81. The Clinicopathological Spectrum Of Rosenthal Fibre Encephalopathy And Alexander Other mechanisms in adult onset Alexander’s disease and Rosenthal fibre encephalopathy that Infantile alexander disease spectrum of GFAP mutations and http://jnnp.bmjjournals.com/cgi/content/full/74/6/807

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Abstract Full Text (PDF) Submit a response ... Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Jacob, J Articles by Hilton, D A Related Collections Other Neurology Journal of Neurology Neurosurgery and Psychiatry BMJ Publishing Group SHORT REPORT J Jacob N J Robertson and D A Hilton Department of Neurology, Derriford Hospital, Plymouth, UK Department of Histopathology and Neuropathology, Derriford Hospital Correspondence to: Dr J Jacob, Department of Neurology, Derriford Hospital, Derriford Road, Plymouth, Devon PL6 8DH, UK; Received 27 August 2002 Accepted 11 December 2002 ABSTRACT in early childhood, but can infrequently arise in adults. It

82. Alexander's Disease: Case Report Including Histopathological And Electron Micros We report a case of Alexander s disease in a black South African boy of 9 years. alexander disease Diagnosis with MR Imaging AJNR Am. J. Neuroradiol., http://jnnp.bmjjournals.com/cgi/content/abstract/42/7/619

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS Author Keyword(s) Vol Page [Advanced] This Article Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Cole, G. Articles by Bill, P. Journal of Neurology, Neurosurgery, and Psychiatry, 1979, Vol 42, 619-624 PAPERS Alexander's disease: case report including histopathological and electron microscopic features G Cole, F De Villiers, NS Proctor, I Freiman and P Bill We report a case of Alexander's disease in a black South African boy of 9 years. The child presented with a grossly abnormal stooped posture, generalised weakness, and slurred nasal speech. Computerised tomography

83. Juvenile Form Of Alexander Disease With GFAP Mutation And Mitochondrial Abnormal Juvenile form of alexander disease with GFAP mutation and mitochondrial abnormality. Y. Nobuhara, MD , K. Nakahara, MD , I. Higuchi, MD , T. Yoshida, http://www.neurology.org/cgi/content/abstract/63/7/1302

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Figures Only Full Text Full Text (PDF) ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Nobuhara, Y. Articles by Nakagawa, M. Related Collections Metabolic disease (inherited) Mitochondrial disorders NEUROLOGY 2004;63:1302-1304 American Academy of Neurology Brief Communications Juvenile form of Alexander disease with GFAP mutation and mitochondrial abnormality Y. Nobuhara, MD K. Nakahara, MD I. Higuchi, MD T. Yoshida, MD S. Fushiki, MD, PhD M. Osame, MD K. Arimura, MD and M. Nakagawa, MD From the Department of Neurology and Geriatrics (Drs. Nobuhara, Higuchi, Osame, and Arimura), Kagoshima University Graduate School of Medical and Dental Sciences; Division of Neurology (Drs. Nobuhara and Nakahara), Miyazaki Prefectural Hospital; Department of Neurology and Gerontology (Drs. Yoshida and Nakagawa), Research Institute for Neurological Diseases and Geriatrics, Kyoto Prefectural University of Medicine; and Department of Pathology and Applied Neurobiology (Dr. Fushiki), Kyoto Prefectural University of Medicine Graduate School of Medical Science, Japan. The authors report a 29-year-old woman with marked atrophy of the cerebellum, medulla oblongata, and spinal cord, dementia

84. Molecular Findings In Symptomatic And Pre-symptomatic Alexander Disease Patients Background and Objective alexander disease is a slowly progressive CNS disorder Juvenile form of alexander disease with GFAP mutation and mitochondrial http://www.neurology.org/cgi/content/abstract/58/10/1494

