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: A thorough understanding of the broad spectrum of clinical manifestations of PD is essential to the proper diagnosis of the disease and genetic mutations or variants, neuroimaging abnormalities and other tests are potential biomarkers that may improve diagnosis and allow the identification of persons at risk.
Abstract: Objective: Parkinson’s disease (PD) is a progressive neurological disorder characterised by a large number of motor and non-motor features that can impact on function to a variable degree. This review describes the clinical characteristics of PD with emphasis on those features that differentiate the disease from other parkinsonian disorders. Methods: A MedLine search was performed to identify studies that assess the clinical characteristics of PD. Search terms included “Parkinson’s disease”, “diagnosis” and “signs and symptoms”. Results: Because there is no definitive test for the diagnosis of PD, the disease must be diagnosed based on clinical criteria. Rest tremor, bradykinesia, rigidity and loss of postural reflexes are generally considered the cardinal signs of PD. The presence and specific presentation of these features are used to differentiate PD from related parkinsonian disorders. Other clinical features include secondary motor symptoms (eg, hypomimia, dysarthria, dysphagia, sialorrhoea, micrographia, shuffling gait, festination, freezing, dystonia, glabellar reflexes), non-motor symptoms (eg, autonomic dysfunction, cognitive/neurobehavioral abnormalities, sleep disorders and sensory abnormalities such as anosmia, paresthesias and pain). Absence of rest tremor, early occurrence of gait difficulty, postural instability, dementia, hallucinations, and the presence of dysautonomia, ophthalmoparesis, ataxia and other atypical features, coupled with poor or no response to levodopa, suggest diagnoses other than PD. Conclusions: A thorough understanding of the broad spectrum of clinical manifestations of PD is essential to the proper diagnosis of the disease. Genetic mutations or variants, neuroimaging abnormalities and other tests are potential biomarkers that may improve diagnosis and allow the identification of persons at risk.
4,349 citations
Cites background from "Parkinson’s disease with camptocorm..."
...In addition to PD, other causes of camptocormia include dystonia and extensor truncal myopathy.(49) 50 Another truncal...
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TL;DR: Improved understanding of the mechanisms underlying postural deformities in PD might ultimately lead to more effective management strategies for these disabling and drug-refractory complications.
Abstract: Postural deformities are frequent and disabling complications of Parkinson's disease (PD) and atypical parkinsonism. These deformities include camptocormia, antecollis, Pisa syndrome, and scoliosis. Recognition of specific postural syndromes might have differential diagnostic value in patients presenting with parkinsonism. The evidence to date suggests that postural deformities have a multifactorial pathophysiology. Contributing factors include muscular rigidity; axial dystonia; weakness caused by myopathy; body scheme defects due to centrally impaired proprioception; and structural changes in the spine. The relative contribution of these different factors varies between patients and across specific syndromes. Improved understanding of the mechanisms underlying postural deformities in PD might ultimately lead us to more effective management strategies for these disabling and drug-refractory complications.
402 citations
TL;DR: This MDS‐commissioned task force assessed clinimetric properties of existing rating scales, questionnaires, and timed tests that assess features in Parkinson's disease.
Abstract: BACKGROUND: Disorders of posture, gait, and balance in Parkinson's disease (PD) are common and debilitating. This MDS-commissioned task force assessed clinimetric properties of existing rating scales, questionnaires, and timed tests that assess these features in PD. METHODS: A literature review was conducted. Identified instruments were evaluated systematically and classified as "recommended," "suggested," or "listed." Inclusion of rating scales was restricted to those that could be used readily in clinical research and practice. RESULTS: One rating scale was classified as "recommended" (UPDRS-derived Postural Instability and Gait Difficulty score) and 2 as "suggested" (Tinetti Balance Scale, Rating Scale for Gait Evaluation). Three scales requiring equipment (Berg Balance Scale, Mini-BESTest, Dynamic Gait Index) also fulfilled criteria for "recommended" and 2 for "suggested" (FOG score, Gait and Balance Scale). Four questionnaires were "recommended" (Freezing of Gait Questionnaire, Activities-specific Balance Confidence Scale, Falls Efficacy Scale, Survey of Activities, and Fear of Falling in the Elderly-Modified). Four tests were classified as "recommended" (6-minute and 10-m walk tests, Timed Up-and-Go, Functional Reach). CONCLUSION: We identified several questionnaires that adequately assess freezing of gait and balance confidence in PD and a number of useful clinical tests. However, most clinical rating scales for gait, balance, and posture perform suboptimally or have been evaluated insufficiently. No instrument comprehensively and separately evaluates all relevant PD-specific gait characteristics with good clinimetric properties, and none provides separate balance and gait scores with adequate content validity for PD. We therefore recommend the development of such a PD-specific, easily administered, comprehensive gait and balance scale that separately assesses all relevant constructs. (c) 2016 International Parkinson and Movement Disorder Society.
