21. Olivopontocerebellar Atrophies Topic - Unified Search Environment Ataxia Telangiectasia Cerebellar Neoplasms DandyWalker Syndrome; SpinocerebellarDegenerations Spinal Cord Neoplasms; spinocerebellar degenerations http://www.use.hcn.com.au/portals/shared/subject.`Olivopontocerebellar Atrophies

Olivopontocerebellar Atrophies Topic Tree Definition: A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085) Synonyms and Source Vocabularies: Olivopontocerebellar Atrophies Dejerine-Thomas Syndrome Olivopontocerebellar Degeneration PRESENILE ATAXIA SYNDROME Olivo-Ponto-Cerebellar Atrophy DEJERINE-THOMAS ATROPHY Olivo-Ponto-Cerebellar Degeneration Presenile Ataxia Pontoolivocerebellar Atrophy Cerebellar Diseases Ataxia Telangiectasia Cerebellar Neoplasms Dandy-Walker Syndrome Spinocerebellar Degenerations Myoclonic Cerebellar Dyssynergia Friedreich Ataxia Olivopontocerebellar Atrophies Spinocerebellar Ataxias ... Muscular Atrophy, Spinal

22. BioMed Central | Abstract | Spinocerebellar Degenerations: An Update Over the past decade, the spinocerebellar degenerations have gone from a diversegroup of loosely defined phenotypes to a family of diseases with many http://www.biomedcentral.com/1528-4042/2/331/abstract

home journals A-Z subject areas advanced search ... Issue 4 Viewing options Abstract Full text PDF Related literature: Other articles by authors Perlman SL Tools: E-mail to a friend Download references Report Susan L Perlman MD Department of Neurology, 300 UCLA Medical Plaza Suite B200, UCLA School of Medicine, Los Angeles, CA, 90095, USA Current Neurology and Neuroscience Reports Published Abstract Over the past decade, the spinocerebellar degenerations have gone from a diverse group of loosely defined phenotypes to a family of diseases with many identifiable genotypes and the promise of gene-specific treatments. The evaluation of the spinocerebellar ataxias has been simplified, and the counseling of patients and families has been enhanced by the growing number of molecular diagnostic tests now available. Management strategies remain symptomatic and focused on rehabilitation, with empirical use of antioxidants based on research in other neuro-genetic diseases. Terms and Conditions Privacy statement Information for advertisers Contact us

23. BioMed Central | Full Text | Spinocerebellar Degenerations: An Update To obtain access to Current Neurology and Neuroscience Reports through yourinstitution use the options below. If you would like information about a http://www.biomedcentral.com/1528-4042/2/331

home journals A-Z subject areas advanced search ... my BioMed Central To obtain access to Current Neurology and Neuroscience Reports through your institution use the options below. If you would like information about a personal subscription, please contact subs@phl.cursci.com Click here to view an abstract of this article Click here to login if you are already a subscriber to Current Neurology and Neuroscience Reports Subscribe to Current Neurology and Neuroscience Reports Register for a free online trial Ask your librarian to investigate institutional access Athens users please click here to gain access If you believe you are seeing this page in error, or cannot activate your subscription, e-mail us info@biomedcentral.com Terms and Conditions Privacy statement Information for advertisers ... Contact us

24. PsychiatryOnline - Search Results Search Results spinocerebellar degenerations. Book Results (0), Journal Results (1) Do a fulltext search for spinocerebellar degenerations http://www.psychiatryonline.com/searchResult.aspx?rootterm=spinocerebellar degen

25. Spinocerebellar Ataxia spinocerebellar degenerations. Timothy C. Hain, MD. Please read our disclaimer Return to Index. Page last modified August 6, 2005 http://www.dizziness-and-balance.com/disorders/central/cerebellar/sca.htm

Spinocerebellar Degenerations Timothy C. Hain, MD Please read our Return to Index Page last modified: August 6, 2005 Figure 1: Sagittal MRI of person with an inherited cerebellar degeneration (of unknown origin). This MRI shows prominent atrophy (shrinkage) of the midline (called the vermis). The main goal of this page is to serve as a repository for recent information about inherited cerebellar degenerations. It is not comprehensive, but we hope that it might be of some use to individuals searching for information about these rare conditions on the web. We highly recommend also using the OMIM database , which can be accessed on the web. A large number of the genetic ataxias can be tested for using contemporary methodology. An example of a lab that does this is Athena Most of the information here concerns inherited conditions, as there is considerable new data derived from researchers using a nearly complete map of the human genome (your tax dollar is doing some good !), and improvements in the technology of molecular biology. It seems quite feasible that within the next decade, we may be able to determine the gene that is damaged in most inherited cerebellar degenerations. As these data become known, it may also be possible to target specific therapies, probably over the next 2 decades. In other words, stay tuned, but we aren't there yet. There are numerous non-genetic causes of cerebellar disease.

