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: Medication and DBS both improved postural alignment in PD patients, but effects were small for the entire cohort, which may indicate distinct pathological mechanisms for stooped posture and postural disorders.
Abstract: Introduction: Postural abnormalities are common in Parkinson's disease (PD) and increasing with disease progression. While many studies focus on balance and gait, postural alignment is only infrequently studied. Purpose: The aim of the present study was to examine the immediate and long-term effects of medication and deep brain stimulation (DBS) in the subthalamic nucleus on postural alignment in PD. Materials and Methods: PD patients (n = 192) in an advanced stage of disease were videotaped during a standardized l-dopa trial before and after DBS. The patients were tested with and without medication pre-surgical and retested post-surgical (6-24 months) in all treatment combinations of medication and DBS regarding the on and off conditions. The forward bending as total camptocormia (TCC) and upper camptocormia (UCC) angles and lateral bending as Pisa angle were assessed with the free downloadable NeuroPostureApp (http://www.neuroimaging.uni-kiel.de/NeuroPostureApp/). Three subgroups were defined according to normative values of healthy controls and according to clinical criteria: patients with normal posture, with stooped posture, and with postural disorders. Results: A stooped posture was found in 82% of the patients with regard to the TCC angle and in 54% for the UCC angle. Sixty-two percent had an abnormal Pisa angle. Camptocormia was diagnosed in ~7% and a Pisa syndrome in 1% of the patients. Medication and DBS both significantly improved postural alignment in the entire cohort. Female and male patients benefit similarly by medication and stimulation. Subgroup analyses revealed that the effects were also significant for patients with stooped posture, and the effects were strongest for patients with camptocormia: they led to angles below the diagnostical criterion for camptocormia for 13 of 14 patients with TCC and 11 of 26 patients with UCC. DBS had an additional effect to medication over time for the Pisa angle. Conclusion: Medication and DBS both improved postural alignment in PD patients, but effects were small for the entire cohort. Patients with camptocormia according to the TCC angle benefit strongest. The large differences of the treatment effects may indicate distinct pathological mechanisms for stooped posture and postural disorders. The TCC angle was shown to be sensitive to change. The UCC angle was less sensitive but may be a useful assessment tool for a subgroup.
14 citations
Cites background or methods from "Parkinson’s disease with camptocorm..."
...While treatment with deep brain stimulation (DBS) or with medication, such as L-dopa or dopamine agonists, is effective in improving several symptoms of the disease, controversy exists with regard to the axial symptoms (3)....
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...Patients were included if they suffered from idiopathic PD according to the UK Brain Bank PD criteria (9), had received DBS of the subthalamic nucleus, and if medical records and a full set of a videotaped standardized L-dopa trials were available before electrode implantation and at follow-up for up to 2 years (Table 1)....
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...In addition, an axial subscore was calculated as the sum of the UPDRS items 18, 19, 22, 29, and 30 (12)....
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...On the other hand, there is also a body of literature arguing against the positive effect of L-dopa in the camptocormia of PD (22, 23)....
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...The predictability of the effect of stimulation on the TCC was low when considering the baseline L-dopa responsiveness of the TCC as the outcome parameter....
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TL;DR: PD patients manifesting camptocormia are likely subjected to NMS more than patients without camptoormia, especially autonomic and miscellaneous symptoms, and suffered from more severe neuropsychiatric symptoms and sleep disorders.
14 citations
Cites background or result from "Parkinson’s disease with camptocorm..."
...Researchers consider camptocormia as a type of dystonia, which may result from additional, non-dopaminergic neuronal dysfunction in the basal ganglia [21,22]....
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...This finding is inconsistent with that of a previous study, which found no difference in anxiety between PD patients with and without camptocormia [21]....
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TL;DR: In two patients with severe camptocormia there was a significant functional improvement following long-term bilateral GPi stimulation and both patients gained ability to walk and GPi DBS is a possible treatment option for this rare disease.
Abstract: Camptocormia, characterised by a forward flexion of the thoracolumbar spine may occur in various movement disorders, mainly in Parkinson’s disease or in primary dystonia. In severe cases, patients with camptocormia are unable to walk. While treatment options are limited, deep brain stimulation (DBS) with bilateral stimulation of the subthalamic nucleus or globus pallidus internus (GPi) has been proposed as a therapeutic option in refractory cases of Parkinson’s disease. Here we present two patients with severe camptocormia as an isolated form of dystonia and as part of generalised dystonia, respectively, which were both treated with bilateral stimulation of the GPi. Symptoms of dystonia were assessed using the Burke–Fahn–Marsden dystonia rating scale (BFM) before and during deep brain stimulation. In both patients there was a significant functional improvement following long-term bilateral GPi stimulation and both patients gained ability to walk. In the first patient with an isolated dystonic camptocormia the BFM motor subscore for the truncal flexion improved by 75 %. The total BFM motor score in the second patient with a camptocormia in generalised dystonia improved by 45 %, while the BFM score for truncal flexion improved by 87 %. In both patients the effect of the bilateral GPi stimulation on camptocormia was substantial, independent of generalisation of dystonia. Therefore, GPi DBS is a possible treatment option for this rare disease.
13 citations
TL;DR: The first case of a patient with confirmed myotonic dystrophy addressed to a medical center for impaired posture who underwent extensive medical exams and explorations because of a myOTonic hand is reported.
Abstract: Camptocormia is an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position. Camptocormia has been described in various neurological (Parkinsonism), muscular (myopathy), psychogenic or orthopedic disorders. There are several hypotheses that can explain this impaired posture but they are usually related to the concomitant pathologies. We report the first case of a patient with confirmed myotonic dystrophy addressed to our medical center for impaired posture who underwent extensive medical exams and explorations because of a myotonic hand. Axial weakness and muscle atrophy, validated by CT-scan imaging, are discussed independent of the concomitant pathology (Parkinson, myopathy).
13 citations
Cites background from "Parkinson’s disease with camptocorm..."
...posture, they are frequently reported in clinical observations of camptocormia [3, 4, 13]....
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...[3] did not find a relationship between camptocormia and a medical history of disc herniation or back surgery, but unveiled a rapidly progressing camptocormia in patients with low-back pain probably linked to mechanical adaptations of the lumbopelvic area....
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TL;DR: The reliability of a truncal posture measurement is established and the C7 to wall measurement is highly reliable in people with PD, and global axial symptoms were independently associated with trun cal posture.
Abstract: Background and Purpose:Flexed truncal posture is common in people with Parkinson disease (PD); however, little is known about the mechanisms responsible or its effect on physical performance. This cross-sectional study aimed to establish the reliability of a truncal posture measurement and explore r
13 citations
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