Optic Nerve Glioma: Glial cell derived tumors arising from the optic nerve, usually presenting in childhood.Neurofibromatosis 1: An autosomal dominant inherited disorder (with a high frequency of spontaneous mutations) that features developmental changes in the nervous system, muscles, bones, and skin, most notably in tissue derived from the embryonic NEURAL CREST. Multiple hyperpigmented skin lesions and subcutaneous tumors are the hallmark of this disease. Peripheral and central nervous system neoplasms occur frequently, especially OPTIC NERVE GLIOMA and NEUROFIBROSARCOMA. NF1 is caused by mutations which inactivate the NF1 gene (GENES, NEUROFIBROMATOSIS 1) on chromosome 17q. The incidence of learning disabilities is also elevated in this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1014-18) There is overlap of clinical features with NOONAN SYNDROME in a syndrome called neurofibromatosis-Noonan syndrome. Both the PTPN11 and NF1 gene products are involved in the SIGNAL TRANSDUCTION pathway of Ras (RAS PROTEINS).Optic Nerve: The 2nd cranial nerve which conveys visual information from the RETINA to the brain. The nerve carries the axons of the RETINAL GANGLION CELLS which sort at the OPTIC CHIASM and continue via the OPTIC TRACTS to the brain. The largest projection is to the lateral geniculate nuclei; other targets include the SUPERIOR COLLICULI and the SUPRACHIASMATIC NUCLEI. Though known as the second cranial nerve, it is considered part of the CENTRAL NERVOUS SYSTEM.Glioma: Benign and malignant central nervous system neoplasms derived from glial cells (i.e., astrocytes, oligodendrocytes, and ependymocytes). Astrocytes may give rise to astrocytomas (ASTROCYTOMA) or glioblastoma multiforme (see GLIOBLASTOMA). Oligodendrocytes give rise to oligodendrogliomas (OLIGODENDROGLIOMA) and ependymocytes may undergo transformation to become EPENDYMOMA; CHOROID PLEXUS NEOPLASMS; or colloid cysts of the third ventricle. (From Escourolle et al., Manual of Basic Neuropathology, 2nd ed, p21)Neurofibromin 1: A protein found most abundantly in the nervous system. Defects or deficiencies in this protein are associated with NEUROFIBROMATOSIS 1, Watson syndrome, and LEOPARD syndrome. Mutations in the gene (GENE, NEUROFIBROMATOSIS 1) affect two known functions: regulation of ras-GTPase and tumor suppression.Optic Nerve Neoplasms: Benign and malignant neoplasms that arise from the optic nerve or its sheath. OPTIC NERVE GLIOMA is the most common histologic type. Optic nerve neoplasms tend to cause unilateral visual loss and an afferent pupillary defect and may spread via neural pathways to the brain.Optic Disk: The portion of the optic nerve seen in the fundus with the ophthalmoscope. It is formed by the meeting of all the retinal ganglion cell axons as they enter the optic nerve.Neurocutaneous Syndromes: A group of disorders characterized by ectodermal-based malformations and neoplastic growths in the skin, nervous system, and other organs.Genes, Neurofibromatosis 1: Tumor suppressor genes located on the long arm of human chromosome 17 in the region 17q11.2. Mutation of these genes is thought to cause NEUROFIBROMATOSIS 1, Watson syndrome, and LEOPARD syndrome.Optic Neuritis: Inflammation of the optic nerve. Commonly associated conditions include autoimmune disorders such as MULTIPLE SCLEROSIS, infections, and granulomatous diseases. Clinical features include retro-orbital pain that is aggravated by eye movement, loss of color vision, and contrast sensitivity that may progress to severe visual loss, an afferent pupillary defect (Marcus-Gunn pupil), and in some instances optic disc hyperemia and swelling. Inflammation may occur in the portion of the nerve within the globe (neuropapillitis or anterior optic neuritis) or the portion behind the globe (retrobulbar neuritis or posterior optic neuritis).Optic Chiasm: The X-shaped structure formed by the meeting of the two optic nerves. At the optic chiasm the fibers from the medial part of each retina cross to project to the other side of the brain while the lateral retinal fibers continue on the same side. As a result each half of the brain receives information about the contralateral visual field from both eyes.Optic Atrophy: Atrophy of the optic disk which may be congenital or acquired. This condition indicates a deficiency in the number of nerve fibers which arise in the RETINA and converge to form the OPTIC DISK; OPTIC NERVE; OPTIC CHIASM; and optic tracts. GLAUCOMA; ISCHEMIA; inflammation, a chronic elevation of intracranial pressure, toxins, optic nerve compression, and inherited conditions (see OPTIC ATROPHIES, HEREDITARY) are relatively common causes of this condition.Optic Nerve Injuries: Injuries to the optic nerve induced by a trauma to the face or head. These may occur with closed or penetrating injuries. Relatively minor compression of the superior aspect of orbit may also result in trauma to the optic nerve. Clinical manifestations may include visual loss, PAPILLEDEMA, and an afferent pupillary defect.Optic Lobe, Nonmammalian: In invertebrate zoology, a lateral lobe of the FOREBRAIN in certain ARTHROPODS. In vertebrate zoology, either of the corpora bigemina of non-mammalian VERTEBRATES. (From McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1329)Optic Neuropathy, Ischemic: Ischemic injury to the OPTIC NERVE which usually affects the OPTIC DISK (optic neuropathy, anterior ischemic) and less frequently the retrobulbar portion of the nerve (optic neuropathy, posterior ischemic). The injury results from occlusion of arterial blood supply which may result from TEMPORAL ARTERITIS; ATHEROSCLEROSIS; COLLAGEN DISEASES; EMBOLISM; DIABETES MELLITUS; and other conditions. The disease primarily occurs in the sixth decade or later and presents with the sudden onset of painless and usually severe monocular visual loss. Anterior