194 citations
TL;DR: This paper showed that physical therapy, especially highly challenging balance exercises, can improve postural stability and reduce the risk of falls, although the long-term effects of physical therapy interventions on postural instability need to be explored given the progressive nature of PD.
Abstract: Postural instability is one of the cardinal signs in Parkinson's disease (PD). It can be present even at diagnosis, but becomes more prevalent and worsens with disease progression. It represents one of the most disabling symptoms in the advanced stages of the disease, as it is associated with increased falls and loss of independence. Clinical and posturographic studies have contributed to significant advances in unravelling the complex pathophysiology of postural instability in patients with PD, but it still remains yet to be fully clarified, partly due to the difficulty in distinguishing between the disease process and the compensatory mechanisms, but also due to the fact that non-standardized techniques are used to measure balance and postural instability. There is increasing evidence that physical therapy, especially highly challenging balance exercises, can improve postural stability and reduce the risk of falls, although the long-term effects of physical therapy interventions on postural stability need to be explored given the progressive nature of PD. Pharmacotherapy with dopaminergic medications can provide significant improvements in postural instability in early- to mid-stage PD but the effects tend to wane with time consistent with spread of the disease process to non-dopaminergic pathways in advanced PD. Donepezil has been associated with a reduced risk of falls and methylphenidate has shown potential benefit against freezing of gait, but the results are yet to be replicated in large randomized studies. Surgical treatments, including lesioning and deep brain stimulation surgery targeting the subthalamic nucleus and the globus pallidus internus, tend to only provide modest benefit for postural instability. New surgical targets such as the pedunculopontine nucleus have emerged as a potential specific therapy for postural instability and gait disorder but remain experimental.
189 citations
Cites background from "Parkinson’s disease with camptocorm..."
...Separately, another important but less common source of postural instability is camptocormia or Pisa syndrome in which there is abnormal forward and lateral flexion of the trunk, respectively [10, 84]....
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TL;DR: Overall, postural impairment is poorly improved by levodopa, which implies that it is unlikely due to the nigrostriatal dopaminergic denervation, and the pedonculopontine nucleus seems promising as a new target for DBS in combination with the subthalamic nucleus.
Abstract: Posture is often affected in Parkinson's disease. Postural abnormalities belong to the motor axial involvement. Generally, postural dysfunction induces clinical impairment at the latest stages of the disease, except in late-onset idiopathic Parkinson's disease and in atypical parkinsonian syndromes. Posture may be affected in its orientation component (stooped posture, camptocormia, Pisa syndrome) or in its balance component (loss of postural reflexes). Overall, postural impairment is poorly improved by levodopa, which implies that it is unlikely due to the nigrostriatal dopaminergic denervation. Several methods of investigation have been proposed but are generally not available in clinical practice. Medical treatment and deep brain stimulation (DBS) of the subthalamic nucleus or globus pallidus pars interna are less efficient on axial than on distal motor signs. The pedonculopontine nucleus seems promising as a new target for DBS in combination with the subthalamic nucleus. Physical therapy is, in most cases, the best way to improve postural dysfunction.
151 citations
Cites background from "Parkinson’s disease with camptocorm..."
...[8] Bloch F, Houeto JL, Tezenas du Montcel S, Bonneville F, Etchepare F, Welter ML, et al....
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...The time intervals between the onset of PD nd the clinical onset of camptocormia ranged from four to 4 years [8,17,32] Another truncal deformity is the Pisa syndrome (Fig....
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References
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TL;DR: The study supports the reliability and validity of the SIP Roland scale items for assessing dysfunction of chronic pain patients with pain in sites other than the low back as well as those with low back pain.
Abstract: This study examined the reliability and validity of the Roland scale (taken from the Sickness Impact Profile: SIP) as a measure of dysfunction among chronic pain patients. One hundred forty-four subjects completed the SIP when they were screened for admission to an inpatient pain management program. One hundred sixteen subjects were subsequently re-administered the SIP at admission to inpatient treatment. A 3-month post-treatment administration of the SIP was performed for 52 of these subjects. Roland scale scores were calculated from the SIP for each patient. Test-retest stability coefficients indicated that the SIP Roland scale was generally as reliable as the SIP Total, Physical, and Psychosocial scale scores. Consistent with previous research, correlational analyses indicated that the SIP Roland scale is strongly associated with the SIP Physical but not the SIP Psychosocial scale. The SIP Roland scale and the other SIP scales demonstrated similar sensitivity to changes associated with multidisciplinary inpatient treatment for chronic pain. Finally, the pattern of relationships between the SIP Roland scale and several pain-related measures supported the concurrent validity of the SIP Roland scale. The results of the analyses were very similar for patients presenting with and without low back pain. The study supports the reliability and validity of the SIP Roland scale items for assessing dysfunction of chronic pain patients with pain in sites other than the low back as well as those with low back pain.
