81. Olivopontocerebellar Atrophy - Wikipedia, The Free Encyclopedia olivopontocerebellar atrophy of neonatal onset and Two brothers presented with olivopontocerebellar atrophy of neonatal onset.The clinical features (failure to thrive, hypotonia, liver disease, effusions, http://en.wikipedia.org/wiki/Olivopontocerebellar_atrophy

You did it! Over US$240,000 was donated in the 21 day fund drive. Thank you for your generosity! You are still welcome to make a donation or purchase Wikimedia merchandise Olivopontocerebellar atrophy From Wikipedia, the free encyclopedia. Olivopontocerebellar atrophy Olivopontocerebellar atrophy (OPCA) are a group of diseases characterized by neuronal degeneration in the cerebellum , pontine nuclei, and inferior olive. They are also referred to as spinocerebellar atrophies or ataxias SCA ). Some also involve brain stem motor nuclei and/or cerebral cortex. All produce gait ataxia, and some also result in tremors, proprioceptive abnormalities, dysarthria , brain stem motor impairment, or dementia. Most are autosomal dominant in inderitance pattern. The primary cause of these hereditary ataxias also appears to be an unstable expansion of the polyglutamine trinucleotide repeat CAG, similar to Huntington's disease edit Subcategories Olivopontocerebellar atrophy is group of disorders which overlap certain other groups, such as Spinocerebellar ataxia (SCA). Some but not all types of SCA are in the Olivopontocerebellar atrophy group. Some but not all Olivopontocerebellar atrophy conditions, are types of SCA. This situation causes some controversy and confusion about what terms and system of categorization should be used. The subcategories of Olivopontocerebellar atrophy are:

82. Familial Olivopontocerebellar Atrophy With Neonatal Onset: A Recessively Inherit Familial olivopontocerebellar atrophy with neonatal onset a recessively inheritedsyndrome with systemic and biochemical abnormalities http://jnnp.bmjjournals.com/cgi/content/abstract/51/3/385

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Harding, B. N. Articles by Erdohazi, M. Journal of Neurology, Neurosurgery, and Psychiatry, 1988, Vol 51, 385-390 PAPERS Familial olivopontocerebellar atrophy with neonatal onset: a recessively inherited syndrome with systemic and biochemical abnormalities BN Harding, DB Dunger, DB Grant and M Erdohazi Institute of Neurology, National Hospitals for Nervous Diseases, London, UK. Clinical and pathological findings are reported in two siblings who presented in the neonatal period with failure to thrive, hypotonia

83. Reflex Myoclonus In Olivopontocerebellar Atrophy -- Rodriguez Et Al. 57 (3): 316 Reflex myoclonus in olivopontocerebellar atrophy patients with a presumeddiagnosis of olivopontocerebellar atrophy (OPCA) and in 30 age matched control http://jnnp.bmjjournals.com/cgi/content/abstract/57/3/316

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Rodriguez, M. E. Articles by Obeso, J. A. Journal of Neurology, Neurosurgery, and Psychiatry, 1994, Vol 57, 316-319 PAPERS Reflex myoclonus in olivopontocerebellar atrophy ME Rodriguez, J Artieda, JL Zubieta and JA Obeso Clinica Universitaria, University of Navarra, Pamplona, Spain. The presence of reflex myoclonus in response to touching and pin- pricking the wrist or stretching the fingers and to photic stimulation was assessed in 24 patients with a presumed diagnosis of olivopontocerebellar atrophy (OPCA) and in 30 age matched control subjects. Reflex myoclonus to

84. Olivopontocerebellar Atrophy In Two Adult Cats, Sporadic Cases Or New Genetic En olivopontocerebellar atrophy (OPCA) in a domestic animal species was rarely reported.Some features allow tentative linking to either familial or sporadic http://www.vetpathology.org/cgi/content/abstract/41/1/20

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS This Article Full Text Full Text (PDF) Alert me when this article is cited ... Alert me if a correction is posted Services Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager Search for citing articles in: ISI Web of Science (1) PubMed PubMed Citation Articles by Poncelet, L. Vet Pathol American College of Veterinary Pathologists Olivopontocerebellar Atrophy in Two Adult Cats, Sporadic Cases or New Genetic Entity and L. Poncelet Two otherwise healthy adult cats were presented with progressive cerebellar signs of different severity. Owners requested euthanasia. Necropsy disclosed whole cerebellum and pontine atrophy, with a severity paralleling the neurologic dysfunction. We used cell The severity of the cerebellar cortex atrophy followed a general gradient from the midvermis toward the hemispheres and a local gradient from the depth of the folia toward their tip. Along

