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Conditions that require Frail care

Hospicare / Conditions that require Frail care

There are various conditions out there that impact our loved ones in different ways, and it is up to all of us to make sure that they get the frail care they deserve. You can find out more about some of the ailments that affect our loved ones here.

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Dementia

Dementia is a general term for a decline in mental ability severe enough to interfere with daily life. People with dementia need frail care assistance.

Dementia is not a specific disease. It's an overall term that describes a wide range of symptoms associated with a decline in memory or other thinking skills severe enough to reduce a person's ability to perform everyday activities.
Alzheimer's disease accounts for 60 - 80 percent of cases. Vascular dementia, which occurs after a stroke, is the second most common dementia type. But there many other conditions that can cause symptoms of dementia, including some that are reversible , such as thyroid problems and vitamin deficiencies.
Dementia is often incorrectly referred to as 'senility' or 'senile dementia', which reflects the formerly widespread but incorrect belief that serious mental decline is a normal part of aging. Frail care assistance is important for people who suffer from dementia.

While symptoms of dementia can vary greatly, at least two of the following core mental functions must be signigicantly impaired to be considered dementia:

Memory

Communication & language

Ability to focus and pay attention

Reasoning and judgement

Visual perception

People with dementia may have problems with short-term memory, keeping track of a purse or wallet, paying bills, planning and preparing meals, remembering appointments or traveling out of the neighborhood.
Many dementias are progressive, meaning symptoms start out slowly and gradually get worse. If you or a loved one is experiencing memory difficulties or other changes in thinking skills, don't ignore them. See a doctor soon to determine the cause. Professional evaluation may detect a treatable condition.

Dementia is caused by damage to brain cells. This damage interferes with the ability of brain cells to communicate with each other. When brain cells cannot communicate normally, thinking, behavior and feelings can be affected.
The brain has many distinct regions, each of which is responsible for different functions (for example, memory, judgment and movement). When cells in a particular region are damaged, that region cannot carry out its functions normally.
Different types of dementia are associated with particular types of brain cell damage in particular regions of the brain. For example, in Alzheimer's disease, high levels of certain proteins inside and outside brain cells make it hard for brain cells to stay healthy and to communicate with each other. The brain region called the hippocampus is the center of learning and memory in the brain, and the brain cells in this region are often the first to be damaged. That's why memory loss is often one of the earliest symptoms of Alzheimer's.
While most changes in the brain that cause dementia are permanent and worsen over time, thinking and memory problems caused by the following conditions may improve when the condition is treated or addressed:

Depression

Medication side effects

Excess use of alcohol

Thyroid problems

Vitamin deficiencies

There is no one test to determine if someone has dementia. Doctors diagnose Alzheimer's and other types of dementia based on a careful medical history, a physical examination, laboratory tests, and the characteristic changes in thinking, day-to-day function and behavior associated with each type. Doctors can determine that a person has dementia with a high level of certainty. But it's harder to determine the exact type of dementia because the symptoms and brain changes of different dementias can overlap. In some cases, a doctor may diagnose ``dementia`` and not specify a type. If this occurs it may be necessary to see a specialist such as a neurologist or gero-psychologist.

Alzheimer's Disease

Alzheimer's is the most common form of dementia, a general term for memory loss and other cognitive abilities serious enough to interfere with daily life and which needs advanced frail care. Alzheimer's disease accounts for 60 to 80 percent of dementia cases.

Alzheimer's is not a normal part of aging. The greatest known risk factor is increasing age, and the majority of people with Alzheimer's are 65 and older.

Alzheimer's worsens over time. Alzheimer's is a progressive disease, where dementia symptoms gradually worsen over a number of years. In its early stages, memory loss is mild, but with late-stage Alzheimer's, individuals lose the ability to carry on a conversation and respond to their environment.

Alzheimer's has no current cure, but treatments for symptoms are available and research continues. Although current Alzheimer's treatments cannot stop Alzheimer's from progressing, they can temporarily slow the worsening of dementia symptoms and improve quality of life for those with Alzheimer's and their caregivers. Today, there is a worldwide effort under way to find better ways to treat the disease, delay its onset, and prevent it from developing.

