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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: Despite prolonged postoperative courses, including a high complication rate and the need for multiple revisions, both patients benefited from the procedures and the authors report two cases of camptocormia in Parkinson's disease treated with spinal corrective surgery.
Abstract: Camptocormia or 'bent spine syndrome' is a rare manifestation of Parkinson's disease. The postural deformity can be a great source of disability. Camptocormia is typically not responsive to dopaminergic medication. Results with deep brain stimulation to treat camptocormia have been mixed but generally poor. The authors report two cases of camptocormia in Parkinson's disease treated with spinal corrective surgery. Despite prolonged postoperative courses, including a high complication rate and the need for multiple revisions, both patients benefited from the procedures.

45 citations

Journal ArticleDOI
TL;DR: Camptocormia in connection with primary myopathies should be treated according to the underlying illness, and case reports have shown botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective.
Abstract: Camptocormia is a disabling pathological, non-fixed, forward bending of the trunk. The clinical definition using only the bending angle is insufficient; it should include the subjectively perceived inability to stand upright, occurrence of back pain, typical individual complaints, and need for walking aids and compensatory signs (e.g. back-swept wing sign). Due to the heterogeneous etiologies of camptocormia a broad diagnostic approach is necessary. Camptocormia is most frequently encountered in movement disorders (PD and dystonia) and muscles diseases (myositis and myopathy, mainly facio-scapulo-humeral muscular dystrophy (FSHD)). The main diagnostic aim is to discover the etiology by looking for signs of the underlying disease in the neurological examination, EMG, muscle MRI and possibly biopsy. PD and probably myositic camptocormia can be divided into an acute and a chronic stage according to the duration of camptocormia and the findings in the short time inversion recovery (STIR) and T1 sequences of paravertebral muscle MRI. There is no established treatment of camptocormia resulting from any etiology. Case series suggest that deep brain stimulation (DBS) of the subthalamic nucleus (STN-DBS) is effective in the acute but not the chronic stage of PD camptocormia. In chronic stages with degenerated muscles, treatment options are limited to orthoses, walking aids, physiotherapy and pain therapy. In acute myositic camptocormia an escalation strategy with different immunosuppressive drugs is recommended. In dystonic camptocormia, as in dystonia in general, case reports have shown botulinum toxin and DBS of the globus pallidus internus (GPi-DBS) to be effective. Camptocormia in connection with primary myopathies should be treated according to the underlying illness.

42 citations


Cites background from "Parkinson’s disease with camptocorm..."

  • ...A relevant number of PD camptocormia patients present with additional laterodeviation [8, 10, 31], but there are no accepted criteria of how to differentiate the combination of Pisa syndrome and camptocormia....

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  • ...In the majority of patients, treatment with L-dopa gives only minimal or no improvement of camptocormia in PD [6, 8, 19]....

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  • ...While inflammatory camptocormia usually shows a more rapid course over weeks to months, there are basically two patterns of camptocormia in PD: A more rapid one and one with symptoms worsening even after more than one year [8, 11, 12, 22, 105]....

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Book ChapterDOI
TL;DR: This chapter reviews the pathophysiological mechanisms and the clinical features of motor manifestations of Parkinson's disease (PD) and can greatly assist in the diagnosis of early PD, as the rigidity is ipsilateral to the rest tremor, if present.
Abstract: Publisher Summary This chapter reviews the pathophysiological mechanisms and the clinical features of motor manifestations of Parkinson's disease (PD). The most typical and easily recognized symptom of PD is unilateral, 4–6 Hz, rest tremor. This is differentiated from the typical 5–8 Hz postural tremor of essential tremor (ET), enhanced physiologic tremor (8–12 Hz), and cerebellar outflow tremor (2–5 Hz). The rest tremor characteristically disappears with action (a feature differentiating it from ET) and during sleep. Stimulation of the subthalamic nucleus (STN), which is typically hyperactive in PD, can also normalize the amplitude and frequency of PD tremor towards physiologic ranges. In PD, the rigidity is usually accompanied by a “cogwheel” phenomenon, probably a manifestation of underlying tremor. Rigidity often increases with reinforcing maneuvers such as voluntary movements of the contralateral limb. This examination technique can greatly assist in the diagnosis of early PD, as the rigidity is ipsilateral to the rest tremor, if present.

