81. Entrez PubMed OBJECTIVE To describe the course of depression in a patient with hepatolenticular degeneration (Wil http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9

82. Entrez PubMed hepatolenticular degeneration*/diagnosis hepatolenticular degeneration*/etiology hepatolenticular degeneration*/metabolism hepatolenticular degeneration*/ http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=5

83. INVESTIGATION OF THE AMINOACIDURIA IN WILSON'S DISEASE (HEPATOLENTICULAR DEGENER INVESTIGATION OF THE AMINOACIDURIA IN WILSON S DISEASE (hepatolenticular degeneration) DEMONSTRATION OF A DEFECT IN RENAL FUNCTION 1 http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=439748

84. Studies On Copper Metabolism. XIII. Hepatolenticular Degeneration1 Tissue copper determinations in a case of hepatolenticular degeneration treated with BAL Metabolism of copper in hepatolenticular degeneration. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1072574

85. LRCP - Jim Koropatnick, PhD Experimental model for Wilson s disease hepatolenticular degeneration. Role of metallothionein in cellular drug resistance and metaldependent protein http://www.lrcc.on.ca/research/staff/jkoropatnick/current_projects.xml

Home Change text size Cancer and treatment Community services ... Prevention and detection Research How to get to LRCP How to contact LRCP MORE TOPICS Research at LRCP - Cancer Research Laboratory Program - Radiation physics - Translation Breast Cancer Research Unit - Clinical Research Unit - Transgenic Core Facility Research Staff - Biological research - Radiation physics - Jerry Battista - Ann Chambers - Fred Dick - Gabriel DiMattia - Kevin Jordan - Jim Koropatnick - Joe Mymryk - Nathalie Berube - David Rodenhiser - Richard Rozmahel - Joe Torchia - Eva Turley - Jake Van Dyk - Siu-Pok Yee Education and training opportunities - Medical physics residency program Library and Information Services News and events - Recent news - Upcoming events - Innovation and discovery Donating Search: Research Research Staff RESEARCH AT LRCP Learn about research activities and opportunities RESEARCH SCIENTISTS Principal investigators at LRCP TRANSLATIONAL BREAST CANCER RESEARCH UNIT Pamela Greenaway-Kohlmeier Translational Breast Cancer Research Unit EDUCATION AND TRAINING Opportunities for employment and education LIBRARY AND INFORMATION SERVICES Books, journals and electronic resources

86. Wilson's Disease --  Encyclopædia Britannica also called hepatolenticular degeneration, a hereditary defect associated with the metabolism of copper and characterized by the progressive degeneration of http://www.britannica.com/eb/article?tocId=9077137

87. - So far, the institute has cured hepatolenticular degeneration patients for about 3000 from Taiwan, Hong Kong and other parts of China, the hospital is http://www.lifesciences.cn/enshanghai/SPT--FullRecord.php?ResourceId=2254

88. - Translate this page ??, Mental Derangement/Research/hepatolenticular degeneration So far, the institute has cured hepatolenticular degeneration patients for about http://www.lifesciences.cn/SPT--FullRecord.php?ResourceId=2254

89. MDVU Resource Library - Movement Disorders Corticobasal degeneration (CBD) is a rare neurological disease in which parts of the brain Wilson disease, also known as hepatolenticular degeneration, http://www.mdvu.org/library/disease/

WE MOVE 204 West 84th Street New York, NY 10024 E-mail: wemove@wemove.org Resource Library Resource Library Pediatric Movement Disorders Pediatric Treatment Centers Glossary Research News ... Support Organizations Special Events Meeting Calendar Local/Regional Courses Grant Announcements Classrooms All Classrooms CME Activities Case-based Learning Modules Our Mission ... Contact Us Movement Disorders Corticobasal Degeneration Corticobasal degeneration (CBD) is a rare neurological disease in which parts of the brain deteriorate. CBD is also known as corticobasal ganglionic degeneration, or CBGD. Dyskinesias (Paroxysmal) Paroxysmal dyskinesias are neurologic conditions characterized by sudden episodes of abnormal involuntary movements or hyperkinesias. Dystonia Dystonia is a movement disorder characterized by sustained muscle contractions, usually producing twisting and repetitive movements or abnormal postures or positions. Dystonia (Cervical) Cervical dystonia (CD) is a focal dystonia characterized by abnormal movements or postures of the neck and head. Essential Tremor Essential tremor or ET is a common movement disorder characterized by involuntary, rhythmic oscillatory movements resulting from alternating or irregularly synchronous contractions of antagonist muscles.

