Sindrom Sturge Weber

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Sindrom Sturge Weber (SSW) atau disebut juga encephalofacialangiomatosis, merupakan
kelainan neurokutaneus yang ditandai dengan angioma leptomeningeal dan angioma
kutaneus pada kulit wajah (Port Wine stain), terutama khas pada daerah perjalanan
nervus trigeminalis yaitu nervus oftalmikus (V1) dan nervus maksilaris (V2). Penyakit
SSW disebabkan oleh anomali perkembangan bantalan vaskular pada stadium awal
vaskularisasi otak yang mengakibatkan kelainan pada otak. Di Amerika, angka kejadian
SSW diperkirakan sebesar 1 tiap 50.000. Kejadian SSW tidak dipengaruhi oleh ras dan
jenis kelamin. Kelainan neurologis dan perkembangan meliputi kejang, kelemahan, stroke,
sakit kepala, hemianopsi, retardasi mental, dan kelainan perkembangan. Diagnosis
ditegakkan berdasarkan manifestasi klinis yang ditemukan pada tiga organ yaitu nervus,
kulit dan mata, serta ditunjang oleh pemeriksaan pencitraan yaitu foto kepala, CT scan,
MRI, single photon emission computed tomografi (SPECT), dan EEG.


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Sindrom Sturge Weber

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Referensi


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DOI: http://dx.doi.org/10.14238/sp8.1.2006.69-74

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