21. Central Nervous System Diseases Zellweger Syndrome. About Zellweger Syndrome OMIM/NLM (US). Brief note about ZellwegerSyndrome A Hutchins - (UK). cerebellar diseases. Dandy-Walker Syndrome. http://www.mic.ki.se/Diseases/C10.228.html

search search staff sitemap ABOUT KAROLINSKA INSTITUTET ... print this page Diseases and Disorders Links pertaining to Central Nervous System Diseases Alert! Patients and laypersons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider. Start Page Contents: Adrenoleukodystrophy Alzheimer Disease Arachnoiditis Brain Abscess ... Zellweger Syndrome Central Nervous System Diseases Nerve Cells [Lodish et al.] - Molecular Cell Biol., Chap 21, via NLM (US) Pathol. Images of the Central Nervous System - Univ of Utah (US) The Human Brain [JD MacArthur] The Global Brainstem '97 , the Cerebellum '97 , the Thalamus '97 , the Spinal Cord '97 - Univ. of Wisconsin (US) Mental Disorders Links Brain Diseases Brain Functions and Map - Centre for Neuro Skills The Whole Brain Atlas - Atlas Project/Harvard Medical School et al. (US) Dissections of the Real Brain [Williams et al] - Univ of Iowa (US) Anatomy of the Brain - AANS Leukodystrophy (not on MeSH) Leukodystrophy - NINDS The United Leukodystophy Foundation , including

22. Psychiatric News Arehart-Treichel 37 (16) 17 Joan ArehartTreichel Patients with cerebellar diseases may experience not justdeterioration in physical movements, but also psychiatric disorders. http://pn.psychiatryonline.org/cgi/content/full/37/16/17

23. CEREBELM A. Signs and symptoms 1. Synergia cerebellar diseases may lead to asynergia (ie,limb ataxia). 6. cerebellar diseases often lead to hypotonia of the muscles. http://www2.umdnj.edu/~paneuweb/cerebelm.htm

Maintained by David P. Crockett . E-mail suggestions to crockett@umdnj.edu (Revised 2/12/97) David P. Crockett, M.A., Ph.D. Department of Neuroscience and Cell Biology UMDNJ-Robert Wood Johnson Medical School Room R-306, Telephone: 235-3404 Notes on the cerebellum PA Neuroscience Home Page I. Introduction: 1. Cerebellum = 'little brain'; by weight is only 10% of the total brain and yet it contains more than half of all the neurons in the brain. 2. Highly regular, almost crystalline organization. 3. Cerebellar functions: The cerebellum acts as a 'comparator'; i.e., a device that compensates for errors by comparing intention with performance. The cerebellum is said to compare the central commands for movement with the actual movements themselves. Three features are important to keep in mind: a. The cerebellum receives information from brain sites that are involved in the planning of movements and commands for movements: corollary discharge b. The cerebellum receives information about motor performance; i.e., the results of the movement command, through sensory feedback arising in the periphery during the course of movement: reafference. The cerebellum is then in a position to compare the corollary discharge with reafference (the sensory feedback associated with the movement); if there is a mismatch, corrections may be made.

24. MeSH-D Terms Associated To MeSH-C Term Cerebellar Diseases MeSHD terms associated to MeSH-C term cerebellar diseases, G2D Home.The number indicates the strength of the association of the http://www.bork.embl-heidelberg.de/g2d/c2d.pl?Cerebellar_Diseases:unknown

25. DVM - Comprehensive Case History Important When Evaluating Congenital cerebellar diseases included hypoplasia, malformation, hypomyelinogenesis,dysmyelinogenesis, abiotrophy and lysosomal storage disease. http://www.dvmnewsmagazine.com/dvm/article/articleDetail.jsp?id=62242

26. NSF: KDI: How The Brain Orchestrates Reaching And Walking does and better understand some of these control signals, it becomes easier to havesome insight into the processes of cerebellar diseases—why, for example http://www.cise.nsf.gov/kdi/people/brain.html

