61. Dementia - Patient UK spinocerebellar degenerations Progressive myoclonic epilepsy, Multiple corticalinfarcts Cranial arteritis Cerebral arteritides (PAN, SLE ) Chronic subdural http://www.patient.co.uk/showdoc/40001832/

PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people. Dementia This is a syndrome where the patient has multiple cognitive deficits and memory loss (enough to impair occupational or social functioning); with either associated deterioration in language (aphasia), perception and comprehension (apraxia); impaired ability to recognise objects (agnosia); and/or a disturbance in executive functioning (inability to think abstractly and plan, initiate, sequence, monitor and stop complex behaviour). Deterioration must represent a progressive decline from a previous higher level of functioning, and consciousness should not be clouded (cf acute confusional state, delirium). Memory loss is typically for recent events and long-term memory can be remarkably intact. Acute confusional state is of recent onset and usually has a reversible cause. Dementia is more chronic and usually untreatable. The onset may be less acute than at first apparent as the patient manages to cover the deficiency by making notes or bluff but when compensatory mechanisms fail deterioration appears more rapid than it really is. Causes of adult onset dementia: (not an exhaustive list) and mixed pathologies are common (33.8%)

62. Portal Toolkit Invalid Site URL Cerebral blood flow in spinocerebellar degenerations a single photon emissiontomography study in 28 patients. J Neurol 1998;2456038. Context Link http://ppv.ovid.com/pt/re/obes/fulltext.00000132-199911000-00006.htm

Sorry, the URL specified, http://ppv.ovid.com:80/pt/re/obes/fulltext.00000132-199911000-00006.htm , is invalid. To go to site for "OvidPPV" please click here http://ppv.ovid.com To go to site for "Acta Neurologica Scandinavica" please click here http://www.lwwonline.com To go to the LWW Online site, please click here http://www.lwwonline.com Thank you

63. OMIM Entry 164600 earlier editions, it may be that linkage will provide the necessary methodfor unravelling the snarled nosology of the spinocerebellar degenerations. http://www.hgmp.mrc.ac.uk/cgi-bin/wrapomim?164600

64. Ataxia Rare Disorders . Spinocerebellar Degenerations Neurological Disorders . Conditions and Diseases . Health . Home Ataxia RareDisorders . spinocerebellar degenerations . Neurologic Manifestations . http://www.thema-gute-gesundheit.de/Health|DE-6404-6640|Conditions,and,Diseases|

Ataxia . Neurological Disorders Conditions and Diseases Health Home Ataxia : Rare Disorders . Spinocerebellar Degenerations . Neurologic Manifestations . Genetic Disorders Genetic Disorders + Information on a rare congenital disease. + Resources and information for families of children with genetic disabilities or syndromes that are still awaiting a diagnosis. + Complete in depth articles about each disease listed. + Papillon Lefevre, Haim-Munk Syndrome and Prepubertal + support group for XLH, a genetic condition also known as Neurologic Manifestations + National Institute of Neurological Disorders and Stroke (NINDS). + narrative with many related links describes the types. + The National Institute of Neurological Disorders and Stroke. Spinocerebellar Degenerations Rare Disorders + biosynthesis and pathophysiology of BH4 and tetrahydrobiopterin deficiency. + for parents and patients suffering from + general information and a personal account, including pictures and X-rays. Also offers patient contact and an email discussion group. This category in other languages: German + and fact sheet about Friedreich's Ataxia, compiled by NINDS, the National Institute of Neurological Disorders and Stroke.

65. Spinocerebellar Degenerations Friedreich S Ataxia spinocerebellar degenerations . Spinal Cord . Neurological Disorders . Conditions andDiseases . spinocerebellar degenerations Friedreich s Ataxia . http://www.thema-gute-gesundheit.de/Health|DE-6404-6640|Conditions,and,Diseases|

Spinocerebellar Degenerations . Spinal Cord Neurological Disorders Conditions and Diseases Health ... Home Spinocerebellar Degenerations : Friedreich's Ataxia . Olivopontocerebellar Atrophy . Ataxia . Machado Joseph Ataxia Friedreich's Ataxia + National Institute of Neurological Disorders + the synonyms, a general discussion and + site about living a positive life in spite of having a + summary, diagnosing, molecular genetic testing, clinical description and management. Machado-Joseph + access to medical care and improving life quality. + symptoms, diagnosis, how it is inherited and research. + at machado-joseph disease with some + the disease and the Jewish connection. Symptoms, diagnosis, ongoing research information, and Olivopontocerebellar Atrophy + Offers synonyms, a general discussion and further resources. + by National Institute of Neurological Disorders and Stroke. + has links, personal stories and a discussion board.