85. Olivopontocerebellar Atrophy, Hereditary Synonyms, Eastern Carolina olivopontocerebellar atrophy, Hereditary Synonyms University Health Systems ofEastern Carolina serves tarboro, ahoskie, edento, winsor, maxhead, http://www.uhseast.com/113722.cfm

Health News Health Library Health Topics Healthy Living ... Nutrition Information on diseases and health concerns, including symptoms, treatment options, and prevention. Olivopontocerebellar Atrophy, Hereditary Synonyms Disorder Subdivisions General Discussion Resources ... For a Complete Report Important It is possible that the main title of the report Olivopontocerebellar Atrophy, Hereditary is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Hereditary OPCA Disorder Subdivisions OPCA, Menzel type Spinocerebellar Ataxia 1 OPCA, Holguin type Spinocerebellar Ataxia 2 SCA, Cuban type Spinocerebellar Ataxia 7 OPCA with retinal degeneration OPCA, Fickler-Winkler type OPCA, Schut-Haymaker type OPCA with dementia and extrapyramidal signs General Discussion Hereditary olivopontocerebellar atrophy (OPCA) is a rare group of disorders characterized by progressive balance problems (disequilibrium), progressive impairment of the ability to coordinate voluntary movements (cerebellar ataxia), and difficulty speaking or slurred speech (dysarthria). There are at least five distinct forms of hereditary OPCA. All forms of hereditary OPCA, except one, are inherited as autosomal dominant traits. Resources WE MOVE (Worldwide Education and Awareness for Movement Disorders) 204 West 84th Street New York, NY 10024

86. Resource Library Find Information On Olivopontocerebellar Atrophy Find information on olivopontocerebellar atrophy at MerckSource. Learn more aboutolivopontocerebellar atrophy, olivopontocerebellar atrophy is a http://www.mercksource.com/pp/us/cns/cns_hl_adam.jspzQzpgzEzzSzppdocszSzuszSzcns

87. Multiple System Atrophy Following Chronic Carbon Disulfide Exposure This report describes a case of olivopontocerebellar atrophy, Magnetic resonanceimage showing olivopontocerebellar atrophy in a patient who had been http://ehp.niehs.nih.gov/members/1998/106p611-613frumkin/frumkin-full.html

Author Keyword Title Full Environmental Health Perspectives EHP ) is a monthly journal of peer-reviewed research and news on the impact of the environment on human health. EHP content is free online and available in print issues through paid subscription. Volume 113, Number 9 September 2005 Environmental Health Perspectives Volume 106, Number 9, September 1998 Grand Rounds Multiple System Atrophy Following Chronic Carbon Disulfide Exposure Howard Frumkin Department of Environmental and Occupational Health, Rollins School of Public Health of Emory University, Atlanta, GA 30322 USA Introduction Case Presentation Discussion Conclusion Abstract Key words : carbon disulfide, cellulose, environmental diseases, movement disorders, multiple system atrophy, occupational diseases, olivopontocerebellar atrophy, rayon, textiles. Environ Health Perspect 106:611-613 (1998). [Online 18 August 1998]. http://ehpnet1.niehs.nih.gov/docs/1998/106p611-613frumkin/ abstract.html Address correspondence to H. Frumkin, Department of Environmental and Occupational Health, Rollins School of Public Health of Emory University, 1518 Clifton Road, Atlanta, GA 30322 USA. This work was supported in part by NIEHS Environmental/Occupational Medicine Academic Award 5 KO7 ESO0257.

88. Environ Health Perspect 108-3, 2000: Correspondence However, Frumkin pointed out that olivopontocerebellar atrophy is part of aspectrum of diseases termed multisystem atrophy, which also includes http://ehp.niehs.nih.gov/docs/2000/108-3/correspondence.html

