Camptocormia Research Articles

Research Progress of Camptocormia in Parkinson Disease.

Camptocormia, also known as bent spine syndrome, primarily affects individuals with Parkinson disease (PD). This review provides an overview of camptocormia in PD, covering its definition, epidemiology, causes, diagnosis, and treatment. In the epidemiology section, we delve into its prevalence, gender disparities, and ongoing genetic research. Regarding diagnosis and assessment, we discuss evolving diagnostic criteria and measurement techniques, as well as new diagnostic tools. For management and treatment, a wide array of options is available, from conservative methods such as physical therapy and botulinum toxin injections to surgical interventions such as spinal orthopedic surgery and deep brain stimulation. We stress the significance of personalized care and multidisciplinary collaboration. This comprehensive review aims to provide clinicians, researchers, and healthcare professionals with a comprehensive understanding of camptocormia in PD, highlighting its clinical features, diagnostic strategies, management approaches, and future perspectives.

In inflammatory myopathies, dropped head/bent spine syndrome is associated with scleromyositis: an international case–control study

BackgroundSome myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM).ObjectivesTo assess the significance of DH/BS...

Characterizing Camptocormia in Parkinson's Disease Using Muscle Ultrasonography.

Camptocormia (CC) is the forward-bending of the spine of more than 30 degrees that can be found in Parkinson's disease (PD) as a disabling complication. Detection of changes in paraspinal lumbar musculature in CC is of value for choosing treatment strategies. To investigate whether these changes can be detected using muscle ultrasonography (mUSG). Age and sex-matched groups comprised 17 PD patients with CC (seven acute, PD-aCC; 10 chronic PD-cCC), 19 PD patients with no CC, and 18 healthy controls (HC). Lumbar paravertebral muscles (LPM) on both sides were assessed using mUSG by two different raters blinded to the group assignment. Groups were compared with regard to the linear measurements of the muscle thickness as well as semi-quantitative and quantitative (grayscale) analyses of muscle echogenicity using a univariate general linear model. All assessments showed substantial interrater reliability. The PD-cCC group had significantly thinner LPM compared to groups with no CC (PD and HC). Groups of PD-aCC and PD-cCC differed from the groups of no CC in quantitative and semi-quantitative analyses of LPM echogenicity, respectively. Assessment of LPM in PD patients with CC can be reliably performed using mUSG. Also, mUSG may be used as a screening tool to detect CC-related changes in thickness and echogenicity of the LPM in patients with PD.

Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study.

Inaugural axial muscle involvement, defined as dropped head syndrome (DHS) and/or camptocormia (CC), is poorly described in inflammatory myopathies (IM). This study aimed to further characterize IM patients with inaugural DHS/CC, their outcome and care management. This retrospective study included IM patients diagnosed between 2000 and 2021. The main inclusion criterion was IM revealed by axial muscle deficit (DHS/CC). Twenty-seven patients were included; median (IQR) age at first symptoms was 66.0 years (55.5-75.0); 21 were female (77.8%). There were nine IBM, 33.3%, nine overlap myositis (OM, 33.3%), five DM, 18.5%, two immune checkpoint inhibitor-related myositis (7.4%), one focal myositis (3.7%) and one myositis with anti-Hu antibodies (3.7%). Age at first symptoms was ≤70 years in 16 patients (59.3%), including all DM patients and 8/9 OM patients (88.9%). In this group, partial remission of the disease was obtained in 9/16 (56.3%) and complete remission in 1/16 patients (6.3%); regression of DHS/CC was achieved in 3/16 patients (18.8%). Conversely, in the group of 11 patients aged >70 years at first symptoms, there were eight IBM (72.7%). Partial remission was obtained in 5/11 patients (45.5%), the disease was stable in 6/11 patients (54.5%); no complete remission was obtained nor regression of DHS/CC. The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term complications.

Teaching Video Neuro Images : Bent spine syndrome as an early presentation of late-onset Pompe disease

Bent spine syndrome (BSS), an abnormal trunk anteroflexion of at least 45° that increases during walking and abates in supine position, is described in parkinsonism and myopathies.1 We report a 56-year-old man with genetically proven late-onset Pompe disease (LOPD) showing BSS (figure 1 and video 1 at [Neurology.org][1]). He complained of axial weakness that progressively forced him to walk stretching his arms backward since his 20s. Whole-body muscle MRI revealed severe fatty replacement of lumbar paraspinal (figure 2) and iliopsoas muscles, and spirometry showed restrictive ventilatory defect. Periodic acid-Schiff–positive vacuoles and reduced acid α-glucosidase activity were demonstrated in muscle biopsy. While lumbar hyperlordosis is a typical feature of LOPD, other trunk abnormalities may appear early in the disease. LOPD must be included in the differential diagnosis of BSS.2 [1]: http://neurology.org/lookup/doi/10.1212/WNL.0000000000004119

