BBGD is an underdiagnosed pan-ethnic treatable condition. Clinicians caring for patients with unexplained encephalopathy and neuroimaging showing vasogenic edema in the bilateral putamen and caudate nuclei, infra- and supratentorial cortex, and brainstem should consider this disorder early in the hospital course because a therapeutic trial with biotin and thiamine can be lifesaving.

The error-related negativity (ERN) and feedback-related negativity (FRN) have been used as electrophysiological indices of performance monitoring produced in response to internally generated (errors) and externally generated (feedback) activations of the anterior cingulate cortex (ACC). No studies to date have systematically examined the measurement reliability of these components. In this article, we present the retest reliability of the ERN and FRN during response tasks designed to elicit errors or feedback responses on two occasions. Data from four experiments are presented in which participants performed tasks over various periods of time. Results indicate good retest reliability of the ERN and FRN amplitudes and source generation of these components. The present article provides important validation of the ERN and FRN as stable and trait-like electrophysiological reflections of performance monitoring and ACC functional integrity.