively benign to somewhat disabling to devastating, as communication between the brain and other parts of the body is disrupted. MS can render a person unable to write, speak, or walk.

Treatment

Currently there is no cure for MS. A person with MS may do well with no therapy at all, especially since the medications have serious side effects and some cause significant risks. Multiple sclerosis (MS) is a chronic disease affecting young and middle‐aged adults. One of the most common and disabling symptoms of MS is fatigue. Different approaches have been used to try and improve this, including energy conservation, specialised fitness training and drug treatments.

Some of the medications have been shown to significantly reduce the frequency and severity of attacks in a person with relapsing forms of MS. When attacks do occur, they tend to be shorter and less severe. Some meds may slow the progression of physical disability.

Immunosuppressant treatment is used for the treatment of advanced or chronic MS. Physical therapy and exercise can help preserve remaining function, and patients may find that various aids such as foot braces, canes, and walkers can help a person remain independent and mobile. Avoiding excessive activity and avoiding heat are probably the most important measures a person can take to counter physiological fatigue. If psychological symptoms of fatigue such as depression or apathy are evident, antidepressant medications may help.Although improvement of optic symptoms usually occurs even without treatment, a short course of treatment may be helpful.

Epileptic seizures can occur in a person with multiple sclerosis but the percentage is relatively low. It can have serious consequences. Because the cause of epileptic seizures in a person with MS may be different from that in other forms of epilepsy, it is uncertain whether a person with MS should be treated differently.

References

National Institute of Neurological Disorders and Stroke

PubMed Health

MS Society

Prepared by: Jody McCoppen - October 2016

Creutzfeldt-Jakob Disease

Etiology

Creutzfeldt-Jakob disease (CJD) -is a rare, degenerative fatal brain disorder causing rapid progressive dementia usually within a few months. It usually occurs in a person without known risk factors. This condition might be due to an abnormal form of a protein. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and is often referred to as “mad cow” disease, scrapie which affects sheep and goats, mink encephalopathy and feline encephalopathy. Similar diseases have occurred in elk, deer, and exotic zoo animals.

Some researchers believe an unusual "slow virus" or another organism causes CJD. However, they have never been able to isolate a virus or other organism in people with the disease. Furthermore, the agent that causes CJD has several characteristics that are unusual for known organisms such as viruses and bacteria. It is difficult to kill, it does not appear to contain any genetic information in the form of nucleic acids (DNA or RNA), and it usually has a long incubation period before symptoms appear. In some cases, the incubation period may be as long as 50 years.

The leading scientific theory at this time maintains that CJD and the other TSEs are caused by a type of protein called a prion. Prion proteins occur in both a normal form, which is a harmless protein found in the body’s cells, and in an infectious form, which causes disease. Prions do not contain genetic information and do not require genes to reproduce themselves. The harmless and infectious forms of the prion protein have the same sequence of amino acids (the "building blocks" of proteins). The infectious form arises from a mutation or change in the gene that controls formation of the normal prion protein. Sporadic CJD may develop because some of a person’s normal prions spontaneously change into the infectious form of the protein and then alter the prions in other cells in a chain reaction. Once they appear, abnormal prion proteins aggregate, or clump together. It is believed that these protein aggregates, may lead to the neuron loss and other brain damage seen in CJD. However, they do not know exactly how this damage occurs.

CJD can be inherited or caused by exposure to diseased brain or nervous system tissue.

There are three major categories of CJD

Symptoms

Typically, onset of symptoms occurs about age 60, and about 90 percent of people with CJD die within 1 year. It is characterized by rapidly progressive dementia. Initially, the person experiences problems with muscular coordination, personality changes including impaired memory, judgment, thinking and impaired vision. A person with CJD also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms.

As the illness progresses, mental impairment becomes severe. A person with CJD often develops involuntary muscle jerks and they may go blind. Eventually the person’s ability to move and speak is lost and the person goes into a coma. Pneumonia and other infections often occur and can lead to death.

There are several known variants of CJD. (vCJD). These variants differ somewhat in the symptoms and course of the disease. New variant begins primarily with psychiatric symptoms. Onset is at a much younger age than the other types of CJD, and has a longer than usual duration from onset of symptoms to death.

Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia.

Diagnosis

There is currently no single diagnostic test for CJD. When a doctor suspects CJD, the first concern is to rule out treatable forms of dementia such as encephalitis (inflammation of the brain) or chronic meningitis.

The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient’s brain so that it can be examined by a neuropathologist. This procedure may be dangerous for the individual, and the operation does not always obtain tissue from the affected part of the brain. Because a correct diagnosis of CJD does not help the person, a brain biopsy is discouraged unless it is needed to rule out a treatable disorder.

In an autopsy, the whole brain is examined after death. Both brain biopsy and autopsy pose a small but definite risk to the person who handles the brain tissue. There is the risk of accidentally infecting themselves by self-inoculation. Special surgical and disinfection procedures can minimize this risk.

