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         Hallervorden-spatz Syndrome:     more detail

61. BAHA B
CASTELNAU P., ZILBOVICIUS M., RIBEIRO MJ, HERTZPANNIER L., OGIER H., EVRARD P. Striatal and pontocerebellar hypoperfusion in hallervorden-spatz syndrome.
http://www.necker.fr/irnem/Unites 2001/01radioped.htm
BAHA B., MEYER P.G., BRUNELLE F., ORLIAGUET G., MICHEL J.L., CARLI P.
Percutaneous embolization of a hepatic artery pseudoaneurysm in a multiple trauma child.
Ann. Fr. Anesth. Réanim., ; (Facteur d'Impact 1999 :
(Services cités : Anesthésie Réanimation, Radiologie Pédiatrique
BENACHI A., SONIGO P., JOUANNIC J.M., SIMON I., REVILLON Y., BRUNELLE F., DUMEZ Y.
Determination of the anatomical location of an antenatal intestinal occlusion by magnetic resonance imaging.
Ultrasound Obstet. Gynecol., ; (Facteur d'Impact 1999 :
(Services cités : Chirurgie Pédiatrique, Maternité, Radiologie Pédiatrique
Intestinal occlusion occurs in approximately 1 in 3000 births. Its diagnosis can be made in utero with ultrasound however, determination of its precise location is difficult to achieve. We report herein the feasibility of diagnosing and locating a fetal small-intestine occlusion with the use of magnetic resonance imaging. [References: 5] BRUNELLE F.
Fetal imaging in a new era.
Ultrasound Obstet. Gynecol., ; (Facteur d'Impact 1999 :

62. LONGSTANDING
deep brain stimulation (DBS) has appeared as an a Pallidal deep brain stimulation for longstanding severe generalized dystonia in hallervordenspatz syndrome.
http://mind-brain.com/abstracts.php?qa=longstanding

63. ²Ä¤Q¥|¨÷²Ä¤T´ÁVolume 14Number 3
Magnetic Resonance Imaging of hallervordenspatz syndromeA Case Report I-Hao Su, Andy Shau-Bin Chou1, Chau-Chin Lee1, Pao-Sheng Yen1 Department of Diagnostic
http://www.tzuchi.com.tw/tcmj/92-2/8.htm
¤¤µØ¥Á°ê92¦~4¤ë ²Ä¤Q¤­¨÷ ²Ä¤G´Á April 2003 Volume 15 Number 2
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Magnetic Resonance Imaging of Hallervorden-Spatz SyndromeA Case Report
I-Hao Su, Andy Shau-Bin Chou1, Chau-Chin Lee1, Pao-Sheng Yen1
Department of Diagnostic Radiology, Chang Gung Memorial Hospital, Taipei, Taiwan; Department of Radiology1, Buddhist Tzu Chi General Hospital, Hualien, Taiwan ABSTRACT

A 48-year-old man had suffered from progressive involuntary movement of bilateral arms since the age of 24 years. Clinical examination revealed acanthocytosis and optic nerve atrophy. Cranial magnetic resonance imaging (MRI) demonstrated abnormal hypointensity in the globus pallidus with central zones of hyperintensity on T2-weighted and Proton-weighted images, producing the "eye of the tiger" sign. Based on the clinical features and MRI findings, Hallervorden-Spatz syndrome was diagnosed. (Tzu Chi Med J 2003; 15:131-134)
Key words: Hallervorden-Spatz syndrome, magnetic resonance image, brain degeneration

