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Fingerprint body myopathy is a rare muscle disease characterized by weakness and reduced muscle mass and by subsarcolemmal, non-membrane-bound inclusions
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A diagnosis of dementia can be frightening for those affected by the syndrome, their family members, and caretakers. Learning more about dementia can help. This booklet provides a general overview of various types of dementia, describes how the disorders are diagnosed and treated, and offers highlights of research that is supported by the National Institute of Neurological Disorders and Stroke and the National Institute on Aging, both part of the National Institutes of Health (NIH).
(AD) is the most common form of dementia in those over the age of 65. As many as 5 million Americans age 65 and older may have AD, and that number is expected to double for every 5-year interval beyond age 65. But Alzheimer’s is only one of many dementia disorders; another 1.8 million people in the U.S. have some other form of dementia. Among all people with dementia, many are believed to have a mixed type of dementia that can involve more than one of the disorders.
Age is the primary risk factor for developing dementia. For that reason, the number of people living with dementia could double in the next 40 years with an increase in the number of Americans who are age 65 or older—from 40 million today to more than 88 million in 2050. Regardless of the form of dementia, the personal, economic, and societal demands can be devastating.
Research over the past 30 years has helped us learn more about dementia—possible causes, who is at risk, and how it develops and affects the brain. This work offers the hope of better drugs and treatments for these disorders.
The basics of dementia
Dementia is the loss of cognitive functioning, which means the loss of the ability to think, remember, or reason, as well as behavioral abilities, to such an extent that it interferes with a person’s daily life and activities. Signs and symptoms of dementia result when once-healthy neurons (nerve cells) in the brain stop working, lose connections with other brain cells, and die. While everyone loses some neurons as they age, people with dementia experience far greater loss.
Researchers are still trying to understand the underlying disease processes involved in the disorders. Scientists have some theories about mechanisms that may lead to different forms of dementias, but more research is needed to better understand if and how these mechanisms contribute to the development of dementia.
While dementia is more common with advanced age (as many as half of all people age 85 or older may have some form of dementia), it is not a normal part of aging. Many people live into their 90s and beyond without any signs of dementia.
Memory loss, though common, is not the only sign of dementia. For a person to be considered to have dementia, he or she must meet the following criteria:
Two or more core mental functions must be impaired. These functions include memory, language skills, visual perception, and the ability to focus and pay attention. These also include cognitive skills such as the ability to reason and solve problems.
The loss of brain function is severe enough that a person cannot do normal, everyday tasks.
In addition, some people with dementia cannot control their emotions. Their personalities may change. They can have delusions, which are strong beliefs without proof, such as the idea that someone is stealing from them. They also may hallucinate, seeing or otherwise experiencing things that are not real.
Types of dementia
Various disorders and factors contribute to the development of dementia. Neurodegenerative disorders such as AD, frontotemporal disorders, and Lewy body dementia result in a progressive and irreversible loss of neurons and brain functions. Currently, there are no cures for these progressive neurodegenerative disorders.
However, other types of dementia can be halted or even reversed with treatment. Normal pressure hydrocephalus, for example, often resolves when excess cerebrospinal fluid in the brain is drained via a shunt and rerouted elsewhere in the body. Cerebral vasculitis responds to aggressive treatment with immunosuppressive drugs. In rare cases, treatable infectious disorders can cause dementia. Some drugs, vitamin deficiencies, alcohol abuse, depression, and brain tumors can cause neurological deficits that resemble dementia. Most of these causes respond to treatment.
Some types of dementia disorders are described below.
Tauopathies. In some dementias, a protein called tau clumps together inside nerve cells in the brain, causing the cells to stop functioning properly and die. Disorders that are associated with an accumulation of tau are called tauopathies.
In AD, the tau protein becomes twisted and aggregates to form bundles, called neurofibrillary tangles, inside the neurons. Abnormal clumps (plaques) of another protein, called amyloid, are prominent in spaces between brain cells and are a hallmark of the disease. Both plaques and tangles are thought to contribute to reduced function and nerve-cell death in AD, but scientists do not fully understand this relationship. It is not clear, for example, if the plaques and tangles cause the disorder, or if their presence flags some other process that leads to neuronal death in AD.
