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         Olivopontocerebellar Atrophy:     more detail
  1. The Official Patient's Sourcebook on Olivopontocerebellar Atrophy: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2003-04-08
  2. Olivopontocerebellar Atrophies: Advances in Neurology
  3. Olivopontocerebellar atrophy: An entry from Thomson Gale's <i>Gale Encyclopedia of Neurological Disorders</i> by Richard Robinson, 2005

41. Cognitive Affective Psychosis Syndrome In A Patient With Sporadic
of psychosis.1 A patient of sporadic olivopontocerebellar atrophy (OPCA) who the patient was diagnosed with sporadic olivopontocerebellar atrophy,
http://neuro.psychiatryonline.org/cgi/content/full/17/2/260

42. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Neurological_Disor
Opca Awareness A site devoted to olivopontocerebellar atrophy. NINDSolivopontocerebellar atrophy - Information sheet compiled by National Institute
http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Neurologica
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43. Lethal Olivopontocerebellar Atrophy - General Practice Notebook
lethal olivopontocerebellar atrophy. These are a heterogeneous group of ataxiascaused by. malformation and hypoplasias of the cerebellum
http://www.gpnotebook.co.uk/cache/-429195218.htm
lethal olivopontocerebellar atrophy These are a heterogeneous group of ataxias caused by:
  • malformation and hypoplasias of the cerebellum:
      usually autosomal dominant inheritance onset during childhood characterised by hypotonia and cerebellar ataxia IQ is normal good prognosis, some improve with age multiple system atrophy:
        usually autosomal recessive prognosis is poor with early onset disease characterised by:
          spasticity failure to thrive amyotrophy skeletal malformations
        babies die within a few weeks of birth

      Click here for more information...

44. Olivopontocerebellar Atrophy - General Practice Notebook
olivopontocerebellar atrophy. Olivopontine cerebellar degeneration may occursporadically or as an autosomal dominant trait.
http://www.gpnotebook.co.uk/cache/-113967058.htm
olivopontocerebellar atrophy Olivopontine cerebellar degeneration may occur sporadically or as an autosomal dominant trait. It is characterised by a general atrophy of the cerebellum spreading in time to involve the pons, medullary olives and other brain stem structures. It can occur at any age but onset in middle life is most common. Presentation is initially with ataxia, dysarthria, and tremor. Parkinsonian features may develop, accompanied by mild dementia, ophthalmoplegia, pyramidal tract signs and autonomic disturbance. Survival ranges from 23 - 30 years from onset.
Click here for more information...

45. Penn State Faculty Research Expertise Database (FRED)
DejerineThomas Syndrome, olivopontocerebellar atrophy, Idiopathic. Familialolivopontocerebellar atrophy, Inherited olivopontocerebellar atrophy
http://fred.hmc.psu.edu/ds/retrieve/fred/meshdescriptor/D009849

46. Olivopontocerebellar Atrophy
olivopontocerebellar atrophy. 4 Patient Support Information. Medline NLM Definitionfor Olivopontocerebellar Atrophies A group of inherited and sporadic
http://www.ion.ucl.ac.uk/library/patient/olivo.htm
Olivopontocerebellar Atrophy Patient Support Information
Medline NLM Definition
for Olivopontocerebellar Atrophies: 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. PubMed Medline search on Olivopontocerebellar Atrophies
Olivopontocerebellar Atrophies
factsheet: National Institute for Neurological Disorders and Stroke
Support Groups

See Multiple System Atrophy for support group.

47. Olivopontocerebellar Atrophy - Alegent Health Serving Eastern Nebraska And South
olivopontocerebellar atrophy courtesy of Alegent Health serving eastern Nebraskaand southwest Iowa - Council Bluffs, Iowa - Corning, Iowa - Missouri
http://www.alegent.com/13262.cfm
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Print This Page Email to a Friend Central nervous system
Olivopontocerebellar atrophy
Definition: Olivopontocerebellar atrophy is a neurodegenerative illness that causes certain brain areas (which may include the olivary nucleus, the pons, and the cerebellum) to shrink.
Alternative Names: OPCA; Olivopontocerebellar degeneration
Causes, incidence, and risk factors: The cause of sporadic olivopontocerebellar atrophy is not known, but the disease is progressive. This condition can be inherited or affect people without a known family history (sporadic form). Symptoms tend to start sooner in people with the familial form. This disease is slightly more common in men than in women.
Symptoms: Many symptoms are associated with olivopontocerebellar atrophy but the predominant feature is progressive ataxia (clumsiness) and difficulties with balance. There may be slurring of speech and difficulty walking. Other symptoms may include:
  • tremor abnormal movements abnormal eye movements sexual dysfunction bowel or bladder problems stiffness or rigidity spasticity neuropathy difficulty swallowing