66. Centre For Molecular Biology And Neuroscience Neurogenetics of spinocerebellar degenerations. Key features of neurodegenerativedisorders associated with triplets repeats and polyglutamine expansions, http://www.cmbn.no/seminar-20031212.html

Home About CMBN Research groups News ... Internal CMBN PO Box 1105 Blindern N-0317 Oslo, Norway Tel: +47 22851528 Fax: +47 22851488 Norwegian-French mini symposium on rare neurodegenerative diseases and neurogenetics Friday 12 December - Saturday 13 December 2003 Please confirm your attendance to by email to chantal.tallaksen@ulleval.no, or by fax to +47 22115949. Programme Friday 12 December New tools and new challenges in neurogenetics Neurogenetics of spinocerebellar degenerations Key features of neurodegenerative disorders associated with triplets repeats and polyglutamine expansions, and implications for further research topics. Presentation of the Center for Molecular Biology and Neuroscience (CMBN) Novel DNA and RNA repair mechanisms DNA repair and genomic instability Glutamate transport and neurological disease How can or should clinicians, geneticists and molecular biologists work together in neurogenetics?

67. TheFetus.net - Bilateral Striatal Necrosis -Alessandro Giuffrida, MD, Saveria Ca Definition Bilateral striatal necrosis is a symmetrical degeneration of the Y.; Satoyoshi, E. spinocerebellar degenerations in Japan a nationwide http://www.thefetus.net/page.php?id=90

68. Vol20 Iss06 URL Http//www.flonnet.com/fl2006/stories Huntington s disease, Wilson s disease, spinocerebellar degenerations, Lewybody disease, idiopathic ganglia degenerations and so on. http://www.hinduonnet.com/thehindu/thscrip/print.pl?file=20030328001108000.htm&d

69. National University Corporation University Of Yamanashi Multiple system atrophies such as spinocerebellar degenerations and ShyDragersyndrome. Demyelinating diseases such as multiple sclerosis. http://www.yamanashi.ac.jp/english/medical/18sinkeinaika.html

▼Menu ■School of Medicine Department of Neurology General description of the department Research Themes Neurophysiology Analyses of autonomic nerve function in neurodegenerative disease using a microneurographic technique. Differentiation between physiologic and pathologic aging, mainly with respect to autonomic nerve function. Studies of the general effect on neurologic function from bathing in an artificially carbonated spring, including effects on pathologic states. Characterization of sleep disorders in neurologic disease, in relation to respiration and involuntary movements. Activities of afferent fibers from muscle spindles in neuromuscular diseases. Neuropathology Characterization of cerebrovascular pericytes in rats. Investigation of metabolism of prostaglandin D2 synthetic enzymes. Neuropathologic investigation of biopsy specimens of muscle and nerve. Neuropathologic investigation of Creutzfeldt-Jakob disease. Departmental neurologic practice: target diseases Dementia and underlying degenerative diseases such as Alzheimer's disease and Pick's disease.

70. Cerebellum Diseases include hereditary spinocerebellar degenerations (such as Friedrich sataxia) and degenerative disease due to too much ethanol imbibition. http://instruct.uwo.ca/anatomy/530/cblm.htm

Return to beginning of Anatomy 530a home page Return to Anatomy 530a list of topics Other parts of the brain involved in the control of movement The cerebellum: its parts and their connections. The cerebellum has three functional divisions, based on the principal sources of afferent fibres. The vestibulocerebellum (salmon pink, surrounded by a thin yellow line on the diagram) consists of the nodule and the two flocculi. Afferents are from the vestibular ganglion and nuclei. The spinocerebellum (light beige on diagram, and demarcated by a thick yellow line) consists of the vermis and adjacent cortex of the anterior lobe and of part of the posterior lobe. The dorsal and ventral spinocerebellar tracts terminate in these regions. The pontocerebellum is the largest division, comprising most of the cerebellar cortex (pale cream colour, almost white, in the diagram). Afferent fibres are from the pontine nuclei. Divisions and landmarks of the cerebellum. Click here to view. In addition, all parts of the cerebellum receive fibres from the contralateral inferior olivary complex of nuclei in the medulla.

