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Topic: Neurocutaneous syndrome


In the News (Tue 22 Dec 09)

  
  Neurocutaneous Syndrome (NCS)
By Dr. Omar M. Amin, Ph.D. Abstract: Neurocutaneous Syndrome (NCS) is a dental toxicity disorder causing various neurological and dermatological symptoms that are often confused by the lay patient as parasitological infections.
Neurocutaneous Syndrome (NCS), a newly discovered dental toxicity syndrome (Amin, 2001) is characterized by neurological and dermatological disorders as well as systemic and related dysfunctions.
Amin, O.M. Dental sealant toxicity: Neurocutaneous Syndrome (NCS), a dermatological and neurological disorder.
members.cox.net /llyee/ncs_diagnosis.htm   (2247 words)

  
 Neurocutaneous Syndromes
Neurocutaneous syndromes are genetic disorders that lead to abnormal growth of tumors in various parts of the body.
It is caused by a spontaneous genetic mutation.
The illnesses associated with neurocutaneous syndromes can place enormous stress and emotional burdens on you and your child, and it is easy to feel overwhelmed.
www.kidshealth.org /parent/system/ill/neurocutaneous.html   (1931 words)

  
  Neurocutaneous Syndromes
Neurocutaneous syndromes are genetic disorders that lead to abnormal growth of tumors in various parts of the body.
Although there is no cure for any of the neurocutaneous syndromes, treatments are available to help a child manage the symptoms and any health problems that they can cause.
It is caused by a spontaneous genetic mutation.
kidshealth.org /parent/system/ill/neurocutaneous.html   (1941 words)

  
 Health Tips - Neurological Disorders - Neurocutaneous Syndromes
Neurocutaneous syndrome is a broad term for a group of rare neurological (brain, spine, and peripheral nerve) disorders.
Since neurocutaneous syndromes are life-long conditions that are not curable, the focus is on medically managing the symptoms.
Life-long considerations for a child with neurocutaneous syndromes:
www.driscollchildrens.org /health/peds/neuro/neurocut.asp   (1286 words)

  
 Von Hippel-Lindau syndrome - Genetics Home Reference
Von Hippel-Lindau syndrome is an inherited disorder characterized by the formation of tumors and fluid-filled sacs (cysts) in many different parts of the body.
Mutations in the VHL gene cause von Hippel-Lindau syndrome.
Mutations in the VHL gene are inherited in an autosomal dominant pattern, which means that one copy of the altered gene in each cell is sufficient to increase the risk of developing tumors and cysts.
ghr.nlm.nih.gov /condition=vonhippellindausyndrome   (894 words)

  
 Guillain-Barr Syndrome
Guillain-Barré syndrome is a neurological disorder in which the body's immune system attacks part of the peripheral nervous system.
Guillain-Barré is called a syndrome rather than a disease because it is not clear that a specific disease-causing agent is involved.
A syndrome is a medical condition characterized by a collection of symptoms (what the patient feels) and signs (what a physician can observe or measure).
medicalcenter.osu.edu /patientcare/healthcare_services/nervous_system/gbs   (798 words)

  
 Korean Journal of Radiology
Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system.
Neurocutaneous melanosis (NCM) is a rare congenital nonheritable neurocutaneous syndrome that is characterized by large or multiple pigmented nevi along with leptomeningeal melanosis or melanoma; there is usually no evidence of malignant melanoma outside the central nervous system (1).
Neurocutaneous melanosis may be associated with other neurocutaneous syndromes such as Sturge-Weber or von Recklinghausen's disease.
www.kjronline.org /abstract/view_articletext.asp?year=2006&page=145   (1758 words)

  
 Article Summary: On the Diagnosis and Management of Neurocutaneous Syndrome (NCS),A Toxicity Disorder from Dental ...
Neurocutaneous syndrome (NCS), a newly discovered toxicity disorder, is characterized by neurological sensations, pain, depleted energy and memory loss as well as itchy cutaneous lesions which may invite various opportunistic infections.
Components in the calcium hydroxide dental sealants Dycal, Life and Sealapex have been identified as sources of the observed symptoms.
The original description of the neurocutaneous syndrome (NCS) was “introductory in nature.” Examination of many NCS patients and a careful study of their symptoms, exposures, clinical conditions and histories made it possible to identify the underlying cause of the syndrome and proceed with its management.
explorepub.com /articles/summaries/12_6_amin.html   (308 words)