64. Hallervorden-Spatz, Maladie : Sites Et Documents Francophones
Translate this page associée à la . Arborescence(s) du thesaurus MeSH contenant le mot-clé Hallervorden-Spatz, maladie hallervorden-spatz syndrome
http://www.chu-rouen.fr/ssf/pathol/hallervordenspatzmaladie.html
Hallervorden-Spatz, maladie Menu général CISMeF Définition
Synonyme(s) CISMeF
Dystrophie neuroaxonale tardive ; Pantothénate kinase, neurodégénérescence associée à la .
Arborescence(s) Hallervorden-Spatz, maladie Hallervorden-Spatz syndrome
maladies et malformations congénitales, héréditaires et néonatales
système nerveux, maladies Position du mot-clé dans l' (les) arborescence(s) : Vous pouvez consulter Ou consulter ci-dessous une sélection des principales ressources :
guide ressources

65. Wauu.DE: Health: Conditions And Diseases: Neurological Disorders: Brain Diseases
http//www.ninds.nih.gov/health_and_medical/disorders/hallervorden.htm. hallervordenspatz syndrome Association (HSSA) Contains news, research information
http://www.wauu.de/Health/Conditions_and_Diseases/Neurological_Disorders/Brain_D
Home Health Conditions and Diseases Neurological Disorders ... Basal Ganglia : Hallervorden-Spatz Syndrome Search DMOZ-Verzeichnis:
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66. Pediatric Neurology, Volume: 25, Issue: 2, August, 2001
First scientific workshop on hallervordenspatz syndrome executive summary. Hayflick, Susan J. pp. 99-101. hallervorden-spatz syndrome. Swaiman, Kenneth F. pp.
http://else.hebis.de/cgi-bin/sciserv.pl?collection=journals&journal=08878994&iss

67. Other Brain Conditions
There is an article on cluster headaches that is particularly recommended reading by OUCH UK. hallervordenspatz syndrome back to top.
http://www.nervecentre.org.uk/Other Conditions.htm
Supporting people in Caithness and North Sutherland affected by neurological disorders www.nervecentre.org.uk info@nervecentre.org.uk Other Neurological Conditions up Brachial plexus injury Lambert-Eaton syndrome Brain tumour, haemorrhage and AVM Mal De Debarquement syndrome (MDDS) Benign essential tremor Migraine Cluster headaches Neuromuscular conditions ... Tuberous Sclerosis Full (?) List of Neurological conditions The US National Institute for Neurological Disorders and Stroke has a fairly comprehensive listing of neurological conditions from which you can find out more on each one. Click here to find the page you need to start your search. Brachial Plexus injury Brain Tumour, Haemorrhage and AVM (arterio venous malformations) Brain Tumour Action is an organisation set up to support and help people diagnosed with brain tumours, in direct and useful ways such as through their Befrienders scheme. The site has a lot of information on brain tumours and statistics on their incidence in the population.

68. Any Search Info - Directory: Health Conditions And Diseases Neurological Disorde
hallervordenspatz syndrome Association (HSSA) Contains news, research information, family MCW HealthLink An article about HallervordenSpatz disease, with an
http://search-info.com/search/engine/index/Health/Conditions_and_Diseases/Neurol
Top Health Conditions and Diseases Neurological Disorders ... Basal Ganglia : Hallervorden-Spatz Syndrome [5] Description Directory Results: Categories: Health: Conditions and Diseases: Genetic Disorders
Health: Conditions and Diseases: Rare Disorders

Web Site Matches: Hallervorden-Spatz Disease
Information sheet compiled by NINDS.
url: www.ninds.nih.gov/health_and_medical/disorders/hal.... Hallervorden-Spatz Syndrome Association (HSSA)
Contains news, research information, family pages with personal stories, publications and resources, details of fundraising efforts and association history.
url: www.hssa.org MCW HealthLink
An article about HallervordenSpatz 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 HallervordenSpatz Syndrome.
url: www.rarediseases.org/search/rdbdetail_abstract.htm.... OMIM National Center for Biotechnology Information. A clinical synopsis of HallervordenSpatz syndrome. url: www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?2342....