Other types of tauopathies include the following disorders:
(CBD) is a progressive neurological disorder characterized by nerve-cell loss and atrophy (shrinkage) of specific areas of the brain, including the cerebral cortex and the basal ganglia. The disorder tends to progress gradually, with the onset of early symptoms around age 60. At first, one side of the body is affected more than the other side, but as the disease progresses both sides become impaired. An individual may have difficulty using one hand, or one’s hand may develop an abnormal position.
Other signs and symptoms may include memory loss; trouble making familiar, focused movements (apraxia) such as brushing one’s teeth; involuntary muscular jerks (myoclonus) and involuntary muscle contractions (dystonia); alien limb, in which the person feels as though a limb is being controlled by a force other than oneself; muscle rigidity (resistance to imposed movement); postural instability; and difficulty swallowing (dysphagia). People with CBD also may have visual-spatial problems that make it difficult to interpret visual information, such as the distance between objects.
There is no cure for CBD. Supportive therapies are available to reduce the burden of certain symptoms. For example, botulinum toxin can help control muscle contractions. Speech therapy and physical therapy may help one learn how to cope with daily activities.
(FTD) are caused by a family of brain diseases that primarily affect the frontal and temporal lobes of the brain; they account for up to 10 percent of all dementia cases. Some, but not all, forms of FTD are considered tauopathies. In some cases, FTD is associated with mutations in the gene for tau (MAPT), and tau aggregates are present. However, other forms of FTD are associated with aggregates of the protein TDP-43, a mutated protein found among people with a type of ALS that is inherited. Mutations in a protein called progranulin may also play a role in some TDP43-opathies.
In FTD, changes to nerve cells in the brain’s frontal lobes affect the ability to reason and make decisions, prioritize and multitask, act appropriately, and control movement. Some people decline rapidly over 2 to 3 years, while others show only minimal changes for many years. People can live with frontotemporal disorders for 2 to 10 years, sometimes longer, but it is difficult to predict the time course for an affected individual. In some cases, FTD is associated with progressive neuromuscular weakness otherwise known as amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease). The signs and symptoms may vary greatly among individuals as different parts of the brain are affected. No treatment that can cure or reverse FTD is currently available.
Clinically, FTD is classified into two main types of syndromes:
Other types of FTDs include:
(PSP) is a rare brain disorder that damages the upper brain stem, including the substantia nigra (a movement control center in the midbrain). This region also is affected in Parkinson’s disease, which may explain an overlap in motor symptoms shared by these disorders. Eye movements are especially affected, causing slow and then limited mobility of the eye. The most common early signs and symptoms include loss of balance, unexplained falls, general body stiffness, apathy, and depression. A person with this type of dementia may suddenly laugh or cry very easily (known as pseudobulbar affect). As the disorder progresses, people develop blurred vision and a characteristic vacant stare that involves loss of facial expression. Speech usually becomes slurred, and swallowing solid foods or liquids becomes difficult. PSP gets progressively worse, but people can live a decade or more after the onset of symptoms. Dextromethorphan, a common ingredient in cough medicine, has been approved for the treatment of pseudobulbar affect.
Argyrophilic grain disease is a common, late-onset degenerative disease characterized by tau deposits called argyrophilic grains in brain regions involved in memory and emotion. The disease’s signs and symptoms are indistinguishable from late-onset AD. Confirmation of the diagnosis can be made only at autopsy.
Synucleinopathies. In these brain disorders, a protein called alpha-synuclein accumulates inside neurons. Although it is not fully understood what role this protein plays, changes in the protein and/or its function have been linked to Parkinson’s disease and other disorders.
