41. The First Scientific Workshop Of Hallervorden-Spatz Syndrome: National Institute The First Scientific Workshop of hallervordenspatz syndrome. http://www.ninds.nih.gov/news_and_events/proceedings/Hallervorden-Spatz.htm

Accessible version Science for the Brain The nation's leading supporter of biomedical research on disorders of the brain and nervous system News Press Releases News Articles Funding News Events Calendar of Events Proceedings Online Events Congressional Testimony Search NINDS... (help) Contact Us My Privacy NINDS is part of the National Institutes of Health You are here: Home News and Events Proceedings The First Scientific Workshop of Hallervorden-Spatz Syndrome Get Web page suited for printing Email this to a friend or colleague The First Scientific Workshop of Hallervorden-Spatz Syndrome Grant Number: NS40452-01 May 19 - 20, 2000 Principal Investigator: Susan J. Hayflick, MD Organization Conducting Meeting: Hallervorden-Spatz Syndrome Association and Oregon Health Sciences University Agenda May 19 May 20 Conclusions ... Participants Friday, May 19, 2000 7:00 - 8:00AM Registration 8:00 - 8:15AM Opening Remarks Section I - Chaired by Swaiman 8:15 - 9:45AM Session I - Clinical Delineation of HSS Data from International Patient Registry - Hayflick (30) 9:45 - 10:00AM Roundtable discussion 10:00 - 10:15AM Break 10:15 - 11:00AM Session II - Pathology of HSS 11:00 - 11:15AM Roundtable discussion 11:15 - 11:45PM Session III - Genetics of HSS Mapping the gene(s) for HSS - Hayflick (15) Candidate disease genes - Zhou (15) 11:45 - 12:00PM Roundtable discussion 12:00 - 1:00PM Lunch Section II - Chaired by Rouault 1:00 - 1:45PM Session IV - Systemic Iron Transport and Metabolism Normal iron transport and metabolism - Rouault (45)

42. Central Nervous System Diseases HSSA hallervordenspatz syndrome Association - (US). Genetic aspects ofhallervorden-spatz syndrome - OMIM/NLM (US). A nineteen year old boy . 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: Alzheimer Disease Arachnoiditis Brain Abscess Brain Abscess ... Uveomeningoencephalitic 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] How brain cells work [Cardoso et al.] EEG Course and Glossary [S Louis] About Normal EEG Variants [S Louis] - eMedicine 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 Hallervorden-Spatz Syndrome HSSA: Hallervorden-Spatz Syndrome Association - (US) Genetic aspects of Hallervorden-Spatz Syndrome - OMIM/NLM (US) A nineteen year old boy ... (Hallervorden-Spatz Syndrome)

43. OHSU News Release OHSU RESEARCHERS DISCOVER KEY GENE BEHIND hallervordenspatz syndrome. Finding mayalso assist in developing greater understanding of Parkinson s disease, http://www.ohsu.edu/news/archive/2001/072301hss.html

News and Information July 23, 2001 Contact: Jim Newman newmanj@ohsu.edu Hallervorden-Spatz Syndrome Association OHSU Department of Molecular and Medical Genetics Index of current releases ... News release archive OHSU RESEARCHERS DISCOVER KEY GENE BEHIND HALLERVORDEN-SPATZ SYNDROME Finding may also assist in developing greater understanding of Parkinson's disease, Alzheimer's disease and Huntington's disease PORTLAND, "Nine years ago, when we first began our investigations, we had very little understanding behind the cause of HSS," said Hayflick, who is senior author of the paper. "Today we have made a tremendous leap towards developing therapies to slow or stop the disease, or perhaps even cure it. Additionally, while HSS is considered quite rare, it is a part of a family of neurodegenerative diseases. We're hoping that this research will also provide new hope for patients with related disorders such as Parkinson's disease." HSS is a rare but well-known genetic disorder characterized by the accumulation of iron deposits in the brain. Specifically, these deposits surface in the basal ganglia, a region that controls body movement. This results in a gradual loss of muscle control. Patients with HSS can also experience night blindness and witness the gradual loss of the ability to speak and chew food. Degeneration caused by the disease is progressive and can shorten lifespan. HSS is an autosomal recessive disorder, meaning both parents must contribute a defective PANK2 gene for the disease to surface in their offspring. When two parents both carry PANK2 mutations, there is a 25 percent chance of occurrence for the disease during each pregnancy.

