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{{ClinPsy}}
{{DiseaseDisorder infobox |
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{{Infobox_Disease |
Name = Pick's Disease |
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Name = {{PAGENAME}} |
ICD10 = G31.0 |
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Image = |
ICD9 = {{ICD9|331.11}} |
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Caption = |
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DiseasesDB = 10034 |
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ICD10 = {{ICD10|G|31|0|g|30}}, {{ICD10|F|02|0|f|00}} |
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ICD9 = {{ICD9|331.11}} |
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ICDO = |
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OMIM = 172700 |
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MedlinePlus = |
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eMedicineSubj = neuro |
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eMedicineTopic = 311 |
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MeshID = D020774 |
 
}}
 
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{{distinguish|Niemann-Pick disease}}
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'''Pick's disease''', also known as '''Pick disease''' and '''PiD''', is a rare [[neurodegenerative disease]]. While 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 among professionals in the field) to mean a specific [[pathology]] that is just one of the causes of the clinical syndrome now known as [[frontotemporal lobar degeneration]]. Some people still use the term Pick's disease to mean the more general clinical syndrome of [[frontotemporal lobar degeneration]], but this has previously led to confusion among both professionals and patients and so its use should be restricted to the specific pathological subtype described below.
   
'''Pick's disease''' is a [[dementia|dementing illness]] associated with deterioration of the [[frontal lobe|frontal]] and [[temporal lobe]]s of the brain.
+
Pick's disease (the pathology) causes progressive destruction of [[nerve]] cells in the [[brain]] and causes [[tau protein]]s to accumulate into "[[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.
   
==Characteristics==
+
===History===
[[Symptom]]s may include a decline in social [[behavior]] (including [[disinhibition]], tactlessness, and breaches of interpersonal [[etiquette]]), emotional blunting, [[apathy]], changes in eating habits (including increased [[appetite]], [[weight gain]], and increased preference for sweets), attention problems, decreased insight, speech and language problems (including reduced speech ability, repetition of phrases heard, reduced use of nouns, difficulty naming objects, loss of word meaning, diminished writing ability, and mutism), and difficulty recognizing faces. Though [[Alzheimer's disease]] and other forms of dementia can sometimes cause similar symptoms, Pick's disease is more likely to cause certain deficits in behavior and speech (such as disinhibition or loss of nouns), while memory and visuospatial function (which are frequently affected by Alzheimer's Disease) tend to be relatively spared. Also, the onset of Pick's Disease (usually between the ages of 45 and 65) is earlier than is normally seen in Alzheimer's disease.
+
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]].<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/>
   
Pick's disease causes a [[syndrome]] called [[frontotemporal lobar degeneration]] (FTLD) that has three main variants:
+
==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.
   
* [[Frontotemporal dementia]]
+
==Causes==
* [[Semantic dementia]]
+
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.
* [[Progressive non-fluent aphasia]]
 
   
The clinical syndrome of FTLD can also result from [[dementia lacking distinctive histology]] (DLDH). Because DLDH and Pick's disease cannot be separated clinically, it is currently not possible to make a final diagnosis of Pick's disease until [[autopsy]]. At autopsy, Pick's disease is diagnosed by the presence of cortical [[Pick bodies]], which are silver-staining, spherical aggregations of [[tau protein]] in neurons.
+
==Pathology and Biochemistry==
  +
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, but 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> 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.<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 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.<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 [[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.<ref name = Gman/>
   
==History==
+
Numerous different areas of the brain are affected by PiD, but the specific areas that are affected allow for differentiation between PiD and Alzheimer’s disease. 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 demonstrates 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. 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, but cases of extreme degeneration do exist.<ref name = Wang/>
The [[Czechoslovakia]]n [[neurologist]] and [[psychiatrist]] [[Arnold Pick]] first described the clinical syndrome and characteristic neuronal inclusions, or Pick bodies, associated with Pick's disease in [[1892]].
 
   
== References ==
+
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, contains only 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, 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.<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>
* Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S, Freedman M, Kertesz A, Robert PH, Albert M, Boone K, Miller BL, Cummings J, Benson DF. (1998) Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. ''Neurology'' '''51'''(6):1546-54. Available: [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9855500&query_hl=14]
 
* Pick A. (1892) Über die Beziehungen der senilen Hirnatrophie zur Aphasie. ''Prager medicinische Wochenschrift'' Prague '''17''':165-167.
 