Search KDI Site KDI Home Contact Us About KDI Behind the Scenes ... Visit Dr. Ebner's Web site How the Brain Orchestrates Reaching and Walking The project was called Artificial Implementation of Cerebro-Cerebellar Control of Reaching and Walking . The principal investigator was Dr. Steve Massaquoi of the Massachusetts Institute of Technology (MIT). Dr. Massaquoi and other engineers from MIT collaborated with Dr. Timothy Ebner, a neuroscientist who is professor and head of the Department of Neuroscience at the University of Minnesota. The team studied several existing models of cerebellar function. According to Dr. Ebner, "Many experts believe the cerebellum does its business by carrying what we call an internal model, a kind of map or schematic diagram of the body and how that body works, or should work." By this view, the cerebellum holds the internal model, using it as a template for reference. "But," according to Dr. Ebner, "Steve Massaquoi thinks that view is incorrect and that movement control is achieved through a more traditional engineering model applied to physiology, where the brain processes control signals using normal feedback loops." The question, then, is whether the cerebellum is a kind of "stationmaster" for neural messages moving to and from the muscles of the body, or if movement is coordinated and controlled on the basis of an internal schema of the body's circuitry. Dr. Ebner collected basic data by recording the cerebellar activity of monkeys who had been trained to do certain tasks. As the monkeys reached, grasped, and performed other movements, Dr. Ebner and his colleagues recorded single-cell activity in the monkeys' cerebelli using microelectrodes. "Then we took those signals and processed them and tried to understand how information is represented and whether it matches either the internal map thesis or the signal-feedback theory," says Dr. Ebner.

27. HDLighthouse.org about three times the incident of mood disorders. OBJECTIVE This study estimatedthe psychiatric morbidity of patients with degenerative cerebellar diseases. http://hdlighthouse.org/abouthd/statistics/updates/0033psyco.shtml

New to the Huntington's Disease Lighthouse: Welcome, start here Beginners Search about hd ... HD Statistics HD Lighthouse Editors Comment: The following small study suggests that HD patients have more mental problems than those with other brain conditions. The study also suggests that a surprisingly large percentage of neurologically healthy folks, including the researchers, have the same problems. Jerry Posted to HDLighthouse: HDL Update: A Comparison to Huntington's Disease Psychopathology HD Researchers Christopher Ross and group compaired HD patients to others. Here is what they found. Compaired to neurologicaly healthy subjects, HD patients have: less than twice the incidence of non-cognitive disorders. about three times the incident of mood disorders. OBJECTIVE: This study estimated the psychiatric morbidity of patients with degenerative cerebellar diseases. METHOD: The study included a series of 31 patients with degenerative cerebellar diseases, compared with 21 patients with Huntington's disease and 29 neurologically healthy comparison subjects. Comprehensive psychiatric evaluations, including the Structured Clinical Interview for DSM-IV and psychopathology rating scales, were administered. noncognitive psychiatric disorders mood disorders personality change Source: Psychopathology in Patients With Degenerative Cerebellar Diseases: A Comparison to Huntington's Disease. Am J Psychiatry. 2002 Aug;159(8):1306-14. Leroi I, et al.

28. Www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=Cerebellar+Diseases BrainTalk Communities What is SPINAL CEREBELLUM DISEASEOnline patient support groups for healthcare and neurology. http://www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=Cerebellar Diseases

29. Independent Research In a neuroimaging study of patients with cerebellar diseases, researchers foundimpariments of verbal fluency, abstract reasoning and working memory; spatial http://www.dyslexiaonline.com/evidence/independent.html

FORUMS SEARCH SITE MAP CONTACT US SEARCH Home Evidence Developmental trajectories of brain volume abnormalities in children and adolescents with attention-deficit/hyperactivity disorder. JAMA 1740-8. Castellanos FX, et al Various anatomic brain abnormalities have been reported for attention-deficit/hyperactivity disorder (ADHD), with varying methods, small samples, cross-sectional designs, and without accounting for stimulant drug exposure. The objective of this study was to compare regional brain volumes at initial scan and their change over time in medicated and previously unmedicated male and female patients with ADHD and healthy controls. The researchers concluded that Developmental trajectories for all structures, except caudate, remain roughly parallel for patients and controls during childhood and adolescence, suggesting that genetic and/or early environmental influences on brain development in ADHD are fixed, nonprogressive, and unrelated to stimulant treatment. [abstract] Deficits of motion transparency perception in adult developmental dyslexics with normal unidirectional motion sensitivity.

30. Neurogate.com Neurosurgery Search Engine And Neurosurgery Custom Medline Searchi Central Nervous System Neoplasms Central Pontine Myelinolysis - CerebellarAtaxia - Cerebellar Cortex - cerebellar diseases - Cerebellar Dyssynergia http://www.neurogate.com/neuro/index2.html

Global Search Add Url Free Medline Contact Us ... Conferences Bookmark this page for your Future Medline/Internet Searches Try the Medline Search Designed for Neurosurgery With FAST results Neurosurgery Internet Search Medline Search Designed for Neurosurgery Enter Keyword to search help Or And Match entire phrase Enter Keyword to search help Categories Premier Neurosurgery Sites General Neurosurgery (MESH 1998 © NLM) Abducens Nerve Abetalipoproteinemia Abnormal Reflex ... Metabolic Brain Diseases General Neurosurgery (MESH 1998 © NLM) Metachromatic Leukodystrophy Microglia Middle Hypothalamus ... Neurogate.com

