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Camptocormia in Parkinson disease: an epidemiological and clinical study D Tiple, G Fabbrini, C Colosimo, D Ottaviani, F Camerota, G Defazio and A Berardelli J. Neurol. Neurosurg. Psychiatry 2009;80;145-148; originally published online 17 Oct 2008; doi:10.1136/jnnp.2008.150011
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Research paper
Camptocormia in Parkinson disease: an epidemiological and clinical study D Tiple,1,2 G Fabbrini,1,3 C Colosimo,1 D Ottaviani,1 F Camerota,4 G Defazio,5 A Berardelli1,3 1
Department of Neurological Sciences, ‘‘Sapienza’’ University of Rome, Rome, Italy; 2 Department of Neurological Sciences, Neurological Center of Latium, Rome, Italy; 3 NEUROMED Institute, ‘‘Sapienza’’ University of Rome, Rome, Italy; 4 Department of Physical Medicine and Rehabilitation, ‘‘Sapienza’’ University of Rome, Rome, Italy; 5 Department of Neurological and Psychiatric Neurosciences, University of Bari, Italy Correspondence to: Dr A Berardelli, Department of Neurological Sciences, ‘‘Sapienza’’ University of Rome, Italy;
[email protected] All authors contributed equally to the manuscript. Statistical analysis was conducted by GD. Received 20 March 2008 Revised 4 July 2008 Accepted 30 July 2008 Published Online First 17 October 2008
ABSTRACT Background: Camptocormia is an abnormal flexion of the thoracolumbar spine during standing and walking that abates in the recumbent position. Methods: In a single-centre epidemiological and clinical study, the prevalence of camptocormia in Parkinson disease (PD) and its relationship with the clinical features of PD were investigated. A total of 275 consecutive outpatients were systematically screened for camptocormia with a clinical evaluation. Patients who screened positive for camptocormia were subsequently reassessed by formal goniometric analysis. The demographic and clinical features of the patients with and without camptocormia were then compared. Results: A 6.9% (19/275, 95% CI, 4.2 to 10.6) prevalence of camptocormia was found. Camptocormia was found in patients with more severe PD, as clinically assessed by the Hoehn–Yahr (HY) staging and the motor Unified Parkinson Disease Rating Scale (UPDRS) part III, longer L-dopa treatment duration and greater L-dopa daily dose and presence of DSM-IV dementia. Camptocormia was reported to develop after the clinical onset of PD. No correlation was found between the degree of trunk flexion and age, duration of PD, UPDRS motor score, HY staging, and L-dopa treatment duration and dose. As a risk factor, the study identified previous vertebral surgery. Conclusions: Camptocormia, a relatively common sign in PD seems to be related to the clinical severity of PD.
Camptocormia is an abnormal flexion of the thoracolumbar spine during standing and walking that abates or disappears in the recumbent position.1–3 A severe trunk flexion was noted in the original description of Parkinson disease (PD)4 and more recently in various other basal ganglia diseases, including autosomal recessive juvenile parkinsonism due to parkin mutation, postencephalitic parkinsonism, drug-induced parkinsonism, multiple system atrophy (MSA), and primary and secondary dystonia.5–10 Even though camptocormia is now a recognised feature of PD, published data leave certain areas unclear. For example, in a study reporting a 3% prevalence of camptocormia in PD, Lepoutre et al11 diagnosed the condition on the basis of generic clinical criteria (‘‘severe, reducible, forward flexion of the thoracolumbar spine’’) without systematically investigating the condition in the overall sample of PD patients. Four studies5 9 11 12 found no differences in the parkinsonian clinical features between PD patients with and without camptocormia, but three of the four studies based their findings on small study samples.5 9 11 Having more information on camptocormia in PD would help to improve strategies for these J Neurol Neurosurg Psychiatry 2009;80:145–148. doi:10.1136/jnnp.2008.150011
patients’ clinical care. In this single-centre epidemiological and clinical study, we investigated the prevalence of camptocormia in PD, the relationship of camptocormia with the clinical features of PD and the presence of possible risk factors in a large outpatient population of 275 consecutive patients with PD.