192 citations
TL;DR: The results showed that the dysexecutive syndrome of SND is similar to that of Parkinson's disease and less severe than in PSP.
Abstract: To study the neuropsychological pattern of striatonigral degeneration (SND), 14 consecutive patients with probable SND were submitted to an extensive battery of neuropsychological tests. Compared with controls the performance of patients with SND was impaired on category and phonemic fluency, frontal behaviours, trail making test A and B, and free recall of the Grober and Buschke test, but normal on the revised WAIS verbal scale, Raven 47 coloured progressive matrices, Wechsler memory scale, California verbal learning test, Wisconsin card sorting test, and the Stroop interference condition. The performance of patients with SND was also compared with that of 14 patients with Parkinson's disease and 14 patients with progressive supranuclear palsy (PSP) matched for age at onset, duration of disease, severity of intellectual deterioration, and depression. The results showed that the dysexecutive syndrome of SND is similar to that of Parkinson's disease and less severe than in PSP.
154 citations
TL;DR: The evaluation of these disorders can indeed be challenging and often no definite diagnosis is made, as illustrated by four cases of head ptosis and camptocormia seen by us at the Johns Hopkins Hospital.
Abstract: The spectrum of bent spine disorders
Head ptosis (drop) results from weakness of the neck extensor, or increased tone of the flexor muscles. It is characterised by marked anterior curvature or angulation of the cervical spine and is associated with various neuromuscular (table 1) and extrapyramidal disorders.12–15 Camptocormia or the bent spine syndrome was first described in hysterical soldiers in 1915 by the French neurologist Souques.16 Typically there is marked anterior curvature of the thoracolumbar spine. In some patients the spine is angulated forward, the arms propped against the thigh for support. More cases, all among soldiers, were reported during the first and second world wars. These patients responded well to psychotherapy. Recently camptocormia arising as a result of weakness or abnormality in the tone of the paraspinal muscles has been described (table 2). In contrast with other skeletal disorders of the spine such as kyphosis, the deformity in head ptosis and camptocormia is not fixed and is corrected by passive extension or lying in the supine position. It is not possible to straighten the neck or back voluntarily. The evaluation of these disorders can indeed be challenging and often no definite diagnosis is made, as illustrated by four cases of head ptosis and camptocormia seen by us at the Johns Hopkins Hospital.
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Table 1
Neuromuscular causes for head ptosis
View this table:
Table 2
Causes of camptocormia
An 80 year old man developed head ptosis insidiously over a period of few weeks. A week before this he had an upper respiratory tract infection and also experienced transient sharp pain over the left and then the right shoulder. He had no diplopia, dysarthria, dysphagia, limb weakness, or fatiguability. Examination showed severe neck extensor weakness, Medical Research Council (MRC) grade 2. Muscle strength was normal in all other cranial, proximal, and distal limb muscles. Serum …
135 citations
TL;DR: Consecutive EOG recordings help diagnose atypical CBD and PSP disorders earlier and when clinical “telltale signs” appeared and the clinical diagnosis was reviewed independent of EOG recording, the three patients with atypicals CBD features were diagnosed as having PSP although new or overlapping syndromes cannot be excluded.
Abstract: Objective: To evaluate the usefulness of ocular motor information in the early diagnosis of corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Methods: Seven PSP patients, six CBD patients, and three atypical CBD patients were followed longitudinally with repeated electrooculographic (EOG) recordings, at 6-month intervals, to search for features that could confirm or modify the diagnosis. Visually guided saccades and antisaccades were studied. Data from clinical evaluations were independently collected. Results: PSP patients had decreased saccade velocity throughout the disease course. Patients with probable CBD showed preserved saccade velocity but important increased saccade latency ipsilateral to the apraxia side. Similar to patients with PSP, those with atypical CBD features exhibited clinically evident abnormalities of vertical saccades and early slowing of horizontal saccade velocity, but no increase in saccade latency or early square-wave jerks. When clinical “telltale signs” appeared and the clinical diagnosis was reviewed independent of EOG recording, the three patients with atypical CBD features were diagnosed as having PSP although new or overlapping syndromes cannot be excluded. Conclusions: Consecutive EOG recordings help diagnose atypical CBD and PSP disorders earlier.
123 citations
Journal Article•
119 citations