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Both Creutzfeldt-Jakob Disease (CJD) and its close relative Variant Creutzfeldt-Jakob Disease (vCJD) are caused by Prions, incorrectly folded proteins, which destroy brain tissue. Neither are infectious but types of CJD can be spread by exposure to infected blood products, meat or medical instruments.

BSE (Bovine Spongiform Encephalopathy) or ‘Mad Cow Disease’ is a form of Transmissible Spongiform Encephalopathy (TSEs). The ‘spongiform’ in the name refers to the fact that the infected brain is filled with holes, resembling a sponge.

CJD is a degenerative brain disorder, estimated to affect one in a million people annually worldwide. Appearing in later life usually, the onset is rapid and fatal. Early symptoms include problems with vision, coordination problems, changes in behaviour, and memory problems, which lead to blindness, involuntary movements and coma.

vCJD was acquired by humans by exposure to meat infected with BSE. It is estimated that only 1% of CJD is ‘variant’. It was discovered in 1996 in the UK and, according to the government, 260 cases have been reported since then.

The Different Types of CJD

When it comes Creutzfeldt-Jakob Disease, there are 4 types:

1. Sporadic CJD

• Sufferers have no known risk factors.
• Most common type of CJD
• 85% of all CJD cases

2. Hereditary CJD

• Family history of the disease
• 10 to 15% of CJD cases

3. Acquired or Iatrogenic CJD

• Transmitted by exposure to bodily tissue during medical procedures
• Improperly sterilised medical instruments are also thought to spread this
• Before 1985, was spread by a treatment using glands from deceased humans

4. Variant CJD (vCJD)

• Caused by consuming meat from a cow infected with BSE AKA ‘Mad Cow Disease’
• 1% of all CJD cases
• Possible 10 year+ incubation period

A lot of young and middle-aged people joke when they forget something or miss an object that is right in front of them or say the wrong word, that they have dementia. Even though it is relatively rare, Young Onset Dementia is a serious concern.

Affecting people under 65 years of age, Young Onset Dementia, also known as early onset or working-age dementia, is so-called because of the old belief that dementia was an affliction that only affected the elderly.

Dementias that affect the young are seemingly rare but this may be because younger people cannot understand or are unwilling to believe that the malady affecting them is actually Dementia. They are much less likely to visit a doctor when they are presenting symptoms of dementia and, as sad as it is, doctors may be missing the signs too.

Who is likely to suffer from Young Onset Dementia?

Young Onset Dementia sufferers are more likely to have inherited a genetic form, for example, frontotemporal dementia presents in 40% with a family history of the disease.

Other groups at higher risk are people from BAME backgrounds, who are less likely to receive a timely diagnosis, and people with learning disabilities are also at greater risk of developing Young Onset.

Alzheimer’s disease accounts for about 1/3 of all Young Onset sufferers and vascular dementia affects 20% of Young Onset sufferers. 10% of Young Onset sufferers have Lewy Body dementia. 20% have something rarer, like Parkinson’s or Creutzfeldt Jakob.

Early Onset Dementia affects around 42,325 people in the UK, which is 5% of all dementia sufferers in the UK. The actual number may be as high as 6-9%. That is a pretty shocking statistic and a surprise for most people reading this. It is important that people, no matter their age, receive the correct diagnosis and are given the most up-to-date treatment.

Some symptoms that the majority of people would ascribe to Dementia can be brought on by other conditions, so it is vitally important that this is taken into consideration during diagnosis. Here is our guide to help you tell the conditions apart.

Urinary Tract Infection

A Urinary Tract Infection can cause a sudden bout of confusion in an older person. Rapid changes in behaviour like agitation, inability to communicate properly and withdrawal are symptoms. If the person has complained of pelvic pain, a fever, a burning during urination or they use a catheter, UTI may be the culprit. The reason for the confusion symptom is due to the UTI overwhelming the kidneys and entering the bloodstream, causing blood poisoning which affects the brain. This is a serious condition and can be life-threatening.

Subdural Hematoma

The dura is the thin membrane that covers the brain. Blood trapped in between the membrane and the brain, caused by injury and bruising, can be mistaken for Alzheimer’s. The symptoms can emerge slowly, adding to the confusion and inhibiting a correct diagnosis. If the person has had a fall prior to the onset of symptoms, it may be a Subdural Hematoma.