71. Eugenio Medea - Scientific Research Brain dysmorphisms; congenital CNS – PNS pathologies; epilepsy; rare syndromes;degenerative diseases – spinocerebellar degenerations; http://www.emedea.it/english_medea/research.html

Scientific Institute Eugenio Medea for Research, Hospitalization and Health Care Who we are Where Departments Pathologies ... Italian site SCIENTIFIC RESEARCH Research Areas Neuropathology Neuromotor Rehabilitation and Functional Neuropsychology Rehabilitation of Infantile Cerebral Palsy; Rehabilitation of neuromuscular diseases; Rehabilitation of acquired brain lesions; Study and rehabilitation of spinal and musculoskeletal disorders; Orthopaedic rehabilitation post-surgery; Rehabilitation of congenital and acquired bone marrow lesions; Rehabilitation of neurological bladder dysfunction Psychopathology of Development, Language and Learning Cognitive disorders and learning disabilities; Clinical psychology; Neurolinguistics and Psycholinguistics; Clinical and biological psychiatry Clinical and experimental neurophysiology; static and functional imaging. Neurobiology Chromosomal diseases; Biomolecular diagnostics; Genic Therapy; Biological Material Bank; Murine Transgenic models and Know-out Bioengineering Error analysis; Virtual reality; Application of Neuroimaging techniques; HW and SW development and aids; Development, experimentation and assessment of electro-medical devices; Tele-medicine; Support technologies for disabilities

72. HEALTHMEDNET ILLNESS DISEASE URL DIRECTORY Spinocerebellar degeneration see Friedreich s ataxia.http//www.nlm.nih.gov/medlineplus/encyclopedia.html. spinocerebellar degenerations http://www.healthmednet.com/SPIN-STE.htm

73. Myoclonus types of slowly progressive neurologic condions that may or may not run infamilies (eg. spinocerebellar degenerations, basal ganglia degenerations, http://www.cmdg.org/Movement_/myoclonus/myoclonus.htm

The Canadian Movement Disorder Group MYOCLONUS Definition: A sudden "Shock" like muscular movement Myoclonus can be Classified By: 1) The Electrophysiologic Phenomenon (certain electrical characteristics) Negative Myoclonus (asterixis) a sudden  relaxation of a group of muscles. 2) Classification by the Anatomical Origin Electical Discharge From the surface of the brain ( Cortical Myoclonus ).............Epileptic From deeper within the brain ( Subcortical Myoclonus From an electrical impulse within the spinal cord ( Spinal or Segmental Myoclonus From an electrical impulse within a peripheral nerve ( Peripheral Myoclonus eg. hemifacial spasm) 3) Classification byBody Parts Involved Focal If only one body part is affected myoclonus is called "focal" . (eg. myoclonic jerks of 1/2 the face ( "hemifacial spasm" ), or when the eyes are affected "Opsoclonus" Segmental If only one segment of the body is affected myoclonus is called "segmental"

74. Postgraduate Medicine: Primary Care Guide To Myoclonus And Chorea B. spinocerebellar degenerations C. Basal ganglia degenerations D. Dementias E.Infection or postinfectious syndromes F. Metabolic 1. Electrolyte disorders http://www.postgradmed.com/issues/2000/10_00/caviness.htm

Primary care guide to myoclonus and chorea Characteristics, causes, and clinical options John N. Caviness, MD VOL 108 / NO 5 / OCTOBER 2000 / POSTGRADUATE MEDICINE CME learning objectives To become familiar with the major diagnostic categories of myoclonus To recognize the major clinical characteristics of Huntington's disease To learn the common causes of chorea and to recognize characteristics of various types of choreic disorders The author discloses no financial interests in this article. This is the third of four articles on hyperkinetic movement disorders This page is best viewed with a browser that supports tables. Preview : Myoclonic and choreic disorders encompass a wide range of manifestations, from simple jerking during sleep in neurologically normal persons to wild, potentially injurious flailing in patients with ballismus. Recognition of the type and cause of the disorder is the first step toward helping patients achieve better functioning when possible. Dr Caviness reviews classification of myoclonus and chorea and discusses appropriate evaluation and the available treatment options. Caviness JN. Primary care guide to myoclonus and chorea: characteristics, causes, and clinical options. Postgrad Med 2000;108(5):163-72

75. Sites Médicaux Francophones : Classement Par Mots Clés MeSH, Qualificatifs Et degeneration;dégénérescences spinocérébelleuses spinocerebellar degenerations http://www.chu-rouen.fr/ssf/santpathd.html

Classement par mots clés MeSH, qualificatifs et types de ressources : D A B C D ... dacarbazine [dacarbazine] dacryocystite [dacryocystitis] dacryocysto-rhinostomie [dacryocystorhinostomy] dactinomycine [dactinomycin] 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 ribosomique [frameshifting, ribosomal] decapoda (crustacea) [decapoda (crustacea)] déchets [waste products] déchets dangereux [hazardous waste] déchets dentaires [dental waste] déchets industriels [industrial 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] décontamination [decontamination] décortication cérébrale [cerebral decortication] décubitus ventral [prone position] défaillance de prothèse [prosthesis failure] défaillance du matériel [equipment failure] défaillance hépatique [liver failure] défaillance multiviscérale [multiple organ failure]

