Pick's disease 1731868 223708406 2008-07-05T11:06:18Z DOI bot 6652755 Citation maintenance. Formatted: title. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. {{Infobox_Disease | Name = {{PAGENAME}} | Image = | Caption = | DiseasesDB = 10034 | ICD10 = {{ICD10|G|31|0|g|30}}, {{ICD10|F|02|0|f|00}} | ICD9 = {{ICD9|331.11}} | ICDO = | OMIM = 172700 | MedlinePlus = | eMedicineSubj = neuro | eMedicineTopic = 311 | MeshID = D020774 | }} '''Pick’s disease''', also known as '''Pick disease''' and '''PiD''', is a rare [[neurodegenerative disease]]. Whilst the term Pick's disease was once used to represent a specific group of clinical syndromes with symptoms attributable to frontal and temporal lobe dysfunction, it is now used (at least amongst professionals in the field) to mean a specific pathology that is just one of the causes of this clinical syndrome (now known as [[frontotemporal lobar degeneration]]). Some people still use the term Pick's disease to mean the clinical syndrome of [[frontotemporal lobar degeneration]] but this has previously led to confusion amongst both professionals and patients and so its use should be restricted to the specific pathological subtype described below. Pick's disease (the pathology) causes progressive destruction of [[nerve]] cells in the [[brain]] and causes [[tau protein]]s to accumulate into the "Pick bodies"<ref name = Wang>{{cite journal | last = Wang | first = LN | coauthors = Zhu MW, Feng YQ, Wang JH.| title = Pick's disease with Pick bodies combined with progressive supranuclear palsy without tuft-shaped astrocytes: a clinical, neuroradiologic and pathological study of an autopsied case | journal = Neuropathology | volume = 26 | issue = 3 | pages = 222–230 | year = 2006 | pmid = 16771179| doi = 10.1111/j.1440-1789.2006.00671.x}}</ref> that are a defining characteristic of the disease. ===History=== Pick 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 <ref name = Armando>{{cite journal | last = Amano | first = N | coauthors = Iseki, E | title = Introduction: Pick’s disease and frontotemporal dementia | journal = Neuropathology | volume = 19 | issue = 1 | pages = 417–421 | year = 1999 | doi = 10.1046/j.1440-1789.1999.00258.x}}</ref><ref name =Wang/>. 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. <ref name = Armando/> ==Symptoms== Pick's disease is one of the causes of the clinical syndrome of frontotemporal lobar degeneration which has three subtypes. Pick's disease pathology is associated more with the [[frontotemporal dementia]] and [[progressive nonfluent aphasia]] subtypes than the [[semantic dementia]] subtype. ==Causes== Whilst other pathologies causing [[frontotemporal lobar degeneration]] are associated with a genetic cause, there is no evidence in the modern literature that classical Pick's disease pathology can run in families or has a genetic cause. ==The Pathology and Biochemistry of Pick’s Disease== PiD was first recognized as a distinct disease separate from other neurodegenerative diseases because of the presence of large dark staining aggregates of proteins in neurological tissue as well as the aforementioned ballooned cells which are known as Pick cells. Pick bodies are almost universally present in patients with PiD; however, some new cases of atypical Pick’s disease have come to light that lack noticeable Pick bodies<ref name = K>{{cite journal | last = Yamakawa | first = K | coauthors = Takanashi M, Watanabe M, Nakamura N, Kobayashi T, Hasegawa M, Mizuno Y, Tanaka S, Mori H | title = Pathological and biochemical studies on a case of Pick disease with severe white matter atrophy| journal = Neuropathology | volume = 26 | issue = 6 | pages = 586–591 | year = 2006 | pmid = 17203597 | doi = 10.1111/j.1440-1789.2006.00738.x}}</ref>. There are a variety of stains that can allow for visualization however at current [[immunohistochemical