ischemic optic neuropathy also features optic disk edema with microhemorrhages. The optic disk appears normal in posterior ischemic optic neuropathy. (Glaser, Neuro-Ophthalmology, 2nd ed, p135)Optic Flow: The continuous visual field seen by a subject through space and time.Brain Neoplasms: Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.Optics and Photonics: A specialized field of physics and engineering involved in studying the behavior and properties of light and the technology of analyzing, generating, transmitting, and manipulating ELECTROMAGNETIC RADIATION in the visible, infrared, and ultraviolet range.Optic Atrophies, Hereditary: Hereditary conditions that feature progressive visual loss in association with optic atrophy. Relatively common forms include autosomal dominant optic atrophy (OPTIC ATROPHY, AUTOSOMAL DOMINANT) and Leber hereditary optic atrophy (OPTIC ATROPHY, HEREDITARY, LEBER).Optic Atrophy, Hereditary, Leber: A maternally linked genetic disorder that presents in mid-life as acute or subacute central vision loss leading to central scotoma and blindness. The disease has been associated with missense mutations in the mtDNA, in genes for Complex I, III, and IV polypeptides, that can act autonomously or in association with each other to cause the disease. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/Omim/, MIM#535000 (April 17, 2001))Retinal Ganglion Cells: Neurons of the innermost layer of the retina, the internal plexiform layer. They are of variable sizes and shapes, and their axons project via the OPTIC NERVE to the brain. A small subset of these cells act as photoreceptors with projections to the SUPRACHIASMATIC NUCLEUS, the center for regulating CIRCADIAN RHYTHM.Child Welfare: Organized efforts by communities or organizations to improve the health and well-being of the child.Mice, Inbred C57BLMice, Knockout: Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.Disabled Children: Children with mental or physical disabilities that interfere with usual activities of daily living and that may require accommodation or intervention.Child Behavior: Any observable response or action of a child from 24 months through 12 years of age. For neonates or children younger than 24 months, INFANT BEHAVIOR is available.Optic Atrophy, Autosomal Dominant: Dominant optic atrophy is a hereditary optic neuropathy causing decreased visual acuity, color vision deficits, a centrocecal scotoma, and optic nerve pallor (Hum. Genet. 1998; 102: 79-86). Mutations leading to this condition have been mapped to the OPA1 gene at chromosome 3q28-q29. OPA1 codes for a dynamin-related GTPase that localizes to mitochondria.Retina: The ten-layered nervous tissue membrane of the eye. It is continuous with the OPTIC NERVE and receives images of external objects and transmits visual impulses to the brain. Its outer surface is in contact with the CHOROID and the inner surface with the VITREOUS BODY. The outer-most layer is pigmented, whereas the inner nine layers are transparent.Optic Disk Drusen: Optic disk bodies composed primarily of acid mucopolysaccharides that may produce pseudopapilledema (elevation of the optic disk without associated INTRACRANIAL HYPERTENSION) and visual field deficits. Drusen may also occur in the retina (see RETINAL DRUSEN). (Miller et al., Clinical Neuro-Ophthalmology, 4th ed, p355)Papilledema: Swelling of the OPTIC DISK, usually in association with increased intracranial pressure, characterized by hyperemia, blurring of the disk margins, microhemorrhages, blind spot enlargement, and engorgement of retinal veins. Chronic papilledema may cause OPTIC ATROPHY and visual loss. (Miller et al., Clinical Neuro-Ophthalmology, 4th ed, p175)Superior Colliculi: The anterior pair of the quadrigeminal bodies which coordinate the general behavioral orienting responses to visual stimuli, such as whole-body turning, and reaching.Eye: The organ of sight constituting a pair of globular organs made up of a three-layered roughly spherical structure specialized for receiving and responding to light.Glioblastoma: A malignant form of astrocytoma histologically characterized by pleomorphism of cells, nuclear atypia, microhemorrhage, and necrosis. They may arise in any region of the central nervous system, with a predilection for the cerebral hemispheres, basal ganglia, and commissural pathways. Clinical presentation most frequently occurs in the fifth or sixth decade of life with focal neurologic signs or seizures.Astrocytoma: Neoplasms of the brain and spinal cord derived from glial cells which vary from histologically benign forms to highly anaplastic and malignant tumors. Fibrillary astrocytomas are the most common type and may be classified in order of increasing malignancy (grades I through IV). In the first two decades of life, astrocytomas tend to originate in the cerebellar hemispheres; in adults, they most frequently arise in the cerebrum and frequently undergo malignant transformation. (From Devita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2013-7; Holland et al., Cancer Medicine, 3d ed, p1082)Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.Fiber Optic Technology: The technology of transmitting light over long distances through strands of glass or other transparent material.Ophthalmoscopy: Examination of the interior of the eye with an ophthalmoscope.Visual Fields: The total area or space visible in a person's peripheral vision with the eye looking straightforward.Nerve Fibers: Slender processes of NEURONS, including the AXONS and their glial envelopes (MYELIN SHEATH). Nerve fibers conduct nerve impulses to and from the CENTRAL NERVOUS SYSTEM.Intraocular Pressure: The pressure of the fluids in the eye.Child Health Services: Organized services to provide health care for children.Visual Pathways: Set of cell bodies and nerve fibers