Pick disease

ピック病

WordNet

1. select carefully from a group; "She finally picked her successor"; "He picked his way carefully"
2. remove in small bits; "pick meat from a bone"
3. a heavy iron tool with a wooden handle and a curved head that is pointed on both ends; "they used picks and sledges to break the rocks" (同)pickax, pickaxe
4. a small thin device (of metal or plastic or ivory) used to pluck a stringed instrument (同)plectrum, plectron
5. a basketball maneuver; obstructing an opponent with ones body; "he was called for setting an illegal pick"
6. a thin sharp implement used for removing unwanted material; "he used a pick to clean the dirt out of the cracks"
7. attack with or as if with a pickaxe of ice or rocky ground, for example; "Pick open the ice" (同)break_up
8. look for and gather; "pick mushrooms"; "pick flowers" (同)pluck, cull
9. pilfer or rob; "pick pockets"
10. provoke; "pick a fight or a quarrel"
11. an impairment of health or a condition of abnormal functioning
12. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

1. (入念に)…‘を'『選ぶ』,選び取る / 〈草花・果実など〉‘を'『摘む』,もぐ / 〈鳥など〉‘の'羽をむしる;(…から)…‘を'むしる《+『名』+『from』+『名』》 / (とがった物で)…‘を'つつく,つついて穴をあける《+『名』+『with』+『名』》 / 〈鳥などが〉〈えさ〉‘を'つつく,つついて食べる / 〈争いなど〉‘を'しかける;(人に)〈口論など〉‘を'ふっかける《+『名』+『with』+『名』〈人〉》 / (針金などで)〈錠〉‘を'こじあける / 〈ポケット・さいふなど〉‘の'中身をすり取る / 〈弦楽器・弦〉‘を'指で鳴らす,つまびく / 入念に選ぶ / 〈鳥が〉えさをつつく;(食浴がなくて)〈人が〉(…を)少しずつ食べる《+『at』+『名』》 / 〈U〉(…の)選択(choice),(…を)選ぶこと(権利)《+『of』+『名』》 / 〈C〉(…から)選択された人(物);《the~》(…から)選ばれた最上の人(物)《+『of』+『名』》
2. 《複合語を作って》「ほじる(つつく)道具」の意を表す / 《話》=plectrum
3. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
4. 女性の話術芸人 =diseur
5. 病気にかかった / 病的な,不健全な(morbid)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/05/18 04:06:41」(JST)

wiki en

[Wiki en表示]

Not to be confused with Niemann–Pick disease.

Pick's disease
Classification and external resources
Brain MRI in Pick's disease
ICD-10	G31.0, F02.0
ICD-9	331.11
OMIM	172700
DiseasesDB	10034
MedlinePlus	000744
eMedicine	neuro/311
MeSH	D020774

Pick's disease is a rare neurodegenerative disease that causes progressive destruction of nerve cells in the brain. Symptoms include loss of speech (aphasia) and dementia. While some of the symptoms can initially be alleviated, the disease progresses and patients often die within two to ten years. A defining characteristic of the disease is build-up of tau proteins in neurons, accumulating into silver-staining, spherical aggregations known as "Pick bodies".

While the term Pick's disease was once used to represent a class of clinical syndromes with symptoms attributable to frontal and temporal lobe dysfunction, it is now used among professionals to mean a specific pathology that is just one of the causes of 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. It is also known as Pick disease and PiD (not to be confused with Pelvic Inflammatory Disease (PID) or Parkinson's Disease (PD)).

Signs and symptoms

The symptoms of Pick's disease include difficulty in speech and thinking, behavioral changes, impaired regulation of social conduct (e.g. breaches of etiquette, tactlessness, dis-inhibition), passivity, inertia, over-activity, pacing and wandering. The changes in personality allow doctors to distinguish between Pick's disease and Alzheimer's disease. 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

While 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.

Pathophysiology

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. 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.

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 coeruleus. 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.

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. 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 and neuronal surface glycoside (A2B5) 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.

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.

History

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.

