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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1978-1990
Dr. Niels Allert
Neurologisches Rehabilitationszentrum Godeshöhe
Waldstraße 2 – 10
53177 Bonn
Email: allert@godeshoehe.de