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Figures Only Full Text Full Text (PDF) ... Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Gorospe, J. R. Articles by Hoffman, E. P. Related Collections Metabolic disease (inherited) Leukodystrophies All Pediatric All Genetics Neurology American Academy of Neurology Molecular findings in symptomatic and pre-symptomatic Alexander disease patients J. R. Gorospe, MD PhD S. Naidu, MD A. B. Johnson, MD V. Puri, MD G. V. Raymond, MD S. D. Jenkins, MD R. C. Pedersen, MD D. Lewis, MD P. Knowles, MD R. Fernandez, MD D. De Vivo, MD M. S. van der Knaap, MD A. Messing, VMD PhD M. Brenner, PhD and E. P. Hoffman, PhD Background and Objective: Alexander disease is a slowly progressive CNS disorder that most commonly occurs in children. Until recently, the diagnosis could only be established by the histologic finding of Rosenthal fibers in brain specimens. Mutations in the glial

85. Team Discovers Genetic Basis Of Alexander Disease (Jan 2, 2001) Scientists have pinpointed the gene responsible for a rare and devastating childhood brain disorder called alexander disease, solving a 50year-old mystery http://www.news.wisc.edu/wisweek/view.msql?id=5672

86. Health Library - Alexanderdisease mutation of GFAP causes filament disorganization alexander disease is a fatal neurological illness characterized by Many Alexander-disease patients are heterozygous for one of a set of point mutations http://12.42.224.150/library/healthguide/en-us/illnessconditions/topic.asp?hwid=

87. Rural Nurse Organization Clinic Digital Library alexander disease Access document. Clinical Guidelines. National Guideline Clearinghouse MeSH browse Detailed search. Metabolism, Inborn Errors (MeSH http://ruralnurseorganization-dl.slis.ua.edu/clinical/metabolism/inborn/lipid/le

Clinical Resources by Topic: Metabolic Disorders Alexander Disease Clinical Resources Pediatrics Radiology Genetics Clinical Guidelines ... Miscellaneous Resources See also: Genetic Testing Clinical Resources Mental Retardation Clinical Resources General Genetic Disorders Clinical Resources General Metabolic Disorders Clinical Resources ... Alexander Disease Patient/Family Resources Neurology (eMedicine): Table of contents Inherited Metabolic Disorders: Access document Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes: List of documents Alexander Syndrome: Access document Pediatrics Resources See also General Pediatrics Resources Neurology (eMedicine): Table of contents Inherited Metabolic Disorders: Access document Emergency Medicine (eMedicine): Table of contents Pediatrics, Inborn Errors of Metabolism: Access document Radiology Resources See also General Radiology Resources CHORUS-Collaborative Hypertext of Radiology: Homepage Musculoskeletal System: List of documents Alexander Disease: Access document Genetics Resources See also General Genetics Resources Online Mendelian Inheritance in Man: Homepage Alexander Disease: Access document Clinical Guidelines National Guideline Clearinghouse: MeSH browse Detailed search Metabolism, Inborn Errors (MeSH browse):

88. The Dylan Samuel Freeman Story He was diagnosed with alexander s disease at age 4. The purpose of his web site is to help educate as many people as possible about this rare known disease http://www.dylanfreeman.20m.com/

The Dylan Samuel Freeman Story About Alexander's Disease Sign My Guest Book Lakeland Ledger Article ... OUR HERO - 11/20/1996 - 4/30/2004 Dylan Samuel Freeman Welcome To My Home Page!!!!! My name is Dylan was 7 years old. He was diagnosed with Alexander's Disease at age 4. The purpose of his web site is to help educate as many people as possible about this rare known disease and hopefully raise funds for research, for a treatment or cure. Maybe the research can't help Dylan. but please continue to donate, so Dylan's friends can be saved Dylan Samuel Freeman November 20th 1996 April 30th 2004 Everyone who meets him out on the town calls him HOLLYWOOD; since he insists on wearing his sunglasses to protect his Beautiful Blue Eyes. Everyone he meets falls in love with him at first sight. He is so polite with his "Hi's, Pleases, and Thank you's". He never wants to see anyone upset and he tries to keep everyone around him happy. His beautiful smile will win your heart the minute you lay eyes on him. Dylan Samuel Freeman, son of Sam and Kathy Freeman, was born on November 20th, 1996 in Brandon, Fl. At the age of 18 months, we noticed that he was having trouble with his balance, but he finally learned to walk at age 2. His doctor mentioned the possibility of mild Cerebral Palsy and said he would probably grow out of it. As it became more noticeable in his speech, we took him to another Neurologist in January 2001 at age 4. He had an MRI done and the doctor dianosed Alexander Disease and advised us to get a second opinion, since this was something that he had only read about at Harvard, but had never seen before.