42 citations

Journal ArticleDOI
TL;DR: The aim of this work was to investigate the prevalence of camptocormia and the clinical characteristics of patients with camptOCormia in a large population of PD patients.
Abstract: Objective: The aim of this work was to investigate the prevalence of camptocormia and the clinical characteristics of patients with camptocormia in a large population of PD patients. Background: Although camptocormia has been recognized as a prominent phenomenon in PD, the previous epidemiological reports were limited, especially in terms of sample size. Methods: We evaluated 531 PD patients (disease duration: 7.0 ± 5.5 years, mean ± standard deviation). We examined their clinical features and the prevalence of camptocormia. Results: Camptocormia was detected in 22 patients (4.1%) and found in patients who were older and had more severe motor symptoms and a higher levodopa (L-dopa) dose (P < 0.05), compared to the patients without camptocormia. Patients with camptocormia showed significantly higher frequencies of autonomic symptoms, such as constipation and urinary incontinence (P < 0.05). Conclusions: Camptocormia is uncommon in PD and is associated with disease severity, higher L-dopa dose and higher frequencies of autonomic symptoms. © 2011 Movement Disorder Society

39 citations

Journal ArticleDOI
TL;DR: PD with camptocormia may represent a selective form of PD in which a specific neuronal dysfunction possibly occurs within the brainstem and a significant negative correlation between the severity of the symptoms and the normalized brain volume was found.
Abstract: Camptocormia is characterized by an excessive anterior flexion of the spine that appears only when standing or walking. The origin of this symptom remains unknown, but recent clinical reports of camptocormia associated with Parkinson's disease (PD) suggest cerebral involvement in the pathogenesis of camptocormia. This study was undertaken to investigate the hypothesis that camptocormia in PD has a central origin. Seventeen PD patients with camptocormia were prospectively enrolled and were compared to 10 matched PD patients without camptocormia and 12 normal controls. The normalized volumes of the brain, striatal nuclei, and the cross-sectional areas of the midbrain and pons were measured on three-dimensional magnetic resonance imaging. Data were correlated with the severity of the symptoms. The normalized axial surface of the midbrain was statistically smaller in PD patients with camptocormia than in normal controls (P = 0.01). The normalized volumetric data were not statistically different in PD patients with camptocormia. There was a significant negative correlation between the severity of camptocormia and the normalized brain volume (P < 0.009; R = -0.649) and sagittal pons area (P < 0.01; R = -0.642). The results suggest that PD with camptocormia may represent a selective form of PD in which a specific neuronal dysfunction possibly occurs within the brainstem.

38 citations


Cites background or result from "Parkinson’s disease with camptocorm..."

  • ...Therefore, 17 patients who met stringent selection criteria for our clinical study of PD associated with camptocormia [8] were prospectively enrolled in this MR imaging protocol....

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  • ...This finding is consistent with previous studies suggesting that a neuronal dysfunction or additional damage to brainstem structures implicated in the control of posture—the pedunculopontine nucleus [8, 34] or the reticulospinal pathway [9]—may account for camptocormia in PD patients....

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  • ...Therefore, we suggest that—except for the coincidental occurrence of neuromuscular disease [8, 12]—fatty involution observed...

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  • ...Finally, although a sample size effect cannot be ruled out, the fact that brainstem MR volumetry fails to detect any significant differences between PD with and without camptocormia is in agreement with the hypothesis that PD with and without camptocormia may represent a spectrum of the same disease [8]....

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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

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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