69. Rare Disease Catalog H-K
This page last updated November 13, 2000. H. Disease Name, Omim , Organizations/Sites. hallervordenspatz syndrome, 234200, hallervorden-spatz syndrome Association.
http://www.med.nyu.edu/rgdc/dis_hijk.htm
Rare Genetic Diseases In Children: The Rare Disease Catalogue (H-K)
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November 13, 2000 H Disease Name Omim # Organizations/Sites Hallervorden-Spatz Syndrome Hemihypertrophy Hemiplegia Hemochromatosis
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70. PhatNav Directory - Health/Conditions_and_Diseases/P
Porencephaly. Health/Conditions and Diseases/Neurological Disorders/Brain Diseases/Basal Ganglia/hallervordenspatz syndrome. Health
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71. Ô˶¯ÕÏ°­ÐÔ¼²²¡---www.sunbinmd.com
8? 1 Pena JA,PrietoCarrasquero M,Gonzalez-Ferrer S. hallervorden-spatz syndrome in 2 Venezuelan patients. hallervorden-spatz syndrome.
http://www.sunbinmd.com/newsinfo.asp?nid=114

72. PANTOTHENATE Prescription Drug Information - Buy, Purchase Or Order PANTOTHENATE
B5 Pantothenate General Search ResultsFull Record Article 1 of 1 A novel pantothenate kinase gene (PANK2) is defective in hallervorden-spatz syndrome Zhou B
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Search for Drug Information Resources: The Drug Database P > pantothenate
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Accession Number : KLM0000299 This work is released into the public domain; please see our release statement. Config Rule : ... 'R- pantothenate ' config('R- pantothenate
http://www.biocheminfo.org/
A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome

Published online: 23 July 2001, doi:10.1038/ng572 volume 28 no. 4 pp 345 - 349 A novel pantothenate kinase gene (PANK2) is defective in Hallervorden-Spatz syndrome
http://www.nature.com/
Calcium Pantothenate

... Synonyms: Calcium D-(+)- Pantothenate ; Vitamin B-5; Pantothenic Acid, Calcium Salt ... http://www.jtbaker.com/ Molecular Expressions: The Vitamin Collection - Pantothenic Acid (Pantothenate, Vitamin B-5) Pantothenic acid was discovered in 1933 by Dr. R. J. Williams and was found to be a true vitamin shortly thereafter. http://micro.magnet.fsu.edu/

73. ²Ô°×Çò-ºÚÖÊÉ«ËرäÐÔÒ»¼Òϵ3Àý
41 Pena JA,PrietoCarrasquero M,Gonzalez-Ferrer S. hallervorden-spatz syndrome in 2 Venezuelan patients. hallervorden-spatz syndrome.
http://www.chinaneuro.com/educate/list.asp?article_id=276

74. Érudit | MS V19 N6-7 2003 : Gilgenkrantz : À Propos Des NeurodégénÃ
Translate this page Le syndrome d’Hallervorden-Spatz, une bien sombre histoire. A novel pantothenate kinase gene (PANK2) is defective in hallervorden-spatz syndrome.
http://www.erudit.org/revue/ms/2003/v19/n6-7/006826ar.html
Le navigateur que vous utilisez est d'une ancienne version (ou bien la prise en charge des feuilles de styles CSS est d©sactiv©e). La mise en page de l'article ne peut ©tre enti©rement reproduite avec cette version. M©decine/Sciences Volume 19, num©ro 6-7, Juin-Juillet 2003 Directeurs : Michel Bergeron et Jean-Claude Dufour Co-R©dacteurs en chef : G©rard Friedlander et Daniel G. Bichet ‰diteurs : La Soci©t© de la revue m©decine/sciences (Montr©al) et EDK : ‰ditions m©dicales et scientifiques (Paris) ISSN : 0767-0974 (imprim©)
€ propos des neurod©g©n©rescences associ©es   un d©ficit en pantoth©nate-kinase ou PKAN
Auteur Simone Gilgenkrantz 9, rue Basse, 54330 Clerey-sur-Brenon, France. s.gilgenkrantz@chu-nancy.fr Les troubles neurologiques extra-pyramidaux avec augmentation du fer s©rique se rencontrent au cours de trois maladies dont le d©faut mol©culaire a ©t© identifi©:
  • la neuroferritinopathie due   des mutations du g¨ne codant pour la cha®ne l©g¨re de la ferritine; l’ac©rul©oplasmin©mie due   une mutation du g¨ne codant pour la c©ruloplasmine;