One type of synucleinopathy, , involves protein aggregates called Lewy bodies, balloon-like structures that form inside of nerve cells. The initial symptoms may vary, but over time, people with these disorders develop very similar cognitive, behavioral, physical, and sleep- related symptoms. Lewy body dementia is one of the most common causes of dementia, after Alzheimer’s disease and vascular disease. Types of Lewy body dementia include:
Vascular dementia and vascular cognitive impairment
Vascular dementia and vascular cognitive impairment (VCI) are caused by injuries to the vessels supplying blood to the brain. These disorders can be caused by brain damage from multiple strokes or any injury to the small vessels carrying blood to the brain. Dementia risk can be significant even when individuals have suffered only small strokes. Vascular dementia and VCI arise as a result of risk factors that similarly increase the risk for cerebrovascular disease (stroke), including atrial fibrillation, hypertension, diabetes, and high cholesterol. Vascular dementia also has been associated with a condition called amyloid angiopathy, in which amyloid plaques accumulate in the blood-vessel walls, causing them to break down and rupture. Symptoms of vascular dementia and VCI can begin suddenly and progress or subside during one’s lifetime.
Some types of vascular dementia include:
. This inherited form of cardiovascular disease results in a thickening of the walls of small- and medium-sized blood vessels, eventually stemming the flow of blood to the brain. It is associated with mutations of a specific gene called Notch3, which gives instructions to a protein on the surface of the smooth muscle cells that surround blood vessels. CADASIL is associated with multi-infarct dementia, stroke, migraine with aura (migraine preceded by visual symptoms), and mood disorders. The first symptoms can appear in people between ages 20 and 40. Many people with CADASIL are undiagnosed. People with first-degree relatives who have CADASIL can be tested for genetic mutations to the Notch3 gene to determine their own risk of developing CADASIL.
. This type of dementia occurs when a person has had many small strokes that damage brain cells. One side of the body may be disproportionally affected, and multi-infarct dementia may impair language or other functions, depending on the region of the brain that is affected. Doctors call these “local” or “focal” symptoms, as opposed to the “global” symptoms seen in AD that tend to affect several functions and both sides of the body. When the strokes occur on both sides of the brain, however, dementia is more likely than when stroke occurs on one side of the brain. In some cases, a single stroke can damage the brain enough to cause dementia. This so-called single-infarct dementia is more common when stroke affects the left side of the brain—where speech centers are located—and/or when it involves the hippocampus, the part of the brain that is vital for memory.
, also called Binswanger’s disease. This is a rare form of dementia that involves extensive microscopic damage to the small blood vessels and nerve fibers that make up white matter, the “network” part of the brain believed to be critical for relaying messages between regions. The symptoms of Binswanger’s are related to the disruption of subcortical neural circuits involving short-term memory, organization, mood, attention, decisionmaking, and appropriate behavior. A characteristic feature of this disease is psychomotor slowness, such as an increase in the time it takes for a person to think of a letter and then write it on a piece of paper.
Other symptoms include urinary incontinence that is unrelated to a urinary tract condition, trouble walking, clumsiness, slowness, lack of facial expression, and speech difficulties. Symptoms tend to begin after age 60, and they progress in a stepwise manner. People with subcortical vascular disease often have high blood pressure, a history of stroke, or evidence of disease of the large blood vessels in the neck or heart valves. Treatment is aimed at preventing additional strokes and may include drugs to control blood pressure.
. Autopsy studies looking at the brains of people who had dementia suggest that a majority of those age 80 and older probably had “mixed dementia,” caused by both AD-related neurodegenerative processes and vascular disease-related processes. In fact, some studies indicate that mixed vascular-degenerative dementia is the most common cause of dementia in the elderly. In a person with mixed dementia, it may not be clear exactly how many of a person’s symptoms are due to AD or another type of dementia. In one study, approximately 40 percent of people who were thought to have AD were found after autopsy to also have some form of cerebrovascular disease. Several studies have found that many of the major risk factors for vascular disease also may be risk factors for AD.
