somewhere something incredible is waiting to be known-
Carl Sagan

Monday, April 11, 2011

Pick's Disease or Fronto-Temporal Dementia

Fronto-temporal dementia (FTD) is associated with degeneration of the frontal and temporal lobes of the brain. After Alzheimer's, it is one of the most common cause of early-onset dementia. Pick's disease - named after Arnold Pick who first described the syndrome in a case report in 1892 - is one variant of FTD. Some studies based on brain pathology samples have suggested that as many as 10% of cases of dementia in the U.S. might be due to Pick's disease. A study by Stevens from the Netherlands reported approximately 5 cases per million.
The symptoms of FTD are behavioural as well as those that have to do with executive functions. Behavioural symptoms include lethargy or apathy. Patients may be withdrawn socially. Occasionally, patients may act as if they have lost their normal inhibitions. Symptoms of loss of executive function include inability to plan or to organize.

Reports suggest the former premier has lost his command of language. While some patients remain quite fluent, others have difficulty recalling words and proper names. Some patients can nevertheless recognize words when spoken to them despite difficulty being able to say those words themsevles.
The diagnosis is made when typical symptoms are present. An MRI of the brain many show atrophy of the frontal and temporal lobes. More recently, positron emission tomography or PET scanning shows decreased metabolism of the frontal and temporal lobes, a feature that is not present in Alzheimer's disease.
While FTD is thought to run in families, the exact proportion of cases associated with abnormal genes and the role of genetics are unknown. Like Alzheimer's disease, FTD has no known cure. Patients require similar supportive care plus medications that treat depression and other related behavioural problems.

The condition can be quite devastating to families in which the patient is young and in the prime of life. The median survival time is 7 years.

Dementia is a devastating condition.

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