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About Alzheimer's & Dementia

Types of dementia Young (Early) Onset

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What is Young Onset dementia? (Also known as “Early Onset Dementia”)

Alzheimer’s disease is not necessarily a disease that affects older people.  About 5% of Canadians currently living with dementia are younger than age 65 years of age. These individuals are often in their 40’s or 50’s; maintaining independent lives, working, supporting their own families when they are diagnosed with Alzheimer’s disease. This can be devastating to the person and his or her family members.

Young-onset dementia is more likely to cause problems with walking, movement, balance or coordination. However, some individuals with young-onset may not have any serious health conditions other than dementia.

There is a greater chance for young-onset dementia to be hereditary in origin. In approximately 10% of individuals with young-onset dementia, the dementia might have been inherited from a parent.

What causes Young Onset Dementia?

Young-onset dementia may be caused by the same diseases that cause dementia in an older individual. There is a wider range of diseases that could cause dementia in a younger individual, including some rare causes of dementia. 

Alzheimer's Disease (AD)

AD is the most common cause of dementia in young individuals, contributing to 30-35% of young-onset dementia cases. Young people are more likely to have am atypical form of the disease that presents with symptoms other than memory loss, such as problems with visual perception (Posterior Cortical Atrophy), speech (logopenic aphasia), decision-making and behaviour (frontal variety).

Approximately 10% of individuals with young-onset dementia have the familial variety of AD that is attributed to mutations in Chromosome 1, 14, and 21.  In these individuals the symptoms manifest in their 30, 40 or early 50s and tend to be more rapidly progressive.

People with Down’s Syndrome (Trisomy 21) tend to develop AD symptoms at an early age because of a mutation on Chromosome 21.  This mutation codes for Amyloid Precursor Protein responsible for causing amyloid plaques in the brain. 

Vascular Dementia

About 15% of young-onset dementia is attributed to vascular dementia caused by interruption of blood flow to the brain. This is the second most common cause of dementia in young people.

When vascular dementia follows a stroke, physical symptoms such as weakness paralysis can appear. Early memory loss in not very common; however, problems with problem solving and decision-making may occur.

A genetic form of vascular dementia is found rarely in people with young-onset dementia. It is known as CADASIL, which stands for Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. It is found in people aged 30-50 years, and the presenting symptoms include migraines, repeated strokes, seizures, low mood, and progressive decline in mental abilities,It is believed to be caused by defect in a gene called NOTCH 3, and is inherited in a pattern similar to familial Alzheimer’s Disease.

Frontotemporal lobe dementia

About 10-15% of young-onset dementia could be due to frontotemporal dementia, which causes progressive damage to the front and sides of the brain. It is usually diagnosed in people between the ages of 45-65.

About 30% of cases of frontotemporal lobe dementia are inherited genetically. Genetic counselling is indicated in such cases.

Three types of presentations of FTD are seen - namely the behavioural variant (that presents initially with personality change, apathy, and inappropriate behaviour) and the language variants - progressive non-fluent aphasia, and semantic dementia.

Alcohol-related brain damage

About 10% of young people with dementia could have alcohol-related brain damage. This includes Korsakoff’s syndrome and alcoholic dementia. It occurs most often in people in their 50’s who regularly consumed excessive amounts of alcohol.

Alcohol-related brain damage results from deficiency of Viatmain B1 (Thiamine), direct injury of nerve cells from alcohol, head injury (falls, fights), and poor diet. The symptoms may be similar to those of Alzheimer’s disease or vascular dementia.

In some people, the symptoms could be halted or even reversed with treatment, abstinence, and a good diet.

Dementia with Lewy Body

About 5% of younger people with dementia could have dementia with Lewy Body, caused by buildup of tiny alpha-synuclein protein deposits in the brain. The common symptoms are fluctuating cognition, visual hallucinations, and symptoms of Parkinson’s disease, including rigidity, stiffness, and sluggish movements.

Rare causes

Around 20 to 25 per cent of younger people with dementia are thought to have some rarer degenerative neurological conditions (where there is progressive damage to the nervous system) such as Huntington's disease, progressive supranuclear palsy, corticobasal degeneration and Creutzfeldt-Jakob disease (CJD).

In most cases these diseases cause problems with movement as well as dementia. Some of these rarer causes of dementia, such as CJD, can progress very rapidly over just a few months.

Other causes of dementia symptoms in younger people include hormone disorders (e.g. thyroid problems, Addison's disease), vitamin B12 deficiencies, inflammatory conditions (e.g. multiple sclerosis) and infections (e.g. HIV). Memory problems can also be caused by sleep apnoea, where breathing stops for a few seconds or minutes during sleep.

 It is important that they are diagnosed because some causes (e.g. vitamin deficiency, thyroid problems, sleep apnoea) can be treated.

What changes can I expect?