Researchers are still working to understand how underlying disease processes in mixed dementia influence each other. It is not clear, for example, if symptoms are likely to be worse when a person has brain changes reflecting multiple types of dementia. Nor do we know if a person with multiple dementias can benefit from treating one type, for example, when a person with AD controls high blood pressure and other vascular disease risk factors.
Other conditions that cause dementia
Doctors have identified many other conditions that can cause dementia or dementia-like symptoms.
(CJD). A rare brain disorder that affects about one in every million people worldwide each year, CJD belongs to a family of diseases known as the transmissible spongiform encephalopathies, or TSEs. Spongiform refers to the fact that the brain becomes filled with microscopic swellings that give the appearance of holes, like a sponge. CJD and other TSEs are believed to be caused by infectious proteins called prions that become misfolded. Scientists believe that the presence of misfolded prions can trigger normal proteins to misfold as well, causing a chain reaction. These abnormal prion proteins tend to clump together, which is believed to be related to the brain damage.
Symptoms usually begin after age 60, and most people die within a year of onset. In most cases, CJD occurs in people who have no known risk factors for the disease; however, an estimated 5 to 10 percent of cases in the U.S. are associated with genetic mutations. In addition, a type of CJD, called variant CJD (vCJD), has been found in Great Britain and several other European countries. vCJD has been observed to affect people who are younger than those with other forms of CJD and is believed to be caused by eating beef from cattle infected with a TSE called bovine spongiform encephalopathy, more commonly known as “mad cow disease.” Inherited forms of CJD include:
. This hereditary disorder is caused by a faulty gene for a protein called huntingtin. Symptoms begin around age 30 or 40 years and include abnormal and uncontrollable movements called chorea, as well as gait changes and lack of coordination. Huntington’s disease may affect a person’s judgment, memory, and other cognitive functions. As the disease progresses, these cognitive problems worsen, and motor difficulties lead to complete loss of ability for self-care. Children of people with Huntington’s have a 50 percent chance of having the disorder.
. These dementias occur in people with disorders that damage brain tissue. Such disorders may include multiple sclerosis; meningitis; encephalitis; and Wilson’s disease, in which excessive amounts of copper build up to cause brain damage. In rare cases, people with brain tumors may develop dementia because of damage to their brain circuits or a buildup of pressure inside the skull. Symptoms may include changes in personality, psychotic episodes, or problems with speech, language, thinking, and memory.
, initially known as dementia pugilistica, is caused by repeated traumatic brain injury (TBI), such as in boxers or in people who suffered multiple concussions while playing a contact sport. People with this condition often develop poor coordination, slurred speech, and other symptoms similar to those seen in Parkinson’s disease, along with dementia, 20 years or more after the TBI events. This form of dementia also is characterized by brain atrophy and widespread deposits of tau aggregates. In some individuals, even just 5 to 10 years beyond the TBI events, behavioral and mood changes may occur. Dementia may not yet be present and the brain may not have atrophied, but small focal deposits of tau are seen in the brain at autopsy.
, or bleeding between the brain’s surface and its outer covering (the dura), is common in the elderly after a fall. Subdural hematomas can cause dementia-like symptoms and changes in mental function. With treatment, some symptoms can be reversed.
Reversible dementias. Many conditions that cause dementia can be reversed with the appropriate treatment.
Environmental factors. Environmental factors may play a role in the development of certain types of dementia. This relationship is complex, however, since a person may carry genetic mutations that influence his or her response to environmental factors. Examples of environmental factors include:
Infectious disease. 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. People 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
The following risk factors can increase a person’s chance of developing one or more kinds of dementia. Some of these factors can be modified, while others cannot.
Doctors first assess whether the individual has an underlying treatable condition such as depression, abnormal thyroid function, drug-induced encephalopathy, normal pressure hydrocephalus, or vitamin B12 deficiency. Early diagnosis is important, as some causes for symptoms can be treated. In many cases, the specific type of dementia that a person has may not be confirmed until after the person has died and the brain is examined.