conducting impulses from the eyes to the cerebral cortex. It includes the RETINA; OPTIC NERVE; optic tract; and geniculocalcarine tract.Child Rearing: The training or bringing-up of children by parents or parent-substitutes. It is used also for child rearing practices in different societies, at different economic levels, in different ethnic groups, etc. It differs from PARENTING in that in child rearing the emphasis is on the act of training or bringing up the child and the interaction between the parent and child, while parenting emphasizes the responsibility and qualities of exemplary behavior of the parent.Vision Disorders: Visual impairments limiting one or more of the basic functions of the eye: visual acuity, dark adaptation, color vision, or peripheral vision. These may result from EYE DISEASES; OPTIC NERVE DISEASES; VISUAL PATHWAY diseases; OCCIPITAL LOBE diseases; OCULAR MOTILITY DISORDERS; and other conditions (From Newell, Ophthalmology: Principles and Concepts, 7th ed, p132).Visual Acuity: Clarity or sharpness of OCULAR VISION or the ability of the eye to see fine details. Visual acuity depends on the functions of RETINA, neuronal transmission, and the interpretative ability of the brain. Normal visual acuity is expressed as 20/20 indicating that one can see at 20 feet what should normally be seen at that distance. Visual acuity can also be influenced by brightness, color, and contrast.Photography: Method of making images on a sensitized surface by exposure to light or other radiant energy.Coloboma: Congenital anomaly in which some of the structures of the eye are absent due to incomplete fusion of the fetal intraocular fissure during gestation.Child, Institutionalized: A child who is receiving long-term in-patient services or who resides in an institutional setting.Blogging: Using an INTERNET based personal journal which may consist of reflections, comments, and often hyperlinks.Online Systems: Systems where the input data enter the computer directly from the point of origin (usually a terminal or workstation) and/or in which output data are transmitted directly to that terminal point of origin. (Sippl, Computer Dictionary, 4th ed)Self-Help Groups: Organizations which provide an environment encouraging social interactions through group activities or individual relationships especially for the purpose of rehabilitating or supporting patients, individuals with common health problems, or the elderly. They include therapeutic social clubs.Internet: A loose confederation of computer communication networks around the world. The networks that make up the Internet are connected through several backbone networks. The Internet grew out of the US Government ARPAnet project and was designed to facilitate information exchange.Tumor Markers, Biological: Molecular products metabolized and secreted by neoplastic tissue and characterized biochemically in cells or body fluids. They are indicators of tumor stage and grade as well as useful for monitoring responses to treatment and predicting recurrence. Many chemical groups are represented including hormones, antigens, amino and nucleic acids, enzymes, polyamines, and specific cell membrane proteins and lipids.Urinary Bladder Neoplasms: Tumors or cancer of the URINARY BLADDER.
"Unusual Gliomas". Retrieved 12 September 2016. "Update on Treatment Modalities for Optic Nerve Sheath Meningiomas". Retrieved ... "About Children's Brain Tumor Foundation". Retrieved 31 August 2016. "Children's Brain Tumor Foundation". Retrieved 31 August ... "Molecular subclasses of high-grade glioma predict prognosis, delineate a pattern of disease progression, and resemble stages in ... "Gliomatosis Cerebri in a Child: Another Case". Retrieved 12 September 2016. Stout AP, Murray MR (1942). "Hemangiopericytoma: a ...
Gliomas of the optic chiasm are usually derived from astrocytes. These tumors are slow growing and more often found children. ... The optic chiasm is formed by the union of the two optic nerves. The nasal fibers of each optic nerve decussate (cross) across ... Clinically, no optic field deficits have been observed in a small series of optic nerve sections at the optic nerve-chiasm ... Posterior lesions may also involve the optic tract and cause a contralateral homonymous hemianopia. Optic disc pallor may be ...
Optic gliomas along one or both optic nerves or the optic chiasm can cause bulging of the eyes, involuntary eye movement, ... percent chance of passing the disorder on to their kids, but people can have a child born with NF-1 when they themselves do not ... MedlinePlus Encyclopedia Optic glioma Hyman SL, Shores A, North KN (October 2005). "The nature and frequency of cognitive ... Renal artery anomalies or pheochromocytoma and associated chronic hypertension Schwannoma Plexiform fibromas Optic glioma ...
... optic pathway gliomas), the preferred treatment is chemotherapy. However, radiotherapy isn't recommended in children who ... The severity of the condition of the parent does not affect the child; the affected child may have mild NF1 even though ... It is recommended that children diagnosed with NF1 at an early age have an examination each year, which allows any potential ... Neurofibromatosis (NF1) in early life may cause learning and behavior problems - about 60% of children who have NF1 have a mild ...
... and optic gliomas are among the most frequently encountered tumors in patients with this disorder. The majority of pilocytic ... Children Diagnosed can also suffer long term side effects due to the type of treatment they may receive. Grade I pilocytic ... Most affected are children ages 5-14 years. According to the National Cancer Institute more than 80% of astrocytomas located in ... Tumors of the optic pathway account for 3.6-6% of pediatric brain tumors, 60% of which are juvenile pilocytic astrocytomas. ...