The most common early symptom of Alzheimer's is difficulty remembering newly learned information. Just like the rest of our bodies, our brains change as we age. Most of us eventually notice some slowed thinking and occasional problems with remembering certain things. However, serious memory loss, confusion and other major changes in the way our minds work may be a sign that brain cells are failing.
The most common early symptom of Alzheimer's is difficulty remembering newly learned information because Alzheimer's changes typically begin in the part of the brain that affects learning. As Alzheimer's advances through the brain it leads to increasingly severe symptoms, including disorientation, mood and behavior changes; deepening confusion about events, time and place; unfounded suspicions about family, friends and professional caregivers; more serious memory loss and behavior changes; and difficulty speaking, swallowing and walking.
People with memory loss or other possible signs of Alzheimer's may find it hard to recognize they have a problem. Signs of dementia may be more obvious to family members or friends.

Microscopic changes in the brain begin long before the first signs of memory loss.
The brain has 100 billion nerve cells (neurons). Each nerve cell connects 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.
To do their work, brain cells operate like tiny factories. They receive supplies, generate energy, construct equipment and get rid of waste. Cells also process and store information and communicate with other cells. Keeping everything running requires coordination as well as large amounts of fuel and oxygen.
Scientists believe Alzheimer's disease prevents parts of a cell's factory from running well. They are not sure where the trouble starts. But just like a real factory, backups and breakdowns in one system cause problems in other areas. 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.
Plaques are deposits of a protein fragment called beta-amyloid (BAY-tuh AM-uh-loyd) that build up in the spaces between nerve cells.
Tangles are twisted fibers of another protein called tau (rhymes with “wow”) that build up inside cells.
Though autopsy studies show that most people develop some plaques and tangles as they age, those with Alzheimer’s tend to develop far more and in a predictable pattern, beginning in the areas important for memory before spreading to other regions.
Scientists do not know 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.
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.

Today, Alzheimer's is at the forefront of biomedical research.
Researchers are working to uncover as many aspects of Alzheimer's disease and related dementias as possible. Ninety percent of what we know about Alzheimer's has been discovered in the last 20 years. Some of the most remarkable progress has shed light on how Alzheimer's affects the brain. The hope is this better understanding will lead to new treatments. Many potential approaches are currently under investigation worldwide.

Vascular Dementia

Vascular dementia is a decline in thinking skills caused by conditions that block or reduce blood flow to the brain, depriving brain cells of vital oxygen and nutrients.

Inadequate blood flow can damage and eventually kill cells anywhere in the body. The brain has one of the body's richest networks of blood vessels and is especially vulnerable.
In vascular dementia, changes in thinking skills sometimes occur suddenly following strokes that block major brain blood vessels. Thinking problems also may begin as mild changes that worsen gradually as a result of multiple minor strokes or other conditions that affect smaller blood vessels, leading to cumulative damage. A growing number of experts prefer the term ``vascular cognitive impairment (VCI)`` to ``vascular dementia`` because they feel it better expresses the concept that vascular thinking changes can range from mild to severe.
Vascular brain changes often coexist with changes linked to other types of dementia, including Alzheimer's disease and dementia with Lewy bodies. Several studies have found that vascular changes and other brain abnormalities may interact in ways that increase the likelihood of dementia diagnosis.
Vascular dementia is widely considered the second most common cause of dementia after Alzheimer's disease, accounting for 10 percent of cases. Many experts believe that vascular dementia remains underdiagnosed — like Alzheimer's disease — even though it's recognized as common.

Symptoms can vary widely, depending on the severity of the blood vessel damage and the part of the brain affected. Memory loss may or may not be a significant symptom depending on the specific brain areas where blood flow is reduced.
Vascular dementia symptoms may be most obvious when they happen soon after a major stroke. Sudden post-stroke changes in thinking and perception may include:

Confusion

Disorientation

Trouble speaking or understanding speech

Vision loss

These changes may happen at the same time as more familiar physical stroke symptoms, such as a sudden headache, difficulty walking, or numbness or paralysis on one side of the face or the body.
Multiple small strokes or other conditions that affect blood vessels and nerve fibers deep inside the brain may cause more gradual thinking changes as damage accumulates. Common early signs of widespread small vessel disease include impaired planning and judgment; uncontrolled laughing and crying; declining ability to pay attention; impaired function in social situations; and difficulty finding the right words.

