61. 1910-1919 By George K York, MD & David Steinberg, MD und Nervenkrankenheiten. In 1912, SA Kinnier Wilson published hisclassical 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.

62. Final Diagnosis -- Case 47 Am J Neuroradiol 19485487. Leigh AD, Card WI (1949) hepatolenticular degenerationA case associated with postero-lateral column degeneration. 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.

63. Hepatolenticular Degeneration hepatolenticular degeneration. A Hospital. A resource with informationon over 4000 medical topics including hepatolenticular degeneration. http://www.kernanhospital.com/medical-terms/02086.htm

Hepatolenticular degeneration A Medical Encyclopedia Article provided by Kernan Hospital A resource with information on over 4000 medical topics including: Hepatolenticular degeneration Previous Next

64. EMedicine - Wilson Disease : Article Excerpt By: Celia H Chang, MD Wilson Disease Wilson disease, or hepatolenticular degeneration, isa neurodegenerative disease of copper metabolism. In 1912, Wilson http://www.emedicine.com/neuro/byname/wilson-disease.htm

(advertisement) Excerpt from Wilson Disease Synonyms, Key Words, and Related Terms: Wilson’s disease, hepatolenticular degeneration, liver cirrhosis, copper overload, Kayser-Fleischer rings Please click here to view the full topic text: Wilson Disease Background: Wilson disease, or hepatolenticular degeneration, is a neurodegenerative disease of copper metabolism. In 1912, Wilson first described it as a familial disorder associated with neurological symptoms and cirrhosis. In 1956, Walshe first treated patients with the chelating agent penicillamine. Although the animal models are not equivalents of Wilson disease, they are helpful in studying copper metabolism and potential treatments. The Bedlington terrier has an autosomal recessive inherited disease characterized by copper toxicosis (reported by Owen and Ludwig in 1982). The Bedlington terrier does not develop neurological symptoms, but the liver pathology is similar to that of Wilson disease. The Long Evans Cinnamon (LEC) rat and toxic milk mouse both develop autosomal recessive inherited diseases associated with copper overload. LEC rats are reported to develop neurological symptoms, although hemolysis and hepatitis are the usual presenting symptoms; however, the liver histologic findings in the LEC rats and toxic milk mice are substantially different from those of Wilson disease. Tetrathiomolybdate was developed initially to treat chronic nutritional copper poisoning in sheep and is now used for Wilson disease. The Menkes disease gene (

65. Resident's Survival Guide - Psychiatric And Medical Disorders Associated hypothyroidism; myxedema; hypoglycemia; SIADH; hypernatremia; Gastrointestinaldiseases; hepatolenticular degeneration; Disorder hepatolenticular degeneration. http://199.243.225.113/ClinicalAssistant/diagnostic aids/medpsych.html

Psychiatric and medical disorder associated. The following is a list of medical disorders that are commonly associated with psychiatric symptoms. The list has been selected to emphasize those disorders that are most often considered in the psychiatric differential diagnosis. Contents Connective Tissue Diseases giant cell arteritis temporal arteritis system lupus erythematosus ... mercury poisoning Connective Tissue Diseases Disorder: giant cell arteritis Alternate name: temporal arteritis Prevalence range: infrequent Age at oneset: 50+ yr Sex ratio: females 2:1 Signs or symptoms: headache, joint, and muscle pain, low-grade fever, vision loss, jaw claudication, polymyalgia rheumatica Psychiatric features: depression, confusion, psychosis Diagnostic tests: ESR, temporal artery biopsy Comments: rapid diagnosis and treatment may prevent blindness. Disorder: system lupus erythematosus Alternate name: Prevalence range: infrequent Age at oneset: 20 - 40 yr Sex ratio: females 10:1 Signs or symptoms: arthritis, malar rash, lymphadenopathy, oral ulcers, fever, weight lsos, renal disease, fatigue Psychiatric features: depression, psychosis, mania