44. Health/Conditions And Diseases/Neurological Disorders/Brain Diseases/Basal Gangl A clinical synopsis of hallervordenspatz syndrome. urlwww3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?2342 mozilla.org http://www.thedoctorslounge.net/dir/Health/Conditions_and_Diseases/Neurological_

Advertisement Powered by Careerbuilder Search for: Home Humor Advertising Contact ... Links Specialties Cardiology Chest diseases Dermatology Endocrinology ... Urology Other Sections Membership Research Tools Medical Tutorials Medical Software ... Specialized Centers Headlines: The Medical Website Directory Popular Categories Popular Links Web Sites report abuse/spam url: report-abuse.dmoz.org/?cat=Health/Conditions_and_D... Hallervorden-Spatz Disease Information sheet compiled by NINDS. url: www.ninds.nih.gov/health_and_medical/disorders/hal... MCW HealthLink An article about Hallervorden-Spatz disease, with an explanation, the symptoms and prognosis. url: healthlink.mcw.edu/article/921723685.html NORD: Neurodegeneration with Brain Iron Accumulation Type 1 Offers synonyms, a general discussion and further resources for the disorder formerly known as Hallervorden-Spatz Syndrome. url: www.rarediseases.org/search/rdbdetail_abstract.htm... OMIM National Center for Biotechnology Information. A clinical synopsis of Hallervorden-Spatz syndrome. url: www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?2342...

45. Orthoguide.com Hallervorden-Spatz Syndrome Search AltaVista for hallervordenspatz syndrome Global Search - Add Url -Free Medline - Contact Us - Search. Enter Keywords to Search and Your Choice http://www.orthoguide.com/ortho/Hallervorden-Spatz_Syndrome.php3

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46. The IFOND Dictionary - H, International Foundation For Optic Nerve Disease hallervordenspatz syndrome Hereditary disease of children. Features includegeneralized dystonia with predominance of oromandibular involvement, http://www.ifond.org/dict_h.php3

I nternational F oundation for O ptic N erve D isease P. O. Box 777, Cornwall NY 12518, USA Providing and disseminating information on causes, prevention and treatment. Feedback HOME Page: A B C D ... Index The IFOND Dictionary - H Hallervorden-Spatz syndrome Hereditary disease of children. Features include generalized dystonia with predominance of oromandibular involvement, behavioral changes followed by dementia, and retinal degeneration in some cases optic atrophy. See OMIM 234200 Haplogroup An association of secondary mutations with the primary mutation in prevalence higher than normal. e.g.Johns and Berman in 1991 showed the frequency of mtDNA mutations at nucleotides 4216 and 13708 were associated more often with 11778 LHON patients than with normal controls. Johns DR, Berman J (1991) Alternative simultaneous complex I mitochondrial DNA mutations in Leber's hereditary optic neuropathy. [Medline] Biochem Biophys Res Commun Heteroplasmy When different cells in an organism may have different mutations of a gene. cf Homoplasmy. HIV (Human Immune deficiency Virus) Retro virus responsible for the Acquired Immune Deficiency Syndrome. Visual field defects consistent with optic nerve disease can occur in HIV infection.

47. Entrez PubMed BACKGROUND hallervordenspatz syndrome is an autosomal recessive disordercharacterized by dystonia http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

48. Thieme-connect - Abstract Here, we report a patient with classic hallervordenspatz syndrome and a hallervorden-spatz syndrome - pantothenate kinase 2 - eye-of-the-tiger sign http://www.thieme-connect.com/ejournals/abstract/neuropediatrics/doi/10.1055/s-2

Home Subject List Alphabetical List Help ... Deutsche Version Quick Search Advanced Search LogIn Username Password Register Now Free Trial Thieme-connect / Abstract Contact Us Short Communication Neuropediatrics 2005; 36: 221-222 DOI: 10.1055/s-2005-865714 The Eye-of-the-Tiger Sign is not a Reliable Disease Marker for Hallervorden-Spatz Syndrome F. A. M. Baumeister D. P. Auer P. Freisinger T. Meitinger Children's Hospital of the Technical University Munich, Munich, Germany Max-Planck-Institute of Psychiatry, NMR Study Group, Munich, Germany Institute of Human Genetics, GSF National Research Center for Environment and Health, Neuherberg, Germany Institute of Human Genetics, Technical University Munich, Munich, Germany Abstract Pantothenate kinase-associated neurodegeneration (PKAN), formerly Hallervorden-Spatz syndrome, is a rare autosomal recessive disorder characterized by extrapyramidal dysfunction as demonstrated by dystonia, rigidity, and choreoathetosis. Iron deposition in conjunction with destruction of the globus pallidus gives rise to the characteristic eye-of-the-tiger sign in MRI. It has been postulated that pantothenate kinase 2 mutations underlying all cases of classic Hallervorden-Spatz syndrome are always associated with the eye-of-the-tiger sign. Here, we report a patient with classic Hallervorden-Spatz syndrome and a homozygous pantothenate kinase 2 mutation in whom the initially present eye-of-the-tiger sign vanished during the course of the disease. Thus, the alleged one-to-one correlation between the eye-of-the-tiger sign and the presence of pantothenate kinase 2 mutation does not hold true over the course of the disease in PKAN.

