Charakterisierung der myopathologischen Veränderungen bei der Kamptokormie des Morbus Parkinson

Abstract

Introduction: This thesis paper presents a morphological evaluation of muscle biopsies of camptocormia-diseased Parkinson patients. Camptocormia is a severe debilitating illness caused by muscular weakness leading to postural deficiency. It is known to be associated with various underlying diseases, but is most often closed associated with Parkinson s disease (PD). Three to 17 % of PD patients develop camptocormia during their disease course .Aim: To characterize the myopathological pattern found in camptocormia- diseased muscles and elucidate the potential role of α-synuclein in the development of camptocormia.Material & methods: For the present study 14 biopsies of paraspinal muscles from PD patients with concomitant camptocormia were taken in order to reduce possible diversifying influences by different underlying diseases. The control group consisted of paraspinal muscle specimens from 10 autopsy cases that did not reveal any signs of neurodegenerative disease. Specimens of both groups were analyzed by histochemical and immunohistochemical staining procedures. The results were supplemented by investigation of semithin sections and electron microscopy. The detection of possible depositions of aggregated forms of α-synuclein within the muscle biopsies was performed using immunohistochemistry, PET blot ( Paraffin embedded tissue-blot ) and a modified variation of the PET blot, called tissue blot.Results: In the present study diagnostic criteria are defined that describe a myopathological pattern typical for a camptocormia. The major criteria were: Structural defects that were negative within the oxidative ferment stains and, that showed a fine granular reactivity in acid phosphatase reaction and presented a disruption of the striation pattern with z-band-streaming and formation of rod-like structures. Supportive minor criteria were: variation in fiber size, hypertrophy of type-I fibers, endomysial fibrosis, whorled fibers, increased numbers of internalized nuclei, atrophy and loss of type-II-fibers and nuclear clumps as signs of myopathic process. Aggregated forms of α-synuclein as a potential damaging factor were not found within the muscle tissue. There is no evidence for a myositis, mitochondriopathy or a myofibrillar myopathy.Summary: Criteria important for the diagnosis of a camptocormia were established.