Because vascular cognitive impairment may often go unrecognized, many experts recommend professional screening with brief tests to assess memory, thinking and reasoning for everyone considered to be at high risk for this disorder. Individuals at highest risk include those who have had a stroke or a transient ischemic attack (TIA, also known as a ``ministroke``). Additional high-risk groups include those with high blood pressure, high cholesterol, or other risk factors for heart or blood vessel disease.
Professional screening for depression is also recommended for high-risk groups. Depression commonly coexists with brain vascular disease and can contribute to cognitive symptoms.
If brief screening tests suggest changes in thinking or reasoning, a more detailed assessment is needed. Core elements of a workup for vascular dementia typically include:

A thorough medical history, including family history of dementia

Evaluation of independent function and daily activities

Input from a family member or trusted friend

In-office neurological examination assessing function of nerves and reflexes, movement, coordination, balance and senses

Laboratory tests including blood tests and brain imaging

According to a 2011 scientific statement issued by the American Heart Association (AHA) and the American Stroke Association (ASA), and endorsed by the Alzheimer's Association and the American Academy of Neurology (AAN), the following three criteria suggest the greatest likelihood that mild cognitive impairement (MCI) or dementia is caused by vascular changes:

The diagnosis of dementia or mild cognitive impairment is confirmed by neurocognitive testing, which involves several hours of written or computerized tests that provide detailed evaluation of specific thinking skills such as judgment, planning, problem-solving, reasoning and memory

There is brain imaging evidence, usually with magnetic resonance imaging (MRI), showing evidence of either: a) A recent stroke, or b) Other brain blood vessel changes whose severity and pattern of affected tissue are consistent with the types of impairment documented in neurocognitive testing

There is no evidence that factors other than vascular changes are contributing to cognitive decline. The guidelines also discuss cases where the diagnosis may be less clear-cut, such as the common situation where vascular changes coexist with brain changes associated with other types of dementia.

As with Alzheimer's disease, advancing age is a major risk factor for vascular cognitive impairment or dementia.
Additional risk factors are the same ones that raise risk for heart problems, stroke and other diseases that affect blood vessels. Many of these vascular factors also raise risk for Alzheimer's. The following strategies may reduce your risk of diseases that affect your heart and blood vessels — and also may help protect your brain:

Limit alcohol consumption

Maintain a healthy weight

Exercise

Eat a healthy, balanced diet

Keep your blood pressure, cholesterol and blood sugar within recommended limits

Don't smoke

Dementia with Lewy Bodies

Dementia with Lewy bodies (DLB) is a type of progressive dementia that leads to a decline in thinking, reasoning and independent function because of abnormal microscopic deposits that damage brain cells over time.

Most experts estimate that dementia with Lewy bodies is the third most common cause of dementia after Alzheimer's disease and vascular dementia, accounting for 10 to 25 percent of cases.
The hallmark brain abnormalities linked to DLB are named after Frederich H. Lewy, M.D., the neurologist who discovered them while working in Dr. Alois Alzheimer's laboratory during the early 1900s. Alpha-synuclein protein, the chief component of Lewy bodies, is found widely in the brain, but its normal function isn't yet known.
Lewy bodies are also found in other brain disorders, including Alzheimer's disease and Parkinson's disease dementia. Many people with Parkinson's eventually develop problems with thinking and reasoning, and many people with DLB experience movement symptoms, such as hunched posture, rigid muscles, a shuffling walk and trouble initiating movement.
This overlap in symptoms and other evidence suggest that DLB, Parkinson's disease and Parkinson's disease dementia may be linked to the same underlying abnormalities in how the brain processes the protein alpha-synuclein. Many people with both DLB and Parkinson's dementia also have plaques and tangles — hallmark brain changes linked to Alzheimer's disease.

Symptoms of dementia with Lewy bodies include:

Changes in thinking and reasoning

Confusion and alertness that varies significantly from one time of day to another or from one day to the next

Parkinson's symptoms, such as a hunched posture, balance problems and rigid muscles

Visual hallucinations

Delusions

Trouble interpreting visual information

Acting out dreams, sometimes violently, a problem known as rapid eye movement (REM) sleep disorder

Malfunctions of the ``automatic`` (autonomic) nervous system

Memory loss that may be significant but less prominent than in Alzheimer's

As with other types of dementia there is no single test that can conclusively diagnose dementia with Lewy bodies. Today, DLB is a ``clinical`` diagnosis, which means it represents a doctor's best professional judgment about the reason for a person's symptoms. The only way to conclusively diagnose DLB is through a postmortem autopsy.
Many experts now believe that DLB and Parkinson's disease dementia are two different expressions of the same underlying problems with brain processing of the protein alpha-synuclein. But most experts recommend continuing to diagnose DLB and Parkinson's dementia as separate disorders.
The diagnosis is DLB when:

Dementia symptoms consistent with DLB develop first

When both dementia symptoms and movement symptoms are present at the time of diagnosis

When dementia symptoms appear within one year after movement symptoms.

Memory loss tends to be a more prominent symptom in early Alzheimer's than in early DLB, although advanced DLB may cause memory problems in addition to its more typical effects on judgment, planning and visual perception.

