NBIA Disorders Association Contains news, research information, family pages with personal stories, publications and resources, details of fundraising efforts and association history. http://www.hssa.org
Hallervorden-Spatz Syndrome (www.whonamedit.com) If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here see a doctor. hallervordenspatz syndrome http://www.whonamedit.com/synd.cfm/1082.html
Extractions: This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor. Localized neuroaxonal dystrophy, late infantile neuroaxonal dystrophy, progressive pallidal degeneration syndrome, progressive pallidal degeneration or adult amaurotic idiocy, pigmentary degeneration syndrome of globus pallidus, substantia nigra and red nucleus, pallidoreticular pigment degeneration, progressive pallidal degeneration syndrome. A very rare disease beginning in childhood, with degeneration of the globus pallidus, red nucleus, and reticular part of the substantia nigra of the brain. It is characterized by progressive Parkinson-like rigidity, athetotic movements, and progressive mental and emotional retardation. Onset is in late childhood; death usually occurs within 10 years, but courses of up to 30 years have been described. Aetiology unknown; considered one form of iron storage disease. A heredo-familial syndrome, inherited as an autosomal recessive trait; some unrelated cases reported
Radiological Quiz - Neuroradiology EC, Schoene WC, Richardson EP Jr. Hallervorden Spatz syndrome. Arch Neurol 1974; 30 7083 DW, EI Gammal T. hallervorden-spatz syndrome clinical and magnetic resonance imaging http://www.ijri.org/20011104/radquiz03.htm
Extractions: A nineteen-year-old boy presented with dystonia, slowly progressing since the age of 5 years. On examination, the patient was depressed and had dystonia and tremors. He had no cranial nerve palsies and his fundi were normal. Hematological examination was normal with no acanthocytosis. MRI was performed (Figs 1, 2). The axial T2 and coronal PD images through basal ganglia show marked hypo intensity of globus pallidus with hyperintense area within. This appearance is referred to as eye of tiger sign and is diagnostic of Hallervorden Spatz Disease (HSD). HSD is a rare neurological disorder, characterized by dystonia, rigidity, retinal degeneration, pyramidal signs and mental deterioration. Criteria for diagnosis of HSD are:
Extractions: Pediatric Neurology devoted its August, 2001, issue Vol. 25, pages 89-176 to the First Scientific Workshop on Hallervorden-Spatz Syndrome. We have a limited number of copies available. This is a professional publication geared for physicians and health professionals. Please contact pwood@NBIAdisorders.org if you are interested in receiving a copy. Scientists map out research strategy to tackle HSS By Dr. Susan J. Hayflick An historic event took place at the National Institutes of Health May 19 and 20. Nearly 50 scientists, physicians and others interested in HSS from around the world gathered for the First Scientific Workshop on Hallervorden-Spatz Syndrome, sponsored by the Office of Rare Disorders and the National Institute of Neurological Disorders and Stroke. This marked the first time researchers met to discuss what is known about HSS and what is needed to further HSS studies. Since few scientists study HSS, the meeting also had the goal of stimulating interest in the disorder among young researchers whose work relates to some aspect of the disease. After defining what we know about HSS - including its clinical, pathological and genetic features - we discussed iron metabolism and how the basal ganglia in the brain work. The group learned about other disorders of brain iron accumulation in humans and mice and discussed possible therapies for HSS, including chelation, nitric oxide and medications to limit the bodys production of damaging compounds (free radicals) as a result of the iron.
Redirect A clinical synopsis of hallervordenspatz syndrome. http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?234200
Extractions: Web Directory: Hallervorden-Spatz Disease Information sheet compiled by NINDS. Hallervorden-Spatz Syndrome Association (HSSA) Contains news, research information, family pages with personal stories, publications and resources, details of fund-raising efforts and association history. MCW HealthLink An article about Hallervorden-Spatz disease, with an explanation, the symptoms and prognosis.