It is usually caused by a brain tumor such as a low-grade glioma or astrocytoma located in the hypothalamic-optic chiasmatic ... The syndrome is a rare but potentially fatal cause of failure to thrive in children. Failure to thrive presents on average at 7 ... Arch Dis Child. 26: 274. doi:10.1136/adc.26.127.270. Drop SL, Guyda HJ, Colle E (August 1980). "Inappropriate growth hormone ... diencephalic syndrome of emaciation or Russell's syndrome is a rare neurological disorder seen in infants and children and ...
Optic glioma (a tumor on the optic nerve that affects vision) Freckles under the arms or in the groin 2 or more Lisch nodules ( ... Children with JMML and neurofibromatosis 1 (NF1) (about 14% of children with JMML are also clinically diagnosed with NF1, ... Relapse is a significant risk after HSCT for children with JMML. It is the greatest cause of death in JMML children who have ... will be performed on a child suspected of having JMML and throughout the treatment and recovery of a child diagnosed with JMML ...
In children under 2, about 70% of brain tumors are medulloblastomas, ependymomas, and low-grade gliomas. Less commonly, and ... A bilateral temporal visual field defect (due to compression of the optic chiasm) or dilation of the pupil, and the occurrence ... However, one type, focal brainstem gliomas in children, seems open to exceptional prognosis and long-term survival has ... In the UK, 429 children aged 14 and under are diagnosed with a brain tumour on average each year, and 563 children and young ...
... optic pathway gliomas and malignant peripheral nerve sheath tumors - are absent in this condition Symptoms however, may include ... "Benefits and harms of methylphenidate for children and adolescents with attention deficit hyperactivity disorder (ADHD) , ...
"Astroblastoma in a Child." Children Nervous System 24.2 (2008): 165-68. Sughrue, Michael E., Jay Choi, Martin Rutkowski, and ... of all brain gliomas since its discovery in 1926. Without a doubt, astroblastoma remains one of the most challenging and ... optic nerve, cauda equina, hypothalamus, and brain stem. The most defining physical symptom of astroblastoma, regardless of ... Most children can continue to lead productive, healthy lives after a low-grade astroblastoma is treated. Surviving the symptoms ...
In children under 2, about 70% of brain tumors are medulloblastomas, ependymomas, and low-grade gliomas. Less commonly, and ... A bilateral temporal visual field defect (due to compression of the optic chiasm) or dilation of the pupil, and the occurrence ... The most common brain tumor types in children (0-14) are: pilocytic astrocytoma, malignant glioma, medulloblastoma, neuronal ... They are 20-25 percent of cancers in children.[58][63]. The average survival rate for all primary brain cancers in children is ...
Isolated optic neuritis Focal seizure in adult A combination of IVMP, PLEX, and IVIg followed by oral prednisolone. Relapsed ... glioma inactivated protein and contactin-associated protein like, that are associated with voltage-gated potassium channels. ... encephalitis in children and adolescents". Annals of Neurology. 66 (1): 11-18. doi:10.1002/ana.21756. PMC 2826225 . PMID ... Journal of Child Neurology. 31 (3): 345-50. doi:10.1177/0883073815595079. PMID 26184485. Morvan, A (1890). "De la chorée ...
In standard MS are a result of inflammation of the optic nerve, known as optic neuritis. The effects of optic neuritis can be ... Distinctions Between Tumefactive Demyelination and Brain Tumors in Children". J Child Neurol. 29: 654-65. doi:10.1177/ ... The tumefactive lesion may mimic a malignant glioma or cerebral abscess causing complications during the diagnosis of ... Japanese optic-spinal MS: Is it MS or neuromyelitis optica and does the answer dictate treatment?". Neurology. 77 (2): 195-196 ...
J Neurosurg 1996;84:430-6. Bailey P, Cushing H. A classification of tumors of the glioma group on a histogenetic basis with a ... Revision of the World Health Organization classification of brain tumors for child hood brain tumors. Cancer 1985;56:1869-86. ... Medulloepithelioma of the ciliary body and optic nerve: clinicopathologic, CT, and MR imaging features. Neuroimaging Clin N Am ... 1998 Jan-Feb;14(1-2):74-8. Chidambaram B; Santosh V; Balasubramaniam V. Medulloepithelioma of the optic nerve with intradural ...
In children, Harley[5] reports typical causes as traumatic, neoplastic (most commonly brainstem glioma), as well as idiopathic ... Optic nerve. Optic disc. *Optic neuritis *optic papillitis. *Papilledema *Foster Kennedy syndrome ... In children, who rarely appreciate diplopia, the aim will be to maintain binocular vision and, thus, promote proper visual ... In children, differential diagnosis is more difficult because of the problems inherent in getting infants to cooperate with a ...
Armstrong was especially glad about this, as he had been prone to motion sickness as a child and could experience nausea after ... "Las Vegas Optic. Las Vegas, New Mexico. Associated Press. p. 6 - via Newspapers.com.. ... Karen was diagnosed with a diffuse intrinsic pontine glioma, a malignant tumor of the middle part of her brain stem.[35] X-ray ... The couple had three children: Eric, Karen, and Mark.[34] In June 1961, ...