Notable cases

• Don Cardwell (1935–2008), Major League Baseball pitcher
• Rocco "Rocky" Catena (1929–2006), music industry executive, former Senior VP, Capitol Records, in charge of marketing The Beatles and other top recording stars in the U.S. Suffered from Pick's disease for 20+ years.
• Ted Darling (c.1935–1996), voice of the Buffalo Sabres
• Robert W. Floyd (1936–2001), computer scientist
• Colleen Howe (1933–2009), wife of Hockey Hall of Famer Gordie Howe
• Kazi Nazrul Islam (1899–1976), Bengali poet, musician and revolutionary who pioneered poetic works espousing intense spiritual rebellion against fascism and oppression, was a lifelong sufferer of this disease (approx. 1941–1976)
• Ralph Klein (1942–2013), former Premier of Alberta, Canada
• Kevin Moore (1958–2013), English footballer
• Nic Potter (1951–2013), British bassist for Van der Graaf Generator
• David Rumelhart (1942–2011), American cognitive psychologist
• Colin Savage, father of footballer Robbie Savage

See also

• Frontotemporal lobar degeneration (FTLD)
• Syndromes of frontotemporal dementia
• Semantic dementia
• Progressive nonfluent aphasia

References

• Pick A. (1892) Über die Beziehungen der senilen Hirnatrophie zur Aphasie. Prager medicinische Wochenschrift Prague 17:165-167.

External links

• Pick bodies (histology)
• Frontotemporal Dementia Information Page from the National Institute of Neurological Disorders and Stroke
• Pick's Disease Information Page
• Frontotemporal dementia information and videos from the UCSF Memory and Aging Center
• The Bluefield Project to Cure Frontotemporal Dementia
• Pick's Disease: Signs, Symptoms, Treatment, and Support

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. ニーマン・ピック病の概要 overview of niemann pick disease
• 2. 前頭側頭型認知症：臨床的特徴および診断 frontotemporal dementia clinical features and diagnosis
• 3. 前頭側頭型認知症：疫学、病理学、および病因 frontotemporal dementia epidemiology pathology and pathogenesis
• 4. 皮膚つみ取り障害 skin picking disorder
• 5. 大脳皮質基底核変性症 corticobasal degeneration

English Journal

• Arachidonic acid metabolism via cytosolic phospholipase A2 α induces cytotoxicity in niemann-pick disease type C cells.

• Nakamura H, Yasufuku K, Makiyama T, Matsumoto I, Fujino H, Murayama T.SourceLaboratory of Chemical Pharmacology, Graduate School of Pharmaceutical Sciences, Chiba University, Chuo-ku, Chiba, Japan. nakahiro@faculty.chiba-u.jp
• Journal of cellular physiology.J Cell Physiol.2012 Jul;227(7):2847-55. doi: 10.1002/jcp.23025.
• Niemann-Pick disease type C (NPC) is a neurodegenerative lipid storage disorder caused by mutations in NPC1 or NPC2 genes. Loss of function of either protein results in the endosomal accumulation of cholesterol and other lipids. Here, we report that NPC1-deficient Chinese hamster ovary cells exhibit
• PMID 21928312

• The impact of severe LDL receptor mutations on SREBP-pathway regulation in homozygous familial hypercholesterolemia (FH).

• Soufi M, Ruppert V, Kurt B, Schaefer JR.AbstractFamilial hypercholesterolemia (FH), Niemann-Pick disease type C (NPC) and Tangier disease (TD) are genetic inherited disorders with impaired processing of cholesterol, caused by mutations in genes that regulate cellular uptake, intracellular movement and transport of cholesterol. Various studies have shown a crucial regulatory role of the SREBP-pathway for cellular cholesterol homeostasis in these diseases. Since cholesterol is an essential structural component of cells, we assessed the impact of a severe FH causing LDLR mutation (FH p.W556R) on the SREBP pathway in primary FH fibroblasts. Primary FH fibroblasts derived from patients with the LDL receptor mutation p.W556R were used for gene expression experiments. Gene expression studies revealed increased expressions of SREBP regulated genes HMGCR, LDLR, SREBP-2, SREBP-1, SR-BI, INSIG-1, but interestingly not SCAP. In contrast expression of ABCA1, was strongly decreased in homozygous, but not in heterozygous p.W556R fibroblasts. Gene expression experiments with LDL receptor lacking primary FH fibroblasts, revealed that SR-BI and ABCA1 are important regulators for cholesterol acquisition in FH cells, consistent with findings in cells from NPC and TD patients.
• Gene.Gene.2012 May 10;499(1):218-22. Epub 2012 Mar 9.
• Familial hypercholesterolemia (FH), Niemann-Pick disease type C (NPC) and Tangier disease (TD) are genetic inherited disorders with impaired processing of cholesterol, caused by mutations in genes that regulate cellular uptake, intracellular movement and transport of cholesterol. Various studies hav
• PMID 22425645

