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.

The perioperative management of patients undergoing carotid endarterectomy (CEA), along with attempts to prevent its associated neurologic decline, continues to be a challenge to clinicians. Due to the marked changes in cerebral perfusion, there is a spectrum of neurologic sequelae directly attributable to this procedure, ranging from intraoperative stroke and death to more subtle perioperative neurocognitive deterioration. This chapter presents a case study of a 68-year-old male with demonstrated occlusive cerebrovascular disease with a history of a right- internal carotid artery (ICA) territory transient ischemic attack (TIA) 3 months previously. Blood pressure control has long been recognized as crucial during and after CEA to prevent critical hypo- or hyperperfusion states. The performance of CEA under local anesthesia has the potential to demonstrate immediate neurologic decline. Further investigation is warranted to ensure the best neurologic outcomes in patients undergoing this common procedure.