31. FA FAQ Eds. Vinken, Bruyn, and Klawans (Elsevier and Amsterdam, New York, 1992)3. The Handbook of cerebellar diseases, Ed. Richard Lechtenberg http://www.fortnet.org/fapg/faq.htm

Friedreich's Ataxia - Frequently Asked Questions What is Friedreich's Ataxia? What are the major symptoms of FA? What other symptoms might occur? How is FA passed on in the family? ... What should somebody with FA do? What is Friedreich's ataxia? Friedreich's ataxia (FA) is a very rare neurological genetic disorder, that affects speech, balance and coordination. In some respects it is similar to multiple sclerosis. It is an inherited genetic disease in which a person is born with. Most people who have Friedreich's Ataxia, eventually need support to walk, like a scooter, walker or wheelchair. Although, not deemed terminal, it is progressive and as a result, complications can occur to the respiratory systems, (heart and lungs etc.) Friedreich's ataxia was the first form of hereditary ataxia to be distinguished from other forms of ataxia. It is named after the German doctor, Nikolaus Friedreich, who first described it in 1863. Presently there is no known cure, but please don't fret or become discouraged, because with the advancement of all the recent genetic discoveries and research, there is great deal of hope indeed! What are the major symptoms of Friedreich's ataxia?

32. Title Details - Cambridge University Press correlations are thoroughly covered, and there is an extensive and authoritativereview of recent advances in the genetics of cerebellar diseases. http://titles.cambridge.org/catalogue.asp?isbn=0521771560

33. Right Arm Tremor There are cerebellar diseases in which the MRI shows nothing abnormal, but usuallythe whole cerebellum is affected (some biochemical problem, for example) and http://www.medhelp.org/perl6/neuro/archive/14506.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: Right Arm Tremor Topic Area: Movement Forum: The Neurology and Neurosurgery Forum Question Posted By: sally on Sunday, December 20, 1998 Dear Doctor, Let me type our findings on the MRI. This is just the impression. There is probably dilated perivesicular space at the inferior aspect of the basal ganglia on the right side. There is slight prominence of the cisterna magna. No mass lesion is seen. No abnormal enhanced lesion is identified with gadolinium injection. There is minimal mucasol thickening demonstraed in the max. sinuses. His right arm tremor is still with him sometimes it seems worse than others, but he is on vacation from school so he hasnt been writing as much. Although he did say that when he was trying to drink the other night he did have some difficulty. We will see his Doctor on Tuesday to get the blood results of a thyroid and test for Wilsons Disease. but I still would appreciate any suggestions you might have. I truely appreciate how quickly you returned my last posted note thank you again and have a Happy New Year!!! Sally The MRI interpretation suggests that there is no structural damage to the cerebellum or the connections with other parts of the brain. If the problem is in the cerebellum, AND if it is localized to one side or one limb, then you would expect some abnormality. There are cerebellar diseases in which the MRI shows nothing abnormal, but usually the whole cerebellum is affected (some biochemical problem, for example) and not just the part that controls one limb. Therefore, I suspect the cerebellum is not involved (based on limited data, of course, in this forum).

34. FAMILIAR CEREBELLUM DEGENERATION For a small fraction of cerebellar diseases, there are genetic tests (for example,Freidreich s ataxia, SCA1, SCA-2, and SCA-3). We usually don t order a http://www.medhelp.org/forums/neuro/archive/7551.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: FAMILIAR CEREBELLUM DEGENERATION Topic Area: Neurology - General Forum: The Neurology and Neurosurgery Forum Question Posted By: Roy Lieuw Kie Song on Thursday, August 20, 1998 Does any one know where one could go to get help for the above subject? My father has had this for 10 years now, and it is getting worst. The main problem is that he is having problems walking and keeping his balance. Docyors her in Canada have diagnosed him withthe familiar cerebellum degeneration disease and claim that not enough is know about the disease to treat it. As I know that there are ohters who have this, I am wondering if any one is aware of any treatment for this. There are a variety of cerebellar degenerative diseases which run in families. The common symptom produced by damage to the cerebellum (of whatever cause) is ataxia, that is, incoordination. Ataxia can affect gait, causing people to walk like they're drunk (alcohol affects the cerebellum, so that's actually why you walk like that if you drink too much). Ataxia can also affect limb function, so that use of the hands is incoordinated. Familial cerebellar system degenerative diseases can be inherited in dominant or recessive patterns, depending on the specific disease. Dominant is when you have it, a parent has it, and a grandparent has it, as well as assorted brothers, sisters, aunts, uncles, and cousins. Recessive is when two siblings have it but no one else in the family has it.