Japanese Journal

• 前頭側頭型認知症(ピック病)の臨床症状 (特集 前頭側頭葉変性症と類縁疾患の基礎と臨床)

• 荻原 朋美,天野 直二
• 老年精神医学雑誌 24(12), 1233-1241, 2013-12
• NAID 40019944690

• タウオパチーの神経病理学 (特集 プロテイノパチーの神経病理学)

• 吉田 眞理
• Brain and nerve : 神経研究の進歩 65(12), 1445-1458, 2013-12
• NAID 40019901548

• Niemann-Pick病C型の神経症状の治療 (第1土曜特集 神経変性疾患 : 研究と診療の進歩) -- (神経変性疾患の治療の新しい展開)

• 大野 耕策
• 医学のあゆみ 247(5), 499-502, 2013-11-02
• NAID 40019848804

Related Links

• Pick' disease（ピック病）とは - インターネット三重

アルツハイマー病でなくピック病と診断されたという方も多数でてきておりますので、ピック病とはどの様な病気かご説明いたしましょう。 ピック病 初老期痴呆（初老期とは歴年齢上の定義は45歳～65歳）の代表疾患が ...

• Pick disease - National Library of Medicine - PubMed Health

Pick disease is a rare form of dementia that is similar to Alzheimer disease, except that it tends to affect only certain areas of the brain. ... Causes, incidence, and risk factors People with Pick disease have abnormal ...

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★リンクテーブル★

リンク元	「ピック病」
拡張検索	「type A Niemann-Pick disease」「type B Niemann-Pick disease」「type C Niemann-Pick disease」「Niemann-Pick disease」
関連記事	「disease」「Pi」「pick」

「ピック病」

　　[★]

英

Pick disease, Pick's disease

同

Pick病

関

前頭側頭型認知症、認知症

概念

• 前頭葉・側頭葉の限局性の萎縮による認知症

症状

• 人格変化、常同行動、反響言語
• クリューバー・ビューシー症候群

ピック病とアルツハイマー型認知症

参考1より引用

	アルツハイマー型認知症	ピック病
病気部位	側頭葉、頭頂葉	前頭葉、側頭葉
症状	「記憶の障害」	「行動の障害」
	昼食を摂ったことを忘れるなど	同じことを同じ時間に繰り返すなど
診断治療	100年ほど前に発見され、原因の究明や治療の開発が不十分ながらも進んでいる	100年ほど前に発見されるが、世界共通の診断基準すらなく、発生頻度も不明
発病年齢	高齢になるほど増える	40代以降65歳頃までに発病することが多い
性差	女性にやや多い	性差なし

参考

• 1. 認知症

http://www.ninchisho.jp/kind/04.html

• 2. emedicine

http://emedicine.medscape.com/article/1135504-overview

• 3. wikipedia en

http://en.wikipedia.org/wiki/Pick's_disease

「type A Niemann-Pick disease」

　　[★]

A型ニーマン・ピック病、A型Niemann-Pick病

関

Niemann-Pick disease type A

「type B Niemann-Pick disease」

　　[★]

B型ニーマン・ピック病、B型Niemann-Pick病

関

Niemann-Pick disease type B

「type C Niemann-Pick disease」

　　[★]

C型ニーマン・ピック病、C型Niemann-Pick病

「Niemann-Pick disease」

　　[★] ニーマン・ピック病

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「Pi」

　　[★] オルトリン酸

関

orthophosphate、orthophosphoric acid

「pick」

　　[★]