90. MDVU Resource Library - Wilson Disease Wilson disease, also known as hepatolenticular degeneration, is a rare inherited systemic disorder of copper metabolism. http://www.mdvu.org/library/disease/wd/

WE MOVE 204 West 84th Street New York, NY 10024 E-mail: wemove@wemove.org Resource Library Movement Disorders Description Symptoms/Findings ... Additional Resources Latest Wilson Disease News from E-MOVE Announcement: Movement Disorders Virtual University Is Open One Hundred Cases of Wilson Disease (WCN 2001) Clinical Trials Database Basal ganglia physiology Resource Library Search the E-MOVE Archives Wilson Disease Support Organizations Our Mission Professional Services ... Contact Us Wilson Disease Description Wilson disease, also known as hepatolenticular degeneration, is a rare inherited systemic disorder of copper metabolism. In patients with this disease, copper initially accumulates in the liver. When the liver's storage capacity is eventually exceeded, copper is then released from the liver and begins to collect in other organs of the body, particularly the brain, eyes, and kidneys. Please see the following topics related to Wilson disease below, and available on the left side of this page. Symptoms/Findings Causes/Genetics/History Diagnosis Treatment ... Additional Resources WE MOVE makes every effort to present medical information that is up-to-date and accurate. The material provided has undergone rigorous medical review. Information regarding the authors, editors, publisher, and medical reviewers of this material of the WE MOVE Web site is listed below. Medical science is constantly changing. Therefore, the authors, editors, and publisher do not warrant that the information in this text is complete, nor are they responsible for omissions or errors in the text or for the results of the use of this information. This information does not replace consultation with a physician. All medical procedures, drug doses, indications, and contraindications should be discussed with your personal physician.

91. Directory Of Open Access Journals Abstract, AIMTo compare the longterm effect of succimer (Suc) with that of penicillamine (Pen) in treating hepatolenticular degeneration (HLD). http://www.doaj.org/abstract?id=92209&toc=y

92. IWFmediacatalogue - Details For "Hepatolenticular Degeneration - Late Pseudoscle Translate this page hepatolenticular degeneration - Late Pseudosclerosis (44), Manifestation about 16 Years before. (Original title Degeneratio hepato-lenticularis - Späte http://mkat.iwf.de/medien/katalog/Kat_Details.asp?Signatur=W 1078&Language=en

93. 1910-1919 By George K York, MD & David Steinberg, MD published his classical description of hepatolenticular degeneration. to SA Kinnier Wilson s description of familial hepatolenticular degeneration. http://www.aneuroa.org/html/c20html/1910_1919.htm

NEUROLOGY IN THE 1910's George K. York, MD David A. Steinberg, MD The world of neurology began the decade 1910-1919 optimistically, in the conviction of the primacy of science. It ended the decade discouraged and uncertain. In between it participated in a war unparalleled in futility. Wassily Kandinsky, Untitled (1911) Kandinsky's paintings matched his writings on the spiritual aspects of modern art. This watercolor, often considered the first completely abstract painting, expresses the daring and confidence of the golden age of modernism. The avant garde opened new vistas in art, music, science and medicine. Pencil, watercolor and India ink, 49.6 x 64.8 cm. Inscription on verso: Watercolor, 1911. Musee National d'Art Moderne, Centre Georges Pompidou, Paris. In the beginning of the decade, amid studies of neurosyphilis and brain tumors, the American Neurological Association engaged in a contentious debate about the place of Freudian psychoanalysis in the practice of neurology. As practical physicians, many neurologists found the time required for couch therapy onerous. Harvey Cushing, Ramsay Hunt, Bernard Sachs, Foster Kennedy and Charles Dana presented important papers at nearly every annual meeting of the ANA. Title page of the 1910 edition of the Transactions of the American Neurological Association The concentration of neurologists in Boston, New York, Philadelphia and Cincinnati was reflected in the officers of the ANA. At the Washington meeting, Harvey Cushing spoke on eye signs in patients with brain tumors, James J. Putnam presented his personal experiences as a practitioner of Freudian psychoanalysis, and Charles K. Mills discussed his cases of spinal cord tumors. Dementia praecox was the subject of three presentations, and Burt G. Wilder described a man with average intelligence whose brain was half the normal size.