Author Keyword Title Full Environmental Health Perspectives EHP ) is a monthly journal of peer-reviewed research and news on the impact of the environment on human health. EHP content is free online and available in print issues through paid subscription. Volume 113, Number 9 September 2005 Environmental Health Perspectives Volume 108, Number 3, March 2000 Correspondence Carbon Disulfide The September 1998 issue of EHP contained two articles about the neurotoxicity of carbon disulfide. The "NIEHS News" article ( ) reported on a collaborative study that involved scientists from the NIEHS (Research Triangle Park, NC), the U.S. Environmental Protection Agency (Research Triangle Park, NC), the University of North Carolina (Chapel Hill, NC), Duke University (Durham, NC), and Vanderbilt University (Nashville, TN). In this study, the neurotoxicity of carbon disulfide was detailed from the earliest molecular alterations to neurobehavioral findings to electrophysiologic and morphologic changes, and the utility of intramolecular cross-linking in hemoglobin as a biomarker was defined. I was pleased to read this report, and even more pleased to have participated in this study, but I was distressed to see the cover story in the same issue. "Multiple System Atrophy Following Chronic Carbon Disulfide Exposure" ( ), in the "Grand Rounds in Environmental Medicine," is a case report of an individual who developed a degenerative nervous system disease, olivopontocerebellar atrophy, and who had been chronically exposed to carbon disulfide while working for 34 years in a viscose rayon plant in the United States. Frumkin (

89. Arch Neurol -- Abstract: Morphometric Studies In Dominant Olivopontocerebellar A Morphometric studies in dominant olivopontocerebellar atrophy. Comparison of celllosses with amino acid decreases. EM Bebin, J. Bebin, RD Currier, http://archneur.ama-assn.org/cgi/content/abstract/47/2/188

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 47 No. 2, February 1990 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Bebin EM Perry TL Contact me when this article is cited Morphometric studies in dominant olivopontocerebellar atrophy. Comparison of cell losses with amino acid decreases E. M. Bebin, J. Bebin, R. D. Currier, E. E. Smith and T. L. Perry Department of Pathology, University of Mississippi Medical Center, Jackson. We present a correlation of the morphometric cell density analysis with previous biochemical findings for the inferior olivary nucleus and Purkinje cell layer of the cerebellum from 10 patients (three kindreds) with dominant olivopontocerebellar atrophy. We have analyzed brain amino

90. Arch Neurol -- Abstract: Neuropsychological Changes In Olivopontocerebellar Atro Neuropsychological changes in olivopontocerebellar atrophy. S. Berent, B.Giordani, S. Gilman, L. Junck, S. Lehtinen, DS Markel, M. Boivin, KJ Kluin, http://archneur.ama-assn.org/cgi/content/abstract/47/9/997

Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress Vol. 47 No. 9, September 1990 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager PubMed citation Articles in PubMed by Berent S Koeppe RA Articles that cite this article Contact me when this article is cited Neuropsychological changes in olivopontocerebellar atrophy S. Berent, B. Giordani, S. Gilman, L. Junck, S. Lehtinen, D. S. Markel, M. Boivin, K. J. Kluin, R. Parks and R. A. Koeppe Department of Psychiatry, University of Michigan, Ann Arbor. We used standardized neuropsychological measures of intellectual, cognitive, psychomotor, and emotional functioning to compare 39 patients with olivopontocerebellar atrophy and 25 normal controls of similar age. The patients reflected greater depression, anxiety, and subjective

91. Author Information Key Words Multiple system atrophy, olivopontocerebellar atrophy, Orthostatichypotension, Olivopontocerebellar pathology in multiple system atrophy. http://www.ptjournal.org/May99/May99_abs/v79n5p488.cfm

Author Information Article Information Reprint Information Table of Contents ... APTA Home Physical Therapy Update Multiple System Atrophy Laurie Swan and Jerome Dupont Click here for the PDF version of this art icle. Download free Adobe Acroba t Reader software Key Words: Multiple system atrophy, Olivopontocerebellar atrophy, Orthostatic hypotension, Parkinson disease, Shy-Drager syndrome, Striatonigral degeneration. M ultiple system atrophy (MSA) is a neurological disorder that has frequently been misdiagnosed as idiopathic Parkinson disease. A proliferation of articles in recent literature has noted that MSA is a disease that primarily affects the functioning of the autonomic, basal ganglia, and cerebellar systems. The History and Nomenclature of MSA The recognition of MSA as a unique entity has emerged gradually during the last century. Quinn, in describing several cases occurring in the late 1800s and early 1900s, may have provided the first descriptions of MSA. In 1925, Bradbury and Eggleston wrote the first clear case report of 3 patients with postural hypotension. Today, we know that postural hypotension is a characteristic sign of MSA. In 1960, Shy and Drager

92. Other Ataxias MSA with Cerebellar Ataxia Sporadic olivopontocerebellar atrophy (OPCA).NINDS Pamphlet - olivopontocerebellar atrophy (OPCA). January 1997 http://internaf.org/ataxia/othatax.html

