Naked Science Forum
Life Sciences => Physiology & Medicine => Topic started by: OldMan on 07/06/2005 04:09:34
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A Dementia related illness. Any information for me? Have already seen this...
http://www.mydr.com.au/default.asp?article=2834
Tim
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I'm guessing after this long with no responses that no one has come across it before. I can tell ya one thing ...it sucks!
Tim
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Hi Tim
Sorry it's taken me a while to get onto this one, I've been very busy...
Picks disease is a kind of frontal dementia (a bit like Alzheimer's) affecting principally the brain's frontal and temporal lobes, with relative sparing of the posterior lobes (parietal and occipital).
This manifests as a progressive decline in function, poor social interactions and awareness (so-called disinhibition), loss of 'higher' cognitive function, depression and apathy.
Unlike Alzheimer's, which causes the progressive accumulation of 'amyloid plaques' and 'fibrillary tangles', patients with Pick's disease show a characteristic 'ballooning' of affected nerve cells and intracellular aggregations of a material known as Pick bodies.
Pick's disease is very rare, accounting for between 1% and 5% of all dementias, and it usually begins after the age of 40. The incidence declines from the age of 60, and the average time patients manifest the illness is about 5 years, but this can range from 2-15 years.
Chris
"I never forget a face, but in your case I'll make an exception"
- Groucho Marx
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No worries and thanks Chris. I thought maybe the thread also hadn't been seen. A bit more information there I wasn't farmilar with.
Cheers
Tim
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Don't know if anyone is interested but found a little bit more on this. Certainly have seen, or at least had described to me my grandmother showing all these sorts of signs and behaviour.
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Introduction
Pick's disease is characterized by circumscribed cerebral atrophy and is also known as lobar sclerosis. It can be mistaken for other presenile dementias, but it is extremely rare, particularly when compared to the incidence of Alzheimer's disease. Pick's disease is seen in the elderly, with women more commonly affected than men. Transmission may be hereditary.
Pathological features
The disease usually involves both the frontal and the temporal lobes. Gross pathologic changes are so severe that the diagnosis can easily be made by visual inspection. There is a sharp line of demarcation between the affected portions and the remaining brain. It is rare to see unilateral involvement.
Atrophic changes also occur in subcortical structures such as the thalamus, basal ganglia and the descending fronto-pontine fiber system. Microscopically, there are fibrillary deposits that appear as masses of straight fibrils in the cytoplasm of the affected neurons. Densely packed spherical deposits (Pick's bodies) can be seen in some neurons, using silver impregnation techniques.
The signs and symptoms of Pick's disease manifest as unusually severe frontal or temporal lobe dysfunction. Detreriorating mental function, changes in behavior patterns, and lack of insight are seen in early stage of the disease, while later advanced illness causes loss of all language functions. Loss of retentive memory is seen in temporal lobe involvement, and when frontal lobes are primarily affected, prominent grasp and sucking reflexes can be noted.
The progression of disease is slow. Rigidity, dystonic postures, and tremor may become prominent features in late stages of the disease. These are attributed to the extension of the disease into the basal ganglia.
Imaging in Pick's disease
CT and MRI are useful in identifying shrinkage of cortex and low density of the white matter in the involved lobes. Tc-99m HMPAO brain SPECT imaging shows greatly decreased or absent perfusion to the affected lobe/s.
Differential diagnosis
Severe involvement of the frontal or temporal lobes in Alzheimer's disease can simulate Pick's disease, but they can be differentiated histopathologically. Unsuspected hydrocephalus, slow growing neoplasms, subdural hematoma and other space occupying lesions can also appear as Pick's disease and must hence be excluded by CT or MRI. Metabolic derangements, B12 deficiency and hypothyroidism must also be included in the differential diagnosis. Multi-infarct (vascular) dementia and pseudo-dementia (depression mimicking dementia) as well as chronic sedative usage need to be ruled out.
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References
1. Harrison's Principles of Internal Medicine. 12th ed. McGraw-Hill publishers. pp 2062.
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Unsuspected hydrocephalus, slow growing neoplasms, subdural hematoma and other space occupying lesions can also appear as Pick's disease and must hence be excluded by CT or MRI.
I can understand basically what they mean by the lesions and hematoma but anyone care to shed a little light on the other bits mentioned here for my non medical mind.
Tim