94. Wilson? (,hepatolenticular Degeneration) Wilson Translate this page The summary for this Japanese page contains characters that cannot be correctly displayed in this language/character set. http://brain.bri.niigata-u.ac.jp/~stsuji/resrc/disease/metab/Wilson/wilson.htm

Wilson•a (ŠÌƒŒƒ“ƒYŠj•Ï«ÇChepatolenticular degeneration) Wilson•a‚Ì—ðŽj Wilson•a‚̏Ǘá•ñ (1912”N) Kayser-FleischerŠp–Œ—Ö (1902”N) Wilson•a‚É‚¨‚¯‚铺‚Ì’~Ï‚Ì”­Œ© (1948”N) Wilson•aˆâ“`Žq‚Ì”­Œ© (1993 ”N) Wilson•a‚ÌŠT”O ŠT”O •a‘Ô‹@˜ ŠÌ×–E‚ðáŠQ‚·‚é Wilson•a‚̗Տ° •p“x‚Æ‚µ‚Ä‚Í3–œl‚É1l’ö“x‚Æ‚Ü‚ê‚ȏíõF‘Ì—ò«ˆâ“`«Ž¾Š³‚Å‚ ‚éD 10Î‘ã‚æ‚èŠÌ×–E‚É“º‚ª’¾’ ‚µCŠÌd•Ï‚ɐis‚·‚éD‰”­”N—î‚Æ‚µ‚ẮCŠÌáŠQ‚Å”­Ç‚·‚éê‡‚Í8-16Ë‚±‚ëC_ŒoÇó‚Å”­Ç‚·‚é‚Ì‚Í12ËˆÈ~‚Ì‚±‚Æ‚ª‘½‚¢D@ ŠÌáŠQ‚É‚æ‚éÇó‚Æ‚µ‚ẮC‰©átC‘SgŒ‘‘ÓŠ´‚È‚Ç‚ª‘½‚­CÇó‚͊ɉðÄ”­‚·‚邱‚Æ‚à‚ ‚éD ŠáÇó‚Æ‚µ‚āCKayser-FleischerŠp–Œ—Ö‚ªd—v‚Å‚ ‚éDŠp–Œ‚ÌŽü•Ó‚ɂɏoŒ»‚·‚é—ΐFC—ΐF‚È‚Ç‚ð’æ‚·‚é’ F—ւŁCŠp–ŒŽü•Ó‚ÌDescemet–Œ‚É“º‚ª’¾’ ‚µ‚½‚à‚Ì‚Å‚ ‚éDWilson•a‚É“ÁˆÙ“I‚Őf’f‰¿’l‚ª‚‚¢D t‘Ÿ‚Å‚Í”A×ŠÇáŠQ‚Ì‚½‚߁CŒŒ”A‚â“ñŽŸ«FanconiÇŒóŒQ‚ð’悵‚Ä‚­‚éD œ‘e頏ǁCŠÖßŠÔŒ„‚Ì‹·­‰»C•a“IœÜC‚­‚é•aCœ“ÇC•ÏŒ`«ŠÖßÇ‚Ȃǂ̍œ‡•¹Ç‚à‚æ‚­‚Ý‚ç‚ê‚éD f’f Kayser-Fleischer Šp–Œ—Ö, CT ƒXƒLƒƒƒ“‚ŁC—¼‘¤‚Ì”íŠk‚Ì’á‹zŽûˆæ‚ðŽ¦‚·D MRI ‚Å‚Í—¼‘¤ƒŒƒ“ƒYŠji“Á‚É”íŠkj‚ɁC“C”X–EŒ`¬CƒOƒŠƒI[ƒVƒX‚È‚Ç‚Ì•a•Ï‚𔽉f‚µ‚āCT1‹­’²‰æ‘œ‚Å’áM†CT2‹­’²‰æ‘œ‚ō‚M†‚Æ‚È‚éD‚½‚¾‚µCT2‹­’²‰æ‘œ‚ł́C’áM†‚ƍ‚M†‚ª¬Ý‚µ‚Ä‚Ý‚ç‚ê‚邱‚Æ‚à‚ ‚èCƒŒƒ“ƒYŠj‚Ö‚Ì“ºC“S‚Ì’¾’ ‚𔽉f‚µ‚Ä‚¢‚é‚ƍl‚¦‚ç‚ê‚éD _ŒoÇó‚ɑ΂µ‚ẮCL-dopa‚ª—LŒø‚È‚±‚Æ‚ª‚ ‚éD ŠÓ•Êf’f ŒŒ´“º‚Ì’á’lCƒZƒ‹ƒƒvƒ‰ƒXƒ~ƒ“‚Ì’á’l‚Æ‚¢‚¤“_‚ł́C Menkes•a C ƒZƒ‹ƒƒvƒ‰ƒXƒ~ƒ“Œ‡‘¹Ç ‚È‚Ç‚ÆŠÓ•Ê‚·‚é•K—v‚ª‚ ‚éD‘ÌŠO˜HÇó‚Æ‚¢‚¤“_‚ł́C Hallervorden-Spatz•a Cƒnƒ“ƒ`ƒ“ƒgƒ“•aCchorea-acanthocytosisC¬lŒ^ (type 3) GM1 ƒKƒ“ƒOƒŠƒIƒVƒh[ƒVƒX“™‚àŠÓ•Ê‚·‚é•K—v‚ª‚ ‚éD