89. Managing Pig Health, Treating Pig Diseases, Pig Health And Welfare. Practical manual providing detailed information on pigs, hogs, swine. This 600+ page book, written by Mike Muirhead and Tom alexander is written in an easily understandable format and is aimed at everyone involved in swine production, from the farmer to the professional. Web site gives book contents and has an order form. http://www.managingpighealth.com

Promote your business here Make ThePigSite your homepage! Friday, September 19, 2003 About News Books Book Draw ... FREE Weekly Newsletter Information Center Managing Pig Health Stockmanship Standards Quick Disease Guide Disease Problem Solver ... close Featured Articles Discussion Forums Business Directory Meet the Experts Our Main Sponsors Supporting Partners rl consulting Want your Company listed here? Click for more details Got Poultry? Why not visit us at: The PigSite Pig Health Management Area Browse Option You are in Managing Pig Health section of thePigSite.com where you can browse through over 600 pages of information on managing the health aspects of a pig or hog farm. The information in this section is taken from our book Managing Pig Health and the Treatment of Disease written by Mike Muirhead and Tom Alexander. If you would like to buy a copy of the book (for bedtime reading!) it can be purchased from our book shop If you would prefer to search the contents of this section you can do so by clicking here Select a Chapter to Browse Cptr 1. Anatomy, Physiology and technical terms

90. Chronic Fatigue Syndrome And The Alexander Technique How this centuryold method of releasing harmful stress may be of benefit to a patient with this disease. http://www.alexandertechnique.com/articles/chronicfatigue

Chronic Fatigue Syndrome and the Alexander Technique by Martin Finnegan What exactly is Chronic Fatigue Syndrome?* Sometimes referred to as ME or Myalgic Encephalomyelitis, it is a term that describes a chronic, debilitating disorder that affects the immune and central nervous system. Typical of its symptoms are a profound fatigue, totally out of proportion to a person's physical activity and independent of mood, plus a range of other symptoms that can affect any organ of the body. The causes of CFS are unknown. Indeed there appear to be any number of apparent causes and in many cases the onset seems to be linked to a stress to the immune system such as an acute infection, especially viral in nature. After the stress or virus has run its course the symptoms do not abate as you would expect but set in, becoming chronic and are often associated with profound fatigue and feelings of general malaise. Western medicine advocates rest, counseling, adjusting lifestyle and support as the best approach to treating CFS and this is all good advice. The problem, it seems to me is one of perception. The orthodox point of view perceives CFS as a thing. It is my opinion that CFS is not a thing at all, which is the reason that no cause and no diagnosis can be found. It is a process. A process of a human system running down, in a variety of ways, to such a point that it can't function properly anymore. In my own experience of CFS, it was exactly that and the solution was not to attempt to 'fix' a part of my body but to address the whole of 'me' and my life, in all its various aspects - physical, mental, emotional and spiritual. And the new model that I followed worked.

91. About Alexander's Disease Alexanders disease belongs to a group of progressive neurological disorders in which the Alexanders disease begins in infancy, affects mostly males, http://www.dylanfreeman.20m.com/about.html

