Young-Onset Dementia

Dementia occurring in people below the age of 65 years is generally referred to as young-onset dementia.

Young-onset accounts for about two to eight per cent of all cases of dementia.

People diagnosed with young onset dementia are often in their 40’s or 50’s and have independent lives, careers and are often supporting their own families when diagnosed. This can feel devastating for them and their family members.

Young-onset dementia is more likely to cause problems with walking, movement, balance or coordination. However, some individuals with young-onset dementia 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 about ten per cent of people, 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 person, including some rare causes of dementia. 

Alzheimer’s Disease (AD)

  • AD is the most common cause of young-onset dementia in younger people, contributing to 30-35 per cent of cases. Younger people are more likely to have an 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).
  • About 10 per cent of people with young-onset dementia have the familial variety of AD that is attributed to mutations in genes Presenilin 1 (PSEN1), Presenilin 2 (PSEN2), and Amyloid Precursor Protein (APP) genes present on chromosome 14, 1, and 21. The symptoms manifest in their 30s, 40s or early 50s and tend to be more rapidly progressive.
  • People with Down’s syndrome (Trisomy 21) tend to develop Alzheimer’s disease symptoms at an early age because of mutation of APP gene present on Chromosome 21 that codes for Amyloid Precursor Protein which is responsible for causing amyloid plaques in the brain. 

Vascular Dementia

  • About 15 per cent of young onset dementia is attributed to vascular dementia caused by an interruption of blood flow to the brain. This is the second most common cause of dementia in younger people. 
  • When vascular dementia follows a stroke, physical symptoms such as weakness or paralysis can appear. Early memory loss is not very common; however, problems with planning, organizing and executing tasks, problem-solving and decision-making may occur.
  • A genetic form of vascular dementia is found rarely in people with young-onset dementia, known as CADASIL (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 a 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 per cent of young-onset dementia could be due to frontotemporal dementia which causes progressive degeneration of the frontal and temporal lobes of the brain. It can occur in people between the ages of 30 - 70.
  • About 30 per cent of cases of frontotemporal lobe dementia is inherited genetically. Genetic counselling is an option in such cases.
  • Two 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 per cent of younger people with dementia may have alcohol-related brain damage. This includes Korsakoff’s syndrome and alcoholic dementia. It occurs most often in people in their 50s who regularly consume excessive amounts of alcohol. 
  • Alcohol-related brain damage results from a deficiency of Vitamin B1(Thiamine); direct injury of nerve cells from alcohol, head injury (E.g. falls) and poor diet. The symptoms may be similar to those of Alzheimer’s disease or vascular dementia. 
  • The symptoms of Korsakoff’s syndrome could be halted or even reversed with treatment, abstinence and a good diet.

Lewy body dementia

  • About five per cent of younger people with dementia could have dementia with Lewy bodies, caused by a 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 in 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). In rare cases, memory problems can also be caused by sleep apnea where breathing stops for a period of time during sleep.
  • It is important that these conditions are diagnosed because some of these conditions (E.g. vitamin deficiency, thyroid problems, and sleep apnea) can be treated.

What changes can I expect?

  • The first signs of young-onset dementia 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 at work
    • Losing interest in activities that were enjoyed previously
    • Withdrawing from social contact 
    • 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-onset dementia can be challenging and is often delayed. This is partly because physicians might overlook the possibility of Alzheimer’s disease in a younger person. Many of the early symptoms could be dismissed because of stress or overwork and for women as symptoms associated with menopause. 

Another reason for the delay in diagnosis is that the symptoms of young onset dementia can be varied. It 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-onset dementia, 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
  • The physician will ask about a family history of young-onset dementia. If a family history exists, it is helpful to diagnose young-onset familial dementia 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 specialized scans. A lumbar puncture, a procedure to collect and analyze fluid around the spine, may be useful. 
  • A person who is suspected of having frontotemporal dementia or early-onset Alzheimer's disease and has a strong family history of that type of dementia may be offered genetic testing. This is to see whether they have a mutation that has caused dementia. In some cases, such 'diagnostic genetic testing' will confirm the dementia type and show that dementia is genetic.

Unique concerns related to young-onset dementia

It can be devastating for a person to be diagnosed with young-onset dementia for several reasons. They are working, supporting their children, and at times, even taking care of their parents. Concerns about financial security, fears and uncertainties about their own future and the future of their family 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 the person and their family are unprepared. The family will need to prepare for a whole range of unexpected challenges, such as potentially giving up full-time work to care for their loved one, and taking on the new responsibilities. Coping with emotions and feelings of loss, despair, frustration, sadness, helplessness, guilt and anger is also a huge challenge. The stress of balancing work, family, and caregiving responsibilities can be overwhelming for the family caregiver.

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Is there a cure? What treatment options are available?

Currently, there is no specific treatment available to cure the disease. There are treatment options and support to help the person and their family manage 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 control 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 the family physician. Counselling may help the person and their caregiver cope with the diagnosis or with relationship issues and with symptoms of depression and anxiety. 

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

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.

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