Abstract

Prader–Willi syndrome (PWS) is a neurodevelopmental genetic disorder characterized by multiple system involvement with hypotonia, poor suck with feeding difficulties, growth and other hormone deficiencies, intellectual disability, and behavioral problems with childhood onset of hyperphagia resulting in obesity, if not externally controlled. Transcranial direct current stimulation (tDCS) has been increasingly shown to modulate cognitive and behavioral processes in children and adults, including food-intake behaviors in patients with PWS. This study further reports the positive effects of brief tDCS sessions on Go/NoGo task performance involving food and non-food stimuli images, alterations in N2 brain amplitude, and genetic subgroup differences (maternal disomy 15, UPD; 15q11-q13 deletion, DEL) before and after tDCS as assessed by event-related potentials (ERPs) in 10 adults with PWS. The results indicate a group effect on baseline NoGo N2 amplitude in PWS patients with DEL vs UPD (p =0.046) and a decrease in NoGo N2 amplitude following tDCS (p = 0.031). Our tDCS approach also demonstrated a trend towards decreased response time. Collectively, these results replicate and expand prior work highlighting neurophysiological differences in patients with PWS according to genetic subtype and demonstrate the feasibility in examining neuromodulatory effects of tDCS on information processing in this patient population to stimulate additional research and treatment.

Highlights

  • Introduction published maps and institutional affilPrader–Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder affecting multiple systems, leading to life-threatening obesity in childhood if not externally controlled

  • Patients with PWS have a rare neurodevelopmental genetic disorder affecting multiple systems which can lead to life-threatening obesity in childhood, if not treated [1,2,3,4]. transcranial direct current stimulation (tDCS)

  • It represents a proof of concept that targeted modulation of dysfunctional brain regions associated with attention and response inhibition may be associated with hyperphagia behavior in PWS

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Summary

Introduction

Prader–Willi syndrome (PWS) is a rare neurodevelopmental genetic disorder affecting multiple systems, leading to life-threatening obesity in childhood if not externally controlled. The syndrome is further characterized by short stature; small hands and feet; hypogonadism/hypogenitalism; mild cognitive problems; and behavioral disturbances, including intrusive thinking patterns, outbursts, anxiety, self-injury, and risk for autism and psychosis in late adolescence and early adulthood [1,2,3,4]. PWS patients develop a pattern of food-seeking leading to hyperphagia and subsequent obesity without strict caloric restriction and food security programs in place. These behaviors include eating of nonfood, inedible items [1,4,6]

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