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

WE MOVE 204 West 84th Street New York, NY 10024 E-mail: wemove@wemove.org Library 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.

82. Wilson's Disease resonance imaging revealed extensive signal abnormality in the basal ganglia and thalamus consistent with that described in hepatolenticular degeneration (Fig 2 http://www.sma.org/smj/97may15.htm

Wilson's Disease NAWRAS K. BABAN, MD, DORIS T. HUBBS, MD, and THOMAS M. ROY, MD, Johnson City, Tenn ABSTRACT: We describe a patient who had severe neurologic symptoms, psychiatric abnormalities, and secondary amenorrhea superimposed on a history of hemolytic anemia and micronodular cirrhosis attributed to hemochromatosis. The correct diagnosis of Wilson's disease was delayed until the appearance of Kayser-Fleischer rings and a low serum ceruloplasmin level. Appropriate treatment ameliorated symptoms, and maintenance therapy has been effective in retarding progression. It is essential to consider Wilson's disease in patients with unexplained hepatic, neurologic, and psychiatric dysfunction, because appropriate early medical treatment can prevent further organ damage and reduce the risk of permanent damage to the liver and brain. We describe a patient whose illness began with hemolytic anemia, necessitating periodic multiple transfusions. The presence of hepatic, psychiatric, and neurologic signs did not become apparent until 5 years later. We reemphasize the varied presentations of Wilson's disease and suggest that the evaluation of hemolytic anemia include the possibility of this disorder of copper metabolism. Early diagnosis of Wilson's disease can have a positive impact on treatment, as well as early screening for asymptomatic relatives. CASE REPORT A 25-year-old woman presented a 2-week history of increasing difficulty with muscular coordination. She had tremors severe enough to interfere with writing and experienced difficulty with walking. She also admitted to increased agitation, emotional lability, and excessive anxiety during the preceding 4 months that she could not explain.

83. Entrez PubMed Child; DNA Mutational Analysis; Female; Genotype; Haplotypes; hepatolenticular degeneration/diagnosis; hepatolenticular degeneration/genetics http://www.biomedcentral.com/pubmed/15024742

Entrez PubMed Nucleotide Protein ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes 3D Domains Domains Gene GEO GEO DataSets HomoloGene Journals MeSH NCBI Web Site OMIM PMC PopSet SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard ... Text Version Entrez PubMed Overview FAQ Tutorial New/Noteworthy ... E-Utilities PubMed Services Journals Database MeSH Database Single Citation Matcher Batch Citation Matcher ... Cubby Related Resources Order Documents NLM Gateway TOXNET Consumer Health ... PubMed Central Summary Brief Abstract Citation ASN.1 MEDLINE XML UI List LinkOut Related Articles Cited in Books CancerChrom Links Domain Links 3D Domain Links GEO DataSet Links Gene Links Genome Links GEO Links HomoloGene Links Nucleotide Links OMIM Links PMC Links Cited in PMC PopSet Links Protein Links SNP Links Structure Links UniSTS Links Show: Sort Author Journal Pub Date Text File Clipboard E-mail Order Hum Mutat. 2004 Apr;23(4):398. Related Articles, Links Wilson disease: novel mutations in the ATP7B gene and clinical correlation in Brazilian patients. Deguti MM, Genschel J, Cancado EL, Barbosa ER, Bochow B, Mucenic M, Porta G, Lochs H, Carrilho FJ, Schmidt HH.

84. Entrez PubMed Carcinoma, Hepatocellular/enzymology*; Cell Polarity; hepatolenticular degeneration/enzymology; hepatolenticular degeneration/pathology; http://www.biomedcentral.com/pubmed/9288609

Entrez PubMed Nucleotide Protein ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes 3D Domains Domains Gene GEO GEO DataSets HomoloGene Journals MeSH NCBI Web Site OMIM PMC PopSet SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard ... Text Version Entrez PubMed Overview FAQ Tutorial New/Noteworthy ... E-Utilities PubMed Services Journals Database MeSH Database Single Citation Matcher Batch Citation Matcher ... Cubby Related Resources Order Documents NLM Gateway TOXNET Consumer Health ... PubMed Central Summary Brief Abstract Citation ASN.1 MEDLINE XML UI List LinkOut Related Articles Cited in Books CancerChrom Links Domain Links 3D Domain Links GEO DataSet Links Gene Links Genome Links GEO Links HomoloGene Links Nucleotide Links OMIM Links PMC Links Cited in PMC PopSet Links Protein Links SNP Links Structure Links UniSTS Links Show: Sort Author Journal Pub Date Text File Clipboard E-mail Order J Hepatol. 1997 Aug;27(2):337-45. Related Articles, Links Aberrant dipeptidyl peptidase IV (DPP IV/CD26) expression in human hepatocellular carcinoma. Stecca BA, Nardo B, Chieco P, Mazziotti A, Bolondi L, Cavallari A.