The Dylan Samuel Freeman Story About Alexander's Disease Sign My Guest Book Lakeland Ledger Article ... OUR HERO - 11/20/1996 - 4/30/2004 A Rare Genetic Disorder!! ALEXANDERS DISEASE Alexanders disease belongs to a group of progressive neurological disorders in which the destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers. The majority of cases of Alexanders are sporadic; that is, without a known family history of the disorder. There are, however, a number of families with more than one affected child. Alexanders disease begins in infancy, affects mostly males, and results in retarded development and dementia. It is quickly progressive and is the rarest of the identified leukodystrophies. The onset of the infantile form of Alexanders disease is usually around six months, but may occur between and 24 months of age. Children who develop Alexanders in infancy generally do not survive past the age of 5 or 6 years. Physical and mental development is retarded, and there is progressive enlargement of the brain and head, increasing spasticity, and seizures in some cases. Histologically, demyelination of both sensory and motor fiber tracts is found. In addition, the eosinophilic Rosenthal fibers are found evenly distributed throughout the brain near blood vessels, and on the surface of the brain. The demyelinated areas do not correspond to the distribution of the Rosenthal fibers. Alexanders disease is thought to be an autosomal recessive disorder which can affect both males and females. The metabolic error is unknown, and the demyelination and fiber formation seem to be otherwise unrelated parallel expressions of the disorder. It is the degeneration of the astrocytic glial cells which seems to lead to demyelination. An exact diagnosis may not be possible without study of postmortem tissues or brain or nerve biopsy.

92. Alexander's Disease : A Case Report Of A Biopsy Proven Case. Tatke M, Sharma A N Neurol India is an peerreviewed biomedical periodical of Neurological Society of India. http://www.neurologyindia.com/article.asp?issn=0028-3886;year=1999;volume=47;iss

93. Alexander's Disease : A Case Report Of A Biopsy Proven Case., Tatke M, Sharma A: Neurol India is an peerreviewed biomedical periodical of Neurological Society of India. http://www.neurologyindia.com/article.asp?issn=0028-3886;year=1999;volume=47;iss

94. Metabolic Disorders The other primary white matter disorders include alexander s disease, Canavan disease, No biochemical marker has been identified in alexander s disease, http://spinwarp.ucsd.edu/NeuroWeb/Text/br-500.htm

DEGENERATIVE AND METABOLIC DISORDERS John R. Hesselink, MD, FACR and Richard J. Hicks, MD DEGENERATIVE DISEASES Imaging of degenerative disorders with CT has generally been disappointing, and attempts have been made to apply MR to this area with hopes of demonstrating more specific findings. The key to the MR imaging of many of these disorders may rest in understanding the normal and pathologic distribution of iron in the brain. Iron is visualized as areas of hypointensity on T2-weighted and GRE imagescaused by local field inhomogeneity and magnetic susceptibility effects. Drayer and colleagues noted decreased signal in the globus pallidus, reticular substantia nigra, red nucleus, and dentate nucleus. These areas correlated closely with sites of preferential accumulation of ferric iron on Perls' stains in normal brains post mortem. This iron deposition becomes greater with increasing age, with iron stains first becoming positive at 6 months in the globus pallidum and at 3 to 7 years in the dentate nucleus. With advanced age (approximately the ninth decade), there may be enough iron deposition in the putamen to render it as hypointense as the globus pallidus. Iron may play a role in neurotransmitter metabolism, and several degenerative disorders have been reported to be associated with increased iron deposition in the brain. This has been described in Hallervorden-Spatz disease, Huntington's chorea, Parkinson's disease and multisystem atrophy variants, Alzheimer's disease, and multiple sclerosis.

95. Alexander S Disease Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume VI 1/ALEXANDERS DISE

financial services our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Alexander's disease, (W. Stewart Alexander, 20th century, English pathologist), rare dysmyelinogenetic leukodystrophy , of sporadic occurrence without a familial incidence. There are three clinical subgroups: infantile, juvenile and adult. In the most common infantile form macrocephaly is the hallmark together with developmental delay, spasticity and seizures. The average duration of the illness is usually about 3 years. The juvenile form is characterized by onset between 7 and 14 years, spasticity, bulbar symptoms and a duration of about 8 years. In the adult the form onset of symptoms is variable as is the clinical manifestation that may have an intermittent course and resemble multiple sclerosis. There is no biochemical test to confirm the diagnosis. The CT and MR appearance ( Fig.1 ) of Alexander's disease is that of involvement of the white matter, starting bilaterally first in the frontal lobes and subsequently involving the peritrigonal temporoparietal regions. MR shows prolonged T1 and T2 relaxation times. GS The Encyclopaedia of Medical Imaging Volume VI 1 Alexander's disease, Fig. 1