PATIENTS AND METHODS PD patients were selected from among those attending our outpatients PD clinic over a 9-month period. The diagnosis of PD was made according to the UK Brain Bank Criteria.13 All the patients were able to participate in the clinical interviews and in the clinical motor evaluation, and were studied while they were on their usual drug treatment. Clinical and demographic information was obtained with a standardised interview administered face to face by a trained medical interviewer who was unaware of the study hypothesis. Data collected for each subject included age, sex, disease duration and treatment regimen. Motor symptoms and signs were assessed using the motor Unified Parkinson disease Rating Scale (UPDRS-III).14 The severity of the disease was scored by Hoehn–Yahr (HY) staging.15 The presence of motor fluctuations and dyskinesias was also evaluated. Dementia was diagnosed according to the DSM-IV criteria.16 Camptocormia was defined as an anterior flexion of the thoracolumbar spine of 45u or more appearing in orthostatism or during gait and disappearing in the recumbent position.9 Patients were systematically screened for camptocormia by two examiners (DO, DT) who reached full clinical agreement on the absence or presence of camptocormia. They also assessed lateral deviation of the trunk. The validity of the clinical examination assessing camptocormia was evaluated in a sample population using as the reference standard goniometric measurement of the degree of thoracolumbar flexion.17 Given the low reported prevalence of camptocormia in PD, the sample included all the subjects who screened positive for camptocormia on clinical examination (n = 21), and 20 randomly recruited patients (12 men and eight women; mean age 70.6 (6.4) years; mean age at PD onset 61.4 (8.0) years; mean HY staging 2.6 (0.8), range 1 to 5; mean UPDRS score part III, 32.0 (14.1)) who screened negative for camptocormia but whose clinical features resembled those of the 256 patients who did not have camptocormia on clinical impression (see table 1). After clinical evaluation, the 41 patients (21 with and 20 without camptocormia) underwent goniometric measurement to determine the degree of thoracolumbar flexion17 assessed by calculating the angle 145
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Research paper between the vertical plane and a line passing through the trochanter and the edge of the acromion. Goniometric evaluation, undertaken by the same specialist (FC) in all patients, was used as the reference standard to calculate the sensitivity and specificity of the clinical examination. Sensitivity was defined as the proportion of patients identified as having camptocormia on goniometric examination who were also diagnosed at clinical evaluation. Specificity was defined as the proportion of patients identified as unaffected by camptocormia on goniometric examination, who also had not been diagnosed on clinical evaluation. In the validation sample, camptocormia was clinically diagnosed in 21/41 patients. After goniometric testing, 19 patients received a diagnosis of camptocormia. Of the 20 patients who were not diagnosed as having camptocormia on clinical examination, none had camptocormia on goniometric measurement. The clinical examination therefore yielded 100% (19/19) sensitivity, and 90% (20/22) specificity in diagnosing camptocormia. Considering a two-step procedure based on clinical examination first and goniometric measurement of patients screening positive thereafter, specificity increased to 100% (22/22). Camptocormia-related disability was assessed using the short version of the Roland Morris Disability Scale (RM-18), an 18item questionnaire measuring the disability associated with trunk abnormalities.18 The various items concerned functional limitations for various activities of daily living, seeking help from others and changes in mood and sleep due to pain. Patients were requested to select any item that currently applied to them. The scale was scored from 0 to 18, with higher scores indicating more severe disability. Statistical analysis was performed using the Stata 8 package (StataCorp LP, College Station, TX). Unless otherwise specified, all data are expressed as means (SD). Differences between groups were examined by the t test or x2 test. Bivariable logistic regression models were used to check for the possible confounding effect of relevant variables on the association between camptocormia and prior vertebral surgery. The Spearman coefficient (with Bonferroni correction) was used to calculate the correlation between the degree of trunk flexion as assessed by the goniometer, the disability of camptocormia as assessed by the Roland Morris Disability Scale and demographic/clinical variables. p Values ,0.05 were considered statistically significant.
