General anesthesia considerations in CADASIL disease

Abstract

CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy) disease is an inherited systemic arterial disease that affects the small and medium calibre cerebral vessels. Around 500 families are affected in the world, most of them in Europe. It is characterised by migraine attacks, subcortical dementia, neuropsychiatric disorders, and recurrent ischaemic strokes. The objective of this article is to describe, for the first time in the literature, the management by general anaesthesia of an intracranial neurosurgical procedure in a patient with CADASIL disease. Continuous monitoring of blood pressure is considered essential, as well as the maintenance of normocapnia and normothermia to avoid the development of new cerebrovascular accidents. This disease is relevant due to its anaesthetic implications and the few publications to date.