HEMIFACIAL SPASM ASSOCIATED WITH MARFANS-SYNDROME - A CASE-REPORT

The authors describe a case of hemifacial spasm associated with Marfan 's syndrome. A 24-year-old-woman suffered from left hemifacial spasm f or 6 years. She had undergone surgical treatments for bilateral ectopi a lentis and kyphoscoliosis in the second decade of life. She also had unusually long and slim limbs with arachnodactyly. Three-dimensional CT angiography revealed bilateral tortuous and elongated vertebral art eries. Microvascular decompression was performed following a left late ral suboccipital craniotomy. The root exit zone of the left seventh ne rve was directly compressed by the proximal segment of the anterior in ferior cerebellar artery (AICA) and the left vertebral artery These ar teries were decompressed with pieces of Teflon cotton. The patient's s ymptoms completely resolved following surgery We review cerebrovascula r disorders in Marfan's syndrome and discuss the pathogenesis and poss ible mechanisms of vascular compression in these patients.