Other Ataxias and related disorders Multiple System Atrophy (MSA): http://www.ndrf.org/MSA.htm June 1997 Acta Neuropathol: The distribution and dynamic density of oligodendroglial cytoplasmic inclusions (GCIs) in multiple system atrophy August 1994 Brain: Clinical features and natural history of multiple system atrophy. Ananalysis of 100 cases. November 1993 Nippon Rinsho: Multiple system atrophy (MSA) MSA with Cerebellar Ataxia - "Sporadic Olivopontocerebellar Atrophy" (OPCA): NINDS Pamphlet - Olivopontocerebellar Atrophy (OPCA) January 1997 Movement Disorders: Cerebellar presentation of multiple system atrophy. March 1996 Movement Disorders: Olivopontocerebellar pathology in multiple system atrophy. MSA With Autonomic Failure - "Shy-Drager Syndrome" (SDS): Jeff's Shy-Drager Page Shy-Drager Syndrome American Autonomic Society CWRU Autonomic Labratory June 1997 Axone: Shy-Drager Syndrome March 1997 New England Journal of Medicine: Autonomic Disorders and Their Recognition MSA with "Striatonigral Degeneration" (SND): January 1997 Nippon Rinsho: Striatonigral Degeneration Episodic Ataxia (EA): Episodic Ataxia Information by Mark Dias OMIM entry for Episodic Ataxia Type 1 OMIM entry for Episodic Ataxia Type 2 OMIM entry for CSE (Choreoathetosis with Spasticity and Episodic Ataxia) Autosomal Recessive Spastic Ataxia of Charelevoix-Saguenay (ARSACS):

93. Association For Patient-Oriented Research Multiple System Atrophy and olivopontocerebellar atrophy mentioned. Evolution ofsporadic olivopontocerebellar atrophy into multiple system atrophy. http://www.mc.vanderbilt.edu/gcrc/aas/patient resources/SD-MSA news february 200

Shy-Drager/MSA Support Group Multiple System Atrophy News Click figure to go to SDS/MSA Website February 1, 2001 Table of Contents 1. Iron Metabolism and MSA 2. Oxidative Stress and MSA 3. Dr. Sid Gilman to Speak at National Ataxia Foundation Annual Conference 4. Research Articles by Dr. Sid Gilman 5. Curing Parkinson's Disease in Our Lifetime 1. Iron Metabolism and MSA Iron Problems May Lead to Parkinson's: Mouse study shows direct link January 30, 2001 article. Multiple System Atrophy and Olivopontocerebellar atrophy mentioned. Mouse With Iron Disorder Offers Clues To Parkinson's, Similar Diseases January 31, 2001 article. MSA and OPCA mentioned 2. Oxidative Stress and MSA Oxidative Damage Linked Directly to Neurodegeneration November 3, 2000 article. MSA is mentioned http://www.grg.org/ParkO2Dam.htm Evidence Links Protein Damage To Parkinson's November 3, 2000 article - MSA and other synucleinopathies are included in this article http://www.sciencedaily.com/releases/2000/11/001103071439.htm Oxidative Stress and Brain Disorders This article gives some background on what oxidative or "free radical" damage is.

94. Ol : On Medical Dictionary Online olivopontocerebellar atrophy, Inherited olivopontocerebellar atrophy, Nonfamilial Olivopontocerebellar Degeneration Olivopontocerebellar Degenerations http://www.online-medical-dictionary.org/ol.asp?q=~Ol

95. The Spinocerebeller Ataxias Spinocerebellar atrophy I; olivopontocerebellar atrophy I (OPCA 1); Spinocerebellar atrophy II; olivopontocerebellar atrophy I, Holguin type; OPCA 2; http://www.compgene.com/sca.htm

The Spinocerebeller Ataxias The spinocerebellar ataxias are a group of autosomal dominantly inherited ataxias with heterogeneous presentation. Characteristic CAG repeat expansions in the coding sequences at several loci have been detected for certain of these disorders. Hence, a direct DNA test can provide an unequivocal diagnostic result for what are often complex clinical presentations. The loci reponsible for this group of disorders continues to grow and are the subject of research strategies evaluating trinucleotide (especially CAG trinucleotide) repeat expansions. A recent comprehensive clinical and molecular genetic reference is provided by Koshy and Zoghbi (1997), Brain Pathology 7:927-942. Although this is a recent review, it is out of date as the locus for SCA7 has been recently described (David et al. (1997) Nature Genetics 17:65-70; Koob et al. (1998) Nature Genetics 18:72-75.) The Table below provides a cross-reference of SCA genetic loci and a variety of alternative clinical names. In addition, by clicking on the highlighted OMIM reference number, you will be linked to the appropriate OMIM page. These pages provide current reviews of the genetics and clinical features of these disorders. Gene Name Locus Alternative Name OMIM Link SCA 1 Spinocerebellar atrophy I; Olivopontocerebellar atrophy I (OPCA 1); Menzel type OPCA

