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Journal ArticleDOI

Parkinson’s disease with camptocormia

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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Journal ArticleDOI
TL;DR: This new orthosis, relying on the principle of thoraco-pelvic anterior distraction, gives excellent results in the treatment of camptocormia.
Abstract: OBJECTIVE Camptocormia, or bent spine syndrome, is an acquired postural disease leading to lumbar kyphosis observed when the patient stands up Classic orthoses and physiotherapy programmes provide little correction and are often poorly tolerated The aim of this study was to evaluate the effectiveness and tolerance of a new orthosis combined with physiotherapy for treating camptocormia METHODS Fifteen patients (5 men and 10 women, 714 (standard deviation (SD) 73) years old) consulting for camptocormia were consecutively included in the study Patients who had pain when they straightened up were excluded Patients equipped with the orthosis were hospitalized for 5 days in order to learn a self-rehabilitation programme They were evaluated before and after hospitalization, then at 30 and 90 days RESULTS In comparison with day 0 (without orthosis), the mean increase in lumbar lordosis with the orthosis was 101 degrees (SD 99) at day 30 (p < 005) and 125 degrees (SD 97) at day 90 (p < 0001) Average pain values showed a reduction of 69% (SD 36) and 70% (SD 35) in the initial pain at days 30 and 90, respectively The average increase in quality of life was 87% and 92% at days 30 and 90, respectively CONCLUSION This new orthosis, relying on the principle of thoraco-pelvic anterior distraction, gives excellent results in the treatment of camptocormia

68 citations

Journal ArticleDOI
TL;DR: Findings show that significant improvements in axial posture and trunk mobility can be obtained through the 4‐week rehabilitation programme described, with a parallel improvement in clinical status.
Abstract: People with Parkinson's disease (PD) often have a posture characterized by lateral trunk flexion poorly responsive to antiparkinsonian drugs. To examine the effects of a rehabilitation programme (daily individual 90-minute-sessions, 5-days-a-week for 4-consecutive weeks) on lateral trunk flexion and mobility, 22 PD patients with mild to severe lateral trunk flexion, and 22 PD patients without trunk flexion were studied. Patients were evaluated using the Unified Parkinson's Disease Rating Scale motor subscale (UPDRS-III) score, and the kinematic behavior of the trunk was recorded by means of an optoelectronic system to determine: a) trunk flexion, inclination and rotation values in the erect standing posture; b) ranges of trunk flexion and inclination during trunk movements. After the treatment, significant decreases in trunk flexion [24 degrees (4) vs. 14 degrees (3), P < 0.001] and inclination in the static condition [23 degrees (5) vs. 12 degrees (4), P < 0.001)] were observed, both of which were maintained at the 6-month follow up. During the trunk flexion task, a significantly increased range of trunk flexion [64 degrees (15) vs. 83 degrees (15), P < 0.001] was observed; similarly, during the lateral bending task, the range of trunk inclination was found to be significantly increased, both toward the side of the trunk deviation [29 degrees (8) vs. 42 degrees (13), P < 0.01] and toward the contralateral side [14 degrees (6) vs 29 degrees (11), P < 0.01]. No further significant changes were observed at the 6-month follow-up. Trunk flexion and inclination values in the upright standing posture correlated slightly with the UPDRS-III score. Our findings show that significant improvements in axial posture and trunk mobility can be obtained through the 4-week rehabilitation programme described, with a parallel improvement in clinical status.

