ENFERMEDAD STURGE WEBER PDF

Sturge—Weber syndrome , sometimes referred to as encephalotrigeminal angiomatosis , is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma , seizures , intellectual disability , and ipsilateral leptomeningeal angioma cerebral malformations and tumors. Sturge—Weber syndrome can be classified into three different types. Type 1 includes facial and leptomeningeal angiomas as well as the possibility of glaucoma or choroidal lesions. Normally, only one side of the brain is affected. This type is the most common.

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Sturge-Weber syndrome: a case report on the results of the phonoaudiological evaluation. Evaluation of the drive and orofacial functions, semantic and pragmatic skills of oral language, perceptual visual and auditory processes, cognitive functions and test development of Denver II. RESULTS: in the assessment of orofacial drive the patient had abnormalities concerning bone structures of the face, inadequate as for the morphology, mobility and tone of all speech organs, presence of reflections of demand and sucking primitive.

In the assessment of skills and Semantic Pragmatic of oral language and Speech, perceptual processes visual and auditory, cognitive functions the patient had sensoriomotor exploitation, with little interaction and shared attention of the patient and difficulty of eye contact; predominantly gestural communication, with no records of verbal communicative acts.

The communicative functions of a request for action, exclamatory, exploratory and protest, and great use of non-focused light. Listening proved to be altered, representative gestures were sporadic, processes and perceptual functional laterality undefined. In the test development of Denver II, the patient failed in the areas: personal, social, language, motor fine and coarse.

As crises convulsivas constantes apareceram aos cinco meses de idade, mas foram controladas com uso de Fenobarbital. No exame intrabucal observaram hiperplasia gengival em ambos os casos. Este teste foi escolhido devido a sua grande aplicabilidade, sobretudo em estudos brasileiros De fato, cada caso pode exibir achados diferenciados 5 considerando a grande diversidade humana. Todos os itens avaliados encontram-se alterados. Ball K. History of Sturge-Weber syndrome.

Periodontal management of gingival enlargement associated with Sturge-Weber syndrome. J Periodontol. Rev Bras Patol Oral. Marinho AS, Cherubini K. Early single photon emission computed tomography in Sturge-Weber syndrome. J Neurol Neurosurg Psychiatr. Central hypothyroidism and Sturge-Weber syndrome. Pediatr Neurol. Ch'ng S, Tan ST. J Plast Reconstr Aesthet Surg. Brasil Dermat.

Late-onset epilepsy in a surgically-treated Sturge-Weber patient. Epileptic Disord. A case report]. Bull Soc Belge Ophtalmol. Wiedemann R. In: Kunze J, Dibbern H. Hamartoses P. In: Kenneth LJ. Sturge-Weber disease with repercussion on the prenatal development of the cerebral hemisphere.

Am J Neuroradiol. Mymensingh Med J. Onset of speech after left hemispherectomy in a nine-year-old boy. Sturge-Weber syndrome: deep venous occlusion and the radiologic spectrum. J Mal Vasc. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg. Sturge-Weber syndrome and dermatomal facial port-wine stains: incidence, association with glaucoma, and pulsed tunable dye laser treatment effectiveness. Plast Reconstr Surg. No Shinkei Geka. Neurol Surg. An Bras Dermatol.

Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci. Rev Neurol Paris. Anesthesia for encephalo-trigeminal angiomatosis Sturge-Weber syndrome. Middle East J Anesthesiol. Roach ES. Neurocutaneous syndromes. Pediatr Clin North Am. Prognosis in Sturge-Weber disease: comparison of unihemispheric and bihemispheric involvement. J Child Neurol. Abnormal hypermyelination in a neonate with Sturge-Weber syndrome demonstrated on diffusion-tensor imaging.

J Magn Reson Imaging. The corticospinal tract in Sturge-Weber syndrome: a diffusion tensor tractography study. Brain Dev. Comi AM. Advances in Sturge-Weber syndrome. Curr Opin Neurol. The Denver developmental screening test. J Pediatr ; 71 2 Marchesan IQ. In: Lopes Filho O. Tratado de fonoaudiologia. Fernandes FDM. Early diagnosis of abnormal development of preterm newborns: assessment instruments. J Pediatr. Manual Denver II.

In: Frankenburg WK, et al. Denver II screening manual. Alves NSG. All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Services on Demand Journal. How to cite this article.

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Sturge–Weber syndrome

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals.

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Sturge Weber Syndrome (Encephalotrigeminal Angiomatosis)

Sturge-Weber syndrome: a case report on the results of the phonoaudiological evaluation. Evaluation of the drive and orofacial functions, semantic and pragmatic skills of oral language, perceptual visual and auditory processes, cognitive functions and test development of Denver II. RESULTS: in the assessment of orofacial drive the patient had abnormalities concerning bone structures of the face, inadequate as for the morphology, mobility and tone of all speech organs, presence of reflections of demand and sucking primitive. In the assessment of skills and Semantic Pragmatic of oral language and Speech, perceptual processes visual and auditory, cognitive functions the patient had sensoriomotor exploitation, with little interaction and shared attention of the patient and difficulty of eye contact; predominantly gestural communication, with no records of verbal communicative acts. The communicative functions of a request for action, exclamatory, exploratory and protest, and great use of non-focused light.

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Sturge-Weber syndrome

It is part of a wide spectrum of possible phenotypes included in the craniofacial arteriovenous metameric syndrome CAMS. Sturge-Weber syndrome is a rare syndrome, with an incidence estimated at 1 case in 20,, persons The diagnosis is usually obvious on account of a congenital facial cutaneous hemangioma also known as port wine stain or facial nevus flammeus. Approximately a third of patients have choroidal or scleral angiomatous involvement, which may be complicated with retinal detachment, buphthalmos or glaucoma 1.

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