26. MeSH-D Terms Associated To MeSH-C Term Spinocerebellar Degenerations MeSHD terms associated to MeSH-C term spinocerebellar degenerations, G2D Home association of the corresponding term to spinocerebellar degenerations. http://www.bork.embl-heidelberg.de/g2d/c2d.pl?Spinocerebellar_Degenerations:unkn

27. Spinocerebellar Degenerations 9 mutations Dysautonomia Friedreich_ataxia Torsion Dystonia 1 Fanconi_anemia,.http//www.ncbi.nlm.nih.gov/genome/guide/HsChr14.shtml http://freesense.free.fr/Diseases/c/393.htm

http://www.ncbi.nlm.nih.gov/genome/guide/HsChr9.shtml 9 mutations Dysautonomia Torsion Dystonia http://www.ncbi.nlm.nih.gov/genome/guide/HsChr14.shtml MA Some diseases associated with chromosome 14 mutations Alzheimer_disease Machado-joseph_disease Spastic Paraplegia 3a See OMIM for additional examples Tropes Web V1.0

28. Machado-Joseph Disease - Azorean Disease - Information Page With HONselect spinocerebellar degenerations Spinocerebellar Ataxias Central Nervous SystemDiseases Spinal Cord Diseases spinocerebellar degenerations http://www.hon.ch/HONselect/RareDiseases/EN/C10.228.140.252.700.700.500.html

InitBulle("navy","#F8F8F8","#000066",1); HONcode sites All Web sites HONselect News ... Images HONselect Search English French German Spanish Portuguese the word the part of word in MeSH term in MeSH term and description Information on "Machado-Joseph Disease": Medical hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Hierarchy English French German Spanish Portuguese Machado-Joseph Disease Definition: A dominantly-inherited ataxia first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, dysarthria, postural instability, nystagmus, eyelid retraction, and facial fasciculations. Dystonia is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features muscle atrophy and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96) Synonym(s): Azorean Disease / Joseph Disease / Spinocerebellar Ataxia Type 3 / Striatonigral Degeneration, Autosomal Dominant /

29. Friedreich Ataxia - Friedreich Disease - Information Page With HONselect Broader term(s) spinocerebellar degenerations - Cerebellar Diseases - BrainDiseases - Central Nervous System Diseases - Nervous System Diseases http://www.hon.ch/HONselect/RareDiseases/EN/C10.228.140.252.700.150.html

InitBulle("navy","#F8F8F8","#000066",1); HONcode sites All Web sites HONselect News ... Images HONselect Search English French German Spanish Portuguese the word the part of word in MeSH term in MeSH term and description Information on "Friedreich Ataxia": Medical hierarchy and definition Research Articles Web resources Medical Images Medical News Medical Conferences Clinical Trials Hierarchy English French German Spanish Portuguese Friedreich Ataxia Definition: An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA , pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75) Synonym(s): Friedreich Disease / Hereditary Spinal Sclerosis / Sclerosis, Hereditary Spinal / Freidreich's Ataxia /

30. Prevalence And Pattern Of Spinocerebellar Degenerations In Northeastern Libya -- Prevalence and pattern of spinocerebellar degenerations in northeastern Libya.R Sridharan, K Radhakrishnan, PP Ashok and ME Mousa http://brain.oxfordjournals.org/cgi/content/abstract/108/4/831

JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article 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 ISI Web of Science Similar articles in PubMed ... Cited by other online articles Search for citing articles in: ISI Web of Science (18) Request Permissions PubMed PubMed Citation Articles by Sridharan, R. Articles by Mousa, M. E. ARTICLES Prevalence and pattern of spinocerebellar degenerations in northeastern Libya R Sridharan, K Radhakrishnan, PP Ashok and ME Mousa An intensive search over a two-year period for cases of cerebellar and spinocerebellar degenerations in Benghazi, Libya, made through polyclinics, university hospitals and a centre for the handicapped, revealed a total of 52 patients, among whom 30 were index cases; the remainder were detected on family study. Twenty-five patients lived in Benghazi, giving a crude prevalence of 4.8/100 000 population. There were 24 patients (10 families)

31. Spinocerebellar Ataxia Type 3 (Machado-Joseph Disease): Severe Destruction Of Th Finally, in all of the SCA3 patients, a severe degeneration was found in theventral and Yagishita S. Autosomal dominant spinocerebellar degenerations. http://brain.oxfordjournals.org/cgi/content/full/125/9/2115