96. Search By Disease olivopontocerebellar atrophy III (OPCA3). 33, olivopontocerebellar atrophy,Holguin type. 34, olivopontocerebellar atrophy, neonatal (OPCA neonatal) http://www.eddnal.com/directory/disease.php?letter=O&page=3

97. The Spectrum Of Pathological Involvement Of The Striatonigral And Olivopontocere striatonigral and olivopontocerebellar systems in multiple system atrophy system atrophy; striatonigral degeneration; olivopontocerebellar atrophy; http://brain.oxfordjournals.org/cgi/content/short/awh303v1

JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... SEARCH QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Brain Advance Access published online on October 27, 2004 Brain, doi:10.1093/brain/awh303 Guarantors of Brain This Article Full Text (PDF) All Versions of this Article: most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Request Permissions PubMed PubMed Citation Articles by Ozawa, T. Articles by Revesz, T. Received June 7, 2004 Revised July 28, 2004 Accepted July 30, 2004 Article The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlations Tetsutaro Ozawa Dominic Paviour Niall P. Quinn Keith A. Josephs Hardev Sangha Linda Kilford Daniel G. Healy Nick W. Wood Andrew J. Lees Janice L. Holton and Tamas Revesz Queen Square Brain Bank, Department of Molecular Neuroscience, Institute of Neurology, London, UK

98. By A Everybody agrees that olivopontocerebellar atrophy is superinenarrable, as St.Augustine described the supremely indescribable. http://www.aepwall.com/OPCA page.htm

Rare diseases / OPCA Page WallPaper from the computer of A. E. P. (Ed) Wall Why pray for healing of an incurable disease? From The Living Church magazine, see second article below, on this page To learn more about OPCA go to www.alyshia.com/opca/ On The Living Church cover, at right, the Rev. Canon Nelson Pinder speaks in favor of a new school tax in Orlando, Florida. Canon Pinder is a retired priest of the Episcopal Diocese of Central Florida who, despite retirement, is active in the Central Florida church and community and in the Episcopal Church at the national level. Photo by Frank Rivera of the Orlando Sentinel , which is one of the nation's better newspapers. (Full disclosure: I've written more than a hundred op-ed columns for the Orlando Sentinel and read it daily via the Internet.) To get to HOME PAGE click here Is this disease a woozydoozy, or what? By A. E. P. (Ed) Wall E verybody agrees that olivopontocerebellar atrophy is superinenarrable, as St. Augustine described the supremely indescribable. The relative handful of people who have the disease abbreviate it OPCA, but that’s not a lot of help. OPCA could (and does) also stand for Ohio Pest Control Assn. or Oregon Police Canine Assn., rather than a murderous ailment listed in the exclusive ranks of the National Organization for Rare Disorders.

99. QUT | EPrints Archive - Motor Learning Processes In A Movement-scaling Task In O Nine Parkinson s disease (PD), seven olivopontocerebellar atrophy (OPCA) patientsand two agematched control groups learned a linear arm movement-scaling http://eprints.qut.edu.au/archive/00000792/

Motor learning processes in a movement-scaling task in olivopontocerebellar atrophy and Parkinson's disease Smiley-Oyen, Ann L and Worringham, Charles J and Cross, Carol L (2003) Motor learning processes in a movement-scaling task in olivopontocerebellar atrophy and Parkinson's disease. Experimental Brain Research Full text available as: PDF - Requires Adobe Acrobat Reader or other PDF viewer. Abstract EPrint Type: Journal (Paginated) Status: Published Refereed: Yes Keywords: Motor learning; Parkinson's disease, OPCA, cerebellar disease; scaling; schema; skill; retention Subjects: .QUT Faculties and Divisions Faculty of Health ID Code: Deposited By: Worringham, Charles Deposited On: 09 March 2005 Alternative Locations: Additional Information: Subscribers can also access the publisher version of this paper via SpringerLink. Use the hypertext link above. QUT Division of Technolgy, Information and Learning Support Library ITS ... Office of Research Cricos No. 00213J Accessibility Privacy Last updated 28.10.03 QUT ePrints Administrator

100. Cognitive Affective Psychosis Syndrome In A Patient With Sporadic http://neuro.psychiatryonline.org/cgi/reprint/17/2/260

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