This post is on the rarer forms of dementia, which make up the minority of dementia diagnoses. These also include reversible and treatable dementias, such as those resulting from infectious diseases or nutrition deficiencies. As Alzheimer’s disease is the most commonly diagnosed form of dementia, it receives the majority of focus in awareness raising, research, and funding for treatment and prevention. This can leave those with the rarer forms of dementia without much information on their dementia type or options for treatment, fewer disease-specific support options, and feeling frustrated and isolated. My hope is that this post will be informative and raise your awareness on other types of dementia that individuals and families face. Continue reading
Tag Archives: rare forms of dementia
What is Posterior Cortical Atrophy (PCA)?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Posterior Cortical Atrophy?
Posterior cortical atrophy (PCA), also known as Benson’s syndrome, is a rare degenerative condition in which damage occurs at the back (posterior region) of the brain. In the vast majority of people, the cause of PCA is Alzheimer’s disease.
The first symptoms of PCA tend to occur when people are in their mid-50s or early 60s. However, the first signs are often subtle and so it may be some time before a formal diagnosis is made.
Initially, people with PCA tend to have a relatively well-preserved memory but experience problems with their vision, such as difficulty recognising faces and objects in pictures. They may also have problems with literacy and numeracy. These tasks are controlled by the back part of the brain, where the initial damage in PCA occurs. Continue reading
How are Niemann-Pick disease type C (NPC) and dementia related?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
But first, a little bit from the University of Notre Dame:
The National Institutes of Health refers to Niemann-Pick disease type C as “childhood Alzheimer’s” because of similarities in the brains of NPC and Alzheimer’s disease patients. Symptoms can include deterioration of memory and balance, lung and liver failure, delayed motor development, and seizures. The disease usually appears in early childhood, leading to neurological problems that are typically fatal.
From the Addi & Cassi Fund webpage (the twins with NPC pictured, above):
Every person in the world is born with the Niemann Pick Type C gene and could not live without it. The NPC1 gene is located on Chromosome 18 and the gene regulates human cholesterol metabolism at the cellular level. We were born with major problems on this cholesterol gene which in turn causes our fatal disease.
Some doctors believe Niemann Pick Type C and Alzheimer’s could be connected due to cholesterol and lipid issues (see The Scientist Magazine article, November 2008) while virus researchers have discovered that the Niemann Pick Type C gene helps both HIV-AIDS and Ebola infect humans. We are told by top scientists that because our cholesterol gene does not work, we can’t get infected with many viruses that infect people who have healthy functioning NPC genes.
According to Dr. Francis S. Collins, Director of the NIH, “While many of the genes we will initially be pursuing are responsible for rare disorders, what we learn from rare disorders often has profound consequences for our understanding of more common conditions.”
When I was writing my thesis on technologies for dementia, the youngest case of dementia that I found recorded was in a 6 year old girl who had Niemann-Pick disease type C – you can read about it in this article from Alzheimer Society, starting on page 16. You can also read one of my other posts about a 12-year old girl with this disorder here (og på dansk her). Continue reading
What is Progressive supranuclear palsy (PSP)?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Progressive supranuclear palsy?
Progressive supranuclear palsy (PSP) is a rare progressive movement disorder, sometimes known as Steele-Richardson-Olszewski syndrome. It affects many areas of the brain and people typically have symptoms similar to those of Parkinson’s disease. As with Parkinson’s disease, there may be a tremor (involuntary shaking of the body and limbs), but this is much less prominent in PSP. The specific parts of the brain that are damaged include the regions that control eye movements and those that keep a person steady when they are walking, resulting in frequent falls. The cause of the damage that occurs in PSP is unknown, but is linked to abnormal deposits of a protein called tau.
PSP mainly occurs in people over the age of 60, although it occasionally affects younger people. One striking symptom is paralysis of eye movements and problems with double vision. Other symptoms include stiff or slow movements, difficulties walking and speaking, swallowing problems and personality changes. Continue reading
What is Korsakoff’s syndrome?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Korsakoff’s syndrome?