RESULTS During the study period, 275 patients met the eligibility criteria and all participated in the study. The study sample comprised 160 men and 115 women aged 68.8 (8.9) years. The mean age of PD onset was 61.0 (7.2) years. The mean HY staging was 2.4 (0.9) (range 1 to 5). The mean UPDRS score part III measured in the on condition was 27.6 (13.4). Of the 275 PD patients studied, 13 were receiving no medication, whereas 115 were taking L-dopa alone, 44 dopamine agonists alone, and 103 both drugs. A total of 171 patients had drug-induced motor complications, and 29 patients had dementia. Our two-step diagnostic procedure identified camptocormia in 19 of 275 PD patients (6.9%; 95% CI 4.2 to 10.6%). The degree of trunk flexion as assessed by the goniometer ranged between 45u and 55u in 12/19 patients, and from 55u to 70u in 7/ 19 patients. In the 19 patients with camptocormia, the condition was reported to develop after the clinical onset of PD. Comparing the demographic and clinical features of the patients with and without camptocormia, we found that the two groups did not differ for sex, age of PD onset, side 146
distribution of parkinsonian symptoms, presence of motor fluctuations, dyskinesias and dopamine agonist treatment (table 1). Conversely, patients with camptocormia were older, had a significantly longer disease duration and more severe PD, and had a higher frequency of dementia. Patients with camptocormia also had a longer treatment duration and daily levodopa dose. Finally, prior vertebral surgery (laminectomy for lumbosacral disc herniation) was more frequent in PD patients with camptocormia (table 1). The association of camptocormia and prior vertebral surgery remained significant even after adjusting for relevant demographic and clinical variables in bivariable logistic regression models (table 2). In the group of patients with camptocormia, we found no significant correlation between the degree of trunk flexion as assessed by the goniometric analysis and age, duration of PD, UPDRS motor score, HY staging and L-dopa treatment duration and dose (not shown). Of the 19 patients with camptocormia, nine had back pain and functional disability related to camptocormia, but no significant correlation was observed between the score in the Roland Morris Disability Scale (mean (SD) 5.8 (7.3)) and age, duration of PD, UPDRS motor score, HY staging, and L-dopa treatment duration and dose (not shown). Eleven of the 19 patients with camptocormia (57.9%), but only one of the 20 patients who did not have camptocormia (5%) and underwent goniometric analysis also had a lateral deviation of the trunk (p,0.0001). In six of the 11 patients with camptocormia, the trunk deviated away from the side most severely affected by parkinsonism and in one patient toward the most affected side. In the remaining four patients with lateral deviation of the trunk, motor symptoms were symmetrical on the two sides of the body. In two of the 19 patients, camptocormia improved when the patient used ‘‘sensory tricks’’ that transiently minimise the discomfort provoked by the abnormal posture.
DISCUSSION By systematically assessing camptocormia in a large unselected sample of patients with PD, we provide valid assumptions that give more definitive information on the prevalence of camptocormia in PD and its relationship with the clinical features of PD. Using a combined clinical and goniometric evaluation, an approach that guarantees diagnostic accuracy, we observed a 6.9% prevalence (95% CI 4.2 to 10.6) of camptocormia in our series. Camptocormia was found in patients with more severe PD as clinically assessed by the HY staging and the UPDRS part III, longer PD duration, longer L-dopa treatment duration and greater L-dopa daily dose, and a more frequent diagnosis of dementia. The age at PD clinical onset, the unilateral/bilateral distribution of symptoms at PD onset and at the time of the study, and the development of L-dopa-related motor complications did not differ significantly in patients who had camptocormia and those who did not. An additional new finding in our study was that patients with camptocormia had more frequently undergone preceding vertebral surgery. Our clinical and goniometric findings differ in some aspects to those of the only epidemiological study that found camptocormia in 3% of a large series of PD patients.11 The higher prevalence observed in our study probably relates to differences in the clinical features of the study population and in the ascertainment methods. The association of camptocormia with advanced HY staging, higher UPDRS motor score, longer duration and greater levodopa dose and a more frequent diagnosis of dementia J Neurol Neurosurg Psychiatry 2009;80:145–148. doi:10.1136/jnnp.2008.150011
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Research paper Table 1 Demographic and clinical features of Parkinson disease (PD) patients with and without camptocormia PD patients with camptocormia No Sex (men/women) Age (years) Age at PD onset (years) Disease duration (years) Distribution of symptoms at onset Bilateral Unilateral Distribution of symptoms at time of study Bilateral Unilateral UPDRS-III Total Tremor subscore Rigidity subscore Bradykinesia subscore Hoehn–Yahr staging 1 1.5 2 2.5 3 4 5 Levodopa treatment No of patients Daily dose (mg) Duration (months) Dopamine agonist treatment No of patients Duration (months) Motor fluctuations Dyskinesias Dementia Previous vertebral surgery
PD patients without camptocormia
p Value
19 13/6 74.6 (6.8) 61.6 (6.9) 13.0 (7.2)
256 147/109 68.5 (9) 60.9 (5) 7.6 (5.4)
3 16
34 222
0.9
17 1
206 50
0.25
42.3 3.4 8.0 16.0
(14.5) (3.3) (3.7) (7.4)
0 0 1 2 7 7 2
26.5 3.9 5.8 9.2
(13.6) (3) (3.4) (12)
24 25 97 32 45 31 2
18 639.5 (272.5) 133.4 (82.2) 5 88.5 (32) 11 9 5 4
0.5 0.001 0.4 0.001
,0.0001 0.6 0.007 ,0.0001
,0.0001
199 534.4 (279.3) 81.5 (61.6)
0.04 0.01 0.001
125 65.3 (38) 105 77 24 10
0.14 0.5 0.1 0.13 0.04 0.004
All values are reported as mean (SD). UPDRS, Unified Parkinson Disease Rating Scale.
