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2 results

  • Chapter 65 - Meningioma

  • from Section 3 - Symptomatic epilepsy
  • Edited by Simon D. Shorvon, Frederick Andermann, Renzo Guerrini
  • Book:
    The Causes of Epilepsy
    Published online:
    05 March 2012
    Print publication:
    14 April 2011, pp 454-458

  • Summary

    Disrupted formation of the corpus callosum is the second most common central nervous system birth defect, occurring in approximately 1 in 3000 live births. The incidence of epilepsy in patients with agenesis of the corpus callosum (ACC) and its related syndromes ranges from 25% to 62%. Aicardi syndrome is a presumed X-linked syndrome, as it is seen only in females and Klinefelter syndrome (XXY) males. The ARX gene is a homeobox gene that has been shown to be crucial in forebrain, pancreatic, and testicular development. Andermann syndrome, also known as peripheral neuropathy associated with agenesis of the corpus callosum (ACCPN). An autosomal dominant disorder, Mowat-Wilson, is caused by de novo mutations or deletions within the ZFHX1B (ZEB2) gene. Recent work in studying large cohorts of ACC individuals is providing insight into the diversity of causes which lead to ACC, which will provide important insight into mechanisms and outcomes.

  • 34 - ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY

  • from PART III: - VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 237-246

  • Summary

    Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder with a 95% penetrance at age 60 and incomplete expression that is characterized by the development of various benign and malignant tumors and cysts. Hemangioblastoma (HB), the most characteristic central nervous system (CNS) lesions, are highly vascular tumors comprising approximately 3% of all tumors of the CNS. HBs are diagnosed by magnetic resonance imaging (MRI) of the brain and the spinal cord. Presentation of HBs with a stroke syndrome is rare despite their highly vascular nature, and the risk of hemorrhage associated with them is not yet known. Screening for VHL disease following the diagnosis of a cerebral HB is indispensable given the high risk of associated lesions with hereditary forms. The best treatment for this tumor is surgical resection. If the tumor is difficult to remove from its primary site, it can sometimes be treated with radiosurgery.