staining]] using anti-tau and anti-ubiquitin [[antibodies]] have proven the most efficient and specific for aiding the visualization of Pick bodies, and Pick cells<ref name = Armstrong>{{cite journal | last = Armstrong | first = RA | coauthors = Cairns NJ, Lantos, PL | title = A comparison of histological and immunohistochemical methods for quantifying the pathological lesions of Pick’s disease | journal = Neuropathology | volume = 18 | issue = 4 | pages = 295–300 | year = 1998 | pmid = 16006664 | doi = 10.1111/j.1440-1789.1998.tb00118.x}}</ref>. [[Hematoxylin]] and [[Eosin]] staining also serves to allow for 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<ref name = Gman>{{cite journal | last = Uchihara | first = T | coauthors = Ikeda K, Tsuchiya K.| title = Pick body disease and Pick syndrome| journal = Neuropathology | volume = 23 | issue = 4 | pages = 318–326 | year = 2003 | pmid = 14719549| doi = 10.1046/j.1440-1789.2003.00523.x}}</ref><ref name = K>{{cite journal | last = Yamakawa | first = K | coauthors = Takanashi M, Watanabe M, Nakamura N, Kobayashi T, Hasegawa M, Mizuno Y, Tanaka S, Mori H | title = Pathological and biochemical studies on a case of Pick disease with severe white matter atrophy| journal = Neuropathology | volume = 26 | issue = 6 | pages = 586–591 | year = 2006 | pmid = 17203597 | doi = 10.1111/j.1440-1789.2006.00738.x}}</ref>. The latter two techniques are sensitive enough to allow PiD to be distinguished from AD as the Bodian will bind preferentially to cells with PiD as compared to the Gallyas method which preferentially binds to the cells with AD<ref name = Gman/>. There are numerous different areas of the brain that are affected by PiD however, upon closer inspection the specific areas that are affected allow for differentiation between PiD and Alzheimer’s disease (AD). The aforementioned Pick bodies are almost always found in several different places in the brain including the [[dentate gyrus]], the pyramidial cells of the CA1 sector and subiculum of the [[hippocampus]], and the neocortex as well as a plurality of other nuclei. Interestingly it is the location within the different layers of the brain as well as the anatomical location that demonstrate some of the unique features of PiD. A striking feature is that in the neocortex the Pick bodies are located in the II and IV layers of the cortex which send neurons within the cortex and to thalamic synapses respectively. While layers III and V have very few if any Pick bodies they show extreme neuronal loss that can, in some cases be so severe as to leave a void in the brain altogether. Furthermore other regions that are involved include the caudate which is severely affected the dorsomedial region of the [[putamen]], the [[globus pallidus]], and locus cerulus <ref name = Wang/>. The hypothalamic lateral tuberal nucleus is also very severely affected. The cerebellar elements that are important in receiving input, including the mossy fibers as well as the monodendritic brush cells in the granule cell layer, and generating output signals most notably the dentate nucleus are stricken with lots of tau protein inclusions. Strangely, the [[substantia nigra]] is most often uninvolved or only mildly involved however cases of extreme degeneration do exist <ref name = Wang/>. PiD has several unique biochemical characteristics that allow for unique identification of Pick’s disease as opposed to other pathological subtypes of [[frontotemporal lobar degeneration]]. The most striking of these is that this disease which has tau protein tangles present in many affected neurons only contains one or as many as two of the six different isoforms of the tau protein.