49. Hallervorden-Spatz Syndrome hallervordenspatz syndrome Medical.WebEnds.com. hallervorden-spatz syndrome.Pigmentary Pallidal Degeneration; Hallervorden-Spatz Disease; http://medical.webends.com/kw/Hallervorden-Spatz Syndrome

Medical.WebEnds.com - Medical Terminology Dictionary A B C D ... Z WWW Medical.WebEnds.com Hallervorden-Spatz Syndrome Pigmentary Pallidal Degeneration; Hallervorden-Spatz Disease; Pigmentary Pallidal Atrophy; Atrophies, Pigmentary Pallidal; Atrophy, Pigmentary Pallidal; Degeneration, Pigmentary Pallidal; Degenerations, Pigmentary Pallidal A rare autosomal recessive degenerative disorder which usually presents in late childhood or adolescence. Clinical manifestations include progressive MUSCLE SPASTICITY ; hyperreflexia; MUSCLE RIGIDITY DYSTONIA DYSARTHRIA ; and intellectual deterioration which progresses to severe dementia over several years. Pathologic examination reveals neuronal atrophy in the globus pallidus and iron deposition in blood vessels and perivascular spaces. (From Adams et al., Principles of Neurology Google links Processing time was 0.028688907623291 seconds.

50. Photoclinic Adultonset hallervorden-spatz syndrome presenting as cortical dementia. First scientific workshop on hallervorden-spatz syndrome executive summary. http://www.ams.ac.ir/AIM/0472/020.htm

Photoclinic Morteza Khajavi MD*, Mansooreh Ardeshirzadeh MD *, Yousef Shafeghati MD**, Gholam-Ali Shahidi MD*** Authors Affiliations: *Department of Psychiatry, Razi Hospital, **University of Social Welfare and Rehabilitation Sciences, ***Department of Neurology, Hazrate Rasoul Hospital, Tehran, Iran. Corresponding authors and reprints:  Mansooreh Ardeshirzadeh, MD,   Department of Psychiatry, Razi Hospital, Tehran, Iran. E-mail: psych1965@yahoo.com. A 25-year-old male was admitted for involuntary contractions of hands (which was prominent in the left hand) and difficulties in articulation, swallowing, and facial grimacing (pouting).  His problems had started at the age of 16, with involuntary contraction of the left index finger. About 7 to 8 months later, involuntary contraction of the left 3 rd finger occurred, and then all fingers of the left hand showed the same feature and finally, the right hand was affected. In the last 10 years, dysarthria and dysphagia have developed leading to occupational incapacity of the patient as a farmer. He was the first case in the pedigree and the parents were not close relatives. His past personal or family history was not remarkable.

51. Alzheimer Disease And Associated Disorders - Abstract: Volume 14(2) April/May/Ju AdultOnset hallervorden-spatz syndrome Presenting As Cortical Dementia. hallervorden-spatz syndrome should be considered in the differential diagnosis http://www.alzheimerjournal.com/pt/re/adad/abstract.00002093-200004000-00010.htm

LWWOnline LOGIN eALERTS REGISTER ... Archive Adult-Onset Hallervorden-Spatz... ARTICLE LINKS: Fulltext PDF (5.41 M) Permissions Adult-Onset Hallervorden-Spatz Syndrome Presenting As Cortical Dementia. Cooper, Greg E; Rizzo, Matthew; Jones, Robert D Abstract: Subscribe to RSS feed utrdc-pt01 Release 4.0

52. Alzheimer Disease And Associated Disorders - UserLogin AdultOnset hallervorden-spatz syndrome Presenting As Cortical Dementia hallervorden-spatz syndrome should be considered in the differential diagnosis http://www.alzheimerjournal.com/pt/re/adad/fulltext.00002093-200004000-00010.htm

