The Role of Frontal-Subcortical Circuitry in Neuropsychological Deficit of Attention: Hypothesis and Results in Two Coagulation Disorders

Coagulation disorders concern a deficiency of the body’s functional ability to regulate blood clotting (Peyvandi and Mannucci, 1999). Disorders in thisarea, which may be genetic or acquired, will result in hemostasis-related problems, including different clinical syndromes from easy bleeding or bruising (so-called “hemophilia”) to inappropriate thrombosis (so-called “thrombophilia”; Weisberg, 1996). Cognitive processes and neurologic disorders have been studied extensively in several disease contexts (Lucchiari et al., 2010; Oliveri et al., 2012; Smorti and Fioretti, 2015; Fioretti and Smorti, 2015). In the field of coagulation disorders, neuropsychological deficits have not been studied extensively, although they represent a frequent occurrence when the diseases involve the central nervous system (CNS) (Riva et al., 2014b, 2015a,b). In two previous published works of the first author, we studied the neuropsychological deficits in two cohorts of patients affected by rare coagulation disorders with significant impairments, especially in the domain of attention. The two disorders were hemophilia and thrombotic thrombocytopenic purpura (TTP).