67 citations

Journal ArticleDOI
TL;DR: Camptocormia associated with PD usually emerges as the disease progresses, but no factors have been identified that reliably predict which patients with PD will develop this deformity, although it appears to be more common in patients with more severe, postural-instability-gait-difficulty form of PD.
Abstract: James Parkinson in his 1817 ‘‘Essay on the Shaking Palsy’’ drew attention to ‘‘a propensity to bend the trunk forward’’ as one of the classic signs of Parkinson’s disease (PD). Subsequent descriptions of PD have highlighted a variety of other deformities associated with PD resulting not only in a flexed neck and trunk but also flexed elbows, knees, and ‘‘striatal’’ deformities of hands and feet. These deformities, often confused with arthritis or other joint disorders, may lead to inappropriate surgical and other invasive treatments. They also adversely impact the patient’s overall functioning, particularly in advanced stages of the disease. Postural deformities involving the neck, such as the ‘‘head drop’’ syndrome, or involving the thoracolumbar spine resulting in scoliosis, ‘‘bent spine,’’ ‘‘pisa syndrome,’’ and ‘‘camptocormia’’ (extreme truncal flexion), have been receiving growing attention and have been subjected to several recent reviews. Camptocormia is characterized by severe often 90 degree or even greater flexion of the trunk that may be present in a sitting position and typically increases during walking and resolves in supine position or when patient achieves an erect posture while performing the ‘‘climbing on the wall’’ maneuver. The term ‘‘camptocormia’’ was coined during World War I when young soldiers who were apparently attempting to escape the stress of battle developed this peculiar posture, perhaps promoted by a stooped posture when walking in the trenches, viewed by the military personnel as mostly a malingering or a somatoform (psychogenic) disorder. Although bent spine may be of psychogenic origin, head drop (head ptosis) almost always has an organic etiology. In addition to parkinsonism, particularly multiple system atrophy, several possibilities should be considered in the differential diagnosis of head drop, including cervical dystonia, encephalitis, amyotrophic lateral sclerosis, myasthenia gravis, focal myopathy, inclusion body myositis, polymyositis, nemaline myopathy, facioscapulohumeral dystrophy, ankylosing spondylitis, disproportionately increased tone in the anterior neck muscles resulting in fibrotic changes, and valproate or dopamine agonist toxicity. In addition, camptocormia may be of genetic origin as suggested by reports of families with cerebellar hypoplasia and quadrupedal locomotion, preceded by and associated with camptocormia, mapped to chromosomes 17 and 9. Camptocormia associated with PD usually emerges as the disease progresses, but no factors have been identified that reliably predict which patients with PD will develop this deformity, although it appears to be more common in patients with more severe, postural-instability-gait-difficulty form of PD and if they had prior vertebral surgery. The pathogenesis of this disabling PD feature is not well understood, but the two most frequently proposed mechanisms for camptocormia or head drop in the setting of parkinsonism include dystoniarigidity or extensor truncal myopathy. Some support for the latter mechanism has been provided by reports of electromyography (EMG) and muscle biopsies suggestive of inflammatory, mitochondrial, or some other, nonspecific, myopathic (or neurogenic) changes. The reported findings, however, are inconsistent and conflicting and no consensus about the role of muscle pathology in the pathogenesis of camptocormia has been reached. In this issue, two groups, both from Germany, propose that myopathy of the paravertebral muscles is the primary mechanism for camptocormia and head drop associated with PD. The Kiel investigators, based on their evaluation of 15 PD patients with camptocormia who underwent a variety of evaluations including MRI of the spine, EMG, and muscles biopsy, concluded that ‘‘The cause of camptocormia in idiopathic PD is a focal myopathy.’’ There are several reasons why this conclusion is not warranted: (1) Clinically, the authors provide no evidence of truncal weakness in their patients with camptocormia that would suggest myopathy. In fact, we have never found any evidence of weakness in our patients with camptocormia as the patients are able to extend their trunk against gravity or against resistance on manual muscle testing until the advanced stages when secondary skeletal deformities and contractures may occur. (2) The MRI findings, consisting chiefly of ‘‘edema,’’ are nonspecific and do not confirm myopathy (no MRI studies in a suitable control population are reported). (3) Although the EMG protocol is not described, it does not appear that EMG of the rectus abdominus muscles was performed while the patients were walking, which is the condition under which the contractions of the truncal flexor muscles are usually most prominent. Furthermore, small polyphasic potentials in paravertebral muscles, seen in only 8 of 15 patients, is not sufficient to conclude that myopathy is the primary mechanism for the abnormal truncal flexion. Unfortunately, the authors do not describe EMG findings in a control group of PD patients without camptocormia. (4) The findings on muscle biopsy from 12 patients, such as ‘‘an increase in fiber size spectrum and some hypotrophic and hypertrophic fibers,’’ ‘‘global increase in connective tissue,’’ ‘‘fibrosis’’ are quite nonspecific. Moreover, autopsied subjects who died of vascular disease are clearly not appropriate controls for live patients with chronic PD. Indeed, the criteria for normal muscle histology in paravertebral muscles has never been established, and these muscles have not been carefully examined in a large cohort of PD patients without camptocormia. I also have serious concerns about the interpretation of data by the Berlin group, which included 13 patients with Published online in Wiley InterScience (www.interscience. wiley.com). DOI: 10.1002/mds.23139

65 citations

Journal ArticleDOI
TL;DR: Gait, balance, and postural disabilities are often lumped together under the Postural Instability and Gait Difficulties umbrella term, which may lead to sub-optimal patients’ management and advocate for a multidisciplinary approach from the day of diagnosis.
Abstract: Postural instability and gait difficulties inexorably worsen with Parkinson’s disease (PD) progression and become treatment resistant, with a severe impact on autonomy and quality of life. We review the main characteristics of balance instability, gait disabilities, and static postural alterations in advanced PD, and the available treatment strategies. It remains very difficult to satisfactorily alleviate gait and postural disturbances in advanced PD. Medical and surgical interventions often fail to provide satisfactory or durable alleviation of these axial symptoms, that may actually call for differential treatments. Exercise and adapted physical activity programs can contribute to improving the patients’ condition. Gait, balance, and postural disabilities are often lumped together under the Postural Instability and Gait Difficulties umbrella term. This may lead to sub-optimal patients’ management as data suggest that postural, balance, and gait problems might depend on distinct underlying mechanisms. We advocate for a multidisciplinary approach from the day of diagnosis.

