Rare types of dementia

Most people have heard about the more common types of dementia such as Alzheimer’s disease and vascular dementia. This page covers some of the rare types of dementia.

Some of the rare types of dementia include:

Parkinson’s disease dementia is caused by deposits of an abnormal protein (called Lewy bodies). Parkinson’s disease dementia is diagnosed when cognitive symptoms (i.e. dementia) develop more than a year after the onset of movement problems (i.e Parkinson’s disease). Parkinson’s disease dementia is one of the two types of Lewy body dementias.

Corticobasal degeneration (CBD) is a progressive neurological disorder characterised by nerve cell loss and atrophy (shrinkage) of multiple areas of the brain including the cerebral cortex and the basal ganglia.

Creutzfeldt-Jakob disease (CJD) is caused by an abnormally shaped protein called a prion infecting the brain. People affected by CJD usually die within six months of their early symptoms developing.

HIV-associated neurocognitive disorder (HAND) – HIV infection can cause a number of different problems in the brain, which affect up to half of people with HIV. Neurocognitive disorders in people with HIV may be caused by the virus directly damaging the brain. They may also be the result of a weakened immune system enabling infections and cancers to attack the brain.

Huntington’s disease (HD) is an inherited disease and is a progressive brain disorder that causes uncontrolled movements, emotional problems and problems with thinking (cognition).  A persons mental abilities generally decline into dementia i.e. they become severe enough to affect daily life.

People are born with the defective gene, but symptoms usually don’t appear until middle age.

Wernicke-Korsakoff syndrome – Korsakoff syndrome is due to brain damage caused by a lack of vitamin B1 (thiamine). As Korsakoff syndrome often occurs as a result of alcoholism, it is sometimes referred to as alcoholic dementia.

In its early stages, the condition is known as Wernicke encephalopathy.  Its symptoms can be reversed if people are given high doses of thiamine in time.  If not, they often go on to develop Korsakoff’s syndrome.

Multiple sclerosis (MS) is a nervous system disease that affects your brain and spinal cord. Some people with MS experience a loss of some of their mental abilities. This happens if damage caused by the MS occurs in certain parts of the brain. The term ‘dementia’ is not generally used in association with multiple sclerosis because the decline is not usually as severe as it is in other forms of dementia. It is more usual to describe the person as ‘experiencing cognitive difficulties’.

Niemann-Pick disease is a group of diseases passed down through families (inherited) in which fatty substances called lipids collect in the cells of the spleen, liver, and brain.

Normal pressure hydrocephalus (NPH) is an abnormal buildup of cerebrospinal fluid (CSF) in the brain’s ventricles, or cavities. It occurs if the normal flow of CSF throughout the brain and spinal cord is blocked in some way. This causes the ventricles to enlarge, putting pressure on the brain.

Posterior cortical atrophy (PCA) also called Benson’s syndrome, is a form of dementia which is usually considered an atypical variant of Alzheimer’s disease. The disease causes atrophy of the back (posterior) part of the cerebral cortex, resulting in the progressive disruption of complex visual processing.

Progressive supranuclear palsy (PSP) is a rare and progressive condition that can cause problems with balance, movement, vision, speech and swallowing. It’s caused by increasing numbers of brain cells becoming damaged over time.

Additional Information

Niemann-Pick disease type CPosterior cortical atrophy (PCA)


2 thoughts on “Rare types of dementia

  1. My husband has PCA. Diagnosed 7 years ago after 3 years of trying to get a diagnosis. Very few people seem to know about PCA. The hospital we attend only has one other patient with it. My husband is 69 and the neurologist suggests he will have had the onset of PCA since his mid 50’s.

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