66. Alphabetical Topic Index (AZ) Jump To A B C D E F G H I J K L M Autoimmune Hepatitis, DrugInduced Hepatitis, Chronic, Drug-Induced HepatoblastomaHepatoblastoma hepatolenticular degeneration hepatolenticular degeneration http://www.uscuh.com/apps/Intermap/topiclist/SectionH.html

Alphabetical Topic Index (A-Z): Jump To: A B C D ... Hy H Ha Haemonchiasis Haemonchiasis Hair Loss Hair Loss in Men And Women Hairy Tongue Tongue, Hairy Halitosis Halitosis Hallermann's Syndrome Hallermann's Syndrome Hallervorden-Spatz Syndrome Hallervorden-Spatz Syndrome Hallucinations Hallucinations Hallux Limitus Hallux Limitus Hallux Rigidus Hallux Rigidus Hallux Valgus Hallux Valgus Hamartoma Syndrome, Multiple Hamartoma Syndrome, Multiple Hand Deformities, Acquired Hand Deformities, Acquired Hand Deformities, Congenital Hand Deformities, Congenital Hand Dermatoses Hand Dermatoses Hand, Foot and Mouth Disease Hand, Foot and Mouth Disease Hantavirus Infection Hantavirus Pulmonary Syndrome Hartnup Disease Hartnup Disease Hay Fever Hay Fever Back To Top ^ He Head and Neck Cancer Hypopharyngeal Cancer Salivary Gland Cancer Parathyroid Cancer Paranasal Sinus And Nasal Cavity Cancer ... Oral Cavity Cancer Head Injuries, Closed Head Injuries, Closed Head Injuries, Penetrating Head Injuries, Penetrating Headache Headache Headache: Tension Tension Headaches Headaches: Cluster Cluster Headaches Hearing Loss, Age-Associated)

67. References Brain 1912;34295507; Cummings JN. The copper and iron content of theliver and brain in the normal and hepatolenticular degeneration. http://www.medscape.com/content/2000/00/41/08/410849/410849_ref.html

68. IComm: File Not Found! hallux,hemimelia,hemoglobin c,hemoglobinopathy,hemolytic newborn,hemophagocytichistiocytosis,hemophilia,hepatolenticular degeneration,hemorrhagic nodular http://www.icomm.ca/geneinfo/h.htm

File Not Found We're sorry, but the file you're looking for can't be found. You might want to try starting at our front page or a list of iComm accountholders , or you can search the site This site was last updated on 30 August 2002. iComm Questions regarding the iComm web site should be sent to our webmaster

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

qualificatifs et types de ressources : D Menu général CISMeF A B C ... dacryocystite [dacryocystitis] dacryocysto-rhinostomie [dacryocystorhinostomy] dactylologie [manual communication] Danemark [Denmark] danse [dancing] daunorubicine [daunorubicin] De Lange, syndrome [De Lange syndrome] débit expiratoire forcé [forced expiratory flow rates] débit expiratoire pointe [peak expiratory flow rate] décalage horaire, syndrome [jet lag syndrome] décalage ribosomique [frameshifting, ribosomal] 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 [decompression sickness] décontamination [decontamination] décortication cérébrale [cerebral decortication] décubitus ventral [prone position] défaillance matériel [equipment failure] défaillance prothèse [prosthesis failure] défense consommateur [consumer advocacy] défense enfance [child advocacy] défense patient [patient advocacy] défibrillateur implantable [defibrillators, implantable]

70. Arch Ophthalmol -- Page Not Found Ophthalmologic findings in Wilson's hepatolenticular degeneration. Arch Ophthalmol. Magneticresonance imaging diagnosis of hepatolenticular degeneration. http://archopht.ama-assn.org/issues/v119n10/ffull/epe00059-1.html