Movement symptoms are more likely to be an important cause of disability early in DLB than in Alzheimer's, although Alzheimer's can cause problems with walking, balance and getting around as it progresses to moderate and severe stages.

Hallucinations, delusions, and misidentification of familiar people are significantly more frequent in early-stage DLB than in Alzheimer's.

REM sleep disorder is more common in early DLB than in Alzheimer's.

Disruption of the autonomic nervous system, causing a blood pressure drop on standing, dizziness, falls and urinary incontinence, is much more common in early DLB than in Alzheimer's.

There are no treatments that can slow or stop the brain cell damage caused by dementia with Lewy bodies. Current strategies focus on helping symptoms.
If your treatment plan includes medications, it's important to work closely with your physician to identify the drugs that work best for you and the most effective doses. Treatment considerations involving medications include the following issues:

Cholinesterase inhibiltors drugs are the current mainstay for treating thinking changes in Alzheimer's. They also may help certain DLB symptoms.

Antipsychotic drugs should be used with extreme caution in DLB. Although physicians sometimes prescribe these drugs for behavioural symptoms that can occur in Alzheimer's, they may cause serious side effects in as many as 50 percent of those with DLB. Side effects may include sudden changes in consciousness, impaired swallowing, acute confusion, episodes of delusions or hallucinations, or appearance or worsening of Parkinson's symptoms.

Antidepressants may be used to treat depression, which is common with DLB, Parkinson's disease dementia and Alzheimer's.The most commonly used antidepressants are selective serotonin reuptake inhibitors (SSRIs).

Clonazepam may be prescribed to treat REM sleep disorder.

Parkinson's Disease

Parkinson's disease dementia is an impairment in thinking and reasoning that eventually affects many people with Parkinson's disease.

The brain changes caused by Parkinson's disease begin in a region that plays a key role in movement. As Parkinson's brain changes gradually spread, they often begin to affect mental functions, including memory and the ability to pay attention, make sound judgments and plan the steps needed to complete a task.
The key brain changes linked to Parkinson's disease and Parkinson's disease dementia are abnormal microscopic deposits composed chiefly of alpha-synuclein, a protein that's found widely in the brain but whose normal function isn't yet known. The deposits are called ``Lewy bodies``.
Lewy bodies are also found in several other brain disorders, including dementia with Lewy bodies (DLB). Evidence suggests that dementia with Lewy bodies, Parkinson's disease and Parkinson's disease dementia may be linked to the same underlying abnormalities in brain processing of alpha-synuclein.
Another complicating factor is that many people with both dementia with Lewy bodies and Parkinson's disease dementia also have plaques and tangles — hallmark brain changes linked to Alzheimer's disease.
Parkinson's disease is a fairly common neurological disorder in older adults, estimated to affect nearly 2 percent of those older than age 65. The National Parkinson Foundation estimates that 1 million Americans have Parkinson's disease. It is estimated that 50 to 80 percent of those with Parkinson's disease eventually experience Parkinson's disease dementia.

Symptoms of dementia with Lewy bodies include:

Changes in thinking and reasoning

Confusion and alertness that varies significantly from one time of day to another or from one day to the next

Parkinson's symptoms, such as a hunched posture, balance problems and rigid muscles

Visual hallucinations

Delusions

Trouble interpreting visual information

Acting out dreams, sometimes violently, a problem known as rapid eye movement (REM) sleep disorder

Malfunctions of the ``automatic`` (autonomic) nervous system

Memory loss that may be significant but less prominent than in Alzheimer's

As with other types of dementia there is no single test that can conclusively diagnose dementia with Lewy bodies. Today, DLB is a ``clinical`` diagnosis, which means it represents a doctor's best professional judgment about the reason for a person's symptoms. The only way to conclusively diagnose DLB is through a postmortem autopsy.
Many experts now believe that DLB and Parkinson's disease dementia are two different expressions of the same underlying problems with brain processing of the protein alpha-synuclein. But most experts recommend continuing to diagnose DLB and Parkinson's dementia as separate disorders.
The diagnosis is DLB when:

Dementia symptoms consistent with DLB develop first

When both dementia symptoms and movement symptoms are present at the time of diagnosis

When dementia symptoms appear within one year after movement symptoms.

Memory loss tends to be a more prominent symptom in early Alzheimer's than in early DLB, although advanced DLB may cause memory problems in addition to its more typical effects on judgment, planning and visual perception.

Movement symptoms are more likely to be an important cause of disability early in DLB than in Alzheimer's, although Alzheimer's can cause problems with walking, balance and getting around as it progresses to moderate and severe stages.