Depression

Signs of depression can have the appearance of Dementia. The area of overlap is large including a lack of interest in subjects the person is usually passionate about, disrupted concentrating, forgetfulness, withdrawal and excessive sleeping. The main differences between the two are that those suffering from depression display less severe symptoms, ones that come and go, and do not reference suicide as much as Dementia sufferers. Obviously, that is not a lot to go on, so diagnosis is the only real way to differentiate.

Delirium

Although the symptoms of Delirium and Dementia are similar, the difference is that Dementia and other associated neurological conditions emerge over an extended period of time and Delirium starts suddenly. Things to look out for are an unusual amount of confusion, feelings of paranoia, hallucinations, rambling, behavioural changes, distraction, disorientation and excessive alertness problems – either drowsiness or agitation. Triggers can be clashes in medication or an underlying, untreated infection. Of course, people suffering from Dementia can also display these symptoms.

Side Effects

Medicines can create a variety of side effects which can be confused with dementia. The list includes, but is not limited to, sedatives, antidepressants, anti-anxiety medication, anticonvulsants, corticosteroids and antihistamines.

Vitamin Deficiency

A lack of Vitamin B1, Vitamin B6, Folic Acid and Niacin have been linked to dementia-esque symptoms. Alcoholics can succumb to Wernicke-Korsakoff syndrome due to a long-term lack of vitamin B1. Deficiency of B12 can lead to pernicious anaemia which is also mistaken for Dementia, and as the liver stores B12 (as well as vitamins A, D and Iron), liver damage can precipitate dementia-like effects.

Hypothyroidism

Too much (hyperthyroidism) or too little (hypothyroidism) thyroid activity can mimic some of the symptoms of dementia.

Hypothyroidism happens when the gland can’t produce enough hormones. This may be due to many factors such as an autoimmune disease. Individuals experiencing hypothyroidism experience declining mental sharpness, depression, ‘foggy brain’, difficulty concentrating, mood swings, visual-spatial skills and memory loss amongst others. Other symptoms that could be clues as to whether you have hypothyroidism are difficulty losing weight/water retention, brittle fingernails, hair loss, sensitivity to cold, enlarged tongue and enlarged thyroid gland.

The most likely cause of hyperthyroidism is also autoimmune related. This causes the gland to secrete too much hormone. The symptoms which cause it to be confused with more serious neurological conditions are tremors, nervousness, agitation, anxiety, poor concentration, lack of spatial awareness, eye problems and slowed reactions.

These conditions can be reversed a lot of the time, so if you or someone you know is displaying these symptoms, seek a diagnosis.

Normal Pressure Hydrocephalus (NPH)

NPH is a brain disorder, affecting those in their 60s and 70s primarily, where excess cerebrospinal fluid enters the brain ventricles and destroys brain tissue, causing dysfunction which leads to dementia-like symptoms like reasoning problems and walking problems. It can be treated with surgery which improves the mobility aspects but has little positive effects on the sufferers reasoning.

Brain Tumour

Malignant or benign tumours can disrupt the flow of information around the brain in a similar fashion to that of dementia. Two of the most common are changes in personality and disruption of cognitive function. These can depend on the position of the tumour within the brain and there can be mimicry of many different symptoms of dementia. Symptoms of a brain tumour are increased pressure in the brain, seizures, headaches, vomiting, changes in senses. Brain tumours can be deadly and if you suspect you have one, seek treatment immediately.

Stroke

Strokes happen when the blood flow to the brain is interrupted. Cognitive abilities are suddenly disrupted. This may be the key to recognising the difference between Dementia and the stroke version known as Vascular Dementia. The similar symptoms between the two are memory loss, confusion, hallucinations and impaired motor skills. Strokes can be treated by lowering blood pressure or cholesterol with medication.

If you are unsure, do not worry. Some health professionals do not know the difference either, so you are not alone.

Dementia is an umbrella term for serious brain conditions that affect reasoning, focus, memory, communication and visual perception. Some types of serious brain conditions are named Dementia such as Vascular Dementia, Lewy Body Dementia and Frontotemporal Dementia. Alzheimer’s is a type of Dementia too.

Alzheimer’s is a degenerative brain disease that leads from difficulty remembering and apathy to difficulty speaking and walking.

To make things even more confusing for people finding out about Dementia, it is possible to have two types of Dementia at the same time. Getting the correct diagnosis is so important to aide the correct treatment.

Just remember: Alzheimer’s is a form of Dementia but Dementia is not a form of Alzheimer’s.