95. Copper Wilson s disease, or hepatolenticular degeneration, is an autosomal recessive disorder that results from pathological accumulation of copper, principally in http://www.pdrhealth.com/drug_info/nmdrugprofiles/nutsupdrugs/cop_0083.shtml

//This is the Top Banner OAS_AD('Top'); Copper TRADE NAMES CU-5 (Bio-Tech Pharmacal), Coppermin (The Key Company) DESCRIPTION Copper is an essential trace mineral in animal and human nutrition. Anemia, neutropenia and osteoporosis are found with frank copper deficiency. Copper deficiency in humans is rare but it does occur under certain circumstances, such as in patients receiving long-term total parenteral nutrition (TPN). Mild copper deficiency due to marginal copper intake over a long period may also occur. In addition to possible anemia, neutropenia and osteoporosis, manifestations of mild copper deficiency may include abnormal glucose tolerance, hypercholesterolemia, arthritis, myocardial disease, arterial disease, cardiac arrhythmias, loss of pigmentation and neurological problems. Copper is a transition metal with atomic number 29 and an atomic weight of 63.55 daltons. Its symbol is Cu. Copper participates in metabolism as a component of many metalloenzymes, including ceruloplasmin or ferroxidase I, cytochromecoxidase, copper/zinc superoxide dismutase, dopamine beta-hydroxylase, tyrosinase, monoamine oxidase, diamine oxidase, lysyl oxidase (protein-lysine 6-oxidase), peptidylglycine-alpha-amidating monoxygenase and ferroxidase II. Copper essentiality for humans was first demonstrated in malnourished children in Peru. The children had an anemia that was not responsive to iron therapy, as well as neutropenia and bone abnormalities. The anemia, neutropenia and bone abnormalities were responsive to copper supplementation. Copper is required for normal infant development, red and white blood cell maturation, iron transport, bone strength, cholesterol metabolism, myocardial contractility, glucose metabolism, brain development and immune function, among other things.

96. Denny-Brown Bibliography DennyBrown, D., Hereditary chorea; hepatolenticular degeneration; Familial spastic Denny-Brown, D., hepatolenticular degeneration (Wilson s Disease). http://users.ipfw.edu/vilensk/Dennybib.htm

Click Here to go to the Denny-Brown Homepage. PUBLICATIONS OF D. DENNY-BROWN Cooper, S. and Denny-Brown, D., Responses to rhythmical stimulation of the cerebral cortex, Preliminary communication, Proc. Roy. Soc. B. Cooper, S., Denny-Brown, D. and Sherrington, Sir Charles, Reflex fractionation of a muscle, Proc. Roy. Soc. B. Denny-Brown, D. and Liddell, E.G.T, Some observations on the reflex activity of a muscle of the fore-limb (M. Supraspinatus), Quarterly J. Exper. Physiol. Denny-Brown, D. and Liddell, E.G.T., Observations of the motor twitch and on reflex inhibition of the tendon jerk of M. Supraspinatus, J. Physiol. Cooper, S., Denny-Brown, D. and Sherrington, Sir Charles, Interaction between ipsilateral spinal reflexes acting on the flexor muscles of the hind-limb, Proc. Roy. Soc. B. Cooper, S. and Denny-Brown, D., Responses to stimulation of the motor area of the cerebral cortex, Proc. Roy. Soc. B. Denny-Brown, D. and Liddell, E.G.T., The stretch reflex as a spinal process, J. Physiol. Denny-Brown, D. and Liddell, E.G.T., Extensor reflexes in the fore-limb, J. Physiol.