Hallucinations, delusions, and misidentification of familiar people are significantly more frequent in early-stage DLB than in Alzheimer's.

REM sleep disorder is more common in early DLB than in Alzheimer's.

Disruption of the autonomic nervous system, causing a blood pressure drop on standing, dizziness, falls and urinary incontinence, is much more common in early DLB than in Alzheimer's.

Frontotemporal Dementia

Frontotemporal dementia (FTD) or frontotemporal degenerations refers to a group of disorders caused by progressive nerve cell loss in the brain's frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears).

The nerve cell damage caused by frontotemporal dementia leads to loss of function in these brain regions, which variably cause deterioration in behavior and personality, language disturbances, or alterations in muscle or motor functions.
There are a number of different diseases that cause frontotemporal degenerations. The two most prominent are 1) a group of brain disorders involving the protein tau and 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.
The disorders grouped under FTD fall into three subtypes.
FTD used to be called Pick's disease after Arnold Pick, a physician who in 1892 first described a patient with distinct symptoms affecting language. Some doctors still use the term ``Pick's disease.`` Other terms you may see used to describe FTD include frontotemporal disorders, frontotemporal degenerations and frontal lobe disorders.

Behavior variant frontotemporal dementia (bvFTD). This condition is characterized by prominent changes in personality, interpersonal relationships and conduct that often occur in people in their 50s and 60s, but can develop as early as their 20s or as late as their 80s. In bvFTD, the nerve cell loss is most prominent in areas that control conduct, judgment, empathy and foresight, among other abilities.Primary progressive aphasia (PPA). This is the second major form of frontotemporal degeneration that affects language skills, speaking, 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:

In semantic variant of PPA, individuals lose the ability to understand or formulate words in a spoken sentence.

In nonfluent/agrammatic variant of PPA, a person’s speaking is very hesitant, labored or ungrammatical.

Disturbances of motor (movement or muscle) function. 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), which causes muscle weakness or wasting. ALS is a motor neuron disease also known as Lou Gehrig’s disease.

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

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

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 younger-onset Alzheimer’s. Only rough estimates are available, but there may be 50,000 to 60,000 people with bvFTD and PPA in the United States, the majority of whom are between 45 and 65 years of age.

The diagnosis of bvFTD and PPA are based on expert evaluation by a doctor who is familiar with these disorders. The type of problems experienced by the patient and the results of neurological exams are the core of the diagnosis. Brain scans such as magnetic resonance imaging (MRI) and glucose positron emission scans are very helpful additional tests, but they must be interpreted in the context of the patient’s history and neurological exam.

Age at diagnosis may be an important clue. Most people with FTD are diagnosed in their 40s and early 60s. Alzheimer's, on the other hand, grows more common with increasing age.

Memory loss tends to be a more prominent symptom in early Alzheimer's than in early FTD, although advanced FTD often causes memory loss in addition to its more characteristic effects on behavior and language.

Behavior changes are often the first noticeable symptoms in bvFTD, the most common form of FTD. Behavior changes are also common as Alzheimer's progresses, but they tend to occur later in the disease.

Problems with spatial orientation — for example, getting lost in familiar places — are more common in Alzheimer's than in FTD.

Problems with speech. Although people with Alzheimer's may have trouble thinking of the right word or remembering names, they tend to have less difficulty making sense when they speak, understanding the speech of others, or reading than those with FTD.

Hallucinations and delusions are relatively common as Alzheimer's progresses, but relatively uncommon in FTD.

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is the most common human form of a group of rare, fatal brain disorders known as prion diseases.

Prion diseases, such as Creutzfeldt-Jakob disease (CJD), occur when prion protein, which is found throughout the body but whose normal function isn't yet known, begins folding into an abnormal three-dimensional shape. This shape change gradually triggers prion protein in the brain to fold into the same abnormal shape.
Through a process scientists don't yet understand, misfolded prion protein destroys brain cells. Resulting damage leads to rapid decline in thinking and reasoning as well as involuntary muscle movements, confusion, difficulty walking and mood changes.
CJD is rare, occurring in about one in 1 million people annually worldwide.

Sporadic CJD develops spontaneously for no known reason. It accounts for 85 percent of cases. On average, sporadic CJD first appears between ages 60 and 65.

Familial CJD is caused by certain changes in the chromosome 20 gene coding the biological blueprint for prion protein. People who develop familial CJD do so because they inherited the genetic changes from a parent. Familial CJD accounts for about 10 to 15 percent of cases. It develops, on average, at a younger age than sporadic CJD, with some genetic types appearing as early as ages 20 to 40.