JOURNAL HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Abstract FREE Full Text (PDF) 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 ISI Web of Science Similar articles in PubMed ... Download to citation manager Search for citing articles in: ISI Web of Science (7) Request Permissions PubMed PubMed Citation Articles by Braak, H. Brain, Vol. 125, No. 9, 2115-2124, September 2002 Guarantors of Brain R. A. I. de Vos C. Schultz E. R. Brunt H. Paulson and H. Braak Department of Clinical Neuroanatomy, Johann Wolfgang Goethe University, Frankfurt/Main, Germany, Laboratorium Pathologie Oost Nederland, Enschede, Department of Neurology, University Hospital, Groningen, The Netherlands, Department of Neurology, University of Iowa College of Medicine, Iowa City, IA, USA Received December 19, 2001. Revised March 12, 2002. Second revision March 28, 2002. Accepted March 29, 2002. Summary Top Summary Introduction Material and methods Results Discussion References The lateral reticular nucleus (LRT) of the medulla oblongata is a precerebellar nucleus involved in proprioception and somatomotor automatisms. We investigated this nucleus in five individuals

32. Entrez PubMed Prevalence and pattern of spinocerebellar degenerations in northeastern Libya.Sridharan R, Radhakrishnan K, Ashok PP, Mousa ME. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=4

33. Parkinsonism - The Doctors Lounge(TM) Olivopontocerebellar and spinocerebellar degenerations. Familial basal gangliacalcification. Familial Parkinsonism with peripheral neuropathy. http://www.thedoctorslounge.net/clinlounge/diseases/neurology/parkinsonism.htm

Advertisement Powered by Careerbuilder Search for: Home Humor Advertising Contact ... Links Other Specialties Cardiology Chest diseases Dermatology Endocrinology ... Urology Other Students Membership Research Tools Medical Tutorials Medical Software ... Specialized Centers Headlines: Parkinsonism A group of neurodegenerative diseases involving the substantia nigra (an area in the basal ganglia). Etiology and classification 1. 1ry idiopathic Parkinsonism Parkinson’s disease Juvenile Parkinsonism 2. Secondary (aquired, symptomatic) Parkinsonism: Infectious: post-encephalitic Drugs: neuroleptics, reserpine, tetrabenazine, lithium, flunarizine, cinnarizine. Toxins: ethanol, methanol, CO, Hg. Vascular: multi-infarct, hypotensive shock. Trauma: pugilistic encephalopathy. Other: parathyroid abnormalities, hypothyroidism, hepatocerebral degeneration, brain tumour, normal pressure hydrocephalus, syringomesenchephalia. 3. Heredodegenerative Parkinsonism

34. Classification Of Ataxias (Cerebellar and spinocerebellar degenerations). 12 March 2003. Primary. Inherited.Autosomal recessive. Autosomal dominant. Sex linked. Sporadic http://www.angelfire.com/retro/michaelpoon168/classification_of_ataxias.htm

setAdGroup('67.18.104.18'); var cm_role = "live" var cm_host = "angelfire.lycos.com" var cm_taxid = "/memberembedded" Search: Lycos Angelfire 40 Yr Old Virgin Share This Page Report Abuse Edit your Site ... Next Michael Poon's Shrine of Neurology HOME CONTENTS CONTACT US HOME ... CONTACT US Classification of Ataxias (Cerebellar and Spinocerebellar Degenerations) 12 March 2003 Primary Inherited Autosomal recessive Autosomal dominant Sex linked Sporadic Multisystem atrophy Idiopathic cerebellar degeneration Secondary Metabolic disorders Mitochondrial disease Toxic disorders Vascular disease Infective disorders Prion disease Paraneoplastic cerebellar degeneration Classification of inherited ataxias Autosomal recessive Friedreich’s ataxia Spinocerebellar ataxia with retained tendon reflexes Spinocerebellar ataxia with isolated Vitamin E deficiency Ataxia telengiectasia Other rare recessive ataxias Hypogonadotrophic hypogonadism (Matthews – Rundle syn) Learning disability, optic atrophy, spasticity (Behr’s syn)

35. Clinical Neuropharmacology - UserLogin Mitochondrial disfunction and spinocerebellar degenerations. Neurochem Pathol1986; 44363. Medline Link Context Link. 3. Blass JP, Kark P, Menon NK. http://www.clinicalneuropharm.com/pt/re/clnneupharm/fulltext.00002826-200003000-