... (LS) is an autosomal dominant condition characterized by cafe au lait spots. It was first described in 2007 and is often mistaken for neurofibromatosis type I (NF-1), it is caused by mutations in the SPRED1 gene, it is also known as Neurofibromatosis Type 1-like syndrome (NFLS). The condition is a RASopathy, developmental syndromes due to germline mutations in genes Nearly all individuals show multiple café au lait spots.Features common in neurofibromatosis - Lisch nodules, bone abnormalities, neurofibromas, optic pathway gliomas and malignant peripheral nerve sheath tumors - are absent in this condition Symptoms however, may include: Freckles Lipomas Macrocephaly Learning disabilities ADHD Developmental delay In terms of the genetics of Legius syndrome one finds the condition is autosomal dominant in regards to inheritance, and caused by mutations to the SPRED1 gene at chromosome 15, specifically 15q14 (or (GRCh38): 15:38,252,086-38,357,248) In terms ...
A malignant peripheral nerve sheath tumor (MPNST) (also known as "malignant schwannoma", "neurofibrosarcoma", and "neurosarcoma") is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors. The first-line treatment is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment. Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs-the brachial and lumbar plexuses-or from nerves as they arise from the trunk. ...
... (LS) is an autosomal dominant condition characterized by cafe au lait spots. It was first described in 2007 and is often mistaken for neurofibromatosis type I (NF-1), it is caused by mutations in the SPRED1 gene, it is also known as Neurofibromatosis Type 1-like syndrome (NFLS). The condition is a RASopathy, developmental syndromes due to germline mutations in genes Nearly all individuals show multiple café au lait spots.Features common in neurofibromatosis - Lisch nodules, bone abnormalities, neurofibromas, optic pathway gliomas and malignant peripheral nerve sheath tumors - are absent in this condition Symptoms however, may include: Freckles Lipomas Macrocephaly Learning disabilities ADHD Developmental delay In terms of the genetics of Legius syndrome one finds the condition is autosomal dominant in regards to inheritance, and caused by mutations to the SPRED1 gene at chromosome 15, specifically 15q14 (or (GRCh38): 15:38,252,086-38,357,248) In terms ...
... (NF) is a group of three conditions in which tumors grow in the nervous system. The three types are neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis. In NF1 symptoms include light brown spots on the skin, freckles in the armpit and groin, small bumps within nerves, and scoliosis. In NF2 there may be hearing loss, cataracts at a young age, balance problems, flesh colored skin flaps, and muscle wasting. The tumors are generally non cancerous. The cause is a genetic mutation in certain genes. In half of cases these are inherited from a person's parents while in the rest, they occur during early development. The tumors involve supporting cells in the nervous system rather than the neurons. In NF1 the tumors are neurofibromas (tumors of the peripheral nerves), while in NF2 and schwannomatosis tumors of Schwann cells are more common. Diagnosis is typically based on the signs and symptoms and occasionally supported by genetic testing. There is no ...
... + cells may be isolated from blood samples using immunomagnetic or immunofluorescent methods.. Antibodies are used to quantify and purify hematopoietic progenitor stem cells for research and for clinical bone marrow transplantation. However, counting CD34+ mononuclear cells may overestimate myeloid blasts in bone marrow smears due to hematogones (B lymphocyte precursors) and CD34+ megakaryocytes.. Cells observed as CD34+ and CD38- are of an undifferentiated, primitive form; i.e., they are multipotential hemopoietic stem cells. Thus, because of their CD34+ expression, such undifferentiated cells can be sorted out.. In tumors, CD34 is found in alveolar soft part sarcoma, preB-ALL (positive in 75%), AML (40%), AML-M7 (most), dermatofibrosarcoma protuberans, gastrointestinal stromal tumors, giant cell fibroblastoma, granulocytic sarcoma, Kaposi's sarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumors, mengingeal hemangiopericytomas, meningiomas, ...
... is an autosomal dominant condition characterized by Lisch nodules of the ocular iris, axillary/inguinal freckling, pulmonary valvular stenosis, relative macrocephaly, short stature, and neurofibromas. Watson syndrome is allelic to NF1, the same gene associated with neurofibromatosis type 1. Westerhof syndrome List of cutaneous conditions Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. pp. 725, 831. ISBN 1-4160-2999-0. Allanson JE, Upadhyaya M, Watson GH, et al. (November 1991). "Watson syndrome: is it a subtype of type 1 neurofibromatosis?" (PDF). J. Med. Genet. 28 (11): 752-6. doi:10.1136/jmg.28.11.752. PMC 1017110 . PMID 1770531 ...
A birthmark is a congenital, benign irregularity on the skin which is present at birth or appears shortly after birth, usually in the first month. They can occur anywhere on the skin. Birthmarks are caused by overgrowth of blood vessels, melanocytes, smooth muscle, fat, fibroblasts, or keratinocytes. Dermatologists divide birthmarks into two types. Pigmented birthmarks caused by excess skin pigment cells include moles, café au lait spots, and Mongolian spots. Vascular birthmarks, also called red birthmarks, are caused by increased blood vessels and include macular stains (salmon patches), hemangiomas, and Port-wine stains. A little over 1 in 10 babies have a vascular birthmark present by age 1. Several birthmark types are part of the group of skin lesions known as nevi or naevi, which means "birthmarks" in Latin. The exact cause of most birthmarks is unknown, but they are thought to occur as a result of a localized imbalance in factors controlling the development and migration of skin cells. In ...