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery MSJAMA Science News Updates Meetings Peer Review Congress The page you requested was not found. The JAMA Archives Journals Web site has been redesigned to provide you with improved layout, features, and functionality. The location of the page you requested may have changed. To find the page you requested, click here HOME CURRENT ISSUE PAST ISSUES ... HELP Error 404 - "Not Found"

71. Wilson's Disease (hepatolenticular Degeneration) Wilson's disease (hepatolenticular degeneration). A recessive geneticdisease in which affected individuals can't process and excrete http://www.cartage.org.lb/en/themes/Reference/dictionary/Biologie/W/61.html

72. Parkinson's Disease: Epidemiology hepatolenticular degeneration Tyrosine from the liver is normally convertedinto levodopa, which is converted into dopamine; Hallervorden http://dna2z.com/projects/PD/park3_6.html

Juvenile Parkinsonism Parkinsonism before age 40 Major cause: hepatolenticular (liver) degeneration Similar diseases: Hallervorden-Spatz (demyelination) Huntington's disease (demyelination of caudate nucleus) Pseudoparkinsonism Share some symptoms Benign essential (familial) tumor Hepatolenticular degeneration: Tyrosine from the liver is normally converted into levodopa, which is converted into dopamine Hallervorden-Spatz: Nerve fibers connecting striatum and pallium become demyelinated Huntington's disease: Demyelination in caudate nucleus, part of the striatum Other conditions sometimes mistaken for Parkinson's disease are hypo/hyperthyroidism and depression with severe psychomotor retardation

73. Health Content Encyclopedia Article Wilson Disease hepatolenticular degeneration Wilson disease (hepatolenticular degeneration) is arare inherited disorder, affecting approximately 1 out of 1 million people. http://www.centralbap.com/adamcontent/ency/article/000785.asp

74. Metabolic And Deficiency These metabolically active astrocytes have swollen nuclei with chromatin pushed againstthe nuclear membrane. Wilson's Disease (hepatolenticular degeneration) http://edcenter.med.cornell.edu/CUMC_PathNotes/Neuropathology/Neuropath_II/metab

Disorders of the CNS Metabolic and Deficiency Disorders of the CNS M etabolic D iseases Anoxia and Ischemia The brain is exquisitely sensitive to ischemia and somewhat less sensitive to pure hypoxia. Causes Stagnant or decreased blood flow Occurs in hypotensive crises (shock) and cardiac-arrest. Moderate decreases in blood flow will first affect the watershed areas between adjacent arterial supplies. Hypoxia Decreased oxygen tension in the blood, as in ARDS. Sommer's sector (hippocampal sector CA-1) is especially sensitive to hyoxic injury. CO poisoning Morphology of CO poisoning: If the patient survives, there will be damage to the white matter in the centrum semiovale after several days have passed. Thus the patient may awaken feeling fine and develop neurologic symptoms days later ("postanoxic delayed demyelination"). This is thought to occur because oligodendrocytes suffer hypoxic injury. Histotoxic As in cyanide poisoning leading to mitochondrial damage. Hypoglycemia Selective Vulnerability to Hypoxic Injury Hippocampus Cerebral cortex Purkinje cells in the cerebellar cortex L iver D iseases Hepatic Encephalopathy Causes Hyperammonemia and elevated levels of other compounds normally metabolized by the liver.

75. METABOLIC AND DEFICIENCY DISORDERS OF THE CNS Wilson's Disease (hepatolenticular degeneration) Autosomal recessive disease affectingthe liver (cirrhosis), the brain (degeneration of neurons in the basal http://edcenter.med.cornell.edu/CUMC_PathNotes/Neuropathology/Neuropath_II/METAB