<ref name = Ghetto>{{cite journal | last = Iskei | first = E | coauthors = Arai, H | title = Progress in the classification of non-Alzheimer-type degenerative dementias | journal = Psychogeriactrics | volume = 6 | issue = 1 | pages = 41–42 | year = 2006 | doi = 10.1111/j.1479-8301.2006.00166.x}}</ref> All of these isoforms result from alternative splicing of the same gene.<ref>{{cite journal | last = Arai | first = T | coauthors = Ikeda K, Akiyama H, Tsuchiya K, Iritani S, Ishiguro K, Yagishita S, Oda T, Odawara T, Iseki E.| title = Different immunoreactivities of the microtubule-binding region of tau and its molecular basis in brains from patients with Alzheimer's disease, Pick's disease, progressive supranuclear palsy and corticobasal degeneration | journal = Acta Neuropathol.| volume = 105 | issue = 5 | pages = 489–498 | year = 2003 | pmid = 12677450| doi = 10.1007/s00401-003-0671-8}}</ref> Pick bodies typically have the 3R isoform of tau proteins as not only the most abundant form but the only form of this protein however a recent study has shown that there are a much greater number of different tau isoforms including 4R and mixed 3R/4R that can be present in the Pick bodies.<ref name = Munoz>{{cite journal | last = Munoz | first = DG | coauthors = Dickson DW, Bergeron C, Mackenzie IR, Delacourte A, Zhukareva V. | title = The neuropathology and biochemistry of frontotemporal dementia | journal = Ann Neurol | volume = 54 supp. S5 | issue = 1 | pages = S24–S28 | year = 2003 | pmid = 12833365 | doi = 10.1002/ana.10571}}</ref> 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<ref name = Armstrong/> and neuronal surface glycoside (A2B5) <ref name = Munoz/> specific stains. Moreover βII tubulin proteins are also suspected in playing a role in the formation of phosphor-tau aggregates that are seen in PiD as well as AD.<ref>{{cite journal | last = Puig | first = B | coauthors = Ferrer I, Ludueña RF, Avila J.| title = βII-tubulin and phospho-tau aggregates in Alzheimer's disease and Pick's disease| journal = J Alzheimers Dis.| volume = 7 | issue = 1 | pages = 213–220 | year = 2005 | pmid = 16006664}}</ref> ===Differences from Alzheimer’s disease=== It is well known that in AD all six isoforms of tau proteins expressed. In addition the presence of neurofibrillary tangles that are a hallmark of AD are able to be stained with antibodies to basic fibroblast growth factor, amyloid P, and heparin sulfate glycosaminoglycan<ref name = Munoz/>. ==External links== *[http://www.ftdsupportforum.com/ FTD Support Forum] *[http://www.ftd-picks.org/ The Association for Frontotemporal Dementias] *[http://memory.ucsf.edu UCSF Memory and Aging Center] - [http://memory.ucsf.edu/resources.html#ftd FTD Resources] and [http://memory.ucsf.edu/Education/Disease/ftd.html FTD Info] *Mayo Clinic - [http://www.mayoclinic.com/invoke.cfm?id=AN00541 FTD Info] *[http://www.pdsg.org.uk/ Pick's Disease Support Group Online] *[http://www.ftdsupport.com/ Frontotemporal Dementia Caregiver Support Center] ==References== {{reflist}} ==See also== For more information on Pick's Disease, see the article on the pathologic process of [[frontotemporal lobar degeneration]] (FTLD) and its related clinical [[syndromes]] of [[frontotemporal dementia]], [[semantic dementia]], and [[progressive nonfluent aphasia]]. ==External links== *[http://www.ninds.nih.gov/disorders/picks/picks.htm] *[http://www.wrongdiagnosis.com/p/picks_disease/intro.htm] *[http://www.pdsg.org.uk/ Pick's Disease Support Group Online (UK-based)] *[http://www.bhoffcomp.com/coping/picks.html Pick's Disease Information Page] *[http://www.ftdsupport.com Frontotemporal Dementia Caregiver Support Center] {{Mental and behavioural disorders}} {{Diseases of the nervous system}} [[Category:Neurological disorders]] [[Category:Cognitive disorders]] [[de:Pick-Krankheit]] [[fr:Maladie de Pick]] [[it:Malattia di Pick]] [[lb:Pick-Krankheet]] [[nl:Ziekte van Pick]] [[pl:Choroba Picka]] [[pt:Doença de Pick]] [[ru:Болезнь Пика]] [[sv:Picks sjukdom]]