81. ACR Learning File Web Diagnosis alexander's disease. Return to top. Discussion alexander's disease (AD) is a rare dysmyelinating leukodystrophy http://www.learningfile.com/learning_file/viewcase.php?section=nu&case_num=6

82. Disease, Alexander - Medical Dictionary Definitions Of Popular Medical Terms MedicineNet Home MedTerms medical dictionaryAZ List disease, alexander. Advanced Search. http://www.medterms.com/script/main/art.asp?articlekey=13491

83. Partners Leukodystrophy Service At MGH Introduction. alexander s disease is a slowly progressive disease that primarilyaffects children. The adult form of alexander s disease is even more rare. http://fisher.mgh.harvard.edu/leuko/alexander.html

Home Staff Services Leukodystrophy categories Contact us Links X-linked Adrenoleukodystrophy Metachromatic leukodystrophy ... CACH Introduction Alexander's disease is a slowly progressive disease that primarily affects children. First described by Alexander in 1949, it is characterized by the finding of Rosenthal fibers in brain specimens ( Harding 1990 It is genetically determined, by sporadic gain of function mutations in the GFAP gene ( Brenner, Johnson et al. 2001 ). This observation adds Alexander disease to a growing list of intermediate filament diseases which includes Emery-Dreifuss muscular dystrophy (lamin A/C), cardiac and skeletal desmin myopathy (Desmin). To date all cases have been sporadic and more recent mutational analysis has not found mutations in either parent. There have been case reports, not genetically confirmed, of more than one child being affected (Johnson, Personal Communication, United Leukodystrophy Foundation Annual Meeting, 2002). Epidemiology The incidence of more than one affected person in a kindred is rare. Clinical Features: Broad Overview Clinically, it resembles

84. Alexander's Disease alexander s disease,. Print this article, (W. Stewart alexander alexander sdisease, Fig. 1. a. MR, axial IR image. Macrocephaly, dilated ventricles http://www.amershamhealth.com/medcyclopaedia/medical/Volume VI 1/ALEXANDERS DISE

Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters 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

85. Alexander's Disease Browse entry words starting with A, B, C, D, E, F, G, H, I, J, K, L, M,N, O, P, Q, R, S, T, U, V, W, X, Y, Z, Other characters, alexander s disease,.Print this article, http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/ALEXANDERS DISEA

Amershamhealth.com Search for: Type a word or a phrase. All forms of the word are searchable. Browse entry words starting with: A B C D ... Other characters 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 Contacts GE Healthcare Making Waves

86. Alexander's Disease, Dysmyelogenic Leukodystrophy, Dysmyelogenic Leukodystrophy- alexander s disease, Dysmyelogenic Leukodystrophy, Dysmyelogenic LeukodystrophyMegalobare,Fibrinoid Degeneration of Astrocytes, Fibrinoid Leukodystrophy http://www.icomm.ca/geneinfo/alex.htm

Alexander's Disease, Dysmyelogenic Leukodystrophy, Dysmyelogenic Leukodystrophy-Megalobare, Fibrinoid Degeneration of Astrocytes, Fibrinoid Leukodystrophy, Hyaline Panneuropathy, Leukodystrophy with Rosenthal Fibers, Megalencephaly with Hyaline Inclusion, Megalencephaly with Hyaline Panneuropathy For Information on Workshops and Seminars for Special Needs Children click here The GAPS INDEX to Information on the Internet about Genetic Disorders and Birth Defects Genetic Information and Patient Services, Inc. (GAPS) HOME DISORDERS GLOSSARY Alexander's Disease also known as: Dysmyelogenic Leukodystrophy Dysmyelogenic Leukodystrophy-Megalobare Fibrinoid Degeneration of Astrocytes Fibrinoid Leukodystrophy Hyaline Panneuropathy Leukodystrophy with Rosenthal Fibers Megalencephaly with Hyaline Inclusion Megalencephaly with Hyaline Panneuropathy (as defined by the National Organization for Rare Disorders Alexander's Disease is an extremely rare, progressive, metabolic, neurological disorder that is frequently inherited. It is one of a group of diseases known as the leukodystrophies.