65 citations

Journal ArticleDOI
TL;DR: The authors prefer to offer spinal surgery only to patients with coexisting spinal stenosis causing radiculopathy or myelopathy, and believe that cases involving PD and camptocormia have a high rate of complications when spinal surgery is performed.
Abstract: Object The authors review the literature on the treatment of spinal deformity in patients with Parkinson disease (PD) and formulate a treatment algorithm. Methods The authors provide representative cases of patients with PD and spinal deformity who underwent deep brain stimulation (DBS) or spinal surgery. Results In patients with PD and spinal deformity who undergo spinal surgery there is a high rate of acute and delayed complications. Patients who undergo DBS, while having significantly fewer complications, often do not regain sagittal balance. Conclusions Cases involving PD and camptocormia have a high rate of complications when spinal surgery is performed. The authors prefer to offer spinal surgery only to patients with coexisting spinal stenosis causing radiculopathy or myelopathy. Patients with PD and camptocormia without spinal stenosis may be considered for DBS, but the results are mixed.

65 citations

References
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Journal ArticleDOI
TL;DR: A simplified, scored form of the cognitive mental status examination, the “Mini-Mental State” (MMS) which includes eleven questions, requires only 5-10 min to administer, and is therefore practical to use serially and routinely.

76,181 citations

01 Jan 2002
TL;DR: The Mini-Mental State (MMS) as mentioned in this paper is a simplified version of the standard WAIS with eleven questions and requires only 5-10 min to administer, and is therefore practical to use serially and routinely.
Abstract: EXAMINATION of the mental state is essential in evaluating psychiatric patients.1 Many investigators have added quantitative assessment of cognitive performance to the standard examination, and have documented reliability and validity of the several “clinical tests of the sensorium”.2*3 The available batteries are lengthy. For example, WITHERS and HINTON’S test includes 33 questions and requires about 30 min to administer and score. The standard WAIS requires even more time. However, elderly patients, particularly those with delirium or dementia syndromes, cooperate well only for short periods.4 Therefore, we devised a simplified, scored form of the cognitive mental status examination, the “Mini-Mental State” (MMS) which includes eleven questions, requires only 5-10 min to administer, and is therefore practical to use serially and routinely. It is “mini” because it concentrates only on the cognitive aspects of mental functions, and excludes questions concerning mood, abnormal mental experiences and the form of thinking. But within the cognitive realm it is thorough. We have documented the validity and reliability of the MMS when given to 206 patients with dementia syndromes, affective disorder, affective disorder with cognitive impairment “pseudodementia”5T6), mania, schizophrenia, personality disorders, and in 63 normal subjects.

70,887 citations

Journal ArticleDOI
TL;DR: It is suggested that the introduction of the scales into general hospital practice would facilitate the large task of detection and management of emotional disorder in patients under investigation and treatment in medical and surgical departments.
Abstract: A self-assessment scale has been developed and found to be a reliable instrument for detecting states of depression and anxiety in the setting of an hospital medical outpatient clinic. The anxiety and depressive subscales are also valid measures of severity of the emotional disorder. It is suggested that the introduction of the scales into general hospital practice would facilitate the large task of detection and management of emotional disorder in patients under investigation and treatment in medical and surgical departments.

35,518 citations

Journal Article
TL;DR: The Mini-International Neuropsychiatric Interview is designed to meet the need for a short but accurate structured psychiatric interview for multicenter clinical trials and epidemiology studies and to be used as a first step in outcome tracking in nonresearch clinical settings.
Abstract: The Mini-International Neuropsychiatric Interview (M.I.N.I.) is a short structured diagnostic interview, developed jointly by psychiatrists and clinicians in the United States and Europe, for DSM-IV and ICD-10 psychiatric disorders. With an administration time of approximately 15 minutes, it was designed to meet the need for a short but accurate structured psychiatric interview for multicenter clinical trials and epidemiology studies and to be used as a first step in outcome tracking in nonresearch clinical settings. The authors describe the development of the M.I.N.I. and its family of interviews: the M.I.N.I.-Screen, the M.I.N.I.-Plus, and the M.I.N.I.-Kid. They report on validation of the M.I.N.I. in relation to the Structured Clinical Interview for DSM-III-R, Patient Version, the Composite International Diagnostic Interview, and expert professional opinion, and they comment on potential applications for this interview.

19,347 citations

Journal ArticleDOI
TL;DR: Controversy over the effectiveness of therapeutic measures for parkinsonism is due partially to this wide variability and to the paucity of clinical information about the natural history of the syndrome.
Abstract: PARKINSONISM, described in its entirety over one hundred and fifty years ago,’ rarely presents itself as a diagnostic problem. In consequence, little scrutiny has been directed to the marked variability of this frequently encountered neurological syndrome and to the progression of the disease in large groups of patients. As with most chronic neurological disorders, marked diversity can be expected to exist in age and mode of onset, relative prominence of the cardinal signs and symptoms, rate of progression, and resultant degree of functional impairment. Controversy over the effectiveness of therapeutic measures for parkinsonism is due partially to this wide variability and to the paucity of clinical information about the natural history of the syndrome. It is also re-

11,606 citations