Prospective evaluation of Globus pallidus internus deep brain stimulation in Huntington's disease.

INTRODUCTION: Pharmacological treatment of chorea in Huntington's disease (HD) is often limited by poor efficacy or side effects. Pallidal deep brain stimulation (DBS) has been considered in these patients but experience is so far limited. METHODS: We prospectively evaluated the effects of bilateral DBS of the Globus pallidus internus (GPi) over one year in six severely affected HD patients with treatment refractory chorea in an advanced stage of the disease. Primary endpoint of the study was improvement in chorea. Additionally, we evaluated the effects of GPi DBS on the motor part of the Unified Huntington's Disease Rating Scale (UHDRS), bradykinesia, dystonia, functional impairment, psychiatric and cognitive symptoms. Side effects were systematically assessed. RESULTS: The chorea subscore was significantly reduced postoperatively (-47% six months, -40% twelve months postoperatively). The UHDRS total motor score was significantly reduced at six months postoperatively (- 17%) but the effect was not sustained twelve months after the operation (- 5%). Pallidal DBS did not improve other motor symptoms or functional impairment. There was no effect on psychiatric symptoms or cognition. A number of side effects were noted, especially spasticity in three of the patients. CONCLUSIONS: Pallidal DBS is a treatment option for HD patients with severe pharmacologically refractory chorea. Further studies are needed to define optimal candidates for this procedure.

Deficient recognition of emotional prosody in primary focal dystonia.

BACKGROUND: Primary dystonia is a movement disorder attributed mainly to basal ganglia dysfunction. Besides motor control, striatopallidal structures are known to implement also non-motor functions including processing of cognitive and emotional information. Previous research has already demonstrated deficient recognition of emotional faces in patients with primary focal dystonia. However, it remains elusive if emotional prosody processing in dystonia is also affected. METHODS: In this study, 30 patients with primary cervical dystonia (CD) and 30 healthy control subjects (HC) had to classify auditory presented words according to their emotional prosody (angry, happy, relaxed, sad). RESULTS: Analysis of hit rates and reaction times revealed a significantly poorer performance of patients with CD in judging angrily intonated words. Additional psychological assessment (SCL-90 R) demonstrated a higher level of psychological distress in patients with CD who displayed symptoms of somatization, anxiety and depression. CONCLUSIONS: Together these findings bring further insight into the basal ganglia involvement in processing of emotional prosody and emphasize the importance to identify the psychopathological symptoms in patients with CD as complementary to the motor deficit.