Parkinson’s disease with camptocormia
01 Nov 2006-Journal of Neurology, Neurosurgery, and Psychiatry (J Neurol Neurosurg Psychiatry)-Vol. 77, Iss: 11, pp 1223-1228
TL;DR: Patients with camptocormia were characterised by prominent levodopa-unresponsive axial symptoms (ie, axial rigidity, gait disorder and postural instability), along with a tendency for greater error in the antisaccade paradigm, and it is suggested that the salient features of parkinsonism observed in patients with camps are likely to represent a specific form of Parkinson’s disease.
Abstract: Background: Camptocormia is defined as an abnormal flexion of the trunk that appears when standing or walking and disappears in the supine position. The origin of the disorder is unknown, but it is usually attributed either to a primary or a secondary paravertebral muscle myopathy or a motor neurone disorder. Camptocormia is also observed in a minority of patients with parkinsonism. Objective: To characterise the clinical and electrophysiological features of camptocormia and parkinsonian symptoms in patients with Parkinson’s disease and camptocormia compared with patients with Parkinson’s disease without camptocormia. Methods: Patients with parkinsonism and camptocormia (excluding patients with multiple system atrophy) prospectively underwent a multidisciplinary clinical (neurological, neuropsychological, psychological, rheumatological) and neurophysiological (electromyogram, ocular movement recording) examination and were compared with age-matched patients with Parkinson’s disease without camptocormia. Results: The camptocormia developed after 8.5 (SD 5.3) years of parkinsonism, responded poorly to levodopa treatment (20%) and displayed features consistent with axial dystonia. Patients with camptocormia were characterised by prominent levodopa-unresponsive axial symptoms (ie, axial rigidity, gait disorder and postural instability), along with a tendency for greater error in the antisaccade paradigm. Conclusion: We suggest that (1) the salient features of parkinsonism observed in patients with camptocormia are likely to represent a specific form of Parkinson’s disease and camptocormia is an axial dystonia and (2) both camptocormia and parkinsonism in these patients might result from additional, non-dopaminergic neuronal dysfunction in the basal ganglia.
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TL;DR: It can be concluded that camptocormia in PD is predominantly myopathic, and is associated with a greater prevalence of compression fractures and associated with greater UPDRS part II, part III score, axial score, and lower MMSE in this cross-sectional study.
10 citations
01 Jan 2014
TL;DR: The unique properties of the neurotoxin have led to its becoming a significant therapeutic agent of benefit in an ever-expanding range of diseases of neuronal hyperactivity, and establishing the structural basis of its activity has opened up opportunities to engineer the toxin to create novel proteins of increased therapeutic effect and potential.
Abstract: Botulinum neurotoxin is a highly successful therapeutic agent used for the treatment of a range of severe, chronic diseases, and is also widely used and recognised as a cosmetic agent for reduction of facial wrinkles. And yet, this blockbuster therapeutic product is also the most lethal toxin known and a Centers for Disease Control and Prevention (CDC) Category A bioweapons threat. These apparently conflicting applications of the same agent have their origins in the unique biological properties of this fascinating protein. Unravelling the biology of the neurotoxin has informed understanding of the basis of both its toxicity and therapeutic activity. The unique properties of the neurotoxin have led to its becoming a significant therapeutic agent of benefit in an ever-expanding range of diseases of neuronal hyperactivity. Establishing the structural basis of its activity has opened up opportunities to engineer the toxin to create novel proteins of increased therapeutic effect and potential.
9 citations
01 Jan 2011
8 citations
Dissertation•
28 Feb 2014
TL;DR: Radiological examination demonstrated that Pisa syndrome was different from de novo degenerative scoliosis and camptocormia not typical of adult onset degenerative kyphosis and fixed bony changes were rare.