Acquired CJD results from exposure to an external source of abnormal prion protein. These sources estimated to account for about 1 percent of CJD cases. The two most common outside sources are:

Specific Creutzfeldt-Jakob disease symptoms experienced by an individual and the order in which they appear can differ significantly. Some common symptoms include:

Depression

Agitation, apathy and mood swings

Rapidly worsening confusion, disorientation, and problems with memory, thinking, planning and judgment

Difficulty walking

Muscle stiffness, twitches and involuntary jerky movements

Rapid symptom progression is one of the most important clues that a person may have Creutzfeldt-Jakob disease.
There is no single test — or any combination of tests — that can conclusively diagnose sporadic CJD in a living person, but the following tests may help determine whether an individual has CJD:

Electroencephalogram (EEG) measures the brain's patterns of electrical activity similar to the way an electrocardiogram (ECG) measures the heart's electrical activity

Brain magnetic resonance imaging (MRI) can detect certain brain changes consistent with CJD.

Lumbar puncture (spinal tap) tests spinal fluid for the presence of certain proteins.

Sporadic Creutzfeldt-Jakob disease has no known cause. Most scientists believe the disease begins when prion protein somewhere in the brain spontaneously misfolds, triggering a ``domino effect`` that misfolds prion protein throughout the brain. Genetic variation in the prion protein gene may affect risk of this spontaneous misfolding.Mutations in the prion protein gene also may play a yet-to-be-determined role in making people susceptible to acquired CJD from external sources. Scientists don't yet know the why acquired CJD seems to be transmitted through such a limited number of external sources. Researchers have found no evidence that the abnormal protein is commonly transmitted through sexual activity or blood transfusions.
Familial CJD is caused by variations in the prion protein gene that increases the risk of an individual developing CJD. Researchers have identified more than 50 prion protein mutations in those with inherited CJD. Genetic testing can determine whether family members at risk have inherited a CJD-causing mutation. Experts strongly recommend professional genetic counseling both before and after genetic testing for hereditary CJD.

Normal Pressure Hydrocephalus

Normal pressure hydrocephalus is a brain disorder in which excess cerebrospinal fluid accumulates in the brain's ventricle, causing thinking and reasoning problems, difficulty walking and loss of bladder control.

Normal pressure hydrocephalus occurs when excess cerebrospinal fluid accumulates in the brain's ventricles, which are hollow fluid-filled chambers. NPH is called ``normal pressure`` because despite the excess fluid, cerebrospinal fluid pressure as measured during a spinal tap is often normal. As brain ventricles enlarge with the excess cerebrospinal fluid, they can disrupt and damage nearby brain tissue, causing symptoms of NPH.
NPH primarily affects people in their 60s and 70s. Scientists aren't certain how many older adults have this disorder because common symptoms of NPH are also common in other brain disorders.

Difficulty walking that's sometimes compared to the way a person walks ``on a boat,`` with the body bent forward, legs held wide apart and feet moving as if they're ``glued to the deck.``

Decline in thinking skills that includes overall slowing of thought processes, apathy, impaired planning and decision-making, reduced concentration and changes in personality and behavior.

Loss of bladder control, which tends to appear somewhat later in the disease than difficulty walking and cognitive decline.

There is no single test to determine if someone has normal pressure hydrocephalus. And even though the three hallmark symptoms listed above are considered the ``classic`` signs of this disorder, not everyone with NPH has all of these symptoms.
Brain imaging to detect enlargement of the ventricles, often with magnetic resonance imaging (MRI), plays a key role in diagnosing NPH. Several brain disorders, including Alzheimer's disease, can cause overall brain tissue shrinkage that makes the ventricles look larger than normal. In NPH, although the ventricles are enlarged, brain tissue may not appear shrunken.
Because the symptoms of NPH may overlap with those of Alzheimer's and other dementias, experts recommend that a person with suspected NPH undergo examination by a neurologist with extensive experience evaluating brain disorders that affect movement, thinking skills and physical functions.
If symptoms and an MRI strongly suggest NPH, a large-volume spinal tap may be used to identify those who may benefit from a shunt. In this procedure, doctors remove a larger-than-usual amount of spinal fluid, and then observe the person for 30 to 60 minutes to note any improvements in walking or thinking and reasoning. Most people originally suspected of having NPH do not improve following a CSF removal test.

In some cases, normal pressure hydrocephalus is caused by other brain disorders such as hemorrhages, infections or inflammation. But in most cases, the fluid buildup happens for unknown reasons.