6 mm, and evolution) are often used to distinguish nevi from melanomas in adults, while modified criteria (amelanosis, bleeding or bumps, uniform color, small diameter or de novo, and evolution) can be used when evaluating suspicious lesions in children. In addition to histopathological examination, some lesions may also warrant additional tests to aid in diagnosis, including special stains, immunohistochemistry, and electron microscopy. Typically; the nevi which exist since childhood are harmless A modern polarized dermatoscope A dermatoscope Hypermelanotic nevi must be differentiated from other types of pigmented skin lesions, including: Lentigo simplex Solar lentigo Café au lait macule Ink-spot lentigo Mucosal melanotic macule Mongolian spot (dermal melanocytosis) Cafe au lait Mongolian spot The management of a nevus depends on the specific diagnosis, however, the options for treatment generally include the following modalities: Chemical peels Cryotherapy Dermabrasion ...
Messiaen L, Yao S, Brems H, Callens T, Sathienkijkanchai A, Denayer E, Spencer E, Arn P, Babovic-Vuksanovic D, Bay C, Bobele G, Cohen BH, Escobar L, Eunpu D, Grebe T, Greenstein R, Hachen R, Irons M, Kronn D, Lemire E, Leppig K, Lim C, McDonald M, Narayanan V, Pearn A, Pedersen R, Powell B, Shapiro LR, Skidmore D, Tegay D, Thiese H, Zackai EH, Vijzelaar R, Taniguchi K, Ayada T, Okamoto F, Yoshimura A, Parret A, Korf B, Legius E (November 2009). "Clinical and mutational spectrum of neurofibromatosis type 1-like syndrome". JAMA. 302 (19): 2111-8. doi:10.1001/jama.2009.1663. PMID 19920235. Lay summary - Medscape ...
... s are similar in nature to the adult form of ependymoma in that they are thought to arise from radial glial cells lining the ventricular system. However, they differ from adult ependymomas in which genes and chromosomes are most often affected, the region of the brain they are most frequently found in, and the prognosis of the patients. Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), have been found to be more frequently afflicted with this class of tumors, but a firm genetic link remains to be established. Symptoms associated with the development of pediatric ependymomas are varied, much like symptoms for a number of other pediatric brain tumors including vomiting, headache, irritability, lethargy, and changes in gait. Although younger children and children with invasive tumor types generally experience less favorable outcomes, total removal of the tumors is the most conspicuous prognostic factor for both survival and ...
Barney Josephson, the founder of Café Society in Greenwich Village, New York's first integrated nightclub, heard the song and introduced it to Billie Holiday. Other reports say that Robert Gordon, who was directing Billie Holiday's show at Cafe Society, heard the song at Madison Square Garden and introduced it to her.[11] Holiday first performed the song at Café Society in 1939. She said that singing it made her fearful of retaliation but, because its imagery reminded her of her father, she continued to sing the piece, making it a regular part of her live performances.[14] Because of the power of the song, Josephson drew up some rules: Holiday would close with it; the waiters would stop all service in advance; the room would be in darkness except for a spotlight on Holiday's face; and there would be no encore.[11] During the musical introduction to the song, Holiday stood with her eyes closed, as if she were evoking a prayer. Holiday approached her recording label, Columbia, about the song, ...
... (originally Freebird Cafe) was a music venue located in Jacksonville Beach, Florida. They closed their doors with a final concert on January 21, 2016. The venue opened in September 8, 1999, with a concert by The Charlie Daniels Band. The cafe featured Lynyrd Skynyrd memorabilia, food and recorded music. In 2005, the owners of the venue considered moving the venue location to accommodate a larger capacity and big name acts. Buildings in Atlantic Beach, Florida and Jacksonville, Florida were considered. However, the owners decided to renovate its current location in lieu of moving. Circa Survive August Burns Red The Codetalkers Andrew McMahon Against Me! Karl Denson's Tiny Universe Galactic, the last show at Freebird Live Carter, John (September 3, 1999). "With Southern flare, Freebird Cafe plans to remember Lynyrd Skynyrd". The Florida Times-Union. Morris Communications. Retrieved December 28, 2013. Burmeister, Caren; Dixon, Drew (February 17, 2007). "Freebird decides it's staying ...
NF-κB作用机制。在此图中,将以Rel与p50蛋白组成的NF-κB异质二聚体为例。当处于激活状态时,NF-κB位于细胞质中且与抑制蛋白IκBα形成复合体。通过内在膜受体的介导,一些胞外信号物质可激活一种称为IκB激酶(IKK)的酶。IKK转而磷酸化IκBα蛋白,这将导致后者的泛素化,使得IκBα从NF-κB上脱离下来,最终IκBα被蛋白酶体所降解。被激活的NF-κB接下来转移到细胞核内,在这里会结合到DNA上被称为反应元件(RE)的特异性序列上。DNA/NF-κB 复合体接下来会招募其它蛋白,如辅激活物与RNA聚合酶,这些蛋白将下游的DNA转录为mRNA并转而被翻译为蛋白质,这些蛋白最终导致细胞功能发生改变[1][2][3] ...
... are an important subtype of pediatric gliomas. Gliomas that specifically arise from the optic pathway represent approximately 2 ... Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force. ... Optic pathway gliomas in children with and without neurofibromatosis 1. J Child Neurol. 2003; 18(7):471-478. ... Optic pathway gliomas (OPGs) are an important subtype of pediatric gliomas. Gliomas are the most common type of primary central ...
High-Dose Intravenous Vitamin C Treatment of a Child with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Case Report.Jay ... High-Dose Intravenous Vitamin C Treatment of a Child with Neurofibromatosis Type 1 and Optic Pathway Glioma: A Case Report.. ... In children, the most frequently identified tumor is the optic pathway glioma. ... We describe the case of a 5-year-old child who was diagnosed with NF1 and optic pathway tumor onset at the age of 14 months. ...
