Sturge-Weber Syndrome
Original Editors - Marti Bradbury & Kayla Stull from Bellarmine University's Pathophysiology of Complex Patient Problems project.
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Definition/Description[edit | edit source]
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Prevalence[edit | edit source]
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Characteristics/Clinical Presentation[edit | edit source]
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Associated Co-morbidities[edit | edit source]
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Medications4[edit | edit source]
Medications used to treat Sturge-Weber Disease will vary greatly depending the presentation of symptoms in those with the disorder. Medical Intervention can include any of the following:
- Anticonvulsants work to discontinue electrical siezure activity quickly and reduce the likelihood of siezure reoccurance. Examples of these medications include Carbamazepine (Tegretol), Phenytoin (Dilantin), Valproic acid (Depakote, Depakene, Depacon), Gabapentin (Neurontin), Lamotrigine (Lamictal), as well as many others.
- Beta Blockers are used to help decrease intraoccular pressure by reducing the amount of aqueous humor produced in the eye. Aqueous humor is a plasma-like substance largely composed of protiens which help support and nourish occular tissue. Aqueous humor also assists in maintaining appropriate intraoccular pressure but in those with Sterge-Weber Syndrome this substance is overproduced. Levobunolol 0.25% or 0.5% (Betagan) is a beta blocker commonly used in Sterge-Weber Disease.
- Carbonic Anhydrase Inhibitors lower intraoccular pressure in much the same way that beta blockers do, by reducing production of aqueous humor. These drugs include Dorzolamide 2% (Trusopt) and Brinzolamide 1% (Azopt).
- Prostaglandin Analogues also work to lower intraoccular pressure, though instead of decreasing aqueous production directly they, instead, increase the outflow of the fluid away from the eye through the proper pathway. This pathway is known as the uveoscleral pathway and is located inferior to the eye. Latanoprost 0.005% (Xalatan) is a prostaglandin analogue used for this purpose.
- Topical Corticosteroids are used to treat occular. Prednisolone acetate 1% inhibits the edema, fibrin deposition,capillary dilation and phagocytic migration during the acute inflammatory response. Dexamethasone ophthalmic (Maxidex, Ozurdex) and Triamcinolone (Triesence) work by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.
- Antineoplastic Agents work by inhibiting DNA synthesis in order to decrease or stop cell growth and proliferation. Two examples of antineoplastic agents are Fluorouracil (Efudex) and Mitomycin.
Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]
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Etiology/Causes[edit | edit source]
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Systemic Involvement[edit | edit source]
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Medical Management (current best evidence)[edit | edit source]
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Physical Therapy Management (current best evidence)[edit | edit source]
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Alternative/Holistic Management (current best evidence)[edit | edit source]
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Differential Diagnosis[edit | edit source]
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Case Reports/ Case Studies[edit | edit source]
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Resources
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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3. Pascual-Castroviejo I, Pascual-Pascual S, Velazquez-Fragua R, Viaño J. Sterge-Weber Syndrome. Study of 55 patients. J of Can Neuro Sciences. 2008. 35(3); 301-307.