Researchers have not found effective nonsurgical treatments for normal pressure hydrocephalus. Drugs that remove excess fluid throughout the body, such as diuretics, haven't been shown to help. Sign up for our enews to receive updates about Alzheimer’s and dementia care and research.
NPH can sometimes be treated with surgical insertion of a shunt, a long, thin tube that drains excess CSF from the brain to the abdomen. Difficulty walking is the symptom most likely to improve after surgery. Thinking changes and bladder control are less likely to get better. Shunting doesn't help everyone with NPH, and there's uncertainty about how best to identify those most likely to benefit.
More research is needed to:

Understand the prevalence of NPH

Show how the excess CSF involved in NPH causes symptoms affecting movement, thinking and bodily functions

Clarify the possible benefits of shunt insertion and who is most likely to benefit

The effectiveness of shunting in NPH has never been demonstrated in a randomized clinical trial. Most of these studies were small and followed people for a limited time. Available data suggest that difficulty walking is the symptom most likely to improve. Several studies found a significant rate of postsurgical complications. Findings also showed that short-term benefits of shunt insertion tended to decline over time.

Huntington's Disease

Huntington's Disease (HD) is a progressive brain disorder caused by a defective gene. This disease causes changes in the central area of the brain, which affect movement, mood and thinking skills.

Huntington's disease is a progressive brain disorder caused by a single defective gene on chromosome 4 — one of the 23 human chromosomes that carry a person’s entire genetic code.
This defect is ``dominant,`` meaning that anyone who inherits it from a parent with Huntington's will eventually develop the disease. The disorder is named for George Huntington, the physician who first described it in the late 1800s.
The defective gene codes the blueprint for a protein called huntingtin. This protein's normal function isn't yet known, but it's called ``huntingtin`` because scientists identified its defective form as the cause of Huntington's disease. Defective huntingtin protein leads to brain changes that cause abnormal involuntary movements, a severe decline in thinking and reasoning skills, and irritability, depression and other mood changes.

Symptoms of Huntington's disease usually develop between ages 30 and 50, but they can appear as early as age 2 or as late as 80. The hallmark symptom of Huntington's disease is uncontrolled movement of the arms, legs, head, face and upper body. Huntington's disease also causes a decline in thinking and reasoning skills, including memory, concentration, judgment and ability to plan and organize.
Huntington's disease brain changes lead to alterations in mood, especially depression, anxiety, and uncharacteristic anger and irritability. Another common symptom is obsessive-compulsive behavior, leading a person to repeat the same question or activity over and over.

Scientists identified the defective gene that causes Huntington's disease in 1993. A diagnostic genetic test is now available.The test can confirm that the defective gene for huntingtin protein is the cause of symptoms in people with suspected Huntington's disease and can detect the defective gene in people who don't yet have symptoms but are at risk because a parent has Huntington's.

The defective gene identified in 1993 causes virtually all Huntington’s disease.
The huntingtin gene defect involves extra repeats of one specific chemical code in one small section of chromosome 4. The normal huntingtin gene includes 17 to 20 repetitions of this code among its total of more than 3,100 codes. The defect that causes Huntington's disease includes 40 or more repeats. Genetic tests for Huntington's disease measure the number of repeats present in an individual's huntington protein gene.
Scientists don't yet understand the normal function of huntingtin protein or how a few dozen extra repeats in its genetic blueprint lead to the devastating symptoms of Huntington's disease. Researchers are eager to solve these mysteries to find the answer to Huntington's. These solutions also may offer important insights into a wide range of other brain disorders, including Alzheimer's, Parkinson'sdisease and amyotrophic lateral sclerosis (ALS).

There is currently no cure for Huntington's disease and no way to slow or stop the brain changes it causes. Treatments focus on managing symptoms. A group of international experts recommended the following treatments as first-line strategies for three of the disease's most troubling symptoms:

Chorea (involuntary movements): Some experts believe beginning treatment with an atypical antipsychotic drug, such as olanzapine, is best. Others start with another type of drug recently approved by the U.S. Food and Drug Administration (FDA) specifically for Huntington’s, called tetrabenazine.

Irritability: For severe anger and threatening behavior, experts agree that an atypical antipsychotic drug is the preferred approach. For less severe, nonthreatening irritability, experts recommend first trying a selective serotonin reuptake inhibitor (SSRI), which is a type of antidepressant.

Obsessive-compulsive thoughts and actions: Experts also recommend SSRIs as the standard treatment for these symptoms.