Scientists are working to develop laboratory tests for CJD which is much easier and safer than a brain biopsy. It uses a person’s cerebrospinal fluid and detects a protein marker that indicates degeneration of the neurons. This can help diagnose CJD in people who already show the clinical symptoms of the disease. The false positive rate is about 5 to 10 percent. Scientists are working to develop this test for use in commercial laboratories. They are also working to develop other tests for this disorder.

Treatment

There is no treatment that can cure or control CJD. Researchers have tested many drugs however so far none of these treatments has shown any consistent benefit in humans.

Current treatment for CJD is aimed at alleviating symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain if it occurs.

Prognosis

As the disease progresses, it profoundly affects the brain as well as the body. Dementia symptoms worsen. Most people eventually lapse into a coma. Heart failure, pneumonia, or other infections are generally the cause of death. Death usually occurs within a year.

Transmission

CJD cannot be transmitted through the air, touching or most other forms of casual contact. Spouses and other household members of sporadic CJD patients have no higher risk of contracting the disease than the general population. However, exposure to brain tissue and spinal cord fluid from an infected person should be avoided to prevent transmission of the disease through these materials.

In some cases, CJD has spread to other people from grafts of dura mater (a tissue that covers the brain), transplanted corneas, or implantation of inadequately sterilized electrodes in the brain.

The appearance of the new variant of CJD (nv-CJD or v-CJD) in several younger than average people in Great Britain and France has led to concern that BSE may be transmitted to humans through consumption of contaminated beef. Although laboratory tests have shown a strong similarity between the prions causing BSE and v-CJD, there is no direct proof to support this theory.

Many people are concerned that it may be possible to transmit CJD through blood and related blood products such as plasma. Some animal studies suggest that contaminated blood and related products may transmit the disease, although this has never been shown in humans. If there are infectious agents in these fluids, they are probably in very low concentrations. Scientists do not know how many abnormal prions a person must receive before contracting CJD so they do not know whether these fluids are potentially infectious or not. They do know that, even though millions of people receive blood transfusions each year, there are no reported cases of someone contracting CJD from a transfusion. Even among people with hemophilia, who sometimes receive blood plasma concentrated from thousands of donors, there are no reported cases of CJD. Even though there is no evidence that blood from a person with sporadic CJD is infectious, it is still not recommended that a person infected with CJD donate blood.

Studies have found that infectious prions from BSE and vCJD may accumulate in the person’s lymph nodes (which produce white blood cells), the spleen, and the tonsils. These findings suggest that blood transfusions from people with vCJD might transmit the disease.

How Can People Avoid Spreading the disease?

To reduce the already very low risk of CJD transmission from one person to another, a person should never donate blood, tissues, or organs if CJD is suspected or confirmed, or if the person is at increased risk because of a family history of the disease, a dura mater graft, or other factors.

Normal sterilization procedures such as cooking, washing, and boiling do not destroy prions. Care partners, healthcare workers, and undertakers should take the following precautions when they are working with a person with CJD:

References

National Institute of Neurological Disorders and Stroke

Mayo Clinic

Prepared by: Jody McCoppen - October 2016

Other Dementias

Head Trauma

Etiology

Traumatic brain injury (TBI), a form of acquired brain injury, occurs when a sudden trauma causes damage to the brain. TBI can result when the head suddenly and violently hits an object, or when an object pierces the skull and enters brain tissue. Every TBI is different, and symptoms of a TBI can be mild, moderate, or severe, depending on the part of the brain that's injured and the extent of the damage to the brain. A person with a mild TBI may remain conscious or may experience a loss of consciousness for a few seconds or minutes.

Symptoms of Mild TBI include

Symptoms of Moderate to Severe TBI include

More serious head injuries may result in

Prognosis

Approximately half of severe head-injuries will need surgery to remove or repair hematomas (ruptured blood vessels) or contusions (bruised brain tissue). Disabilities resulting from a TBI depend upon the severity of the injury, the location of the injury, and the age and general health of the person.

Some common disabilities as a result of a TBI

Chronic Traumatic Encephalopathy

Chronic Traumatic Encephalopathy

Chronic Traumatic Encephalopathy is caused by repeated traumatic brain injury (TBI), such as in boxers or in a person who suffered multiple concussions while playing a contact sport.

Symptoms

20 years or more after the TBI occurs, a person often develops

This form of dementia also is characterized by brain atrophy (shrinking of the brain) and widespread deposits of tau aggregates. Sometimes a person with chronic traumatic encephalopathy may experience behavioral and mood changes as early as 5 to 10 years afterwards. Dementia may not yet be present and the brain may not have shrunk, but small focal deposits of tau are seen in the brain at autopsy.

