Publikation
Axial myopathy and parkinsonism
Präsentation/Vortrag - 01.07.2004
Kägi Georg, Weder Bruno
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We report 2 patients with parkinsonism
exhibiting pronounced forward flexion of the
trunk, called camptocormia or bent spine
syndrome. Age of the patients was 70 and
76 years, disease duration 6 and 12 years,
respectively. One patient was diagnosed with
multiple system atrophy. His parkinsonism
responded poorly to dopaminergic treatment.
After a two-year course posture deteriorated
severely, associated by impaired postural
reflexes and slight autonomic failure. The
second patient was diagnosed with Parkinson’s
disease and responded favourably to
dopaminergic treatment. She also developed
severe forward flexion of the trunk after a
six-year course. Temporarily, she suffered
from abdominal dystonia which could be controlled
with apomorphine infusion treatment.
The common feature, characterising the main
functional deficit in both patients, was the
inability to actively erect the trunk while
passive extension of the trunk was possible.
Electromyography of paraspinal muscles revealed
a myopathic pattern with high frequency,
spiky motor unit potentials of short
duration and abnormal spontaneous activity
consisting of fibrillations and positive sharp
waves.The nature of the observed association
between parkinsonism and chronic myopathy
confined to trunk extensor muscles is unclear.
Focal myopathy may reflect one factor beside
others causing abnormal posture in parkinsonism.