Signs and tests: A thorough medical and neurological examination as well as a good history of symptoms and family history are necessary to make the diagnosis. There are no specific tests for this condition.

48. Header
Cerebellar and brainstem hypometabolism in olivopontocerebellar atrophy detectedwith positron emission tomography. Source. Annals of Neurology.
http://pet.radiology.uiowa.edu/webpage/Reference/Pub/files/cerebellar.htm
Authors Gilman S. Markel DS. Koeppe RA. Junck L. Kluin KJ. Gebarski SS. Hichwa RD. Institution Department of Neurology, University of Michigan, Ann Arbor 48109-0316. Title Cerebellar and brainstem hypometabolism in olivopontocerebellar atrophy detected with positron emission tomography. Source Annals of Neurology. 23(3):223-30, 1988 Mar. Abstract Back Topics Overview Patient Info Facilities Personnel ... Home

49. Early Onset Cerebellar Ataxia With Retained Tendon Reflexes (EOCA) And Olivopont
Eadie MJ olivopontocerebellar atrophy (DejerineThomas type). In Handbook ofClinical Neurology - System Disorders and Atrophies (Part I), Vol 21,
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=1999;volume=47;iss

50. Early Onset Cerebellar Ataxia With Retained Tendon Reflexes (EOCA) And Olivopont
(EOCA) and olivopontocerebellar atrophy (OPCA) a computed tomographic study . Computed tomographic (CT) studies in olivopontocerebellar atrophies
http://www.neurologyindia.com/article.asp?issn=0028-3886;year=1999;volume=47;iss

51. Olivopontocerebellar Atrophy
Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VI 1/OLIVOPONTOCEREB

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52. Multiple System Atrophy
Evolution of sporadic olivopontocerebellar atrophy into multiple system atrophy.Neurology 200055527532; Gilman S, and others. Consensus statement on the
http://www.dizziness-and-balance.com/disorders/central/movement/msa.html
Multiple System Atrophy (MSA) Timothy C. Hain, MD
Chicago IL, USA. Please read our Return to Index Search this site Page last modified: November 11, 2003
Multiple system atrophy is a rare neurological disorder characterized by a combination of parkinsonism, cerebellar and pyramidal signs, and autonomic dysfunction. The term "Multiple System Atrophy" is synonymous with striatonigral degeneration (SND) when Parkinsonism predominates, olivopontocerebellar atrophy (OPCA) when cerebellar signs predominate, and Shy-Drager syndrome when autonomic failure is dominant. The incidence (new case per 100,000 person years) for ages 50 to 99 years is 3.0 (Bower et al, 1997), or about half as frequent as it's close relative, progressive supranuclear palsy (PSP) . The mean age of onset is 54. The Parkinsonism of MSA is generally an akinetic rigid syndrome, similar to that of PSP . Rest tremor may occur but is not a predominant feature. Postural instability is common. Parkinsonism is generally the most common initial sign and eventually develops in about 90% of all patients. The cerebellar signs inlude finger-to-nose or heel-shin dysmetria, gait ataxia, intention tremor and nystagmus. Cerebellar signs are the first feature on only about 5% of patients. Cerebellar signs are observed in 50% of cases (Ben-Shlomo et al, 1997). Sporadic OPCA evolves into MSA in roughly 25% of cases within 5 years.