  
 Neurocutaneous Syndromes   (Site not responding. Last check: 2007-10-18)
Neurocutaneous syndromes is a term that refers to a group of genetic neurological disorders.
Neurocutaneous syndrome is a broad term for a group of...
Neurocutaneous syndromes is a term that refers to a group of genetic neurological (affecting...
www.health-nexus.com /neurocutaneous_syndromes.htm   (282 words)

  
 Neurocutaneous Syndrome (NCS)
By Dr. Omar M. Amin, Ph.D. Abstract: Neurocutaneous Syndrome (NCS) is a dental toxicity disorder causing various neurological and dermatological symptoms that are often confused by the lay patient as parasitological infections.
Neurocutaneous Syndrome (NCS), a newly discovered dental toxicity syndrome (Amin, 2001) is characterized by neurological and dermatological disorders as well as systemic and related dysfunctions.
Amin, O.M. Dental sealant toxicity: Neurocutaneous Syndrome (NCS), a dermatological and neurological disorder.
www.myplanet.net /yeec60bp/ncs_diagnosis.htm   (2260 words)

  
 Driscoll
Klippel-Trenaunay Syndrome (KTS) consists of a triad of cutaneous capillary hemangioma, bone and soft tissue hypertrophy, and venous varicosities (1).
The etiology of KTS is unknown but some authors have suggested that it results from a mesodermal abnormality that occurs during fetal development leading to the maintenance of microscopic arteriovenous communications in the limb bud (2).
In Parks-Weber Syndrome there are clinically apparent and important arteriovenous fistulae where as in KTS any arteriovenous fistulae that exist are microscopic in size and unassociated with the typical clinical findings of arteriovenous fistulae.
www.k-t.org /driscoll.html   (2153 words)

  
 Syndrome - Kosmix Topic Page - Medical Information for Syndrome
The exact cause of Munchausen syndrome is not known, but researchers believe both biological and psychological factors play a role in the...
The syndrome is closely associated with a generalized metabolic disorder called...
The Proteus Syndrome Foundation has been founded to support and educate familes and professionals, and to raise money for research to find a cure...
www.kosmix.com /search?q=Syndrome&c=health.procedures:plasmapheresis&o=t&t=vhealth.all   (681 words)

  
 Radiological Society of New Jersey - Education - Unknown Case of the Month #43   (Site not responding. Last check: 2007-10-18)
Sturge-Weber-Dimitri syndrome or encephalotrigeminal angiomatosis is a neurocutaneous syndrome seen in children that is characterized clinically by a facial port-wine nevus, mental retardation (70%), seizures, contralateral hemiparesis (50%) and buphthalmos (congenital glaucoma).
Pathologically, the disorder consists of cutaneous facial hemangiomas, involving at least the distribution of the ophthalmic branch of the trigeminal nerve and ipsilateral leptomeningeal angiomatosis, with bilateral involvement occurring in up to 30% of cases.
syndrome: comparison of the efficacy of CT and MR imaging in 14 cases.
www.rsnj.org /answercase43.htm   (214 words)

  
 Nervous System Disorders - Neurocutaneous Syndromes
Neurocutaneous syndrome is a broad term for a group of neurologic (brain, spine, and peripheral nerve) disorders.
The symptoms of neurocutaneous syndromes may resemble other conditions or medical problems.
Since tuberous sclerosis, NF, and Sturge-Weber disease are life-long conditions that are not correctable, management includes focusing on preventing or minimizing deformities and maximizing the person's capabilities at home and in the community.
healthcare.utah.edu /healthinfo/adult/Neuro/neurocut.htm   (1271 words)

  
 Health Information - Yale Medical Group
Neurocutaneous syndrome is a broad term for a group of rare neurological (brain, spine, and peripheral nerve) disorders.
Life-long considerations for a child with neurocutaneous syndromes:
Since tuberous sclerosis, NF, and Sturge-Weber disease are life-long conditions that are not correctable, management includes focusing on preventing or minimizing deformities and maximizing the child's capabilities at home and in the community.
ymghealthinfo.org /content.asp?pageid=P02614   (1333 words)