35. UMHS Department Page Clinical Activities Ataxia clinic. Research Interests cerebellar diseasesBasal ganglia diseases Dementia Positron emission tomography. http://www.med.umich.edu/neuro/resfaculty.htm

Neurology Home Page Neurology Residency Program Educational Philosophy Clinical Training in Neurology Educational Activities Neurology Outpatient Clinics ... Ann Arbor Neurology Faculty This list includes all faculty members who are regularly involved in resident education. The list does not include some faculty members whose primary activity is research, medical student teaching, or clinical practice. SID GILMAN, M.D. William J. Herdman Professor of Neurology Chair, Department of Neurology Director, Michigan Alzheimer's Disease Research Center Clinical Activities: Ataxia clinic Research Interests: Cerebellar diseases Basal ganglia diseases Dementia Positron emission tomography PROFESSORS JAMES W. ALBERS, M.D. Ph.D. Clinical Activities: Director, Neuromuscular clinic

36. ClinicalTrials.gov - Information On Clinical Trials And Human Research Studies: 56. Cerebellar Ataxia (2 studies). 57. cerebellar diseases (5 studies). 58. CerebralAmyloid Angiopathy (1 study). 59. Cerebral Arterial Diseases (8 studies). 60. http://www.clinicaltrials.gov/ct/screen/BrowseAny?path=/browse/by-condition/hier

37. ClinicalTrials.gov - Information On Clinical Trials And Human Research Studies: studies). 49. Central Nervous System Viral Diseases (9 recruiting studies).50. cerebellar diseases (2 recruiting studies). 51. Cerebral http://www.clinicaltrials.gov/ct/screen/BrowseAny?path=/browse/by-condition/hier

38. The Pathological Protein their crossing. This is in contrast to the increased divergence tendencyusually observed in cerebellar diseases. Another important http://www.thepathologicalprotein.com/_wsn/page4.html

The Pathological Protein Mad Cow, Chronic Wasting, and Other Prion Diseases Case Report of "Miss H" Josef Gerstmann described members of the "H" family suffering from an inherited disease now called Gerstmann-Sträussler-Scheinker syndrome, or GSS (Scheinker’s name wasn’t attached to the disease at first). The disease proved to be a transmissible spongiform encephalopathy in experiments reported in 1981 by D. Carleton Gajdusek’s team at the National Institutes of Health. In 1989, Stanley Prusiner and Karen Hsaio showed that GSS resulted from a mutation in the prion protein gene. The change in the genetic sequence is a point mutation: a single nucleotide was changed, resulting in an altered amino acid in the prion protein. Several different point mutations are now known to produce GSS. Gerstmann described an unusual reflex action of one of his GSS patients. Turning her head one way, either by herself or by the force of a physician’s hands, would cause her arms to swing to the opposite side, and vice versa. Below is a translation of that 1928 case report. (The original appeared as: "Über ein noch nicht beschriebenes Reflexphänomen bei einer Erkrankung des zerebellaren Systems," in Wiener Medizinische Wochenschrift, Volume 78, pages 906–908, 1928.) On a Previously Undescribed Reflex Phenomenon in a Disease of the Cerebellar System.

39. Department Of Neuroscience At The Johns Hopkins University School Of Medicine: F Psychopathology in Patients With Degenerative cerebellar diseases A Comparisonto Huntington s Disease. Am J Psychiatry. 2002 Aug;159(8)130614. http://neuroscience.jhu.edu/peopledetail.asp?ID=62

40. L4 - ACTING CEREBELLUM. THE PURKINJE CELL CIRCUIT. FUNCTIONAL ANATOMY. BEYOND MOVEMENTCONTROL. Interlude cerebellar diseases. COGNITION, LEARNING AND MEMORY. http://www.dubinweb.com/brain/4.html

ACTING Missing the menu? Click here This figure summarizes the major components of the motor system. The actual interconnecting pathways are so profuse that a diagram showing them all would be confusingly covered with lines. The main point here is that the system consists of a hierarchy of interacting levels. The next sections discuss their main functions and relationships. SPINAL CORD BRAINSTEM Interlude: Ondine's Curse BASAL GANGLIA PARKINSONS DISEASE AND HUNTINGTON'S DISEASE OTHER DISEASES RELATED TO THE BASAL GANGLIA CORTEX AND THALAMUS THE SELF IN SPACE Interlude: Spatial Hemineglect MOTOR PATTERNS WHEN DOES CONSCIOUSNESS SEE? WATCHING, IMAGINING AND DOING CEREBELLUM THE PURKINJE CELL CIRCUIT FUNCTIONAL ANATOMY BEYOND MOVEMENT CONTROL Interlude: Cerebellar Diseases COGNITION, LEARNING AND MEMORY

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