85. Resident Cases The diseases involving the basal ganglia are Huntington s chorea and Wilson s disease, or hepatolenticular degeneration. The disease http://www.pathology.vcu.edu/WirSelfInst/BASALGANG.html

Home About Us Site Directory Clinical Services ... Neuropathology Mini-Course NEUROPATHOLOGY MINI-COURSE Presented by William I. Rosenblum, MD CHAPTER 6 DISEASES OF THE BASAL GANGLIA AND SUBSTANTIA NIGRA PRETEST: Answers will be found in the text of this chapter What neurotransmitter is produced in the substantia nigra and where does it go? What color is the normal substantia nigra and why? What happens to the subst nigra grossly and microscopically in Parkinsons disease? What is the inclusion body that characterizes idiopathic Parkinsons and what peptide does it contain? Parkinsons disease maybe accompanied by dementia. What other disease of basal ganglia is characterized by dementia and is hereditary? In the later disease what is the key genetic feature? The key gross change? What disease is associated with excessive copper deposition in brain, eye and liver? In the diseases discussed here, the primary pathology within the brain is located either in the basal ganglia or in the substantia nigra. The student should review the anatomy of these structures. The diseases involving the basal ganglia are Huntington's chorea and Wilson's disease, or hepatolenticular degeneration. The disease involving the substantia nigra is Parkinson's disease. The reason for grouping the latter disease together with the former is simple: the neuronal cell bodies in the substantia nigra are connected via their axons with cell bodies in the basal ganglia. The neurotransmitter released at the synapse in the basal ganglia is DOPamine (dihydroxyphenylethylamine), a catecholamine closely related to norepinephrine, and synthesized in the neurons of the substantia nigra. The transmitter travels in the axons to the basal ganglia. Hence destruction of the nigra results in depletion of DOPamine in the basal ganglia.

86. Sites Médicaux Francophones : Classement Par Mots Clés MeSH, Qualificatifs Et hepatolenticular degeneration; dégénérescence macula macular degeneration; http://www.chu-rouen.fr/ssf/santpathd.html

Classement par mots clés MeSH, qualificatifs et types de ressources : D Menu général CISMeF A B C ... dacryocystite [dacryocystitis] dacryocysto-rhinostomie [dacryocystorhinostomy] dactylologie [manual communication] danazol [danazol] Danemark [Denmark] danse [dancing] dapsone [dapsone] datura [datura] datura stramonium [datura stramonium] daunorubicine [daunorubicin] De Lange, syndrome [De Lange syndrome] débit cardiaque [Cardiac Output] débit cardiaque élevé [cardiac output, high] débit expiratoire forcé [forced expiratory flow rates] débit expiratoire pointe [peak expiratory flow rate] débit plasmatique rénal [renal plasma flow] débit sanguin [blood flow velocity] début accouchement [labor onset] décalage horaire, syndrome [jet lag syndrome] décalage ribosomique [frameshifting, ribosomal] decapoda (crustacea) [decapoda (crustacea)] déchets [waste products] déchets dangereux [hazardous waste] déchets dentaires [dental waste] déchets médicaux [medical waste] déchets radioactifs [radioactive waste] décision médicale non réanimation [resuscitation orders] déclaration Helsinki [Helsinki declaration] décollement rétine [retinal detachment] décompression [decompression] décompression, maladie

87. CAS Appendix I Subj Hdngs 30a-z Intestine, neoplasm * * Liver, disease * * * BuddChiari syndrome * * * Cirrhosis * * * Hepatitis * * * hepatolenticular degeneration * * * Jaundice * * * Liver http://www-sul.stanford.edu/depts/swain/casapp/sub8a.html

Chemical Abstracts Appendix I Subject Headings 30a - z A publication of the Chemical Abstracts Service published by the American Chemical Society, and reproduced here with permission from the Chemical Abstracts Service. General Subject Headings Menu Keyword Index Menu Main Menu 30. MEDICINE sections: a - z aa - al

88. BJO -- Sullivan Et Al. 86 (1): 114 LETTER. Dense KayserFleischer ring in asymptomatic Wilson s disease (hepatolenticular degeneration). Charlotte Anne Sullivan 1 , A Chopdar 2 and GA Shun-Shin 3 http://bjo.bmjjournals.com/cgi/content/full/86/1/114