53. Olivopontocerebellar Atrophy - Talk Medical
Humanfriendly medical definition of olivopontocerebellar atrophy.
http://www.talkmedical.com/medical-dictionary/10220/Olivopontocerebellar-Atrophy

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Olivopontocerebellar Atrophy
Olivopontocerebellar atrophy: A group of genetic diseases in which there is progressive degeneration of the olivopontocerebellar pathway in the brain connecting the inferior olive, pons, and cerebellum. The cerebellum is the part of the brain that plays a role in maintaining balance and posture as well as coordinating voluntary movement. The pons is part of the brainstem and contains important neuronal pathways between the cerebrum, spinal cord, and cerebellum. The pons serves as a relay point for messages between these structures. The inferior olives are two round structures that contain nuclei that are involved with balance, coordination and motor activity. Olivopontocerebellar atrophy (OPCA) usually has its clinical onset in youth or the middle years. It may be confused with Parkinson's disease. The features characteristic of OPCA are progressive disequilibrium (increasing problems with balance problems), progressive cerebellar ataxia (increasing impairment of the capacity to coordinate voluntary movements), and progressive dysarthria (increasing difficulty in speaking clearly). There are at least five forms of OPCA. All are inherited in an autosomal manner. Both autosomal dominant and autosomal recessive types are known. There is considerable complexity and some confusion about the names for the different forms of OPCA, which is also known as spinocerebellar ataxia or SPA:

54. Dejerine-Thomas Atrophy (www.whonamedit.com)
olivopontocerebellar atrophy, pontoolivocerebellar atrophy, presenile ataxia,presenile cerebellar ataxic syndrome, olivopontocerebellar atrophy,
http://www.whonamedit.com/synd.cfm/1903.html

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Dejerine-Thomas atrophy Also known as: Dejerine-Thomas syndrome Synonyms: Atrophie olivo-ponto-cérébelleuse de Dejerine-Thomas (French), cortical cerebellar degeneration, delayed cerebellar ataxic syndrome, delayed cortical cerebellar atrophy, delayed cortical cerebellar degeneration, intracerebellar atrophy, olivopontocerebellar ataxia, olivopontocerebellar atrophy, ponto-olivocerebellar atrophy, presenile ataxia, presenile cerebellar ataxic syndrome, olivopontocerebellar atrophy, «sporadic form». Associated persons: Joseph Jules Dejerine André Antoine Henri Thomas Description: A chronic progressive ataxia characterized by progressive cerebellar atrophy with ataxic disorders of extremities and trunk, equilibrium and gait disorders, slow voluntary movements, speech disorders, nystagmus, and oscillatory tremor of the head and trunk. Onset in adult or middle life.

55. Dorlands Medical Dictionary
olivopontocerebellar atrophy, any of a group of progressive hereditary disordersinvolving degeneration of the cerebellar cortex, middle peduncles,
http://www.merckmedicus.com/pp/us/hcp/thcp_dorlands_content.jsp?pg=/ppdocs/us/co

56. Olivopontocerebellar Atrophy 1 Synonyms 2 Symptoms 3 Causes 4
olivopontocerebellar atrophy I (Menzel type OPCA) usually begins in the third orfourth olivopontocerebellar atrophy III (OPCA III; OPCA with retinal
http://pages.infinit.net/macmike/internaf/archives/OPCA.txt

57. Hereditary Ataxia Hereditary Ataxia Is A Group Of Rare Genetic
olivopontocerebellar atrophy I (Menzel type OPCA) is inherited as a dominanttrait and olivopontocerebellar atrophy III (OPCA III; OPCA with retinal
http://pages.infinit.net/macmike/internaf/archives/H_Atax_2.txt

58. BCM-Neurology-Parkinson's Disease Center And Movement Disorders-Multiple System
C. What is olivopontocerebellar atrophy (OPCA)? The term olivopontocerebellar Berciano J. olivopontocerebellar atrophy. In Jankovic J, Tolosa E, eds.
http://www.bcm.edu/neurology/struct/parkinson/atrophy.html
Print This Page Close Window Multiple System Atrophy Joseph Jankovic, M.D. What is Multiple System Atrophy (MSA)? A. What is Shy-Drager Syndrome (SDS)? The above symptoms occur because SDS damages the autonomic nervous system, the part of the nervous system which controls autonomous functions that are essential to survival. These functions include regulation of heart rate and breathing, in addition to control of intestinal, urinary, and sexual functions. The autonomic nervous system also controls skin and body temperature, and the body's response to stress. Because the autonomic nervous system also controls constriction and dilation of the pupils of the eye, as well as focusing the eye, SDS patients sometimes complain of blurry vision. Diagnosis is sometimes aided by testing autonomic nervous system function, for example, by measuring blood pressure and heart rate with the patient laying down compared with standing up. A polysomnogram, or sleep study, can document sleep apnea (gaps in breathing during sleep). Brain imaging with CT and MRI are usually helpful in differentiating typical Parkinson's disease from atypical parkinsonism.

