What is dementia?
Dementia is a broad umbrella term used to describe a range of progressive neurological disorders. There are many different types of dementia and some people may present with a combination of types. Regardless of which type is diagnosed, each person will experience their dementia in their own unique way.
Symptoms of dementia can include:
Memory problems – short term memory is often affected and new information is difficult to retain for a person with dementia. People with dementia can get lost in seemingly familiar places and may experience confusion with names. Families may notice that their loved one is increasingly forgetful and loses items regularly. However, we all forget a name or face once in a while and this is nothing to worry about. When noticed on a more frequent basis it is advisable to seek medical advice.
Cognitive ability – people with dementia may experience confusion in environments which are unfamiliar to them. They may have difficulty orientating in time and place, for example, getting up in the middle of the night to go to work, despite being retired. Their ability to focus on specific tasks may be affected, concentration may be difficult to sustain. These symptoms may be noticed in activities such as shopping, where there maybe confusion over goods and payment. Their ability to reason may also be affected. For many people with dementia they get a sense of restlessness and prefer to keep moving than sitting still.
Communication – problems with communicating may be noticed. People with dementia may repeat themselves often or have difficulty finding the right words. Reading and writing may become challenging for a person with dementia. They may experience changes in personality and behaviour, mood swings, anxiety and depression. As a result, people with dementia may lose interest in engaging with others socially. Often following and engaging in conversation can be difficult and tiring and so a formerly outgoing person may become quieter and more introverted. Self-confidence will be affected.
Dementia can be a combination of one or all of the above symptoms, which have been occurring for a period of time and are progressively getting worse. In familiar places it is easier to hide some of the difficulties experienced by people with dementia; plus the symptoms can be seen gradually over time and are initially easily explained away.
Any concerns about forgetfulness or confusion should always be discussed with your GP. There may be many reasons for these symptoms and it is always best to get them checked.
Types of dementia
Alzheimer’s is the most common type of dementia in the UK. Symptoms of Alzheimer’s affect five main areas – memory, cognitive ability, insight, language and spatial awareness. Symptoms may include: poor memory function, particularly in relation to time; inability to recall recent events; general forgetfulness characterised by losing things, inability to recall messages or repeating oneself.
Typically, memory problems are concealed by an increased dependency on routine and familiar environments. Cognitive ability may be impaired in a number of ways including, poor organisational skills, and an inability to perform every day and familiar tasks; impaired decision making and affected conversation. Conversation can appear slow or muddled and difficulty following a television programme may be noticed. Decision making is also affected and poor judgements may be made. Those with early stage Alzheimer’s may show less initiative and have fewer original ideas. It may be noticed that there is an increasing dependency on relatives.
Language is also affected. As Alzheimer’s progresses there may be increasing difficulty in naming objects, the occasional misuse of words may be noticed. These difficulties may also impact on number problems. Miscalculation is common and the ability to manage financial affairs may be impaired. Alzheimer’s disease tends to develop quite slowly over time. As Alzheimer’s progresses people experience increasing confusion when out, and can easily become disorientated. People with Alzheimer’s can encounter difficulty in performing everyday tasks like dressing.
Find out more about Alzheimer’s
Vascular dementia is the second most common type of dementia to be diagnosed. Vascular dementia, also sometimes known as multi-infarct dementia (MID), is the result of small strokes which cause pockets of cell damage in the brain. As a result vascular dementias often follow a more stepwise deterioration followed by a period of relative recovery. This differs from the progression of Alzheimer’s, which is more gradual. Memory may not be as affected by vascular dementia as with Alzheimer’s, but language and communication can be more affected. It is possible to have a combination of Alzheimer’s disease and vascular dementia. This is known as mixed dementia.