Only seven of the 24 non-optic glioma patients were symptomatic. Five of 24 patients had multiple non-optic gliomas. Four ... We identified 24 patients (4.3%) with non-optic gliomas. Median age at first scan with glioma was 21.2 years, much higher than ... The rate of progressing non-optic gliomas per patient year of follow-up in the first 5 years after tumour diagnosis was 4.7% ( ... regression and the natural history of non-optic gliomas in adults and compare these findings to the results found in children. ...
... glioma in children from Dana-Farber/Boston Childrens. View pediatric treatment options at our Brain Tumor Center. ... Optic Nerve (Pathway) Glioma in Children. An optic nerve glioma (also called an optic pathway glioma) is a slow-growing brain ... Optic Nerve Glioma Treatment at Dana-Farber/Boston Childrens. Children with optic nerve gliomas are treated at Dana-Farber/ ... How are optic nerve gliomas classified?. Optic nerve gliomas are a type of glioma, slow growing tumors that arise from glial ...
DESIGN This was a retrospective case notes analysis of 166 children with newly diagnosed OP/HSGs at our quaternary center ... CONTEXT Fifty percent of pediatric low-grade gliomas affect the optic pathway, hypothalamus, and suprasellar areas (OP/HSGs), ... Neuroendocrine Morbidity After Pediatric Optic Gliomas: A Longitudinal Analysis of 166 Children Over 30 Years.. @article{ ... Neuroendocrine Morbidity After Pediatric Optic Gliomas: A Longitudinal Analysis of 166 Children Over 30 Years.}, author={Hoong- ...
... childs age location of the tumour extent of vision loss or other symptoms ... Treatment for optic nerve and hypothalamic gliomas depends on the: ... Optic and hypothalamic gliomas. Optic and hypothalamic gliomas are both a type of low-grade glioma. They are usually benign, ... Treatment of optic and hypothalamic gliomas. The main goal of treatment is to save the childs sight and hormone functions. ...
... in children with optic pathway glioma (OPG) age ≥ 6 months to , 18 years, who receive combination antineoplaston therapy (ANP ... Optic pathway glioma, recurrent. Optic pathway glioma, progressive. Temozolomide. Temodar. TMZ. Antineoplastons. ANP therapy. ... Glioma. Optic Nerve Glioma. Neoplasms, Neuroepithelial. Neuroectodermal Tumors. Neoplasms, Germ Cell and Embryonal. Neoplasms ... Genetic and Rare Diseases Information Center resources: Glioma Optic Pathway Glioma Neuroepithelioma ...
Conclusions: Children with optic chiasm glioma may present endocrine disorders from the time of diagnosis of the tumor and, ... Endocrine Disorders in Children with Optic Chiasm Glioma. Alicia Torralbo-Carmona, Mar Domínguez-Begines, Laura Barchino-Muñoz ... Background: Pituitary function in children with optic chiasm glioma may be impaired. ... Method: Retrospective study using the records of patients under 14 years old followed for optic chiasm glioma. Sex, age at ...
... optic pathway glioma (OPG) is the most common optic pathway tumor. While many cases are found in young children, 20-40% are ... Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force. ... optic pathway glioma (OPG) is the most common optic pathway tumor. While many cases are found in young children, 20-40% are ... Natural history and outcome of optic pathway gliomas in children. Pediatr Blood Cancer. 2009;53(7):1231-7.PubMedCrossRefGoogle ...
NF-1 optic nerve glioma hypovision temporopolar arachnoid cyst fibroma cerebral hypertension chiasmatic-hypothalamic glioma ... Diagnosis: NF-1 and optic nerve glioma. To that end the most meaningful reports of the latest medical tests carried out are ... We would not be scared into starting chemotherapy just because the child has tortuous optic nerves or a mass at the chiasm, ... There is a widespread involvement of the optic nerves in intracranial and intraorbital areas with enlarged and tortuous ...
title = "Optic nerve and chiasm gliomas in children",. author = "Gaini, {S. M.} and G. Tomei and C. Arienta and M. Zavanone and ... Optic nerve and chiasm gliomas in children. / Gaini, S. M.; Tomei, G.; Arienta, C.; Zavanone, M.; Giovanelli, M.; Villani, R. ... Gaini SM, Tomei G, Arienta C, Zavanone M, Giovanelli M, Villani R. Optic nerve and chiasm gliomas in children. Journal of ... Gaini, SM, Tomei, G, Arienta, C, Zavanone, M, Giovanelli, M & Villani, R 1982, Optic nerve and chiasm gliomas in children, ...
Cook Childrens is among the nations finest when it comes to treating brain tumors. And we have access to some of the newest ... Optic nerve gliomas Optic nerve gliomas affect vision. The optic nerve sends signals to the brain about what the eye sees. A ... Optic nerve gliomas are usually treated with chemotherapy, although radiation also can be used. Most kids do well with ... Cook Childrens Locations. Cook Childrens provides a complete network of care to children across the state of Texas. ...
She visited another hospital and was told she had optic neuritis. Steroid pulse therapy was suggested... ... Long-term outcome in children with gliomas of the anterior visual pathway. Pediatr Neurol. 2003;28:262-70.CrossRefPubMedGoogle ... Neuroophthalmological management of optic pathway gliomas. Neurosurg Focus. 2007;23:E1.CrossRefPubMedGoogle Scholar ... Optic nerve glioma: an update. Int Ophthalmol. 2014;34(4):999-1005.CrossRefPubMedGoogle Scholar ...