METABOLIC AND DEFICIENCY DISORDERS OF THE CNS METABOLIC DISEASES Anoxia and Ischemia Lack of blood supply and/or lack of adequate oxygen delivery causes hypoxic damage to the nervous system: for example, post cardiac-arrest encephalopathy. The brain has a high metabolic demand reflected in the large proportion of cardiac output which it receives. Thus mild decreases in cerebral blood flow or oxygenation can cause changes in brain function. Causes 1. Stagnant or decreased blood flow Occurs in hypotensive crises (shock) and post cardiac-arrest. Moderate decreases in blood flow will first affect the watershed areas between adjacent arterial supplies. 2. Hypoxia Decreased oxygen tension in the blood, as in ARDS. Sommer's sector is especially sensitive to hyoxic injury. 3.Anemia (as in CO poisoning) Morphology of CO poisoning: If the patient survives, there will be demyelination of white matter in the centrum semiovale after several days have passed. Thus the patient may awaken feeling fine and develop neurologic symptoms days later ("postanoxic delayed demyelination"). This is thought to occur because oligodendrocytes suffer hypoxic injury. Because the metabolic turnover of myelin is slow, symptoms don't develop for days. 4. Histotoxic

76. Korsakov's Journal Of Neurology And Psychiatry - Abstracts ¹2 2000 VV Poleschuk, ED Markova, IA IvanovaSmolenskaya Acoustic brain stem and cognitiveevoked potentials (P300) in patients with hepatolenticular degeneration. http://www.mediasphera.aha.ru/neurol/2000/2/e2-00ref.htm

Abstracts Korsakov's journal of neurology and psychiatry ¹2 2000 A.E. Morenkova, O.R. Orlova, E.A. Dubanova Clinical-physiologic analysis of cranial dystonia S.V. Ivanov, A.M. Andreev Typologic differentiation of chronic agoraphobia V.V. Marilov, A.H. Crisp, D.I. Ben-Tovim Personal behaviour and mental state in anorexia nervosa Personality of the patients with anorexia nervosa is characterised by high neurotization and anxiety that resulted in diffuse anxiety in stress situation. Personal peculiarities included also high total hostility and intrapunitivity, inclination to obsessive-phobic and dysthymic reactions and tendency to somatization of anxiety. Such peculiarities promoted the choice of intrapunitivity type of reaction in situation of frustration. Its manifestation increases with an increase in the disease duration. The range of psychotic disorders in anorexia nervosa is restricted to anxious-depressive, obsessive and asthenic sphere. Yu.S. Martynov, E.V. Surskaya, E.V. Malkova, N.A. Shuvakhina Follow-up study of the patients operated for traumatic subdural hematomas In 56 patients operated for traumatic subdural hematomas clinical manifestations were analysed (8 main syndromes of the remote period) as well as the degree of neurologic rehabilitation and the level of social-occupational adaptation. Such adaptation appeared to be rather high: 66,1% of the patients were able to resume work. The highest lethality was in the acute period; the worst rehabilitation and follow-up adaptation were observed in elderly and old patients who were in comatose state before the operation and had severe accompanying contusion of the brain. Policy of drug treatment was determined in patients with traumatic subdural hematomas regarding peculiarities and manifestations of the syndromes (urgent operation, if necessary — cranioplasty, psychologic support in the remote period, resorption therapy and symptomatic drugs).

77. Korsakov's Journal Of Neurology And Psychiatry - Contents ¹10 2001 Morozov SG, Usanova MP, Poleschuk VV, Poletaev AB, IvanovaSmolenskaya IA, MarkovaED Nervous tissue protein autoantibodies in hepatolenticular degeneration. http://www.mediasphera.aha.ru/neurol/2001/10/e10-01con.htm