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53. Index H: Contact A Family - For Families With Disabled Children: Information On hallervordenspatz syndrome see Neuroacanthocytosis disorders Hand SchullerChristian syndrome see Histiocytosis Harlequin Ichthyosis see Ichthyosis http://www.cafamily.org.uk/Idx/h.html

printer friendly home how we can help medical information ... how you can help Please use the index below to access the condition you require information on. It may take longer to find what you are looking for this way compared with our "search this site" facility in the navigator on the left but we try to point you in the most appropriate direction using this index and therefore the results should be better. For speed, this index has been split into separate alphabetical files: numbers 0-9 A B C ... Z Contact a Family also has information on many other specific conditions and rare disorders. If you cannot find the information you require in The Contact a Family Directory Online , you may wish to use our Contact a Family Helpline service. HAE see C1 Esterase Inhibitor Deficiency HCH see Hypochondroplasia HCM see Cardiomyopathies HGG see Primary Immunodeficiencies HGPRT Deficiency see Lesch Nyhan syndrome HH see Hypothalmic Hamartoma HHH see Metabolic diseases HHT see Hereditary Haemorrhagic Telangiectasia HIV Infection and AIDS HLH see Histiocytosis HLHS see Hypoplastic Left Heart syndrome HME see Hemimegalencephaly HMG CoA Lyase Deficiency see Organic Acidaemias HMS see Hypermobility HOCM see Cardiomyopathies HOOD see Nail-Patella syndrome HPE see Holoprosencephaly HPRT see Lesch Nyhan syndrome HSAN see Congenital Insensitivity/Indifference to Pain HSAN Type I see Congenital Insensitivity/Indifference to Pain HSAN Type II see

54. NEJM -- Genetic, Clinical, And Radiographic Delineation Of Hallervorden-Spatz Sy hallervordenspatz syndrome, PANK2, and the tiger s eyes. Arch. Dis. Child.88 487-487 Full Text New Tests Diagnosis of hallervorden-spatz syndrome. http://content.nejm.org/cgi/content/abstract/348/1/33

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 348:33-40 January 2, 2003 Number 1 Next Susan J. Hayflick, M.D., Shawn K. Westaway, Ph.D., Barbara Levinson, M.A., Bing Zhou, Ph.D., Monique A. Johnson, Ph.D., Katherine H.L. Ching, B.S., and Jane Gitschier, Ph.D. Full Text PDF PDA Full Text PowerPoint Slide Set ... PubMed Citation ABSTRACT Background recessive disorder characterized by dystonia, parkinsonism, and iron accumulation in the brain. Many patients with this disease have mutations in the gene encoding pantothenate kinase neurodegeneration. In this study, we compared the clinical and syndrome with and without mutations in Methods One hundred twenty-three patients from 98 families with on the basis of clinical assessment as having classic disease (characterized by early onset with rapid progression) or atypical disease (later onset with slow progression). Their genomic DNA was sequenced for mutations. Results and one third of those with atypical disease had mutations.

55. NEJM -- Sign In hallervordenspatz syndrome and brain iron metabolism. Arch Neurol 1991;481285-1293 . Generalized freezing in hallervorden-spatz syndrome case report. http://content.nejm.org/cgi/content/full/348/1/33

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56. General News For June Commentary The human genome location of hallervordenspatz syndrome, akaneurodegeneration hallervorden-spatz syndrome (HSS) (OMIM 234200) is a rare, http://www.mad-cow.org/last_sci.html

Mad Cow Home or Best Links The news in brief Best online site for CJD abstracts? New neurodegeneration gene (HSS) maps by prion gene ... The psychiatric manifestation of CJD and mis-diagnosis Turn-around from June 5 Nature, page 560, 1997. It seems in the 1930's, Norman Pirie and Fred Bawden purified TMV (tobacco mosaic virus) to homogenity, even crystallizing it, and showed conclusively that every infectious particle contained nucleic acid (its RNA genome). This went against the grain of conventional wisdom of the day in the person of Wendell Stanley, subsequent Nobel laureate, who held that TMV and other viruses consisted entirely of protein! Help wanted: from June 5 Nature, ad in the back Collinge's group is looking for a research technician to help with on-going projects such as determination of human prion 3D structure, mechanism of conversion to disease isoform, site=directed mutagenesis, heterologous gene expression, and NMR, X-ray, fluorescence, steady-state and transient kinetics. (Pay is 16,000 pounds or $26,400.) Three online access points to Medline abstracts were tested on June 19, 1997.

