Pick's disease is named after Arnold Pick, a professor of psychiatry from the University of Prague who first discovered and described the disease in 1892 by examining the brain tissue of several deceased patients with histories of dementia. As a result, the characteristic histological feature of this disease - a protein tangle that appears as a large body in neuronal tissue - is named a Pick body.
In 1911, Alois Alzheimer also noted the complete absence of senile plaques and neurofilbrillary tangles as well as the presence of Pick Bodies and occasional ballooned neurons. A variety of stains can aid in the visualization of Pick bodies and Pick cells, but immunohistochemical staining using anti-tau and anti-ubiquitin antibodies have proven the most efficient and specific. Hematoxylin and eosin staining allows visualization of another population of Pick cells, which are both tau and ubiquitin protein negative. Several different silver impregnation stains have been used, including the Bielschowsky, Bodian, and Gallyas methods. The latter two techniques are sensitive enough to allow PiD to be distinguished from Alzheimer's disease as the Bodian will bind preferentially to cells with PiD as compared to the Gallyas method, which preferentially binds to the cells with Alzheimer's. All of these isoforms result from alternative splicing of the same gene. Pick bodies typically have the 3R isoform of tau proteins as not only the most abundant form but the only form of this protein, but a recent study has shown that a much greater number of different tau isoforms including 4R and mixed 3R/4R can be present in the Pick bodies. Not only do these tangles have the 3R tau protein predominately but they are also characteristically shaped with a round body and there is often an indentation in the area that faces the nucleus of the cell. The Pick bodies are also able to be labeled by N-terminal amyloid precursor protein segment, hyperphosphorylated tau, ubiquitin, Alz-50, neurofiliment proteins, clathrin, synaptophysin
Differences from Alzheimer’s disease
In Alzheimer’s disease, all six isoforms of tau proteins are expressed. In addition, the presence of neurofibrillary tangles that are a hallmark of Alzheimer’s can be stained with antibodies to basic fibroblast growth factor, amyloid P, and heparan sulfate glycosaminoglycan.
Another difference is that in Pick's disease, a personality change occurs prior to any form of memory loss, unlike Alzheimer's, where memory loss typically presents first. This is used clinically to determine whether patient is suffering from Alzheimer's or Pick's.
Sample Presentation
(From biopsy "histological" verified, after death, case presentation)
64 y/o retired professional male with progressive strange behavior according to his wife and children for several months without memory loss at start but mild problems with memory now (peoples names, and dates).
Additional signs/symptoms: Uncharacteristic impulsive behavior, easily distractable (again uncharacteristic for this former professional), lack of personal hygiene (stops bathing, brushing teeth, combing hair, etc without prompting from wife), appears unkempt/disheveled, uncharacteristically rude to friends and family, eats with fingers occasionally. No focal neurological signs/symptoms. Prominent grasp and snout reflex.
Patient died from acute myocardial infarction with "sudden death" 6 months after this presentation.
Further Reading
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