NORD - National Organization For Rare Disorders, Inc. Offers synonyms, a general discussion and further resources for the disorder formerly known as hallervordenspatz syndrome. http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Neurodegenera
NeuroGate.com Search results for "hallervordenspatz syndrome" Cardioguide Matches 1 - 1 of 1 Search AltaVista for more on 'hallervorden-spatz syndrome' http://www.neurogate.com/neuro/result.php3?search=Hallervorden-Spatz Syndrome&am
HON - List Of Rare Diseases GuillainBarre Syndrome, Gynatresia. Hallermann s Syndrome, hallervorden-spatz syndrome. Hamartoma Syndrome, Multiple, Hand-Schueller-Christian Syndrome. http://www.hon.ch/HONselect/RareDiseases/
Search: - Info.co.uk Results for hallervordenspatz syndrome from Info.co.uk metasearch. Find better search results from the Web, Yellow Pages and White Pages quickly and easily! STREIFF - OCULO-MANDIBULO-DYSCEPHALY; http://dpxml.infospace.com/infocom.uk/results?otmpl=dog/webresults.htm&qkw=H
The First Scientific Workshop Of Hallervorden-Spatz Syndrome The First Scientific Workshop of hallervordenspatz syndrome Debbie Forstall Secretary, hallervorden-spatz syndrome Association. http://www.ninds.nih.gov/news_and_events/proceedings/Hallervorden-Spatz.htm
Hallervorden-Spatz Syndrome Gene Discovery Laboratories. Education. Jobs. About NEI. Resources. Help Viewing Site. Home News and Events Statements and Reports on Vision hallervordenspatz syndrome Gene Discovery. NEI Statement. hallervorden-spatz syndrome Gene Discovery The gene that causes hallervorden-spatz syndrome has been identified by National Eye Institute (NEI) grantees. hallervorden-spatz syndrome is a rare, inherited, neurological http://www.nationaleyeinstitute.net/news/statements/hallervorden-spatz.htm
Extractions: National Institutes of Health The gene that causes Hallervorden-Spatz syndrome has been identified by National Eye Institute (NEI) grantees. Hallervorden-Spatz syndrome is a rare, inherited, neurological disorder associated with high accumulations of iron in the brain, and causes progressive degeneration of the retina and nervous system. The new findings appear in the August 2001 issue of Nature Genetics. Susan J. Hayflick, MD, associate professor of Molecular and Medical Genetics at Oregon Health Sciences University in Portland, and colleagues discovered that the defective gene produces an ineffective enzyme. The body needs the normal enzyme to utilize vitamin B5; without it, vitamin B5 cannot produce some of the body's essential compounds. The ineffective enzyme results in Hallervorden-Spatz syndrome. Because of this research, scientists can now focus their efforts on developing treatment strategies that bypass this defective enzyme, allowing the body to utilize vitamin B5 to help make the essential body compounds. Researchers can also look toward developing a genetic diagnostic test for the syndrome. Understanding the biochemical defects in Hallervorden-Spatz syndrome may also provide insights into the effect iron has on other neurodegenerative diseases associated with high iron accumulations, such as Parkinson's disease.
Hallervorden-Spatz Syndrome From Linkspider UK Health Directory Directory Topic hallervordenspatz syndrome assoicated to Health. Information. - A clinical synopsis of hallervorden-spatz syndrome. http://linkspider.co.uk/Health/ConditionsandDiseases/NeurologicalDisorders/Brain
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The First Scientific Workshop Of Hallervorden-Spatz Syndrome The First Scientific Workshop of hallervordenspatz syndrome. Get Web page suited for Registry of Patients with hallervorden-spatz syndrome and Related Disorders indicate that http://accessible.ninds.nih.gov/news_and_events/Hallervorden-Spatz.htm
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Hallervorden-Spatz Syndrome Directory, Home Health Conditions and Diseases Neurological Disorders Brain Diseases Basal Ganglia hallervordenspatz syndrome (5) See Also http://www.thenewhealthfind.com/Health/ConditionsandDiseases/NeurologicalDisorde
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