   
== External links ==
+
===Differences from Alzheimer’s disease===
*[http://www.pdsg.org.uk/ Pick's Disease Support Group Online]
+
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]].<ref name = Munoz/>
*[http://www.picksdisease.org/FAQ.html Pick's Disease info]
 
*[http://dementiasupport.multiply.com/ Another Pick's Disease Support Group Online]
 
   
[[Category:Neurological disorders]]
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==See also==
[[Category:Eponymous diseases]]
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* [[Dementia]]
[[Category:Cognitive disorders]]
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* [[Genetic disorders]]
  +
* [[Presenile dementia]]
   
{{disease-stub}}
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==References==
  +
{{reflist}}
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  +
  +
  +
==External links==
  +
*[http://www.ninds.nih.gov/disorders/picks/picks.htm Frontotemporal Dementia Information Page] from the [[National Institute of Neurological Disorders and Stroke]]
  +
*[http://www.bhoffcomp.com/coping/picks.html Pick's Disease Information Page]
  +
*[http://memory.ucsf.edu/ftd Frontotemporal dementia information] from the [http://memory.ucsf.edu/ UCSF Memory and Aging Center]
  +
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{{Mental and behavioural disorders}}
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{{Diseases of the nervous system}}
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  +
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[[Category:Cognitive disorders]]
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[[Category:Neurological disorders]]
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[[Category:Presenile dementia]]
   
[[de:Morbus Pick]]
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{{enWP|Pick's disease}}
 
{{enWP|Pick's disease}}

Latest revision as of 23:32, January 27, 2009

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Pick's disease
ICD-10 G310, F020
ICD-9 331.11
OMIM 172700
DiseasesDB 10034
MedlinePlus [1]
eMedicine neuro/311
MeSH {{{MeshNumber}}}


Pick's disease, also known as Pick disease and PiD, is a rare neurodegenerative disease. While 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 among professionals in the field) to mean a specific pathology that is just one of the causes of the clinical syndrome now known as frontotemporal lobar degeneration. Some people still use the term Pick's disease to mean the more general clinical syndrome of frontotemporal lobar degeneration, but this has previously led to confusion among 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 proteins to accumulate into "Pick bodies"[1] that are a defining characteristic of the disease.

HistoryEdit

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.[2][1] 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.[2]

SymptomsEdit

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.

CausesEdit

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.

Pathology and BiochemistryEdit

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, but some new cases of atypical Pick’s disease have come to light that lack noticeable Pick bodies.[3] 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.[4] 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.[5][3] 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.[5]

Numerous different areas of the brain are affected by PiD, but the specific areas that are affected allow for differentiation between PiD and Alzheimer’s disease. 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 demonstrates 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. Other regions that are involved include the caudate, which is severely affected, the dorsomedial region of the putamen, the globus pallidus, and locus cerulus.[1] 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, but cases of extreme degeneration do exist.[1]

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, contains only one or as many as two of the six different isoforms of the tau protein.[6] All of these isoforms result from alternative splicing of the same gene.[7] 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.[8] 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[4] and neuronal surface glycoside (A2B5)[8] 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.[9]

Differences from Alzheimer’s diseaseEdit

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.[8]

See alsoEdit

ReferencesEdit

  1. 1.0 1.1 1.2 1.3 Wang, LN, Zhu MW, Feng YQ, Wang JH. (2006). 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. Neuropathology 26 (3): 222–230.
  2. 2.0 2.1 Amano, N, Iseki, E (1999). Introduction: Pick’s disease and frontotemporal dementia. Neuropathology 19 (1): 417–421.
  3. 3.0 3.1 Yamakawa, K, Takanashi M, Watanabe M, Nakamura N, Kobayashi T, Hasegawa M, Mizuno Y, Tanaka S, Mori H (2006). Pathological and biochemical studies on a case of Pick disease with severe white matter atrophy. Neuropathology 26 (6): 586–591.
  4. 4.0 4.1 Armstrong, RA, Cairns NJ, Lantos, PL (1998). A comparison of histological and immunohistochemical methods for quantifying the pathological lesions of Pick’s disease. Neuropathology 18 (4): 295–300.
  5. 5.0 5.1 Uchihara, T, Ikeda K, Tsuchiya K. (2003). Pick body disease and Pick syndrome. Neuropathology 23 (4): 318–326.
  6. Iskei, E, Arai, H (2006). Progress in the classification of non-Alzheimer-type degenerative dementias. Psychogeriactrics 6 (1): 41–42.
  7. Arai, T, Ikeda K, Akiyama H, Tsuchiya K, Iritani S, Ishiguro K, Yagishita S, Oda T, Odawara T, Iseki E. (2003). 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. Acta Neuropathol. 105 (5): 489–498.
  8. 8.0 8.1 8.2 Munoz, DG, Dickson DW, Bergeron C, Mackenzie IR, Delacourte A, Zhukareva V. (2003). The neuropathology and biochemistry of frontotemporal dementia. Ann Neurol 54 supp. S5 (1): S24–S28.
  9. Puig, B, Ferrer I, Ludueña RF, Avila J. (2005). βII-tubulin and phospho-tau aggregates in Alzheimer's disease and Pick's disease. J Alzheimers Dis. 7 (1): 213–220.


External linksEdit


  1. REDIRECT Template:CNS diseases of the nervous system
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