  
 Sturge-Weber Syndrome - WrongDiagnosis.com
Sturge-Weber syndrome (also called "encephalotrigeminal angiomatosis") is a congenital disorder characterized by a vascular birthmark and neurological abnormalities.
A group of neurocutaneous disorders manifested by facial and leptomeningeal angiomas, ipsilateral gyriform calcifications of the cerebral cortex, seizures, development delay, hemiplegia, emotional and behavioral problems, and glaucoma and other ocular disorders.
Correlation between the distribution of the nevus and the course of the trigeminal nerve is responsible for naming the syndrome "trigemino-encephalo-angiomatosis," but later findings found the relationship to be fortuitous.
www.wrongdiagnosis.com /s/sturge_weber_syndrome/intro.htm   (710 words)

  
 MedlinePlus Medical Encyclopedia: Sturge-Weber syndrome - legs
Sturge-Weber syndrome is a disease that affects the skin and nervous system (neurocutaneous) and is associated with Port Wine Stain, red vascular markings on the face and other parts of the body (shown here on the legs).
This is an unusual case, due to the large size of the lesion (extensive involvement).
Occasionally seizures or learning disorders are also associated with Sturge-Weber syndrome.
www.nlm.nih.gov /medlineplus/ency/imagepages/2536.htm   (187 words)

  
 A case of encephalocraniocutaneous lipomatosis -- Chittenden et al. 86 (8): 934 -- British Journal of Ophthalmology
ECCL is a rare neurocutaneous syndrome of unknown aetiology,
syndrome, the linear naevus sebaceous syndrome, or ECCL and
A new syndrome of autosomal dominantly inherited, bilateral, annular limbal dermoids with corneal and conjunctival extension.
bjo.bmjjournals.com /cgi/content/full/86/8/934   (656 words)

  
 Gillette Childrens Specialty Healthcare
Because neurocutaneous syndromes are complex, Gillette Children’s Specialty Healthcare and Children’s Hospitals and Clinics of Minnesota have formed the Neurocutaneous Syndromes Clinic Without Walls to help patients and their families manage these conditions (see page 4 for information on the clinic).
This article focuses on the most common neurocutaneous syndromes that affect children and adolescents and on the treatments for such conditions.
The Neurocutaneous Syndromes Clinic Without Walls is a collaboration between Children’s Hospitals and Clinics of Minnesota and Gillette Children’s Specialty Healthcare.
www.gillettechildrens.org /default.cfm?PID=1.7.19.6.10   (3393 words)

  
 proceed
Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of arteriovenous or capillary vascular malformations, atypical varicosities, and bony or soft tissue hypertrophy usually affecting one extremity.
In the other, a 6-year-old boy, the internal jugular vein and sigmoid sinus on the affected side were absent, and he had macrocephaly and an abnormally shaped skull with thin, dysplastic diploic spaces.
One patient had a coexisting prolonged QT syndrome, now localized to defects of chromosomes 3,7 and 11.18 The other patient had a balanced translocation of chromosomes 8 and 14 (8;14)(q22.3 q 13).
www.k-t.org /proceed.html   (3406 words)

  
 Encephalocraniocutaneous lipomatosis: A rare neurocutaneous syndrome Gokhale NR, Mahajan PM, Belgaumkar VA, Pradhan SN, ...
ECCL or Fishman syndrome is a rare neurocutaneous syndrome of unknown cause.
In all but two cases in the literature,[9],[11] lipomas and neurocutaneous findings were limited to the head and central nervous system.
ECCL is an important syndrome for the dermatologist to recognize on encountering intracranial and cutaneous lipomas in a patient with epibulbar choristomas and CNS abnormalities.
www.ijdvl.com /article.asp?issn=0378-6323;year=2007;volume=73;issue=1;spage=40;epage=42;aulast=Gokhale   (1482 words)

  
 Neurofibromatosis
Similar skin findings are found in Oliers's syndrome, Albright's disease, and Jaffe Campanacci syndrome - just to name a few.
At one time it was fashionable to use terms like phacodermatoses or neurocutaneous disorder.
So the common correlation of badly migrated neural paths or cells or whatever and oddball left behind color patches on skin give one pause to consider that the pathology - may vey well include migration defects - regardless of the actual chemistry.
www.pediatric-orthopedics.com /Topics/Muscle_Neuro/Diseases/Neurofibromatosis/neurofibromatosis.html   (870 words)

