Acute stroke can occur in CBS deficiency patients due to arterial thrombosis/embolism and carotid artery disease/dissection but it is usually due to cerebral venous sinus thrombosis (Mudd 1985; Hou and Wang 1994; Kelly et al 2003; Weiss et al 2006; Granel et al 2009). This evidence concerns the gene CBS and hyperinsulinemic hypoglycemia, familial, 4.