75. Human Protein: Q9BSE1 - Hypothetical Protein (Fragment). EMBL Bioinformatic Harv
Mutations in this gene are associated with HARP syndrome and pantothenate kinaseassociated neurodegeneration (PKAN), formerly hallervorden-spatz syndrome.
http://harvester.embl.de/harvester/Q9BS/Q9BSE1.htm
Human protein: Q9BSE1 - Hypothetical protein (Fragment). EMBL
FORUM Length: 56 aa , molecular weight: 6058 Da , CRC64 checksum: LCMPLPPRAA ELSWGDPESH PAAPCRPRSS SPPPAQQCSR SVWPLVVGRR KGLNRG 56 //
UniProt
ensEMBL Entrez RZPD ... EBI-Hinxton-"Uniprot-Swissprot-TrEMBL" database General information Entry name Accession number Created TrEMBLrel. 17, 1-JUN-2001 Sequence update TrEMBLrel. 17, 1-JUN-2001 Annotation update TrEMBLrel. 22, 1-OCT-2002 Description and origin of the Protein Description Hypothetical protein (Fragment). Organism source Homo sapiens (Human). Taxonomy Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Primates; Catarrhini; Hominidae; Homo. NCBI TaxID References Strausberg,R., Submitted AUG-2001 to the EMBL GenBank DDBJ databases Position SEQUENCE FROM N.A. Comments TISSUE=Placenta; Database cross-references EMBL Keywords Hypothetical protein Features Key Begin End Length Description
UniProt
ensEMBL Entrez RZPD ... GoTo: SOURCE @ Stanford University
GeneReport for:
pantothenate kinase 2 (Hallervorden-Spatz syndrome)

UniGene
LocusLink OMIM GenAtlas ... Genome Browser Chromosomal Location
Chromosome/Cytoband Microarray Gene Expression Data
Data available Show Gene Expression Data LocusLink Information
Locus Link Summary This gene encodes a protein belonging to the pantothenate kinase family. Pantothenate kinase is a key regulatory enzyme in the biosynthesis of coenzyme A (CoA) in bacteria and mammalian cells. It catalyzes the first committed step in the universal biosynthetic pathway leading to CoA and is itself subject to regulation through feedback inhibition by CoA. Mutations in this gene are associated with HARP syndrome and pantothenate kinase-associated neurodegeneration (PKAN), formerly Hallervorden-Spatz syndrome. Alternative splicing has been observed at this locus, including the use of 4 alternate first exons. Six transcript variants have been identified, encoding four distinct isoforms.

