Pick disease, also known as
Frontotemporal dementia (FTD) is one of the less common types of dementia. It is sometimes called Pick's disease or frontal lobe dementia. The first noticeable FTD symptoms are changes to personality and behaviour and/or difficulties with language.
Unlike people with Alzheimer's disease, people with Pick's disease: Are diagnosed earlier in life. Don't have hallucinations or delusions. Don't tend to get lost in familiar places.
Pick's disease is a specific type of frontotemporal dementia (FTD), a degenerative brain disease that happens most commonly in people under age 65. In years past, Pick's disease was referred to as frontotemporal dementia itself. However, today, experts only use Pick's name when the condition meets certain criteria.
The Progression of Pick's Disease
Although some cases proceed slowly, Pick's disease usually proceeds more rapidly than AD, on average taking only four to six years from diagnosis to death. Patients with behavioral changes tend to pursue a more rapid course.
As well as causing speech and behavioral changes, Pick's or FTD also creates memory problems similar to those experienced by people with Alzheimer's disease, along with cognition and judgment problems.
Causes. Niemann-Pick disease types A and B is caused by mutations in the SMPD1 gene. This gene provides instructions for producing an enzyme called acid sphingomyelinase. This enzyme is found in lysosomes , which are compartments within cells that break down and recycle different types of molecules.
Pick's disease causes disability in the early stages of life, whereas, in later stages, the disease may cause death due to severe infection or multiorgan failure in 8 to 10 years.
Administration: The examiner reads a list of 5 words at a rate of one per second, giving the following instructions: “This is a memory test. I am going to read a list of words that you will have to remember now and later on. Listen carefully. When I am through, tell me as many words as you can remember.
Figuring out which one a patient has is important because it can affect the kind of care needed and what the patient and family members can expect. Willis, 67, has frontotemporal dementia. It's a rare condition that usually shows up earlier than other forms of dementia.
Genetic testing.
DNA testing of a blood sample may show the specific abnormal genes that cause Niemann-Pick types A, B and C. DNA tests can show who the carriers are for all types of Niemann-Pick disease if the mutations have been described in the first person identified in a family (the index case).
With FTD, unusual or antisocial behavior as well as loss of speech or language are usually the first symptoms. In later stages, patients develop movement disorders such as unsteadiness, rigidity, slowness, twitches, muscle weakness or difficulty swallowing.
People with FTD typically live six to eight years with their condition, sometimes longer, sometimes less. Most people die of problems related to advanced disease.
Actor Bruce Willis has been diagnosed with dementia, evolving from a previous diagnosis of aphasia last spring, his family announced Thursday. More specifically, Willis has frontotemporal dementia, which can include aphasia, which brings challenges with speaking and writing.
Pick disease, also known as frontotemporal dementia, is the most common cause of dementia in patients under 60 years of age and is the third most common cause of dementia in patients over 65 years old.
The cause of death is not the illness itself, but complications from its symptoms. The inability to swallow correctly may result in food or liquid getting into the lungs, causing an infection that turns into aspiration pneumonia.
More men than women may be affected by Pick disease. Pick disease occurs in a younger age group than dementia of the Alzheimer type, with peak incidence occurring in individuals aged 55–65 years.
Drugs that are commonly used to treat other types of dementia are not recommended for people with FTD. These drugs, known as cholinesterase inhibitors (for example, donepezil, rivastigmine, galantamine) can actually make the symptoms of FTD worse.
This degeneration causes progressive loss of the ability to control movement, typically beginning around age 60. The most prominent symptom may be apraxia, the inability to use the hands or arms to perform a movement despite normal strength, such as difficulty closing buttons or operating small appliances.
Pick's disease is notable for the difficulty it causes with speech, which may present as an initial symptom. Other forms of dementia may present with behavioral or personality changes as primary symptoms. Pick's disease occurs as a result of tau proteins, which form plaques called Pick bodies in the brain.
The Niemann–Pick diseases
Type C NPD is usually suspected in patients with vertical gaze impairment, dysarthria, dementia, ataxia, dystonia, and mild hepatosplenomegaly. Some of these patients also have cataplexy and seizures.
Many people affected by dementia are concerned that they may inherit or pass on dementia. The majority of dementia is not inherited by children and grandchildren. In rarer types of dementia there may be a strong genetic link, but these are only a tiny proportion of overall cases of dementia.