The first signs can be similar to those of late-onset Alzheimer's disease, although the sequence in which signs appear varies from person to person. Typical signs include:

  • Personality changes, such as abruptness and insensitivity
  • Frequent lapses of memory, particularly involving recent memories
  • Forgetting appointments or the names of colleagues at work
  • Unsettling moments of disorientation in previously familiar places
  • Being unable to find the way home
  • Becoming confused about familiar tasks such as handling money or placing a call
  • Difficulty finding the right words
  • Difficulty with voluntary movements or physical coordination
  • Struggling to learn new things and adapting to changes at home or work
  • Losing interest in activities that were enjoyed previously
  • Withdrawing from social contact or depression
  • Mood swings, paranoia and fearfulness
Occasionally, a person's symptoms may also include insomnia, verbal or physical aggression, sexual disorders, walking disorders and weight loss.

How is it diagnosed?

Obtaining an accurate diagnosis of young/early onset Alzheimer’s disease can be challenging, and is often delayed.  This is partly because physicians may overlook the possibility of Alzheimer’s disease in a younger person. Many of the early symptoms could be dismissed as a consequence of stress or overwork, and for women as symptoms associated with menopause.
 

Another reason for delay in diagnosis is that the symptoms of young-onset Alzheimer's disease can be varied. Young-onset dementia presents less often with memory changes, and more often with changes in behaviour (apathy, irritability) or personality (loss of empathy).

Even when there is a family history of Alzheimer’s disease, it could take time to confirm the presence of young onset Alzheimer’s disease, leaving family members frustrated. At the time of diagnosis, a person with early-onset Alzheimer’s disease can be in the early, middle or late stages of the disease process. The symptoms are similar to late onset Alzheimer’s disease and can vary from person to person.

To diagnose young/early onset Alzheimer’s disease, the physician  follows the same guidelines as in standard Alzheimer’s disease cases:

  • Examining for the presence of Alzheimer’s disease symptoms
  • Ruling out other possible causes of dementia
 
In addition, the physician will ask about a family history of young onset dementia. If a family history exists, it is helpful for diagnosing young onset familial Alzheimer’s disease at an earlier stage. Since young onset dementia could arise from a variety of causes, the neurologist or psychiatrist would still be working to exclude other potential causes.
Assessment will often include extensive tests of mental abilities, behaviour and daily functioning, a full physical exam and at least one brain scan - often including more specialist scans. A lumbar puncture, a procedure to collect and analyse fluid around the spine, may be useful.
A person who is suspected of having frontotemporal dementia or early-onset Alzheimer's disease and who has a strong family history of that form of dementia may be offered genetic testing. This is to see whether they have a mutation that has caused the dementia. In some cases such 'diagnostic genetic testing' will confirm the dementia type and show that the dementia is genetic.

Unique concerns of individuals diagnosed with young-onset dementia

It is devastating for an individual to be diagnosed with young-onset dementia for several reasons. They are working, supporting their dependent children, and even taking care of their parents.  They might have financial commitments, such as loans and mortgages. Concerns about financial security, fears and uncertainties about their own future and future of their families are very commonly expressed, along with feelings of anger, despair, loss and guilt.

It is often very difficult for families to accept because it is totally unexpected, and therefore, both individual and their family are unprepared. The family must be prepared for a whole range of unexpected challenges, such as giving up full-time work to care for their loved one, take over the family responsibilities previously handled by the newly diagnosed individual, and trying to cope with their own emotions and feelings of loss, despair, frustration, sadness, helplessness, guilt and anger, The stress of balancing their work, family and caregiving responsibilities can be overwhelming for the family caregiver.

It is important to remember that there are resources available in the community for support, and a positive move in this direction is to contact your local Alzheimer Society.  They can provide ongoing support, information, and connect you with programs and services available in the community that will help you and/or your family cope with the disease.

Treatment options

There is no specific treatment available at this time to cure the disease. There are treatment options and support available that can help the individual live well with the condition. The options include medications, non-drug options, activities, and support.

Medication options include the use of standard drugs for Alzheimer’s Disease such as Aricept or donepezil, or certain antidepressants for frontotemporal dementia, for management of symptoms. For vascular dementia, drugs to treat the underlying conditions, such as high blood pressure, diabetes, etc. are often prescribed.

Non-drug approaches are also available, usually through a family physician. Counselling may help the person and their caregiver adjust to the diagnosis or with relationship issues, and with symptoms of depression and/or anxiety.

Engaging in expressive art forms, such as music and art, can help improve mood and self-esteem, and improve quality of life.

Measures that could be taken to slow down the progression of the disease include engaging in regular physical activity or exercise, adopting a healthy diet such as the Mediterranean diet, challenging the brain through a variety of cognitively stimulating tasks, minimizing alcohol consumption, and avoiding cigarette smoking.

Sources: www.alz.org  and www.alzheimer.uk

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