Korsakoff’s syndrome is a brain disorder usually associated with heavy alcohol consumption over a long period. Although Korsakoff’s syndrome is not strictly speaking a dementia, people with the condition experience loss of short-term memory. This factsheet outlines the causes, symptoms and treatment of the syndrome. This factsheet also explains the possible relationship between Korsakoff’s syndrome and ‘alcohol related dementia’. Alcohol consumption is increasing in the UK and therefore these conditions are expected to become more common in the future. Continue reading
How are multiple sclerosis (MS) and dementia related?
A bit of information from Medical News Today:
Cognitive Function – according to the Multiple Sclerosis Resource Center, UK, about 50% of people with MS say they experience cognitive problems to some extent, increasing to 80% among the most severe cases. The most commonly reported cognitive abnormalities include problems with memory, abstraction, attention and word finding.
Alzheimer Europe has some good information on MS and symptoms of dementia. You can access their page by clicking on the title, below.
Cognitive Dysfunction in Multiple Sclerosis
by Clive Evers
There has been a lot of recent research into changes in cognition due to MS and it is now evident that such changes do occur and that they are more common than was previously thought. Cognition is about our abilities in thinking things through and how well our memory works.
Cognition is also about how to focus and to maintain our attention; the way we learn and remember new things; how we think reason and solve problems. It also concerns how we plan and carry out our activities; the way we understand and use language and how well we recognise objects, assemble things together and judge distances. Continue reading
What is Huntington’s disease?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Huntington’s disease?
Huntington’s disease can cause one of the rarer forms of dementia. Rarer forms of dementia account for only around 5 per cent of all dementia cases in the UK.
Huntington’s disease is an inherited disease causing abnormal movements and problems with coordination. Other symptoms include mood problems and cognitive impairment that gets progressively worse over time. The age of onset and the course of the disease varies for each person, and dementia can occur at any stage of the illness. Continue reading
What is HIV-associated neurocognitive disorder (HAND)?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is HIV-associated neurocognitive disorder (HAND)?
HIV (human immunodeficiency virus) causes an infection that weakens the immune system, making it harder for the body to fight infections and disease. HIV infection can cause a number of different problems in the brain, which affect up to half of people with HIV. This is known as HIV-associated neurocognitive disorder (HAND). HAND is one of the rarer forms of dementia. Rarer forms account for only around 5 per cent of all dementia cases in the UK.
Difficulties with memory, thinking and reasoning (aspects of cognition) are common with HIV, but they are usually mild and dementia is much rarer. Before the use of antiretroviral drugs (medication that helps to control HIV), around 20-30 per cent of people with advanced HIV infection previously developed dementia. This figure has now decreased to around 2 per cent. Continue reading
What is Corticobasal degeneration (CBD)?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Corticobasal degeneration?
Corticobasal degeneration is one of the rarer forms of dementia. Rarer forms account for only around 5 per cent of all dementia cases in the UK.
Corticobasal degeneration (CBD) is a rare disease in which parts of the brain become damaged and begin to shrink. The outer layer of the brain, known as the cortex, and deep parts of the brain, called the basal ganglia, are both affected. It is not yet known what causes CBD but producing too much of an abnormal form of a protein called tau is thought to play a role. The disease usually affects people between the ages of 60 and 80. Continue reading
What is Creutzfeldt-Jakob disease (CJD)?
This information comes from Alzheimer’s Society, a wonderful organization based out of the UK. They are one of my go-to sources for information and I highly recommend taking a look around their website. They offer the information on their website to be freely used by others, so I am posting the information in full. You can access the same article on their website as well as resources on dementia by clicking on the title, below.
What is Creutzfeldt-Jakob disease?
Creutzfeldt-Jakob disease (CJD) is one of the rarer forms of dementia. Rarer forms account for only around 5 per cent of all dementia cases in the UK.
CJD is caused by an abnormally shaped protein called a prion infecting the brain. Sporadic CJD, which normally affects people over 40, is the most common form of the disease. It is estimated that the disease affects about one out of every 1 million people each year. It is not known what triggers sporadic CJD, but it is not known to be inherited or otherwise transmitted from person to person. Continue reading