An assessment generally includes:
The following procedures also may be used when diagnosing dementia:
Some dementias are treatable. However, therapies to stop or slow common neurodegenerative diseases such as AD have largely been unsuccessful, though some drugs are available to manage certain symptoms.
Most drugs for dementia are used to treat symptoms in AD. One class of drugs, called cholinesterase inhibitors, includes donepezil, rivastigmine, and galantamine. These drugs can temporarily improve or stabilize memory and thinking skills in some people by increasing the activity of the cholinergic brain network. The drug memantine is in another class of medications called NMDA receptor agonists, which prevents declines in learning and memory. NMDA receptor agonists work by regulating the activity of the neurotransmitter glutamate. When glutamate activity levels are excessive, neurons may die. Memantine may be combined with a cholinesterase inhibitor for added benefits. These drugs are sometimes used to treat other dementias as well. None of these drugs can stop or reverse the course of the disease.
Currently, there are no cures for the common dementias caused by progressive neurodegeneration, including AD, frontotemporal disorders, and Lewy body dementia. However, some forms of dementia are treatable. A better understanding of dementia disorders, as well as their diagnosis and treatment, will make it possible for affected individuals and their caretakers to live their lives more fully and meet daily challenges. NIH, primarily through research activities funded by NINDS and NIA, continues to make discoveries in the lab, design therapeutic approaches to dementias, and create tools and resources to help speed the development of treatments that can be used in practice. These discoveries may eventually lead to ways to slow disease progression or even cure and prevent the dementias.
Where can I get more information?
For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institute's Brain Resources and Information Network at:
P.O. Box 5801
Bethesda, MD 20824
Information also is available from the following organizations:
Alzheimer's Disease Education and Referral Center (ADEAR)
P.O. Box 8250
Silver Spring, MD 20907-8250
225 North Michigan Avenue, 17th Floor
Chicago, IL 60601-7633
312-335-8700, TDD: 312-335-5886
Alzheimer's Foundation of America
322 Eighth Avenue, 7th Floor
New York, NY 10001
John Douglas French Alzheimer's Foundation
11620 Wilshire Blvd., Suite 270
Los Angeles, CA 90025
Association for Frontotemporal Dementias (AFTD)
Radnor Station Building #2, Suite 320
290 King of Prussia Road
Radnor, PA 19087
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
203-744-0100, Voice Mail 800-999-NORD (6673)
Family Caregiver Alliance/ National Center on Caregiving
180 Montgomery Street, Suite 900
San Francisco, CA 94104
National Institute of Mental Health (NIMH)
National Institutes of Health, DHHS
6001 Executive Blvd. Rm. 8184, MSC 9663
Bethesda, MD 20892-9663
301-443-4513, 866-615-8051, 301-443-8431 (TTY)
National Family Caregivers Association
10400 Connecticut Avenue, Suite 500
Kensington, MD 20895-3944
Lewy Body Dementia Association
912 Killian Hill Road SW
Lilburn, GA 30047
404-935-6444, 800-LEWYSOS (539-9767)
Alzheimer’s Drug Discovery Foundation
57 West 57th Street, Suite 904
New York, NY 10019
Well Spouse Association
63 West Main Street, Suite H
Freehold, NJ 07728
National Respite Network and Resource Center
800 Eastowne Drive, Suite 105
Chapel Hill, NC 27514
National Hospice and Palliative Care Organization /Natl. Hospice Foundation
1731 King Street
Alexandria, VA 22314
703-837-1500 Helpline: 800-658-8898
This information was developed by the National Institute of Neurological Disorders and Stroke.
National Institute of Neurological Disorders and Stroke. Dementia: Hope Through Research. Available at: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Dementia-Hope-Through-Research. Last accessed September 5, 2018.
The information in this document is for general educational purposes only. It is not intended to substitute for personalized professional advice. Although the information was obtained from sources believed to be reliable, MedLink Corporation, its representatives, and the providers of the information do not guarantee its accuracy and disclaim responsibility for adverse consequences resulting from its use. For further information, consult a physician and the organization referred to herein.