Medisur (Oct 2022)

Sturger Weber syndrome. Clinical presentation and therapeutic management

  • Marileydis Almaguel García,
  • Irene Estefania Delgado Pérez,
  • José Sànchez Prieto

Journal volume & issue
Vol. 20, no. 5
pp. 976 – 982

Abstract

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Encephalotrigeminal angiomatosis is the most common of the neurocutaneous syndromes with a predominance of vascular abnormalities. It is a rare, but not exceptional, congenital process that affects both sexes equally. It consists, in its complete form, in the association of brain, skin and eye abnormalities. The case of a 1-year-old female patient, white skin, eutocic birth result, weighing 3660 g is presented. Physical examination confirmed patient with generalized hypotonia, delay in psychomotor development; with extensive bilateral facial hemangioma, right hemiparesis, tonic-clonic convulsive manifestation. Taking into account all these elements, the diagnosis of a Sturge-Weber syndrome is made. The early stimulation of language, the motor and cognitive part, the training in fine motor skills is the physiotherapeutic treatment that should start in an early stage. The objective of this paper is to point out the timeliness of treatment for a better prognosis and quality of life.

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