6. Optic Nerve Glioma. As the name suggests, this type of brain tumor develops in the optic nerve pathways that occur between ... 1. Brain Stem Glioma. Commonly occurring in children aged 3 to 10 years, this tumor occurs in the base of the brain. This tumor ... This is the most commonly occurring among types of brain cancers in kids. It is a slow progressing disease and is restricted in ... This is a highly aggressive type of brain cancer in children and affects the central parts of the brain. Though surgery is a ...
F, Blindness caused by optic glioma. Note the irregular patterns of rest and activity in the 2 children with SOD and free- ... One patient with an optic glioma had nonentrained circadian phase.. Conclusions. A proportion of children with hypopituitarism ... Twenty children were studied, including children with septo-optic dysplasia (SOD), congenital hypopituitarism, brain tumors, ... Twenty children met criteria for eligibility. The cohort included 4 children with SOD and hypopituitarism, 7 children with ...
Optic gliomas. These tumors start in the optic nerves (the nerves leading from the eyes to the brain). They are often hard to ... Younger children may get chemotherapy instead of radiation. Radiation could then be used later if needed as the child gets ... Focal brain stem gliomas: A small number of brain stem gliomas are small tumors with very distinct edges (called focal brain ... But in children with neurofibromatosis type 1, these tumors often grow slowly (or even stop growing), so these children tend to ...
An optic pathway glioma. *Disease Status: All patients must have radiographically progressive low-grade glioma (including NF1 ... low-grade glioma. progressive. children. Participants studied have NF Type 1 and a low grade glioma that has either not ... In this research study, the investigators are looking to see the response of RAD001 in children with low-grade gliomas and NF1 ... Study of RAD001 (Everolimus) for Children With NF1 and Chemotherapy-Refractory Radiographic Progressive Low Grade Gliomas (NFC- ...
Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force. ... glial cells develop optic gliomas. Similar to human NF1-associated optic gliomas, the optic gliomas arising in Nf1+/−GFAPCKO ... as observed in children with NF1-associated optic gliomas ( 8, 36). Second, the optic gliomas are readily detectable using ... Therefore, to validate the Nf1+/−GFAPCKO optic glioma mouse model as a preclinical model of human NF1-associated optic glioma, ...
Optic nerve glioma in children].. Lena G, Pech-Gourg G, Scavarda D, Klein O, Paz-Paredes A. ... Thalamic gliomas in children: an extensive clinical, neuroradiological and pathological study of 14 cases. ... Search for distinctive markers in DNT and cortical grade II glioma in children: same clinicopathological and molecular entities ... Craniopharyngioma in children: MRI study of 43 cases].. Brunel H, Raybaud C, Peretti-Viton P, Lena G, Girard N, Paz-Paredes A, ...
Effect of bevacizumab in treatment of children with optic pathway glioma Effect of bevacizumab in treatment of children with ... [email protected]#To investigate the effect of bevacizumab in the treatment of children with optic pathway glioma (OPG)[email protected]*[email protected]#A ... Antineoplastic Combined Chemotherapy Protocols , Bevacizumab , Carboplatin , Child , Humans , Optic Nerve Glioma , ... Child / Retrospective Studies / Carboplatin / Optic Nerve Glioma / Bevacizumab Language: Chinese Journal: Chinese Journal of ...
"Unusual Gliomas". Retrieved 12 September 2016. "Update on Treatment Modalities for Optic Nerve Sheath Meningiomas". Retrieved ... "About Childrens Brain Tumor Foundation". Retrieved 31 August 2016. "Childrens Brain Tumor Foundation". Retrieved 31 August ... "Molecular subclasses of high-grade glioma predict prognosis, delineate a pattern of disease progression, and resemble stages in ... "Gliomatosis Cerebri in a Child: Another Case". Retrieved 12 September 2016. Stout AP, Murray MR (1942). "Hemangiopericytoma: a ...
... optic glioma explanation free. What is optic glioma? Meaning of optic glioma medical term. What does optic glioma mean? ... Looking for online definition of optic glioma in the Medical Dictionary? ... Thirteen children were found to have structural abnormalities, such as optic glioma, enlarged ventricles, enlarged sulci, and ... optic glioma. optic glioma. a slow-growing tumor on the optic nerve or in the chiasm, composed of glial cells. Symptoms may ...
Health Supervision for Children With Neurofibromatosis. Joseph H. Hersh, Committee on Genetics ...
Gliomas of the optic chiasm are usually derived from astrocytes. These tumors are slow growing and more often found children. ... The optic chiasm is formed by the union of the two optic nerves. The nasal fibers of each optic nerve decussate (cross) across ... Clinically, no optic field deficits have been observed in a small series of optic nerve sections at the optic nerve-chiasm ... Posterior lesions may also involve the optic tract and cause a contralateral homonymous hemianopia. Optic disc pallor may be ...
Gliomas are a cancer that grows in the brain or spinal cord. Learn more about the types, symptoms and treatment and where to ... Optic pathway gliomas: These are gliomas that grow in the optic nerve, which connects the brain with the eyes, or in the nerves ... Mayo Clinic (Glioma), Rare Cancers Australia (Brain stem glioma - child), Cancer Research UK (Gliomas in children), Cancer ... the optic nerves (the nerves that control eyesight). We dont know why some children develop gliomas. They are more common in ...