Subdural Hematoma

Subdural Hematoma a bleeding between the brain’s surface and its outer covering (the dura) is common in an elderly person after a fall. Subdural hematomas can cause dementia-like symptoms and changes in mental function. With treatment, some symptoms can be reversed.

Paraneoplastic Syndromes

Etiology

Paraneoplastic Syndromes are a group of rare disorders that may develop when cancer cells release substances that disrupt the normal function of surrounding cells and tissue. This triggers an abnormal immune system response to the cancerous tumor known as a "neoplasm." When the body’s circulating antibodies or white blood cells (known as T cells) respond to the cancer, they mistakenly attack the normal cells in the brain. This causes symptoms that resemble dementia. These disorders typically affects a middle-aged to older person and are most common in a person with lung, ovarian, lymphatic, or breast cancer.

Symptoms

Neurologic symptoms generally develop over a period of days to weeks and usually occur prior to the tumor being discovered. These symptoms may include difficulty in walking or swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems.

Treatment

When present, the tumor and cancer are treated first, followed by efforts to decrease the autoimmune response, either through steroids such as cortisone or prednisone, high-dose intravenous immunoglobulin, or irradiation. Plasmapheresis, a process that cleanses antibodies from the blood, may ease symptoms in people with paraneoplastic disorders that affect the peripheral nervous system. Speech and physical therapy may help individuals regain some functions.

Prognosis

There are no cures for paraneoplastic syndromes. There are no available treatments to stop progressive neurological damage. Generally, the stage of cancer at diagnosis determines the outcome.

Eventually sleep disturbances, dementia, seizures, sensory loss in the limbs, and vertigo or dizziness may occur.

Dementia-like Conditions That Can Be Reversed

A small percentage of dementias are reversible, occurring as a secondary development in treatable conditions.

Such conditions include

Infections - Dementia-like symptoms or delirium can result from fever or other side effects of your body's attempt to fight off an infection.

Metabolic Problems and Endocrine Abnormalities such as thyroid problems, low blood sugar (hypoglycemia), too little or too much sodium or calcium, an impaired ability to absorb vitamin B-12 and markedly decreased Cortisol's can develop dementia-like symptoms or other personality changes.

Nutritional Deficiencies

Reactions To Medications - Side effects of medications or drug combinations may cause dementia like symptoms that arise quickly or develop slowly over time.

Subdural hematomas - Bleeding between the surface of the brain and the covering over the brain, which is common in an elderly person after a fall, can cause symptoms similar to dementia.

Normal-pressure Hydrocephalus -This condition is an abnormal buildup of cerebro¬spinal fluid in the brain, which is caused by enlarged ventricles in the brain. It can be treated or even reversed by implanting a shunt system to divert fluid from the brain. An elderly person with this condition usually has trouble with walking and bladder control before onset of dementia.

Cerebral Vasculitis - Necrosis (tissue death) of blood vessel walls can cause a form of dementia that may resolve when a person is treated with immune suppressants. Some studies have shown that a person with depression is at increased risk of developing dementia. One form of depression (pseudo dementia), presents as dementia and can be treated.

Infectious diseases - HIV-associated dementia (HAD) can occur in people who are positive for the human immunodeficiency virus, the virus that causes AIDS. HAD damages the brain’s white matter and leads to a type of dementia associated with memory problems, social withdrawal, and trouble concentrating. A person with HAD may develop movement problems as well. The incidence of HAD has dropped dramatically with the availability of effective antiviral therapies for managing the underlying HIV infection.

Risk factors for dementia

Many factors can eventually lead to dementia. Some factors can't be changed but others can be addressed to reduce your risk.

Environmental Factors

Environmental factors may play a role in the development of certain types of dementia. Sometimes a person may carry genetic mutations that influence his or her response to environmental factors.

Symptoms of Anoxia

When hypoxia lasts for longer periods of time, it can cause coma, seizures, and even brain death. In brain death, there is no measurable activity in the brain, although cardiovascular function is preserved. Life support is required for respiration.

Treatment

Treatment depends on the underlying cause of the hypoxia, but basic life-support systems have to be put in place.

Prognosis

Recovery depends on how long the brain has been deprived of oxygen and how much brain damage has occurred, although carbon monoxide poisoning can cause brain damage days to weeks after the event. A person who makes a full recovery has only been briefly unconscious. The longer a person is unconscious, the higher the chances of death or brain death and the lower the chances of a meaningful recovery. During recovery, psychological and neurological abnormalities such as amnesia, personality regression, hallucinations, memory loss, and muscle spasms and twitches may appear, persist, and then resolve.

Risk factors that can't be changed

Risk factors a person might be able to control

Complications

Dementia can affect many systems in the body and the ability of these systems to function.

Dementia can lead to

References

National Institute of Neurological Disorders and Stroke - Dementia

Mayo Clinic

Prepared by: Jody McCoppen - October 2016