Management and Outcome of Pallidal Deep Brain Stimulation in Severe Huntington’s Disease

Zusammenfassung

Die tiefe Hirnstimulation (THS) hat die Behandlungmöglichkeiten bei M. Parkinson nachhaltig erweitert. Auch für andere neurodegenerative Erkrankungen wie Chorea Huntington wird die THS als Therapie-Option erprobt, vordergründig mit dem Ziel die beeinträchtigenden Bewegungsstörungen zu reduzieren. Wir berichten über den klinischen Verlauf einer Patientin mit fortgeschrittener Chorea Huntington Erkrankung unter THS des Globus pallidus internus. Dabei ließen sich positive Auswirkungen sowohl auf motorische Symptome als auch auf Kognition und Stimmung beobachten, die im Kontext der wenigen publizierten Daten zur THS bei Chorea Huntington diskutiert werden.

Die Abnahme der kognitiven Leistungsfähigkeit, die im fortgeschrittenen Stadium neurodegenerativer Erkrankungen und insbesondere bei Chorea Huntington typisch ist, impliziert ein Problem hinsichtlich der Einwilligungsfähigkeit der Patienten zur tiefen Hirnstimulation. Die damit einhergehenden ethischen und rechtlichen Aspekte werden diskutiert und diesbezügliche Lösungsansätze für kognitiv eingeschränkte Patienten im Allgemeinen diskutiert.

Abstract

Neurodegenerative movement disorders, such as Huntington’s disease (HD), have become a promising field for Deep Brain Stimulation (DBS). This study aims to contribute to the establishment of a well-grounded database including both expected and unexpected effects of pallidal DBS in HD, and to discuss the ethical and legal restrictions of DBS in cognitively limited patients. Evaluation of the outcome data indicates that pallidal DBS exerted an independent effect on motor symptoms but probably also on the patient’s cognitive and affective state. The cognitive decline, however, that characterizes the late stage of neurodegenerative disorders implicates ethical and legal problems given the patients’ inability to give informed consent to DBS.

Key words

Huntington - deep brain stimulation - globus pallidus internus - cognition - informed consent

Schlüsselwörter

Tiefe Hirnstimulation - Chorea Huntington - Globus pallidus internus - Kognitive Leistungsfähigkeit - Einwilligungsfähigkeit

^* These authors contributed equally to this work.

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