Abstract: Studies have been performed to detail the phenomenology, investigate the skeletal changes and explore the spinal biomechanics underlying the main axial deformities – Pisa syndrome and camptocormia in Parkinson’s disease. Results demonstrate that the clinical picture of these deformities varies greatly but that certain particular features allow distinction from other neurological, muscular and bony aetiologies. The tone of the axial muscles, the level at which spinal flexion occurs, the patient’s ability and method to try to overcome the chronically abnormal posture, and the flexibility or fixity of the trunk provide clinical pointers to the likely underlying cause. The scoliotic curve in a patient with Pisa syndrome was C-shaped, involved a large element of collapse and occurred without evidence of a secondary upper compensatory curvature (S-shaped curve). On supine imaging patients with camptocormia were severely mechanically disadvantaged as a result of their alordotic lumbar spines in relation to pelvic angulation. This lumbar alordosis may reflect the effects of Parkinson’s disease on the axial musculature, particularly in those with axial akinetic rigid predominant PD. Radiological examination also demonstrated that Pisa syndrome was different from de novo degenerative scoliosis and camptocormia not typical of adult onset degenerative kyphosis. Fixed bony changes were rare but the severity of these postural deformities and their consequent effects (e.g. knee flexion contractures, gluteal muscle atrophy) are likely to render conservative interventions unsuccessful unless instigated very early in the evolution of the abnormal posture.
7 citations
Cites background from "Parkinson’s disease with camptocorm..."
...On average, camptocormia presents 6-8 years following onset of parkinsonism (Azher and Jankovic, 2005, Margraf et al, 2010, Spuler et al, 2010, Bloch et al, 2006, Lepoutre et al, 2006, Djaldetti et al, 1999, Seki et al, 2011)....
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...…established with secondary fixed changes, patients may complain of breathlessness due to restricted lung capacity, difficulty swallowing or of difficulty lying flat in bed due to hip or knee contractures; the latter can be accompanied by skin irritation in the flexed segment (Bloch et al, 2006)....
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...It also appears that PD patients with camptocormia are sometimes less levodopa responsive than those without this deformity and have fewer levodopa-induced limb dyskinesias (Bloch et al, 2006)....
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...It is generally accepted that camptocormia is not a levodopa-responsive phenomenon (Djaldetti et al, 1999, Azher and Jankovic, 2005), although one author reported a modest improvement in forward flexion when patients were ‘on’ drug as opposed to ‘off’ (Bloch et al, 2006)....
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...…show a positive association between camptocormia and disease severity, with camptocormia sufferers tending to have more advanced parkinsonism than those without (Ashour and Jankovic, 2006, Margraf et al, 2010, Tiple et al, 2009, Bloch et al, 2006, Seki et al, 2011, Kashihara and Imamura, 2012)....
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TL;DR: Hiatal hernias are a treatable cause of camptocormia and should be missed, especially in patients copresenting with gastrointestinal symptoms, and should therefore not be missed.
Abstract: kyphosis. However, the present case is the first report of camptocormia in PD because of an intrathoracic stomach, and the improvement of the forward trunk flexion after surgical intervention in our patient supports the view that the intrathoracic stomach was responsible for the camptocormia. We suggest that with the hiatal hernia in our patient, direct compression or vascular embarrassment of paravertebral structures can lead to a camptocormia-like posture. However, the exact pathogenesis of camptocormia because of the hiatal hernia remained unclear and required further evaluation. Therapeutic efforts often have little benefit. Bloch et al reported that at most, 20% of PD patients with camptocormia benefited from levodopa therapy. Although it is possible that the treatment of the hiatal hernia led to better levodopa absorption, the camptocormia did not respond to a 1500-mg dose of levodopa before surgery in our case. Therefore, we assumed that the improvement of camptocormia was not related to levodopa but to the surgical treatment of the hiatal hernia. In conclusion, hiatal hernias are a treatable cause of camptocormia and should therefore not be missed, especially in patients copresenting with gastrointestinal symptoms.
7 citations
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