Avascular necrosis after long-term glucocorticoid treatment in MELAS: a cautionary note

Excerpt

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) are most commonly due to m.3243A > G mutation (El-Hattab et al 2015). Most patients have stroke-like episodes (SLEs), characterized by neurologic deficits not conforming to cerebral vascular territories (El-Hattab et al 2015). There is a consensus on L-arginine as a potent supplementation in mitochondrial stroke (Parikh et al 2017; El-Hattab et al 2015). Studies indicate that L-arginine increases nitric oxide production, resulting in improved blood flow, but further studies on potential efficacy are required (Parikh et al 2015; El-Hattab et al 2015). There is a controversy on corticosteroid use in SLEs. Several reports advocate corticosteroids for treatment, as corticosteroids could maintain blood-brain barrier and reduce vasogenic edema at SLEs. There are case reports advocating the positive effect of steroids in SLEs (Finsterer 2009; Walcott et al 2012; Fryer et al 2016). However, long-term steroid use is related to biphasic neurologic effects (Jing et al 2009) and diverse systemic complications including osteoporosis, hypertension, hyperglycemia, and avascular necrosis (AVN) (Weinstein 2012). …