Find out more about vascular dementia
Frontotemporal dementia is a gradually progressive condition which predominantly affects behaviour and personality. The regions of the brain which are most affected are those in the front of the brain, just behind the forehead. Frontotemporal dementia is sometimes called frontal lobe or Pick’s disease. This form of dementia is more commonly, but not exclusively, found in younger people, between ages 45 and 65. It is a relatively uncommon form of dementia and can be difficult to diagnose. Frontotemporal dementia can cause disinhibition and inappropriate behaviour, particularly in public. Eating patterns can also be affected, with people suddenly ‘bingeing’ on food. This form of dementia may be confused with depression, psychosis or obsessive compulsive disorder.
Find out more about Frontotemporal dementia
Dementia with Lewy Bodies
In dementia with Lewy bodies, cognitive impairment can fluctuate and movements are particularly affected, with poor motor control. A person with Lewy bodies might shuffle as they walk and be more prone to falls. Tremors may be noticed, similar to those experienced by people who have Parkinson’s disease. Due to the nerve cell damage of Lewy bodies, hallucinations are often present in those with this type of dementia. These hallucinations can be both visual and auditory. Memory is often less affected than with other types of dementia, but a person might experience sudden bouts of confusion which can change on an hourly basis. Swallowing can also be affected as can sleep patterns. People with Lewy bodies tend to fall asleep easily during the day, but then experience disrupted sleep at night, often having intense dreams or nightmares. As with all dementias, Lewy bodies is progressive and symptoms will worsen over time.
Find out more about Lewy Body Dementia
Wernicke-Korsakoff syndrome (WKS) is a type of brain disorder caused by a lack of vitamin B-1. The syndrome is actually two separate conditions that can occur at the same time. Usually, people get the symptoms of Wernicke’s encephalopathy first.
Also called Wernicke’s disease, people with Wernicke’s encephalopathy have bleeding in the lower sections of the brain, including the thalamus and hypothalamus. These areas of the brain control the nervous and endocrine systems. The bleeding causes brain damage that presents symptoms involving your vision, coordination, and balance.
The signs of Korsakoff psychosis tend to follow as the Wernicke’s symptoms decrease. If Wernicke’s disease is treated quickly and effectively, Korsakoff syndrome may not develop. Korsakoff psychosis is the result of chronic brain damage. Korsakoff syndrome affects the areas of your brain that control memory.
Alcoholism, or chronic alcohol abuse, is the most common cause of WKS. WKS can also be linked to diet deficiencies or other medical conditions that impair vitamin B-1 absorption.
Find out more about Wernicke-Korsakoff
Creutzfeldt-Jakob (CJD) is one of the rarest forms of dementia. Only 1 in 1 million people are diagnosed with it every year, according to the Alzheimer’s Association. CJD progresses very quickly, and people often die within a year of diagnosis.
Symptoms of CJD are similar to other forms of dementia. Some people experience agitation, while others suffer from depression. Confusion and loss of memory are also common. CJD affects the body as well, causing twitching and muscle stiffness.
Find out more about Creutzfeldt-Jakob
Normal Pressure Hydrocephalus
Normal pressure hydrocephalus (NPH) is a condition that causes a person to build up excess fluid in the brain’s ventricles. The ventricles are fluid-filled spaces designed to cushion a person’s brain and spinal cord. They rely on just the right amount of fluid to work properly. When the fluid builds up excessively, it places extra pressure on the brain. This can cause damage that leads to dementia symptoms.
Find out more about Normal Pressure Hydrocephalus
Mixed dementia refers to a situation where an individual has more than one type of dementia. Mixed dementia is very common, and the most common combination is vascular dementia and Alzheimer’s. Many people living with dementia may have mixed dementia but don’t know it.
Mixed dementia can cause different symptoms in different people. Some people experience memory loss and disorientation first, while others have behaviour and mood changes. Most people living with mixed dementia will have difficulty speaking and walking as the condition progresses.