76. NIH Guide: THE HEREDITARY ATAXIAS INCLUDING MACHADO-JOSEPH DISEASE interchangeably referred to as spinocerebellar degenerations, Onset ofsystem degenerations may span a lifetime but often begin in the first to http://grants.nih.gov/grants/guide/pa-files/PA-92-048.html

Return to 1992 Index Return to NIH Guide Main Index Department of Health and Human Services National Institutes of Health (NIH) 9000 Rockville Pike Bethesda, Maryland 20892

77. Dementia Is A Clinical Syndrome Characterized By Acquired Losses Of Cognitive An spinocerebellar degenerations memory and frontal dysfunction + cerebellar signs.Idiopathic basal ganglia calcifications. http://www.indegene.com/Psy/FeatArt/indPsyFeatArt4.html

Dementia Approach To Evaluation Department of Neurology All India Institute of Medical Sciences New Delhi Dementia is a clinical syndrome characterized by acquired losses of cognitive and emotional abilities severe enough to interfere with daily functioning. Dementing illness is an important cause of disability in the elderly. With improving medical facilities and an increasing life expectancy, the number of patients with demential is expected to rise. The care of these patients will have a growing impact and will pose an increasing burden on the family, the health care systems and the society. Epidemiology The prevalence rates according to western studies range from 5-10 percent for all persons over the age of 65 years. These rates appear to double roughly every 5 years to reach 25-50 percent for persons over 85 years of age . The exact prevalence of these disorders in India is difficult to guess, as no recent population based prevalence study has been published. However, in a population-based survey reported in 1981, of 18,721 individuals, 861 were above the age of 60 years. Of these 181 randomly selected individuals, 6 percent were found to be demented . Most of the other studies conducted in India are inadequate as the validity of the criteria used to define and assess dementia needs to be questioned. Another confounding factor is the diversity of educational status and literacy in our population. There is also a general belief that the amount of vascular dementias (VaD) would outnumber the Alzheimer's dementias (AD) in our population.

78. Received From JNET-Daemon By UNCVX1.BITNET; Mon, 30 Mar 92 0818 in general, encompass a variety of degenerative disorders, interchangeablyreferred to as spinocerebellar degenerations, that are difficult to classify http://www.ibiblio.org/pub/docs/nih-nsf/nihguide2.920306

Received: from JNET-Daemon by UNCVX1.BITNET; Mon, 30 Mar 92 08:18 EDT Received: From UNC(MAILER) by UNCVAX1 with Jnet id 0031 for PJONES@UNCVAX1; Mon, 30 Mar 92 08:18 EST Date: Mon, 30 Mar 92 08:14 EST From: Dot Baker

79. Humana Press Familial AdultOnset spinocerebellar degenerations, James H. Bower, md.Corticobasal Degeneration, Bradley F. Boeve, md. http://www.humanapress.com/ProductDetail.pasp?txtCatalog=HumanaBooks&txtCategory

80. Home       Objectives       Schedule       Lectures      spinocerebellar degenerations, progressive supranuclear palsy). Granulovacuolar degeneration of cells in the hippocampus. Some of these findings can http://umed.med.utah.edu/neuronet/lectures/2002/cognitive testing in dementia.ht

Home Objectives Schedule Lectures ... Download for PDA Cognitive Testing in Patients with Suspected Dementia T. Schenkenberg, Ph.D. Department of Neurology OBJECTIVES To know the diagnostic criteria for the diagnosis of dementia To list five causes of dementia To understand the importance of age-based norms when evaluating cognition in the elderly To recognize that there are reversible causes of dementia To name three basic tests used in the evaluation of possible dementia DEMENTIA DEFINITION A progressive loss of intellectual abilities of sufficient severity to interfere with social and/or occupational functioning. “Dementia” is a descriptive term, not a specific disease or condition. There are dozens of pathophysiologic causes of dementia. Because dementia is seen in the context of so many conditions, dementia is one of the most common findings encountered by neurologists who see adult patients. A. MAGNITUDE OF THE PROBLEM The population over 65 years of age is increasing rapidlyfrom 23 million in 1978 (11% of the population) to 51 million by 2030 (about 19% of the population). If life expectancy continues to rise, and the mean age of onset of dementia does not, some authors have projected that about 45% of the population would develop dementia at some point during their lives (with life expectancy extending into the late 90’s).

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