57. Flanking Genes In The Prion Gene Neighborhood Homozygosity mapping of hallervordenspatz syndrome to chromosome 20p12.3-p13 . hallervorden-spatz syndrome is a rare, autosomal recessive http://www.mad-cow.org/00/ferritin_HSS.html

Mad Cow Home Best Links Search this site GenMap00: how the map was made ... Nearby diseases: AR CHED, AD CHED, and PPMD Making GenMap00 Last updated: 7 Feb 00 webmaster research Previous maps of the human genome, such as and its predecessors , have created a framework that locates a large number of microsatellites used to determine locations of human disease genes. While radiation hybrid mapping may in principle be capable of sufficient detailed mapping, as matters stand, the map in the chromosome 20p12 region lacks sufficient precision. Many markers are allocated to multiple (contradictory) positions, preventing a unique ordering as well as not providing meaningful recombination mapping distances. This causes monogenic disease research to stall out at a critical juncture associating the disease with a particular gene. If the disease can only be mapped to a 3 million base pair region, gene density is such that 30-50 different genes or more would have to be screened in the patient set and controls. A different approach was taken in the Whitehead Institute map . Here, chromosome 20 was tiled with a set of overlapping yeast artificial chromosomes (YACs); markers were positioned relative to hits on this panel. This resulted in an ordinal map: microsatellites were sequentially ordered with respect to their telomere to centromere position without any physical or centimorgan distances resulting. It quickly emerges that this map is superior in accuracy to GenMap99.

58. Hallervorden-Spatz Disease hallervordenspatz syndrome; late infantile neuroaxonal dystrophy; NeuroaxonalDystrophy, Late Infantile; Pigmentary Degeneration of Globus Pallidus, http://www.icongrouponline.com/health/Hallervorden-Spatz_Disease.html

ICON Health Publications Official Health Sourcebooks Search ICON Health Titles: The Official Patient's Sourcebook on HALLERVORDEN-SPATZ DISEASE (Hallervorden-Spatz Syndrome; late infantile neuroaxonal dystrophy; Neuroaxonal Dystrophy, Late Infantile; Pigmentary Degeneration of Globus Pallidus, Substantia Nigra, Red Nucleus; pigmentary pallidal degeneration syndrome; Progressive Pallid Degeneration Syndrome; progressive pallidal degeneration syndrome) Revised and Updated for the Internet Age P A P E R B A C K Paperback Book Paperback Book Order by phone: 800-843-2665 (within USA) 1-201-272-3651 (from outside USA) Paperback Book Shipped in 3 to 5 business days E B O O K Electronic File * E-Book version sent via e-mail in 2 business days Pages Price $28.95(USD) ISBN Published Synopsis A comprehensive manual for anyone interested in self-directed research on Hallervorden-Spatz Disease. Fully referenced with ample Internet listings and glossary. Related Conditions/Synonyms Hallervorden-Spatz Syndrome; late infantile neuroaxonal dystrophy; Neuroaxonal Dystrophy, Late Infantile; Pigmentary Degeneration of Globus Pallidus, Substantia Nigra, Red Nucleus; pigmentary pallidal degeneration syndrome; Progressive Pallid Degeneration Syndrome; progressive pallidal degeneration syndrome Description Table of Contents Introduction Overview Organization Scope Moving Forward PART I: THE ESSENTIALS Chapter 1. The Essentials on Hallervorden-Spatz Disease: Guidelines

59. JW Neurology -- Sign In New Tests Diagnosis of hallervordenspatz syndrome. In 2001, Hayflick and colleaguesidentified mutations in a gene localized to chromosome 20p13 that http://neurology.jwatch.org/cgi/content/full/2003/307/8

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60. Hallervorden-Spatz Syndrome, PANK2, And The Tiger's Eyes -- 88 (6): 487 -- Archi hallervordenspatz syndrome*, PANK2, and the tiger’s eyes In 2001 researchersin California and Oregon linked hallervorden-spatz syndrome with a defect http://adc.bmjjournals.com/cgi/content/extract/88/6/487

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Full Text Full Text (PDF) Submit a response ... Alert me if a correction is posted Services Email this link to a friend Similar articles in ADC Online Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Archives of Disease in Childhood Archivist Hallervorden-Spatz syndrome The first 150 words of the full text of this article appear below. Hallervorden and Spatz reported a rapidly progressive neurodegenerative disease of early onset in a German journal of neurology and psychiatry in 1922. The main clinical features are dystonia, dysarthria and rigidity, rapid progression, and early death. Until recently the diagnosis depended on clinical features and CT or MRI abnormalities in the globus pallidus. Pathologically, there is iron accumulation in the basal ganglia with destruction of the pallidum and substantia nigra. The classic disease is

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