Other Huntington's symptoms, such as anxiety, depression and insomnia, also should be treated according to generally accepted guidelines and benefit greatly from frail care assistance. Experts encourage people with Huntington's to keep all their medical appointments and not to get discouraged if it takes their health care team some time to find the best drugs and the most effective doses.

Wernickle-Korsakoff Syndrome

Korsakoff syndrome is a chronic memory disorder caused by severe deficiency of thiamine (vitamin B-1). Korsakoff syndrome is most commonly caused by alcohol misuse, but certain other conditions also can cause the syndrome.

Thiamine (vitamin B-1) helps brain cells produce energy from sugar. When levels fall too low, brain cells cannot generate enough energy to function properly. As a result, Korsakoff syndrome may develop.
Korsakoff syndrome is most commonly caused by alcohol misuse, but can also be associated with AIDS, chronic infections, poor nutrition and certain other conditions. See causes and risks below.
Korsakoff syndrome is often, but not always, preceded by an episode of Wernicke encephalopathy, which is an acute brain reaction to severe lack of thiamine. Wernicke encephalopathy is a medical emergency that causes life-threatening brain disruption, confusion, staggering and stumbling, lack of coordination, and abnormal involuntary eye movements.
Because the chronic memory loss of Korsakoff syndrome often follows an episode of Wernicke encephalopathy, the chronic disorder is sometimes known as Wernicke-Korsakoff syndrome. But Korsakoff syndrome can also develop in individuals who have not had a prior episode of Wernicke encephalopathy. We recommend frail care assistance to people who suffer from this ailment.

Korsakoff syndrome causes problems learning new information, inability to remember recent events and long-term memory gaps. Memory problems may be strikingly severe while other thinking and social skills are relatively unaffected. For example, individuals may seem able to carry on a coherent conversation, but moments later be unable to recall that the conversation took place or to whom they spoke.Those with Korsakoff syndrome may ``confabulate,`` or make up, information they can't remember. They are not ``lying`` but may actually believe their invented explanations. Scientists don't yet understand why Korsakoff syndrome may cause confabulation.

Korsakoff syndrome is a clinical diagnosis representing a physician's best judgment about the cause of a person's symptoms. There are no specific lab tests or brain scan procedures to confirm that a person has this disorder. The syndrome may sometimes be hard to identify because it may be masked by symptoms of other conditions common among those who misuse alcohol, including intoxication or withdrawal, infection or head injury.
Experts recommend that a medical workup for memory loss or other cognitive changes always include questions about an individual's alcohol use. Anyone admitted to the hospital for an alcohol-related condition should be professionally screened for memory loss and cognitive change.

Scientists don't yet know exactly how Korsakoff syndrome damages the brain. Research has shown that severe thiamine deficiency disrupts several biochemicals that play key roles in carrying signals among brain cells and in storing and retrieving memories. These disruptions destroy brain cells and cause widespread microscopic bleeding and scar tissue.
Most cases of Korsakoff syndrome result from alcohol misuse. Scientists don't yet know why heavy drinking causes severe thiamine deficiency in some alcoholics, while others may be affected primarily by alcohol's effects on the liver, stomach, heart, intestines or other body systems.
Researchers have identified several genetic variations that may increase susceptibility to Korsakoff syndrome. Poor nutrition also may raise risk. Sign up for our enews to receive updates about Alzheimer’s and dementia care and research.
Korsakoff syndrome also can be caused by anorexia, overly-stringent dieting, fasting, starvation or weight-loss surgery; uncontrolled vomiting; AIDS; kidney dialysis; chronic infection; or cancer that has spread throughout the body.

Some experts recommend that heavy drinkers and others at risk of thiamine deficiency take oral supplements of thiamine and other vitamins under their doctor's supervision.
Many experts also recommend that anyone with a history of heavy alcohol use who experiences symptoms associated with Wernicke encephalopathy be given injectable thiamine until the clinical picture grows clearer.
Once acute symptoms improve, individuals should be carefully evaluated to determine if their medical history, alcohol use and pattern of memory problems may be consistent with Korsakoff syndrome. For those who develop Korsakoff syndrome, extended treatment with oral thiamine, other vitamins and magnesium may increase chances of symptom improvement. Giving up alcohol is also an effective treatment.
In those who develop Korsakoff syndrome, with or without a preceding episode of Wernicke encephalopathy, there are few studies on long-term outcomes. Available data suggest that about 25 percent of those who develop Korsakoff syndrome eventually recover, about half improve but don't recover completely, and about 25 percent remain unchanged. Some research suggests that those who recover from an episode may have a normal life expectancy if they abstain from alcohol.