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07-SEPTEMBER-2008 03:17:44 - Pick's disease Pick's disease Classification and external resources ICD-10 G31.0, F02.0 ICD-9 331.11 OMIM 172700 DiseasesDB 10034 eMedicine neuro/311 MeSH D020774 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 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 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 bodies1 that are a defining characteristic of the disease. Contents 1 History 2 Symptoms 3 Causes 4 The Pathology and Biochemistry of Pick's Disease 4.1 Differences from Alzheimer's disease 5 External links 6 References 7 See also 8 External links 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 21. 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 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, but some new cases of atypical Pick's disease have come to light that lack noticeable Pick bodies3. A variety of stains that 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 methods53. 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 AD5. 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 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 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, synaptophysin4 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 disease In AD all six isoforms of tau proteins expressed. In addition, the presence of neurofibrillary tangles that are a hallmark of AD can be stained with antibodies to basic fibroblast growth factor, amyloid P, and heparin sulfate glycosaminoglycan8. External links FTD Support Forum The Association for Frontotemporal Dementias UCSF Memory and Aging Center - FTD Resources and FTD Info Mayo Clinic - FTD Info Pick's Disease Support Group Online Frontotemporal Dementia Caregiver Support Center References ^ a b c d 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. doi:10.1111/j.1440-1789.2006.00671.x. PMID 16771179. ^ a b Amano, N; Iseki, E 1999. Introduction: Pick's disease and frontotemporal dementia. Neuropathology 19 1: 417-421. doi:10.1046/j.1440-1789.1999.00258.x. ^ a b 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. doi:10.1111/j.1440-1789.2006.00738.x. PMID 17203597. ^ a b 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. doi:10.1111/j.1440-1789.1998.tb00118.x. PMID 16006664. ^ a b Uchihara, T; Ikeda K, Tsuchiya K. 2003. Pick body disease and Pick syndrome. Neuropathology 23 4: 318-326. doi:10.1046/j.1440-1789.2003.00523.x. PMID 14719549. ^ Iskei, E; Arai, H 2006. Progress in the classification of non-Alzheimer-type degenerative dementias. Psychogeriactrics 6 1: 41-42. doi:10.1111/j.1479-8301.2006.00166.x. ^ 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. doi:10.1007/s00401-003-0671-8. PMID 12677450. ^ a b c 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. doi:10.1002/ana.10571. PMID 12833365. ^ 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. PMID 16006664. 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 1 2 Pick's Disease Support Group Online UK-based Pick's Disease Information Page Frontotemporal Dementia Caregiver Support Center v d e WHO ICD-10 mental and behavioral disorders F · 290-319 Neurological/symptomatic Dementia Alzheimer's disease, multi-infarct dementia, Pick's disease, Creutzfeldt-Jakob disease, Huntington's disease, Parkinson's disease, AIDS dementia complex, Frontotemporal dementia, Elopement, Sundowning, Wandering · Delirium · Post-concussion syndrome · Organic brain syndrome Psychoactive substance alcohol drunkenness, alcohol dependence, alcoholic hallucinosis, Alcohol withdrawal, delirium tremens, Korsakoff's syndrome, alcohol abuse · opioids opioid dependency · sedative/hypnotic benzodiazepine withdrawal · cocaine cocaine dependence · general Intoxication, Drug abuse, Physical dependence, Withdrawal Psychotic disorder Schizophrenia disorganized schizophrenia · Schizophreniform disorder · Schizotypal personality disorder · Delusional disorder · Folie à deux · Schizoaffective disorder Mood affective Mania · Bipolar disorder · Clinical depression · Cyclothymia · Dysthymia Neurotic, stress-related and somatoform Anxiety disorder Agoraphobia, Panic disorder, Panic attack, Generalized anxiety disorder, Social anxiety, Social phobia · OCD · Acute stress reaction · PTSD · Adjustment disorder · Conversion disorder Ganser syndrome · Somatoform disorder Somatization disorder, Body dysmorphic disorder, Hypochondriasis, Nosophobia, Da Costa's syndrome, Psychalgia · Neurasthenia Physiological/physical behavioral Eating disorder: Anorexia nervosa · Bulimia nervosa Sleep disorder: Dyssomnia Hypersomnia, Insomnia · Parasomnia REM behavior disorder, Night terror · Nightmare Sexual dysfunction: Erectile dysfunction · Premature ejaculation · Vaginismus · Dyspareunia · Hypersexuality · Female sexual arousal disorder Postpartum depression · Postnatal psychosis Adult personality and behavior Personality disorder · Passive-aggressive behavior · Kleptomania · Trichotillomania · Voyeurism · Factitious disorder · Munchausen syndrome · Ego-dystonic sexual orientation · Fetishism Mental retardation Mental retardation Psychological development developmental disorder Specific: speech and language expressive language disorder, aphasia, expressive aphasia, receptive aphasia, Landau-Kleffner syndrome, lisp · Scholastic skills dyslexia, dysgraphia, Gerstmann syndrome · Motor function developmental dyspraxia Pervasive: Autism · Rett syndrome · Asperger syndrome Behavioral and emotional, childhood and adolescence onset ADHD · Conduct disorder · Oppositional defiant disorder · Separation anxiety disorder · Selective mutism · Reactive attachment disorder · Tic disorder · Tourette syndrome · Speech stuttering · cluttering v d e Pathology of the nervous system, primarily CNS G00-G47, 320-349 Inflammatory Meningitis Arachnoiditis, Aseptic meningitis, Tuberculous meningitis - Encephalitis - Myelitis - Encephalomyelitis Acute disseminated - Tropical spastic paraparesis - Cavernous sinus thrombosis Systemic atrophies Huntington's disease - Spinocerebellar ataxia Friedreich's ataxia, Ataxia telangiectasia, Herary spastic paraplegia - Spinal muscular atrophy: Werdnig-Hoffman - Kugelberg-Welander - Fazio-Londe - MND ALS, PMA, PBP, PP, PLS Extrapyramidal and movement disorders Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia/Dyskinesia Spasmodic torticollis, Meige's, Blepharospasm - Essential tremor - Myoclonus - Lafora - Chorea Choreoathetosis - Restless legs - Stiff person Other degenerative/ demyelinating diseases dementia: Alzheimer's - Pick's - Dementia with Lewy bodies - Frontotemporal lobar degeneration mitochondrial disease: Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis - Marchiafava-Bignami disease - CAMFAK syndrome - Alpers' Seizure/epilepsy Focal - Generalised - Status epilepticus - Myoclonic epilepsy Headache Migraine Familial hemiplegic - Cluster - Vascular - Tension Vascular Transient ischemic attack Amaurosis fugax, Transient global amnesia Cerebrovascular disease MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke Sleep disorders Insomnia - Hypersomnia - Sleep apnea Obstructive, Ondine's curse - Narcolepsy - Cataplexy - Kleine-Levin - Circadian rhythm sleep - Delayed sleep phase - Advanced sleep phase Intracranial hypertension Hydrocephalus Normal pressure - Idiopathic intracranial hypertension Other encephalopathy Brain herniation - Cerebral edema - Reye's Other spinal cord disease Syringomyelia - Syringobulbia - Morvan's syndrome - Spinal cord compression Retrieved from http://en..org/wiki/Pick%27s_disease Categories: Neurological disorders | Cognitive disorders Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Français Italiano Lëtzebuergesch Nederlands Polski Português РуÑ?Ñ?кий Svenska This page was last modified on 30 July 2008, at 13:32

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