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DOI: 10.1055/s-0029-1245994
© Georg Thieme Verlag KG Stuttgart · New York
Tiefe Hirnstimulation bei Morbus Parkinson mit Kamptokormie? Ein Fallbericht
Deep Brain Stimulation for Parkinson’s Disease and Camptocormia? A Case ReportPublication History
Publication Date:
20 January 2011 (online)
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Zusammenfassung
Die Kamptokormie stellt eine schwere Störung der Körperhaltung dar und findet sich gehäuft bei neurodegenerativen Erkrankungen wie Morbus Parkinson und Multisystematrophie. Pathophysiologisch werden bei der Parkinson-assoziierten Kamptokormie vor allem 2 Hypothesen diskutiert: 1. eine Störung der Basalganglien-Funktion, die zu einer Zunahme von Rigor und Dystonie der Rücken- und Bauchmuskulatur führt; 2. eine primäre Myopathie der autochthonen Rückenmuskulatur. Bei sehr begrenzten Therapiemöglichkeiten sind Berichte über Erfolge durch die Tiefe Hirnstimulation (THS) von großem Interesse, obwohl der Stellenwert dieser Therapie unklar ist. Wir berichten hier über eine Parkinsonpatientin, die wegen Fluktuationen und Dyskinesien zunächst erfolgreich mit einer THS des Nucleus subthalamicus behandelt wurde, bevor sie nach 6 Monaten eine von der THS unbeeinflusste, rasch progrediente Kamptokormie entwickelte, die klinische und elektrophysiologische Hinweise auf eine Myopathie der paravertebralen Rückenmuskulatur aufwies. Die widersprüchlichen Erfahrungen zur Wirkung der THS auf die Parkinson-assoziierte Kamptokormie weisen auf heterogene Pathomechanismen hin. Dabei scheint ein pathophysiologischer Subtyp mit Basalganglien-Funktionsstörung und Ansprechen der Kamptokormie auf die THS eher selten zu sein. Häufiger scheint dagegen ein myopathischer Typ zu sein, bei dem die THS ohne Wirkung ist. Eine Kamptokormie als führendes Symptom einer Parkinsonkrankheit stellt somit keine etablierte Indikation zur THS dar.
Abstract
Camptocormia is recognised as a severe postural movement disorder complicating neurodegenerative diseases like Parkinson’s disease (PD) and multisystem atrophy. Pathophysiologically two main hypotheses are discussed: (i) a disorder of the basal ganglia resulting in axial dystonia and rigidity on the one hand and (ii) an extensor truncal myopathy on the other hand. Therapeutic efforts often result in limited success. Therefore, reports on improvements by deep brain stimulation (DBS) are of great interest. However, the role of DBS in the treatment of camptocormia remains unclear. Here, we report a female PD patient who responded well to DBS of the subthalamic nucleus for severe dyskinesias and fluctuations. However, after 6 months she started to develop a rapidly progressing camptocormia which did not respond to DBS. The clinical and electrophysiological examination suggested a truncal erector myopathy. The inconsistent reports on the effects of DBS on camptocormia in PD patients suggest heterogeneous pathogenetic pathways. A pathophysiological subtype with predominant basal ganglia dysfunction and responsivity to DBS, however, seems to be rather rare. A myopathy, in contrast, seems to be more frequent and DBS is not effective in this condition. Therefore, camptocormia in PD patients is not an established indication for DBS.
Schlüsselwörter
Kamptokormie - Tiefe Hirnstimulation - Nucleus subthalamicus - Globus pallidus internus - Morbus Parkinson
Keywords
camptocormia - deep brain stimulation - subthalamic nucleus - globus pallidus internus - Parkinson’s disease
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Dr. Niels Allert
Neurologisches Rehabilitationszentrum Godeshöhe
Waldstraße 2 – 10
53177 Bonn
Email: allert@godeshoehe.de