suggests that more severe disease or prolonged levodopa therapy or both lead to camptocormia. Despite these findings, we cannot completely exclude the possibility that bias related to the cross-sectional approach used in the present study contributes to these associations.19 The higher frequency of camptocormia in individuals with more severe PD and longer
Table 2 Influence of potentially confounding factors on the association between camptocormia and prior vertebral surgery in patients with Parkinson disease Adjustment
Odds ratio
95% CI
p Value
None Age Sex Disease duration Unified Parkinson Disease Rating Scale Levodopa therapy Clinical fluctuations Dyskinesia Cognitive impairment
4.9 4.6 5.2 4.6 5.7
1.2 1.1 1.3 1.1 1.1
to to to to to
19.2 20 21.5 19.8 28.2
0.02 0.04 0.02 0.04 0.03
4.5 4.5 6.2 5.5
1.1 1.1 1.5 1.3
to to to to
19.1 18.3 26.3 23.1
0.04 0.04 0.01 0.03
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disease duration is at variance with prior studies that found no specific clinical features distinguishing PD patients with camptocormia from those without.5 9 12 In addition to differences in the ascertainment methods, the small sample sizes in previous series might have prevented possible associations from achieving significance. The lack of correlation between the degree of camptocormia and clinical and treatment-related variables could imply that the pathophysiology of camptocormia involves additional, nondopaminergic mechanisms. In accordance with Djaldetti and coworkers,5 some observations from our study sample support camptocormia as a form of dystonia. These include the improvement in camptocormia some patients achieved with ‘‘sensory tricks’’ and possibly the frequent association of camptocormia with lateral deviation of the trunk (57.9% of the patients with camptocormia vs 5.0% of those without), particularly trunk deviation toward the side least affected by parkinsonian signs. A dystonic disorder receives support also from the finding that prior vertebral surgery was statistically more frequent in patients with camptocormia than in those without. Accordingly the association was independent of age and other potentially confounding variables. Surgery could 147
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Research paper indeed be a trigger event similar to that seen in dystonia after local trauma in the same body segment.20 21 Finally, the presence of dystonic features not induced by L-dopa therapy is a wellknown phenomenon in PD.22 Our study may have limitations. First, since this study was a population-based study, this may have introduced a selection bias. The population we studied could differ from the general population of PD patients. Nevertheless, considering the age of PD onset and sex distribution, our series resembled the general population of PD.23 The mean severity of PD and the frequency of levodopa-related motor complications in our series are compatible with an average disease duration of 8 years. Owing to the association of camptocormia with variables related to more severe PD, the frequency of camptocormia could vary according to disease duration and severity. Second, because we quantitatively assessed the degree of trunk flexion only in a few PD patients, the lack of camptocormia remains a clinical impression. This limitation notwithstanding goniometric examination left the clinical impression of a lack of camptocormia in all the 20 PD patients included in the validation sample unchanged. Despite the small sample size, the clinical features of patients in the validation sample resembled those in the entire sample of patients in whom clinical assessment disclosed no camptocormia. Third, due to the cross-sectional design of our study, we could not exactly determine the timing of onset of camptocormia. Patients may not be able to distinguish between the flexion posture of PD and camptocormia. Despite the foregoing limitations, our results indicate that camptocormia is a relatively common sign in PD, that its development may be related to clinical variables associated with more severe PD and that previous vertebral surgery may be a risk factor. Our findings may have implications for designing studies aiming at understanding the mechanisms underlying camptocormia in PD and identifying specific treatment strategies for this often disabling condition.
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