Axial postural abnormalities and pain in Parkinson’s disease

Axialpostural abnormalities and pain are two main determinants of poor quality of life in patients with Parkinson's disease (PD). Indeed, a detailed characterization of pain and other non-motor symptoms in patients with PAs has not been provided yet. The aim of this study is to assess the phenomenology of pain and other non-motor symptoms in PD patients with Pisa syndrome and camptocormia compared to PD patients withoutaxial postural abnormality. Forty-five PD participants were equally distributed in three groups: patients with Pisa syndrome (PS), patients with Camptocormia (CC), and patients without postural abnormalities (PD). Pain characteristics were assessed by Kings Parkinson's Pain Scale (KPPS), brief pain inventory (BPI), and numeric pain rating scale (NRS). All participants completed clinical assessments by non-motor symptom scale (NMSS), and movement disorder society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) parts II-III. Patients with and without axialpostural abnormalities showed one or more types of pain, being fluctuation, nocturnal, chronic, and musculoskeletal the most frequently reported in Pisa Syndrome and camptocormia. PD group compared with PS and CC groups showed differences in the KPPS, NMSS, BPI pain severity and interference, and NRS total scores. No significant differences were found between PS group compared with CC group with exception of the NMSS total scores. PD patients with Pisa syndrome or camptocormia have a higher burden of musculoskeletal, chronic and fluctuation pain than PD patients without axialpostural abnormalities, suggesting different etiologies of pain and possible different treatments.

Visuospatial Deficits Are Associated with Pisa Syndrome and not Camptocormia in Parkinson's Disease.

Pisa syndrome (PS) and camptocormia (CC) are postural abnormalities frequently associated with Parkinson's disease (PD). Their pathophysiology remains unclear, but the role of cognitive deficits has been postulated. To identify differences in the neuropsychological functioning of patients with PD with PS or CC compared with matched patients with PD without postural abnormalities. We performed a case-control study including 57 patients with PD with PS (PS+) or CC (CC+) and 57 PD controls without postural abnormalities matched for sex, age, PD duration, phenotype, and stage. Patients were divided into four groups: PS+ (n=32), PS+ controls (PS-, n=32), CC+ (n=25), and CC+ controls (CC-, n=25). We compared PS+ versus PS- and CC+ versus CC- using a neuropsychological battery assessing memory, attention, executive functions, visuospatial abilities, and language. Subjective visual vertical (SVV) perception was assessed by the Bucket test as a sign of vestibular function; the misperception of trunk position, defined as a mismatch between the objective versus subjective evaluation of the trunk bending angle >5°, was evaluated in PS+ and CC+. PS+ showed significantly worse visuospatial performances (P = 0.025) and SVV perception (P = 0.038) than their controls, whereas CC+ did not show significant differences compared with their control group. Reduced awareness of postural abnormality was observed in >60% of patients with PS or CC. Low visuospatial performances and vestibular tone imbalance are significantly associated with PS but not with CC. These findings suggest different pathophysiology for the two main postural abnormalities associated with PD and can foster adequate therapeutic and prevention strategies.

Postural abnormalities in Asian and Caucasian Parkinson's disease patients: A multicenter study

IntroductionPostural abnormalities (PA) are disabling features of Parkinson's disease (PD). Indirect analyses suggested a higher prevalence of PA among Asian patients compared to Caucasian ones, but no direct comparisons have been performed so far. MethodsAn international, multicenter, cross-sectional study was performed in 6 European and Asian movement disorders centers with the aim to clarify differences and similarities of prevalence and characteristics of PA in Asian vs. Caucasian PD patients. Axial PA, encompassing antecollis (AC), camptocormia (CC), and Pisa syndrome (PS), and appendicular PA (appPA) were systematically searched and analysed in consecutive patients. Results88 (27%) of 326 PD patients had PA (29.1% in Asians and 24.3% in Caucasians, p: 0.331). Prevalence of axial PA was 23.6% in Asians and 24.3% in Caucasians (p = 0.886), in spite of a longer disease duration among Caucasians, but a longer PA duration among Asians. No differences in prevalence between AC, CC, and PS were found between the two ethnicities. The prevalence of appPA was higher in Asians (p = 0.036), but the regression analysis did not confirm a significant difference related to ethnicity. Considering the whole population, male gender (OR, 4.036; 95% CI, 1.926–8.456; p < 0.005), a longer disease duration (OR, 2.61; 95% CI, 1.024–6.653; p = 0.044), and a higher axial score (OR, 1.242; 95% CI, 1.122–1.375; p < 0.0005) were the factors associated with axial PA. ConclusionThe prevalence of axial PA in PD patients is not influenced by ethnicity. However, Asian PD patients tend to develop PA earlier in the disease course, particularly AC.