Some of the rarer types of dementia include: Familial Alzheimer’s (fAD) familial Frontotemporal Dementia (fFTD)
Posterior Cortical Atrophy (PCA) Primary Progressive Aphasia (PPA)
To find out more about rare forms of dementia visit Rare Dementia Support
For more information about understanding dementia, please visit Dementia UK
What is Primary Progressive Aphasia (PPA)?
Primary Progressive Aphasia (PPA) is a neurological syndrome in which language capabilities become slowly and progressively impaired. Unlike other forms of aphasia that result from stroke or brain injury, PPA is caused by neurodegenerative diseases, such as Alzheimer’s Disease or Frontotemporal Lobar Degeneration. PPA results from deterioration of brain tissue important for speech and language. Although the first symptoms are problems with speech and language, other problems associated with the underlying disease, such as memory loss, often occur later.
PPA commonly begins as a subtle disorder of language, progressing to a nearly total inability to speak, in its most severe stage. The type or pattern of the language deficit may differ from patient to patient. The initial language disturbance may be fluent aphasia (i.e., the person may have normal or even increased rate of word production) or non-fluent aphasia (speech becomes effortful and the person produces fewer words). A less common variety begins with impaired word-finding and progressive deterioration of naming and comprehension, with relatively preserved articulation.
As with aphasia that results from stroke or brain trauma, the manifestations of PPA depend on what parts of the left hemisphere are relatively more damaged at any given point in the illness. The person may or may not have difficulty understanding speech. Eventually, almost all patients become mute and unable to understand spoken or written language, even if their behavior seems otherwise normal.
Signs and symptoms of other clinical syndromes are not found through tests used to determine the presence of other conditions. PPA is not Alzheimer’s disease. Most people with PPA maintain ability to take care of themselves, pursue hobbies, and, in some instances, remain employed.
Is there any treatment or assistance for people with PPA?
People with primary progressive aphasia are fighting against a condition in which they will continue to lose their ability to speak, read, write, and/or understand what they hear. Usually people with aphasia that results from stroke or head injury will experience improvement over time, often aided by speech therapy. This is not the case for people with primary progressive aphasia. However, individuals with PPA may benefit during the course of their illness by acquiring new communication strategies from speech-language pathologists. Some families have also learned new strategies through participation in Aphasia Community Groups.
Many people with aphasia find it helpful to carry identification cards and other materials that can help explain the person’s condition to others. ID cards are available from the the National Aphasia Association website. Some communication-assistive devices may also be helpful. Non-verbal techniques for communicating, such as gesturing and pointing to pictures, may help people with PPA express themselves.
More information about Primary Progressive Aphasia (PPA)
Posterior Cortical Atrophy (PCA)
Posterior cortical atrophy (PCA) refers to gradual and progressive degeneration of the outer layer of the brain (the cortex) in the part of the brain located in the back of the head (posterior). It is not known whether PCA is a unique disease or a possible variant form of Alzheimer’s disease. In many people with PCA, the affected part of the brain shows amyloid plaques and neurofibrillary tangles, similar to the changes that occur in Alzheimer’s disease but in a different part of the brain. In other people with PCA, however, the brain changes resemble other diseases such as Lewy body dementia or a form of Creutzfeld-Jacob disease. Most cases of Alzheimer’s disease occur in people age 65 or older, whereas the onset of PCA commonly occurs between ages 50 and 65.