59. Baylor Neurology Case Of The Month
olivopontocerebellar atrophy (parkinsonism with cerebellar dysfunction), olivopontocerebellar atrophy (OPCA), and striatonigral degeneration (SND).
http://www.bcm.edu/neurology/challeng/pat22/summary.html
Patient #22
Summary and Discussion
Ericka Simpson, M.D.
Resident, Department of Neurology Diagnosis: Multi-System Atrophy The Parkinsonism Plus syndromes include an array of neurodegenerative conditions characterized by parkinsonism plus other evidence of neurological dysfunction as seen in our patient. In Diffuse Lewy Body disease, parkinsonism is accompanied by cortical dementia with varying levels of attention, early hallucinations, and psychosis. Autonomic dysfunction is common, and pyramidal signs may be seen, but cerebellar dysfunction is not found. This patient's mild subcortical dementia is also not consistent with this diagnosis. In cortical-basal ganglionic degeneration, parkinsonism is accompanied by ideomotor apraxias, the alien limb phenomenon, cortical reflex myoclonus, cortical sensory loss, marked asymmetry of involvement, and focal rigidity and dystonia with contractures. None of these features was seen in this patient. In progressive supranuclear palsy, parkinsonism is accompanied by a prominent supranuclear gaze disturbance not seen in this patient. Multisystem atrophy (MSA) is a progressive, sporadic disorder characterized by parkinsonism in association with varying degrees of cerebellar, pyramidal, intellectual, and autonomic dysfunction. Classically, this includes three separate entities - Striatonigral degeneration (parkinsonism poorly responsive to levodopa and frequently associated with cervical dystonia), Olivopontocerebellar atrophy (parkinsonism with cerebellar dysfunction), and Shy-Drager syndrome (parkinsonism with autonomic dysfunction). Because of the clinical overlap and common pathologic finding of an intracytoplasmic oligodendroglial inclusion body, these entities are now lumped together. This patient best fit the diagnosis of multisystem atrophy with evidence of parkinsonism, cerebellar dysfunction, autonomic dysfunction, mild dementia, and long-tract signs.

60. Muscular Dystrophy Foundation Of South Africa
Fact Sheets. olivopontocerebellar atrophy Fact Sheet 21 2. What isolivopontocerebellar atrophy? 3. What causes olivopontocerebellar atrophy?
http://www.mdsa.org.za/fact_FS21.htm
Please upgrade your browser to view the navigation menu. Fact Sheets Olivopontocerebellar Atrophy
Fact Sheet #21 1. Is Olivopontocerebellar Atrophy also known by other names? Yes, this disorder is also known as Marie Ataxia. 2. What is Olivopontocerebellar Atrophy? OPCA is a disorder characterised by progressive degeneration of the nervous system. This loss of function is the result of deterioration of the areas of the brain that control co-ordination. These areas are the olive, the pons, and the cerebellum - hence the name of Olivopontocerebellar Atrophy. Cells in these areas of the brain begin to deteriorate and die. 3. What causes Olivopontocerebellar Atrophy? OPCA is the result of deterioration of the areas of the brain that control co-ordination. 4. What are the symptoms? One of the more dangerous characteristics of OPCA, as with other forms of ataxia, is a loss of the ability to clear the throat. As the disorder progresses, coughing becomes weak and ineffective, causing choking and breathing problems. Respiratory illness, such as colds and flu, are particularly dangerous. These can easily develop into pneumonia because of the inability to cough up fluids from the lungs. Mental ability is not affected by OPCA until quite late in the course of the disorder. Choking and pneumonia holds the biggest risk for affected individuals. 5. Which muscles are affected?

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