87. Metabolic Disorders The other primary white matter disorders include alexander s disease, Canavandisease, Cockayne s syndrome, and PelizaeusMerzbacher s disease. (8). 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.

88. ALEXANDER'S DISEASE Features Listed For alexander S disease. McKusick 203450. Ataxia; Hydrocephaly/largeventricles, nonspecific; Macrocephaly; Papilloedema. Chromosome(s) http://www.hgmp.mrc.ac.uk/dhmhd-bin/hum-look-up?52

89. About Alexander's Disease A Rare Genetic Disorder!! ALEXANDERS disease. Alexanders disease begins in infancy,affects mostly males, and results in retarded development and dementia. 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.

90. ACR Learning File Web Return to top. Diagnosis alexander s disease. Return to top. Discussionalexander s disease (AD) is a rare dysmyelinating leukodystrophy. http://www.learningfile.com/learning_file/viewcase.php?section=nu&case_num=604

91. Leucodistrofie/ALEXANDER'S DISEASE HMSN) ii) MORBO DI alexander (alexander S disease). DEFINITION alexanderdisease was proven histologically in the last. Pyeritz http://www.peacelink.it/appeal/gianmarco/ii.html

a) Parametri Genetici delle Neuropatie Sensomotorie Ereditarie (HMSN) ii) MORBO DI ALEXANDER (ALEXANDER'S DISEASE) DEFINITION: A neurodegenerative disorder characterized by degeneration of CNS white matter and specific CNS pathological findings. EPIDEMIOLOGY: incidence: rare age of onset: newborn to adulthood (depends on the Form) risk factors: sporadic PATHOGENESIS: 1. Background unknown etiology but thought to involve dysfunctional astrocytes PATHOLOGY: 1. Rosenthal Fibres refractile eosinophilic hyaline bodies found within the cytoplasm of astrocytes particularly in the subpial, subependymal, and perivascular regions required for definite diagnosis but not pathognomonic as seen in other disorders as well, i.e., Neurofibromatosis, MS TYPES: Type I: Infantile Form Type II: Juvenile Form Type III: Adult Form CLINICAL FEATURES: 1. Type I - Infantile Form onset: mean of 6 months but can range from shortly after birth to 2 years of age most common of the 3 variants and most are male death in 2nd to 3rd years of age 1. Neurological Manifestations

92. Alexander's Disease : A Case Report Of A Biopsy Proven Case.,Tatke M, Sharma A: alexander s disease a case report of a biopsy proven case. Tatke M, SharmaA Department of Pathology, GB Pant Hospital, New Delhi, 110002, India. http://www.neurologyindia.com/article.asp?issn=0028-3886;year=1999;volume=47;iss

93. Discussion Forum : By Manas Tungare Replyalexander s disease and PRR, From Holly. Replies to this message Re alexander sdisease and PRR by Laura; Re alexander s disease and PRR by Laura; http://atready.com/activepages/bb/message.asp?MessageID=56

94. Discussion Forum : By Manas Tungare Discussion Forums at Developmental Concepts. ReplyRe alexander s disease and PRR,From Laura. This message is a Reply to alexander s disease and PRR from Holly. http://atready.com/activepages/bb/message.asp?MessageID=62

95. Genetic Basis For Rare Disease Found I think parents who have had children with alexander s diseasewill be relieved by finally knowing its cause,” said Messing. http://main.uab.edu/show.asp?durki=35374

96. EMedicine - Addison Disease : Article By Alexander Brough, MD autoimmune disease II, PGAD II, Schmidt syndrome, addisonian crisis, adrenocorticotrophichormone, ACTH, melanocytestimulating hormone, MSH. Author alexander http://www.emedicine.com/derm/topic761.htm

(advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Dermatology Internal Medicine Addison Disease Last Updated: August 28, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: Addison’s disease, primary adrenal insufficiency, chronic adrenal insufficiency, hypoadrenalism, polyglandular autoimmune diseases, polyglandular autoimmune disease I, PGAD I, polyglandular autoimmune disease II, PGAD II, Schmidt syndrome, addisonian crisis, adrenocorticotrophic hormone, ACTH, melanocyte-stimulating hormone, MSH AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography Author: Alexander Brough, MD , Consulting Surgeon, Department of Dermatology, Sewell's Point Clinic Coauthor(s): Elizabeth A Liotta, MD , Assistant Professor, Department of Dermatology, Uniformed Services University of the Health Sciences; Quenby Lea Erickson, DO , Staff Physician, Department of Dermatology, Wilford Hall Medical Center;

97. Demyelinating Diseases - Internet Handbook Of Neurology Cockayne Syndrome eMedicine/Pediatrics. alexander s disease alexanderdisease - GeneReviews; alexander s disease - University of http://www.neuropat.dote.hu/myelin.htm

Internet Handbook of Neurology Compiled by K atalin H Department of Neurology University of Debrecen, Hungary Demyelinating Diseases Chapters: A Collection of High Quality Online Resources for Health Professionals Pathology see Pathology of Infectious Diseases and Disease of Myelin Overview - Washington University Disorders of Myelination - University of Berkeley Demyelinating Diseases of the Brain - University of California, San Diego Diseases of Myelin - University of New Mexico Chemokine Regulation of Immune-mediated Demyelinating Disease - ILAR Journal, Vol 40(4) 1999 Pathology of Inflammatory Demyelinating Diseases - Mayo Clinic Viral Induced Demyelination - Brain Pathology, January 2001 (PDF) Is There a Role for Human Herpesvirus-6 in the Course of Multiple Sclerosis? - International Journal of MS Care, March Multiple Sclerosis Overview Multiple Sclerosis - Neurology Associates of Arlington Multiple Sclerosis - Praxis MD Multiple Sclerosis: Current Status and Strategies for the Future - National Academy Press, Multiple Sclerosis: Lectures - University of Utah, Houston (

98. CDC - Alexander The Great And West Nile Virus Encephalitis s of alexander’s illness do not include common disease signs (eg,rash, icterus, “thin blood,” vomiting, diarrhea or dysentery, hematuria...... http://www.cdc.gov/ncidod/eid/vol9no12/03-0288.htm

Past Issue Vol. 9, No. 12 December 2003 EID Home Ahead of Print Past Issues EID Search ... Table Historical Review Alexander the Great and West Nile Virus Encephalitis John S. Marr* and Charles H. Calisher† *Virginia Department of Health, Richmond, Virginia, USA; and Colorado State University, Fort Collins, Colorado, USA Suggested citation for this article: Marr JS, Calisher CH. Alexander the Great and West Nile virus encephalitis. Emerg Infect Dis [serial online] 2003 Dec [ date cited ]. Available from: URL: http://www.cdc.gov/ncidod/EID/vol9no12/03-0288.htm Alexander the Great died in Babylon in 323 BC. His death at age 32 followed a 2-week febrile illness. Speculated causes of death have included poisoning, assassination, and a number of infectious diseases. One incident, mentioned by Plutarch but not considered by previous investigators, may shed light on the cause of Alexander’s death. The incident, which occurred as he entered Babylon, involved a flock of ravens exhibiting unusual behavior and subsequently dying at his feet. The inexplicable behavior of ravens is reminiscent of avian illness and death weeks before the first human cases of West Nile virus infection were identified in the United States. We posit that Alexander may have died of West Nile virus encephalitis. Figure Click to view enlarged image Figure.

99. The Myelin Project: Demyelinating Diseases In Brief Refsum s disease, adrenoleukodystrophy, Krabbe s disease, phenylketonuria,Canavan disease, PelizaeusMerzbacher disease and alexander s disease. 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.

100. The Dylan Samuel Freeman Story Dylan has a very rare neurological disorder, Alexanders disease (Leukodystrophy). His story, his progress, and photographs. 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.

A  B  C  D  E  F  G  H  I  J  K  L  M  N  O  P  Q  R  S  T  U  V  W  X  Y  Z

Page 5     81-100 of 100    Back | 1  | 2  | 3  | 4  | 5