For more information about Posterior Cortical Atrophy (PCA)
Binswanger's disease (BD), also called subcortical vascular dementia, is a type of dementia caused by widespread, microscopic areas of damage to the deep layers of white matter in the brain. The damage is the result of the thickening and narrowing (atherosclerosis) of arteries that feed the subcortical areas of the brain. Atherosclerosis (commonly known as "hardening of the arteries") is a systemic process that affects blood vessels throughout the body. It begins late in the fourth decade of life and increases in severity with age. As the arteries become more and more narrowed, the blood supplied by those arteries decreases and brain tissue dies. A characteristic pattern of BD-damaged brain tissue can be seen with modern brain imaging techniques such as CT scans or magnetic resonance imaging (MRI). The symptoms associated with BD are related to the disruption of subcortical neural circuits that control what neuroscientists call executive cognitive functioning: short-term memory, organization, mood, the regulation of attention, the ability to act or make decisions, and appropriate behaviour. The most characteristic feature of BD is psychomotor slowness - an increase in the length of time it takes, for example, for the fingers to turn the thought of a letter into the shape of a letter on a piece of paper. Other symptoms include forgetfulness (but not as severe as the forgetfulness of Alzheimer's), changes in speech, an unsteady gait, clumsiness or frequent falls, changes in personality or mood (most likely in the form of apathy, irritability, and depression), and urinary symptoms that aren't caused by urological disease. Brain imaging, which reveals the characteristic brain lesions of BD, is essential for a positive diagnosis.
The ischemic brain damage in Binswanger disease is not reversible, so treatment is focused on reducing risk factors for stroke, thereby retarding progression of the disease. Treatment usually involves the use of anti-hypertensive drugs to control blood pressure, antiplatelet drugs (e.g., aspirin) or warfarin to reduce thromboembolism, statins to reduce atherosclerosis, smoking cessation and diabetic control. Other treatment is symptomatic and supportive.
Dr. Otto Binswanger was born on October 14, 1852, in Switzerland and died on July 15, 1929. In 1894, he noted a condition he called "encephalitis subcorticalis chronica progressiva" which later would be termed Binswanger's disease by Alois Alzheimer.
CADASIL is a rare genetic disorder affecting the small blood vessels in the brain. The age of onset, severity, specific symptoms and disease progression varies greatly from one person to another, even among members of the same family.
CADASIL is an acronym that stands for:
(C)erebral - relating to the brain
(D)ominant - a form of inheritance in which one copy of an abnormal gene is necessary for the development of a disorder (A)rteriopathy - disease of the arteries (blood vessels that carry blood away from the heart)
(S)ubcortical - relating to specific areas of the brain supplied by deep small arteries
(I)nfarcts - tissue loss in the brain caused by lack of blood flow to the brain, which occurs when circulation through the small arteries is severely reduced or interrupted
(L)eukoencephalopathy - lesions in the brain white matter caused by the disease and observed on MRI
Hallmark symptoms of CADASIL may include:
2) cognitive impairment
3) migraine with aura
4) psychiatric disturbances.
These symptoms are caused by damage to small blood vessels, especially those within the brain. The specific symptoms and severity of the disorder can vary greatly among affected individuals, even among members of the same family.
Despite this variability, most individuals (approximately three out of four patients) experience recurrent stroke or transient ischemic attacks (TIAs), beginning at 40-50 years of age.
Strokes cause arm and leg weakness, loss of feeling of one part of the body, speech difficulties or lack of coordination. TIAs result in similar symptoms as strokes but resolve in less than 24 hours. Repeated strokes can cause progression of symptoms listed above and also cause cognitive disturbances, loss of bladder control (urinary incontinence) or loss of balance.
Although strokes are the most common symptom associated with CADASIL, some affected individuals never have strokes. It is not uncommon for CADASIL patients to have evidence of stroke on MRI without any symptoms (silent strokes).
Cognitive impairment eventually develops in many affected individuals on average between the ages of 50-60, although the progression of the disease will vary. Symptoms may include difficulty with concentration, deficits in attention span or memory dysfunction, difficulty making decisions or solving problems, and general loss of interest (apathy). With age, continued cognitive decline may result in dementia, a progressive loss of memory and decline in intellectual abilities that interferes with performing routine tasks of daily life.
Migraine with aura may be a predominant symptom in some affected individuals, occurring in at least half of those with CADASIL. Migraines are severe headaches that often cause excruciating pain and can be disabling. In individuals with CADASIL, these headaches are often preceded by abnormal feelings called “aura.”