76. Human Protein: Q9BZ23 - Pantothenate Kinase 2, Mitochondrial Precursor (EC ) (Pa
Zhou,B., Westaway,SK, Levinson,B., Johnson,MA, Gitschier,J., Hayflick,SJ, A novel pantothenate kinase gene (PANK2) is defective in hallervordenspatz syndrome.
http://harvester.embl.de/harvester/Q9BZ/Q9BZ23.htm
Human protein: Q9BZ23 - Pantothenate kinase 2, mitochondrial precursor (EC ) (Pantothenic acid kinase 2) (hPANK2). EMBL
FORUM Length: 570 aa , molecular weight: 62694 Da , CRC64 checksum: MRRLGPFHPR VHWAAPPSLS SGLHRLLFLR GTRIPSSTTL SPPRHDSLSL DGGTVNPPRV 60 REPTGREAFG PSPASSDWLP ARWRNGRGGR PRARLCSGWT AAEEARRNPT LGGLLGRQRL 120 LLRMGAGRLG APMERHGRAS ATSVSSAGEQ AAGDPEGRRQ EPLRRRASSA SVPAVGASAE 180 GTRRDRLGSY SGPTSVSRQR VESLRKKRPL FPWFGLDIGG TLVKLVYFEP KDITAEEEEE 240 EVESLKSIRK YLTSNVAYGS TGIRDVHLEL KDLTLCGRKG NLHFIRFPTH DMPAFIQMGR 300 DKNFSSLHTV FCATGGGAYK FEQDFLTIGD LQLCKLDELD CLIKGILYID SVGFNGRSQC 360 YYFENPADSE KCQKLPFDLK NPYPLLLVNI GSGVSILAVY SKDNYKRVTG TSLGGGTFFG 420 LCCLLTGCTT FEEALEMASR GDSTKVDKLV RDIYGGDYER FGLPGWAVAS SFGNMMSKEK 480 REAVSKEDLA RATLITITNN IGSIARMCAL NENINQVVFV GNFLRINTIA MRLLAYALDY 540 WSKGQLKALF SEHEGYFGAV GALLELLKIP 570 //
UniProt
ensEMBL Entrez RZPD ... EBI-Hinxton-"Uniprot-Swissprot-TrEMBL" database General information Entry name Accession number Created Rel. 41, 28-FEB-2003

77. Brain Diseases - 114 Of The Best Sites Selected By Humans
NORD Neurodegeneration with Brain Iron Accu MCW HealthLink -hallervorden-spatz syndrome Association (HSSA) Basal_Ganglia -Sydenhams Chorea and Chorea
http://www.cbel.com/brain_diseases/?order=alpha

78. Brain Diseases - 114 Of The Best Sites Selected By Humans
1 NORD Neurodegeneration with Brain Iron Accu 1 -MCW HealthLink 1 -hallervorden-spatz syndrome Association (HSSA) 1 Basal_Ganglia 6 -Sydenhams
http://www.cbel.com/brain_diseases/?order=pop

79. ASENT | NeuroRx Newsletter - Role Of Iron In Neurodegeneration
hallervordenspatz syndrome Gene Defect Identified hallervorden-spatz syndrome (HSS) is a progressive, autosomal neurodegenerative disorder of childhood and
http://www.asent.org/newsletter/2001/september/
Volume 1, Issue 4
September 2001
What's Inside Role of Iron in Neurodegeneration A Vaccine for Prion Disease? and Related Articles Industry News News from Other Countries ... Employment Opportunities The Role of Iron in Neurodegeneration Excessive iron accumulation has been reported in the brains of patients with Parkinson’s or Alzheimer’s disease, and it has been postulated that abnormal iron accumulation in the brain is neurotoxic through the generation of free radicals. However, it remains unclear whether iron-induced oxidative stress is a primary or a secondary event in the process that leads to neuronal death. Two recent reports suggest that - at least in some cases - iron toxicity may be a primary event in neurodegeneration. Neuroferritinopathy Causative Gene Identified
In recent years, Curtis et al have described a previously unrecognized adult-onset neurodegenerative disease that affects the basal ganglia and is associated with iron accumulation. The disease is dominantly inherited and presents with extrapyramidal symptoms and low ferritin serum levels. Histopathology is characterized by lesions in the globus pallidus with abundant spherical inclusions containing aggregates of ferritin and iron. Additionally, axonal swellings are found throughout the brain and are immunoreactive for ubiqutin and tau. Within the brain, only the basal ganglia appear to be affected. Outside the brain, organs such as the pancreas, liver, and heart that are typically affected in iron accumulation disease, appear to function normally.

80. Blackwell Synergy - Cookie Absent
hallervordenspatz syndrome. Pediatric Neurology 2001;25 102-108. hallervorden-spatz syndrome clinical and magnetic resonance imaging correlations.
http://www.blackwell-synergy.com/links/doi/10.1034/j.1600-0447.2003.00159.x/abs/
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