96. Alexander S Disease Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/ALEXANDERS DISEA

financial services our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Alexander's disease, (W. Stewart Alexander, 20th century, New Zealand pathologist), fibrinoid leukodystrophy , a primary white matter disease without specific biochemical abnormalities. Neurological regression, seizures, bulbar signs and macrocrania are the clinical manifestations. MR imaging shows diffuse demyelination, most marked frontally with a coarse appearance of sulci and gyri. For further description, see Alexanders disease HC The Encyclopaedia of Medical Imaging Volume VII Corporate Products Financial Services Our Commitment ... Terms of Conditions

97. The Myelin Project: Demyelinating Diseases In Brief The etiology of alexander s disease remains largely unknown. The clinical course of hereditary demyelinating disorders, which usually tend to manifest http://www.myelin.org/diseasesinbrief.htm

HOME Overview Research News ... Links IN THIS SECTION An Overview Demyelinating Diseases In Brief Scope and Strategies Demyelinating Diseases In Brief Demyelinating diseases are those in which myelin is the primary target. They fall into two main groups: acquired diseases (i.e., multiple sclerosis) and hereditary neurodegenerative disorders (i.e., the leukodystrophies). Although their causes and etiologies are different, they have the same outcome: CNS demyelination. Without myelin, nerve impulses are slowed or stopped, leading to a constellation of neurological symptoms. Axons with normal myelin Demyelinated axons: Nerve impulse conduction slows or stops completely Acquired Diseases The most common of these is multiple sclerosis (MS), which usually manifests itself between the 20th and 50th years of life. Current estimates are that approximately 2.5 million people worldwide have MS, with between 250,000 and 350,000 cases in the United States, 50,000 cases in Canada, 130,000 cases in Germany, 85,000 cases in the United Kingdom, 75,000 cases in France, 50,000 cases in Italy, and 11,000 cases in Switzerland. MS attacks the white matter of the central nervous system (CNS). In its classic manifestation (90% of all cases), it is characterized by alternating relapsing/remitting phases with periods of remission growing shorter over time. Its symptoms include any combination of spastic paraparesis, unsteady gait, diplopia, and incontinence.

98. Medical Image Database, Radiology Teaching Files And Cases, MedPix™: Single alexander s disease (AD) is a rare dysmyelinating leukodystrophy. Males are most often affected, and the disease appears to occur sporadically without a http://rad.usuhs.mil/medpix/radpix.html?mode=single&comebackto=mode=geo_browse&r

99. Type_Document_Title_here An exact diagnosis of alexander s disease may not be possible without study of As well as the infantile form of the disease, alexander s disease is http://www.alds.org.au/alex.html

Back to Main List ALEXANDER'S DISEASE Alexanders disease is the rarest of the identified leukodystrophies. The infantile form tends to appear between birth and two years, and most sufferers of this form do not survive past the age of 5 or 6. The destruction of the white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits. These are known as Rosenthal fibres. Alexanders tends to be sporadic, appearing in families with no known history of the disorder. It is progressive, affects mostly males, and leads to both physical and mental retardation and dementia. The brain and head become progressively larger, leading the increased spasticity and sometimes seizures. The myelin sheath of both the sensory and motor fibre tracts is affected in Alexander's disease. The Rosenthal fibres are found distributed near the blood vessels of the brain and on the brain's surface. The nerves affected by the disease's demyelination do not correspond with the distribution of the Rosenthal fibres, and these two effects seem to be otherswise unrelated expressions of the disorder. The exact metabolic error that causes Alexanders disease is unknown, but it is thought to be an autosomal recessive disorder, which may affect both males and females. The demyelination seems to be cause by the destruction of the astrocytic glial cells.

100. UAB Researchers Collaborate To Find Genetic Basis For Rare Alexander's Disease - alexander s disease most often occurs in infants under the age of one but can also attack older children and adults. Only about 100 cases have been http://www.bizjournals.com/birmingham/stories/2001/01/01/daily8.html

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