These additional symptoms usually affect vision and may consist of the sudden appearance of a bright light in the centre of the field of vision (scintillating scotoma) or, less frequently, disturbances in all or part of the field of vision. The auras preceding the migraine usually last 20 to 30 minutes but are sometimes longer. Some patients suffer from severe attacks with unusual symptoms such as confusion, fever or coma.
Finally, many individuals with CADASIL develop psychiatric abnormalities ranging from personality and behavioral changes to severe depression.
CADASIL is caused by mutations of the NOTCH3 gene. This mutation is inherited as an autosomal dominant trait. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. Most individuals with CADASIL have a parent with the disorder but CADASIL can be due to a spontaneous genetic mutation that occurs for unknown reasons (de novo mutation). In these cases, there is no previous family history of the disorder. The risk of passing the abnormal gene from affected parent to offspring is 50 percent for each pregnancy regardless of the gender of the child.
The NOTCH3 gene contains instructions to create a protein that is predominantly expressed in smooth muscle cells in the walls of small arteries. Mutations in the NOTCH3 gene result in abnormal accumulation of this protein at the surface of smooth muscle cells. Ultimately, NOTCH3 mutations lead to progressive damage to the small blood vessels in the brain, premature destruction of smooth muscle cells, reduced blood flow to the brain and tissue loss within the white matter and the deeper parts of the brain, by means of a mechanism not entirely understood.
Symptoms of the following disorders can be similar to those of CADASIL. Comparisons may be useful for a differential diagnosis.
Sporadic cerebral small vessel disease (SVD) related to age and hypertension is a progressive and common neurological disorder characterized by dysfunction of blood vessels supplying the white-matter and deep structures of the brain. As in CADASIL, patients can experience progressive memory loss, deterioration of intellectual abilities and loss of balance with a progressive worsening of these symptoms, but symptoms are usually less severe and occur later in life.
A variety of rare genetic disorders may have symptoms similar to those found in CADASIL. These disorders include cerebral autosomal recessive arteriopathy with subcortical infarcts and leukodystrophy (CARASIL), mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), Fabry disease, and a variety of leukodystrophies, rare progressive metabolic disorders that affect the brain, spinal cord and often the peripheral nerves.
Multiple sclerosis is a prevalent chronic neuroimmunologic (both the nervous system and the immunological system are involved) disorder of the central nervous system involving the brain, spinal cord and optic nerves. By means of a mechanism not clearly understood, the protective fatty, insulating substance called myelin sheath that covers the nerve is destroyed. The inflammatory attacks that produce the characteristic scarring (plaques or patches) of the myelin sheath occur randomly at multiple sites and vary in intensity. The course of the disease may advance, relapse, remit, or stabilize. The randomness of the location of plaques or patches causes a wide range of neurological symptoms, which may vary from person to person.
CADASIL is suspected based on symptoms, family history, and brain MRI lesions compatible with the disease. Although MRI can identify characteristic changes in the brains of individuals with CADASIL, such changes are not unique to CADASIL and can occur with other disorders. As such, the CADASIL diagnosis can only be confirmed by testing blood samples for characteristic mutations in the NOTCH3 gene. In some cases, a skin biopsy can be used to diagnosis CADASIL.
At the present, there is no treatment that can cure the disease or prevent its onset. However, it is possible to treat symptoms as they occur to improve the patient’s quality of life. For example, migraines can be treated with traditional analgesics such as paracetamol, ibuprofen, aspirin and NSAIDs. Other medicines commonly used to treat migraine such as vasoconstrictors (especially triptans) are not recommended for patients with CADASIL. Aspirin may be used as preventive treatment after the first stroke has occurred.
Dementia Research - Colin & Irene's Story
Colin has Posterior Cortical Atrophy (PCA), a rare type of dementia. He and his wife Irene explain how life has changed since the diagnosis in 2012 and why they have been taking part in various research studies with Humber NHS Foundation Trust.