HOME HELP FEEDBACK SUBSCRIPTIONS ... Email this link to a friend eLetters: Submit a response to this article Similar articles found in: BJO Online Search PubMed for articles by: Sullivan, C. A. Shun-Shin, G A Alert me when: new articles cite this article Download to Citation Manager Collections under which this article appears: Other ophthalmology British Journal of Ophthalmology British Journal of Ophthalmology LETTER Dense Kayser-Fleischer ring in asymptomatic Wilson's disease (hepatolenticular degeneration) Charlotte Anne Sullivan A Chopdar and G A Shun-Shin Department of Ophthalmology, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK Department of Ophthalmology, East Surrey Hospital, Surrey, UK Department of Ophthalmology, Wolverhampton Eye Infirmary, Wolverhampton, West Midlands, UK Correspondence to: Miss C A Sullivan, Birmingham and Midlands Eye Hospital, City Hospital, Western Road, Birmingham B18 7QU, UK Accepted for publication 6 June 2001 Keywords: Kayser-Fleischer ring; Wilson's disease; hepatolenticular degeneration The Kayser-Fleischer ring is the single most important diagnostic sign in Wilson's disease; it is found in 95% of patients. Virtually

89. 1910-1919 By George K York, MD & David Steinberg, MD und Nervenkrankenheiten. In 1912, SA Kinnier Wilson published his classical description of hepatolenticular degeneration. As the 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.

90. Final Diagnosis -- Case 221 Am J Neuroradiol 19485487. Leigh AD, Card WI (1949) hepatolenticular degeneration A case associated with postero-lateral column degeneration. http://path.upmc.edu/cases/case221/dx.html

Final Diagnosis Acquired (Non-Wilsonian) hepatocerebral degeneration with "shunt myelopathy" 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.

91. Mirago Results : Hereditary Angioedema Hemophilia Hemorrhagic Fevers Hemorrhoids Hepatic Encephalopathy Hepatitis Hepatocerebral Encephalopathy hepatolenticular degeneration Hereditary Angioedema http://www.mirago.co.uk/scripts/qhandler.aspx?qry=Hereditary Angioedema&ass=1218

92. Rev. Bras. Ginecol. Obstet.  Vol.23 no.5; Abstract: S0100-72032001000500009 Wilson s disease (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_abstract&pid=S0100-72032001000500009&

93. Atypical Presentation Of Wilson's Disease KEYWORDS 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

94. »»Hepatolenticular-Degeneration Reviews«« hepatolenticulardegeneration Reviews. Related Subjects Heart-Murmur. Book reviews for hepatolenticular-degeneration sorted by average review score http://www.health-issue-books.com/Heart-Murmur/Hepatolenticular-Degeneration/

Hepatolenticular-Degeneration Reviews Related Subjects: Heart-Murmur Book reviews for "Hepatolenticular-Degeneration" sorted by average review score: Orphan Diseases and Orphan Drugs (Fulbright Papers, Proceedings of Colloquia, Vol 3) Published in Hardcover by Manchester Univ Pr (December, 1986) Authors: I. Herbert Scheinberg and J.M. Walshe Amazon base price: Used price: Average review score: No reviews found. Wilson's Disease Published in Textbook Binding by Not Applicable (February, 1984) Authors: I. Herbert Scheinberg and Irmin Sternlieb Amazon base price: Used price: Average review score: No reviews found. Wilson's disease : the etiology, clinical aspects, and treatment of inherited copper toxicosis Published in Unknown Binding by Noyes (1981) Author: Charles Archibald Owen Amazon base price: Average review score: No reviews found. Wilson's Disease for the Patient and Family: A Patient's Guide to Wilson's Disease and Frequently Asked Questions About Copper Published in Paperback by Xlibris Corporation (April, 2002) Authors: George J., MD Brewer and M. D. George J. Brewer

95. Roche Lexikon Medizin (4. Aufl.) - Hepatolentikuläre Degeneration http://www.gesundheit.de/roche/ro15000/r15740.html

hepato engl.: hepatolenticular degeneration Fach: Neurologie (H ALL ) die W ILSON * Krankheit ; hepatogene Encephalopathie (Erkrankung der Leber u. des Putamens des Linsenkerns). Verwandte Themen Wilson* Krankheit

96. IIDRIS Dégénérescence Hépatolenticulaire http://www.med.univ-rennes1.fr/iidris/index.php?action=contexte&num=1709&mode=mu

97. GeneReviews: Wilson Disease http://www.geneclinics.org/profiles/wilson/

Your browser does not support HTML frames so you must view Wilson Disease in a slightly less readable form. Please follow this link to do so.

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