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36. Korsakov's Journal Of Neurology And Psychiatry - Abstracts ¹2 2004 Key words spinocerebellar degenerations, motor learning, voluntary posturalcontrol. Zh Nevrol Psikhiatr Im SS Korsakova ¹2 2004,. Return to contents http://www.mediasphera.aha.ru/neurol/2004/2/e2-04ref.htm

Abstracts Korsakov's journal of neurology and psychiatry ¹2 2004 M.G. Krasnoperova Clinical peculiarities of childhood autism with endogenous manifesting psychosis and mental retardation Author's address: Key words: childhood autism, endogenous psychosis, mental retardation, catatonic symptoms. Zh Nevrol Psikhiatr Im SS Korsakova ¹2 2004, Return to contents T.M. Manevich, E.D. Sokolova, N.N. Yakhno, E.G. Rogovina The peculiarities of personality and mental state in children and adolescents with chronic tension-type headache Author's address: Neurological and psychiatric examination of 334 children, aged 7–15 years, 87 (23%) of them suffering from chronic tension-type headache (CTTH) is presented. All the patients had mental (mostly depressive and anxiety) disorders and 95.3% of them – psychosomatic ones. Clinical heterogeneity of CTTH was revealed: in the cases of daily continuous headache there were depressive disorders, in daily intermittent headache – depressive and anxiety-phobic disorders and in frequent headache pattern neurasthenia was predominantly detected. Personality features of CTTH patients were as follows: high anxiety, aggressiveness, asthenia, low “Ego” power, high frustration, sensitivity, hypochondriac, depressive and hysterical tendencies, affective rigidity. Patients realized mostly non-adaptive coping styles both for high and low pain intensiveness. Combination of psychopharmacology, psychotherapy and biofeedback for the CTTH treatment is recommended. Key words: headache, headache types in children, personality features, mental disorders, treatment.

37. Journal Of Computer Assisted Tomography - UserLogin Both MJD and DRPLA are autosomal dominant spinocerebellar degenerations in Yagishita S, Inoue M. Clinicopathology of spinocerebellar degeneration its http://www.jcat.org/pt/re/jcat/fulltext.00004728-200303000-00023.htm

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38. Searching Please Wait. You Ve Got 63 Hits! * The Results Are SCD, spinocerebellar degeneration, 21, 1, good bad. SCD, sister chromatiddifferentiation SCD, spinocerebellar degenerations, 3, 1, good bad http://bioinformatics.org/textknowledge/acronym.php?textfield=scd&sub=search

39. Re: Olivopontocerebellar Atrophy Cerebellar degenerations (also called spinocerebellar degenerations) can eitherbe sporadic or heredofamilial. Heredofamilial cerebellar degenerations can http://www.medhelp.org/forums/neuro/archive/15044.html

Questions in The Neurology Forum are being answered by doctors from The Cleveland Clinic , consistently ranked one of the best hospitals in America. Subject: Re: Olivopontocerebellar atrophy Forum: The Neurology and Neurosurgery Forum Topic Area: Ataxia Posted by CCF Neurology MD:NT on January 11, 1999 at 20:32:38: In Reply to: Olivopontocerebellar atrophy posted by Dave Felten on January 11, 1999 at 00:59:31: : Hello: : Can you tell me what diagnostic test currently are used to diagnose and confirm OPCA? : Thank you very much!! Dear Dave: Cerebellar degenerations (also called spinocerebellar degenerations) can either be sporadic or heredofamilial. Heredofamilial cerebellar degenerations can be either autosomal dominant (various types termed spinocerebellar ataxia = SCA 1 - 7, DRPLA, Machado-Joseph disease, etc) or autosomal recessive (Friedreich's ataxia, and some other rare types). The syndrome of progressive ataxia (imbalance of gait, incoordination of hands, slurred speech), in association with some other characteristic abnormalities (peripheral neuropathy, optic atrophy, retinitis pigmentosa, dementia, corticospinal dysfunction, oculomotor dysfunction, etc) is seen with heredofamilial forms. Ataxia is associated with autonomic dysfunction and/or parkinsonian features in sporadic cases. Either sporadic or autosomal dominant forms of cerebellar degeneration can be associated with atrophy of the cerebellum, pons and inferior olives, either on MRI, or at autopsy. Hence, OPCA (olivopontocerebellar atrophy) is used as a descriptive term for these disorders.

40. Sp : On Medical Dictionary Online spinocerebellar degenerations spinocerebellar degenerations, Familial spinocerebellar degenerations, Hereditary spinocerebellar degenerations http://www.online-medical-dictionary.org/sp.asp?q=~Sp

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