97. Final Diagnosis -- Case 47 Leigh AD, Card WI (1949) hepatolenticular degeneration A case associated with posterolateral column degeneration. J Neuropathol Exp Neurol 8 318-346 http://path.upmc.edu/divisions/neuropath/bpath/cases/case47/dx.html

Brain Pathology Case of the Month - February 2000 FINAL DIAGNOSIS: Acquired (Non-Wilsonian) hepatocerebral degeneration with "shunt myelopathy" DISCUSSION: It is well known that patients with severe liver disease may suffer from a reversible neurologic condition known as hepatic encephalopathy [1,6]. Some of these patients suffer from a more permanent neurologic disorder, particularly if they have surgically induced or spontaneous porto-systemic shunts and experience episodes of severe hepatic encephalopathy with coma. Victor, Adams, et al. reported the features of AHCD in detail in a series of 27 cases [14]. These symptoms and signs included progressive dementia, dysarthria, involuntary movements (including tremor, asterixis, and choreoathetosis), ataxia of limb and gait, and mild pyramidal tract signs, typically in a patient with severe hepatic disease and recurrent episodes of coma. Less commonly reported is a progressive spastic myelopathy in association with AHCD [4,12]. Only about 15 cases have been reported in the world literature with detailed pathological descriptions [2,3,7,9,13]. The present case report has several distinctive features. Whereas most reported cases resulted from a surgically created porto-systemic shunt as a treatment for cirrhosis, a few other cases, including this one, are thought to result from spontaneously created shunts (e.g., varices) with the same functional consequences. The published cases usually had Alzheimer II cells in the brain, but only a few had the severe spongy degeneration in the deep cortex illustrated here. Recently, hepatocerebral degeneration has occasionally been documented in vivo by MRI [8,9]. Few electron microscopic studies of human Alzheimer II cells and hepatocerebral degeneration have been published [5,11]. The present report suggests that the vacuolation results from the disruption of astrocytes.

98. Atypical Presentation Of Wilson's Disease hepatolenticular degeneration/DI; hepatolenticular degeneration/DT; Liver Failure/CO; Bilirubin; Human; Male; Adolescents; Case Report. References 5 http://medind.nic.in/imvw/imvw586.html

Extracted from IndMED Jain A; Bhatnagar MK Department of Medicine, Lady Harding Medical College and associated SK Hospital, New Delhi - 110001 Atypical presentation of Wilson's Disease Journal, Indian Academy of Clinical Medicine. 2002 Jul-Sep; 3(3): 300-1 ABSTRACT: Wilson's disease is an uncommon inherited disorder, which is characterised by low serum ceruloplasmin levels, low copper levels, Kayser-Fleischer rings, and hypercupriuria. Here we describe the case of a young boy who did not have classical features as mentioned above, rather presented with fulminant hepatic failure, normal serum ceruloplasmin levels, absent Kayser-Fleischer rings, and diagnosis was confirmed by high 24 hr. urinary copper levels. This is an uncommon presentation of Wilson's disease. KEYWORDS: Hepatolenticular Degeneration/DI; Hepatolenticular Degeneration/DT; Liver Failure/CO; Bilirubin; Human; Male; Adolescents; Case Report References: 5 Record Identifier: NI207845

99. Karger Publishers 19 Walshe JM Wilson s disease (hepatolenticular degeneration); in Vinken PJ, Bruyn GW, Klawans HL (eds) Handbook of Clinical Neurology, vol 27. http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowFulltext&ProduktNr=

100. Revista Brasileira De Ginecologia E Obstetrícia - Pregnancy And Wilson's Diseas hepatolenticular degeneration) is a rare autosomal recessive disorder that usually occurs between the first and third decades. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-72032001000500009

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