Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD-associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13-14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC.
We report on a patient with tetraspasticity due to perinatal cerebral palsy requiring total hip joint endoprosthesis because of hip dysplasia. In order to minimize the risk of postoperative luxation Botulinum Toxin A was injected preoperatively into hip flexor and adductor muscles guided by CT-fluoroscopy. Outcome measures included muscle tone, limb position and self-reported pain relief. Seven days post injections the tone of the right hip flexor and adductor muscles improved from three to one points on the five-point Modified Ashworth Scale (MAS), the spastic joint position improved from 45 • to 20 • in flexion and from 20 • to 10 • in adduction, and the patient was free of pain. Ten days after injection of Botulinum Toxin operation of total hip joint arthroplasty was performed without complication. Improvement of spasticity sustained for another eight weeks. Subsequent Botulinum Toxin A injection three months post surgery resulted in identical results. This case demonstrates a new preoperative indication for Botulinum Toxin A in patients with an increased muscle tone at the hip who have to undergo total hip joint endoprosthesis to reduce the risk of postoperative luxation.
3O r i g i n a l a r b e i t Zusammenfassung Der artikel spürt den subtilen Veränderungen nach, die bei Patienten, die mit tiefer Hirnstimulation behandelt werden, möglicherweise beobachtet werden können. Dabei sollen im rückgriff auf Konzeptionen zur narrativen identität mittels einer möglichst genauen beschreibung und analyse der Selbstwahrnehmung der Patienten sowie der Wahrnehmung ihres Umfelds die Änderungen im praktischen Selbstverhältnis untersucht werden, u. a. am beispiel technomorpher Metaphern, die von den Patienten in ihren Selbstbeschreibungen verwendet werden. Ziel ist es, die Neuartigkeit und das Spezifische der neurotechnologien -über die bisherigen Untersuchungen hinaus -hinsichtlich des Umgangs mit sich selbst herauszuarbeiten und greifbar zu machen. abschließend werden auf basis der analyse der Patienten-narrationen die entsprechenden normativen implikationen für eine ethische bewertung des einsatzes von neurotechnologien benannt.Schlüsselwörter tiefe Hirnstimulation · narrative identität · PatientenSelbstbeschreibung · neuroethik · technisierung · Phänomenologische anthropologie Narrative identity and therapy with 'brain pacemaker' Reflections on the integration of patients' self-descriptions in the ethical assessment of deep brain stimulation ethik Med (2010) 22:303-315 Der artikel entstand im rahmen des vom bMbF geförderten Freiburger bernstein Focus: neurotechnology (bFnt).O. Müller () · U. bittner institut für ethik und geschichte der Medizin an der albert-ludwigs-Abstract Definition of the problem the article goes into the matter of subtle changes, which can be noticed by patients being treated by deep brain stimulation. Arguments Thereby it is looked on alterations of the patients' practical self-relation by taking recourse to the conception of narrative identity. this shall be done via detailed description and analysis of both the patients' perception of themselves and of their environment, i.e. through the example of technomorph metaphors used by the patients in their self-descriptions. Conclusion The goal to achieve is to work out the novelty and the specifics of neurotechnologies-beyond current examinations-regarding the patients' behaviour towards themselves. Concluding normative implications for an ethical evaluation of neurotechnological usage are shown basing on the analysis of the patients' narrations.
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