Exploration of brain GABA levels alterations in patient with neurologic Wilson disease

Motivation: To better understand the effect of abnormal copper metabolism on the brain GABA level in patients with neurologic Wilson disease. Goal(s): To explore brain GABA level alterations in patients with neurologic Wilson disease compared to healthy controls. Approach: Five neurologic WD patients and five healthy controls underwent magnetic resonance spectroscopy (MRS) on a 3.0 scanner and GABA signal was acquired from left striatum and left thalamus using MEGA-PRESS. Results: The GABA level in the left striatum was significant reduced while in left thalamus it had no changes. Impact: This study suggests that the abnormality of GABA energy system may exist in the pathogenesis of neurologic WD, and adjusting neurotransmission of γ-aminobutyric acid may be an important target for neuroprotection in WD.