  
 Toxicity from calcium hydroxide sealers causing neurocutaneous syndrome (NCS), a dermatological and neurological ...
Toxicity from calcium hydroxide sealers causing neurocutaneous syndrome (NCS), a dermatological and neurological disorder
Neurocutaneous syndrome (NCS), a newly discovered toxicity disorder, is characterized by neurological sensations, pain, depleted energy and memory loss as well as itchy cutaneous lesions which may invite various opportunistic infections.
Examination of many NCS patients and a careful study of their symptoms, exposures, clinical conditions and histories made it possible to identify the underlying cause of the syndrome and proceed with its management.
members.cox.net /llyee/NCS_revC.html   (3152 words)

  
 eMedicine - Haberland Syndrome : Article Excerpt by: Sergiusz Jozwiak, MD, PhD
Background: Encephalocraniocutaneous lipomatosis (ECCL) is a rare, congenital, neurocutaneous disorder with unilateral lipomatous cutaneous neoplasms devoid of hair and ipsilateral ophthalmologic and neurologic malformations.
Haberland and Perou speculated as to whether the ectodermal malformations are caused by the same basic defect responsible for the mesenchymal malformations or whether they were secondary to the mesodermal dysgenesis.
Happle and Steijlen suggested that the origin of this nonhereditary genodermatosis could be a lethal autosomal mutation that survives in a mosaic state.
www.emedicine.com /derm/byname/haberland-syndrome.htm   (353 words)

  
 Schimmelpenning-Feuerstein-Mims syndrome (www.whonamedit.com)
Associated features may be mental retardation, focal epilepsy, spastic hemiparesis, defects of the aorta, lesions of the ribs, cleft palate; ocular lesions, including coloboma of the iris and retina and pipodermoids of the conjunctiva and cornea, and corneal opacity.
Schimmelpenning described the syndrome in 1957 when he worked as an assistant in the department of neurology and psychiatry at Münster.
A new neurocutaneous syndrome (skin, eye, brain) consisting of linear nevus, bilateral lipodermoids of the conjunctiva, cranial thickening, cerebral cortical atrophy, and mental retardation.
www.whonamedit.com /synd.cfm/2171.html   (350 words)

  
 CAT.INIST
Objective Cutaneous hemangiomas and vascular malformations constitute a systemic disease that is the most frequent of the neurocutaneous syndromes reported to date.
The aimof this study was to describe the characteristics of this new neurocutaneous syndrome.
This syndrome is associated with numerous disorders that can be located in the areas underlying the cutaneous vascular lesions anywhere on the body.
cat.inist.fr /?aModele=afficheN&cpsidt=14721283   (352 words)

  
 Recognizing an Index Case of Tuberous Sclerosis American Family Physician - Find Articles
Dermatologic manifestations may be the only clues the family physician has to the diagnosis of the disorder, which is also marked by childhood seizures and mental retardation.
The term "tuberous sclerosis" was first used in 1880 to describe a syndrome consisting of seizures, mental retardation and facial rash in a young girl.
However, this complex disorder was initially identified in 1862, and an association between seizures, mental retardation and facial angiofibromas (adenoma sebaceum), referred to as "Vogt's triad," was established in 1908.1 With recent advances in genetic research and imaging modalities, more mysteries about this fascinating disorder have been solved.
findarticles.com /p/articles/mi_m3225/is_3_61/ai_59480946?lstpn=article_results&lstpc=search&lstpr=external&lstprs=other&lstwid=1&lstwn=search_results&lstwp=body_middle   (858 words)

  
 Sturge-Weber syndrome diagnosed in a 45-year-old man -- Hussain et al. 170 (11): 1672 -- Canadian Medical Association ...
Sturge-Weber syndrome diagnosed in a 45-year-old man -- Hussain et al.
covered by a port-wine nevus; only 1 was found to have the syndrome.
Sturge–Weber syndrome: indications and results of surgery in 20 patients.
www.cmaj.ca /cgi/content/full/170/11/1672   (521 words)

  
 Neurocutaneous Disorders
Neonates with a subcutaneous fatty pad, angiomatous patch, a hairy tuft, or a dermal sinus in the lumbosacral region should have ultrasound or MRI of this area.
Neonates with angiomatous patch may have Cobb syndrome.
Cobb syndrome consists of the association of cutaneous angiomatosis and angiomatosis of the spinal cord, the adjacent meninges, or both (Figure 307.2).
pediatricneuro.com /alfonso/pg307.htm   (178 words)

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