Contents Korsakov's journal of neurology and psychiatry ¹ Clinical Presentation of Nervous and Mental Diseases Golubev V.L., Korabelnikova E.A. Dreams of children and adolescents with high and low anxiety level Rudenskaya G.E., Shagina I.A., Vasserman N.N., Mersiyanova I.A., Dadali E.L., Polyakov A.V. Sensomotor neuropathy with X-linked dominant inheritance ... Prognostic factors for attack-like progredient schizophrenia Treatment of Nervous and Mental Diseases Rudnev V.A., Prokopenko S.V. The use of “controlled motor synergies” for rehabilitation of walking acquirements in parkinsonism Crawford P., Hall W., Chappell B., Collings J., Stewart A. Generic prescribing for epilepsy. Is it safe? Safarova T.P., Trifonov E.G., Belova M.Yu. Psychopharmacotherapy of out-patients with dementia Experimental and Theoretical Problems Morozov S.G., Usanova M.P., Poleschuk V.V., Poletaev A.B., Ivanova-Smolenskaya I.A., Markova E.D. Nervous tissue protein autoantibodies in hepatolenticular degeneration Golimbet V.E., Shcherbatyukh T.V., Abramova L.I., Kaleda V.G.,Oleichik I.V., Orlova V.A., Rogaev E.I. Serotonin transporter gene polymorphism in families with schizophrenia Clinical Notes Shuleshova N.V., Lapin S.V., Bolshakova O.V., Kazakov V.M., Skoromets A.A., Golikov K.V., Totolyan A.A.

78. Health Library Heparin. Hepatitis. hepatolenticular degenerationWilson’s Disease. Herbal Medicine.Herniated Disc-Low Back Pain. Herpes Simplex-Mouth Ulcers (Canker Sores). http://yalenewhavenhealth.org/Library/HealthGuide/CAM/_SearchResults.asp?letter=

79. GeneClinics : Wilson Disease Notes for physicians on Wilson Disease (hepatolenticular degeneration) covering diagnosis,clinical description, differential diagnosis, management, molecular http://omni.ac.uk/whatsnew/detail/1103703.html

Back to whats new page. GeneClinics : Wilson disease Notes for physicians on Wilson Disease (Hepatolenticular Degeneration) covering diagnosis, clinical description, differential diagnosis, management, molecular genetics and genetic counselling. Reviewed during October 1999, this resource forms part of GeneClinics, a peer-reviewed clinical genetic information resource that is funded by the US National Institutes of Health (NIH) and produced by the University of Washington, Seattle. Hepatolenticular Degeneration / genetics Last modified 10/May/2001 [Low Graphics]

80. Penicillamine used to treat Wilson's disease (hepatolenticular degeneration), cystinuria, chroniclead poisoning, and in patients with recalcitrant, severe rheumatoid http://home.caregroup.org/clinical/altmed/interactions/Drugs/Penicillamine.htm

Penicillamine Brand Names: Cuprimine, Depen Clinical Names: Penicillamine Summary generic name Penicillamine trade names : Cuprimine, Depen type of drug : Chelating agent. mechanism : Penicillamine increases the urinary excretion of copper. used to treat : Wilson's disease (hepatolenticular degeneration), cystinuria, chronic lead poisoning, and in patients with recalcitrant, severe rheumatoid arthritis. Wilson's disease is an inherited disease of copper accumulation caused by a failure of biliary excretion of excess copper. Accumulated copper causes liver disease in these patients, and in perhaps two thirds of patients, it causes brain damage leading to clinical neurologic or psychiatric dysfunction. Maintenance treatment involves reversing the positive copper balance. overview of interactions Vitamin B6 Pyridoxine) Copper Iron Sodium Zinc Food Interactions nutrient affected by drug Vitamin B6 Pyridoxine) (Jaffe IA. Ann N Y Acad Sci 1969 Sep 30;166(1):57-60; Bhagavan HN, Brin M. Curr Concepts Nutr nutrient affecting drug performance Copper nutrient affecting drug toxicity Iron Great caution needs to exercised when changing the level of iron intake in individuals taking penicillamine. Problems have been reported where the prescribing physician has been unaware that a patient has been using concomitant supplemental iron and responded to the lack of therapeutic response to penicillamine by increasing the drug dose. In one report of four patients toxicity only became apparent after iron was stopped and there was a sudden increase in penicillamine absorption with resulting penicillamine-induced kidney damage.

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