Relevance Method to Structure of Orthosis

There are many problems when treatment is needed. Such diseases occur in young and old people. The orthosis and their development have lot of problems. One way to solve this problem is to defi ne and apply standard rules and proce-dures throughout the development process. We show in this work is to develop a methodology to structure the orthosis design process. A case study was used to validate the proposed methodology. It was applied to the development of an orthosis to treat a camptocormia – bent spine syndrome. This disorder is char-acterized by the anterofl exion of the trunk and especially affects elderly people and young non-health people with difference disease.

Relevance Method to Structure of Orthosis

There are many problems when treatment is needed. Such diseases occur in young and old people. The orthosis and their development have lot of problems. One way to solve this problem is to define and apply standard rules and procedures throughout the development process. We show in this work is to develop a methodology to structure the orthosis design process. A case study was used to validate the proposed methodology. It was applied to the development of an orthosis to treat a camptocormia - bent spine syndrome. This disorder is characterized by the anteroflexion of the trunk and especially affects elderly people and young non-health people with difference disease.

Do Upper and Lower Camptocormias Affect Gait and Postural Control in Patients with Parkinson's Disease? An Observational Cross-Sectional Study.

Gait impairments and camptocormia (CC) are common and debilitating in patients with Parkinson's disease (PD). Two types of CC affect patients with PD, but no studies investigated their relative contribution in worsening gait and postural control. Therefore, we investigated spatiotemporal gait parameters, gait variability, and asymmetry and postural control in PD patients (Hoehn & Yahr ≤4) with upper CC and lower CC and patients without CC. This observational cross-sectional study involving patients with PD and upper CC (n=16) and lower CC (n=14) and without CC (n=16). The primary outcome measure was gait speed assessed by the GAITRite System. The secondary outcome measures were other spatiotemporal parameters, gait variability, and asymmetry. Postural control and balance were assessed with posturography and the Mini-BESTest. Patients with lower CC showed a higher H&Y stage (p=0.003), a worse PDQ8 (p=0.042), and a lower Mini-BESTest score (p=0.006) than patients with PD without CC. Patients with lower CC showed a reduced gait speed (p=0.012), stride length, and velocity than patients with PD without CC. Upper CC patients showed a higher stride length than lower CC ones (p=0.007). In the eyes open and closed condition, patients with lower CC showed a higher (worse) velocity of CoP displacement in mediolateral direction and length of CoP than patients with PD without CC. No significant between-group differences were measured in gait variability and asymmetry. In conclusion, lower CC was associated with more severe gait and postural control impairment than patients with upper CC and without CC. Categorizing CC based on the bending fulcrum is compulsory to identify patients with the worst performance and to implement specific rehabilitation programs.

Hand, foot, and spine deformities in parkinsonian disorders.

Hand and foot deformities, known as "striatal deformities", and other musculoskeletal abnormalities such as dropped head, bent spine, camptocormia, scoliosis and Pisa syndrome, are poorly understood and often misdiagnosed features of Parkinson's disease and other parkinsonian syndromes. These deformities share some similarities with known rheumatologic conditions and can be wrongly diagnosed as rheumatoid arthritis, osteoarthritis, psoriatic arthritis, Dupuytren's contracture, trigger finger, or other rheumatologic or orthopedic conditions. Neurologists, rheumatologists, and other physicians must be familiar with these deformities to prevent misdiagnosis and unnecessary diagnostic tests, and to recommend appropriate treatment options.

Design Method to Structure Orthosis Design: Camptocormia Postural Brace Case Study.

The orthosis is considered a class 1 medical device which often originates from a nonstructured development process. As these devices are mainly developed by small- and medium-sized enterprises, with no standard research method, the result can be an unadapted device which may not respond to the user's needs and which in the short term may be abandoned. One way to solve this problem is to define and apply standard rules and procedures throughout the development/design process. Although methodologies may solve the “empiricism” in orthosis design problems, these design strategies are not applied during orthosis development due to the particularities of this field and the difficulties in linking the required knowledge and the actors that may be present during the orthosis development. The objective of this work is to develop a methodology to structure the orthosis design process that takes into account both the device life cycle and the different stakeholders involved in the design process. A case study was used to validate the proposed methodology. It was applied to the development of an orthosis to treat a specific postural disorder called camptocormia, also known as bent spine syndrome. This disorder is characterized by the anteroflexion of the trunk and especially affects elderly people. Contrary to scoliosis, the characteristics of camptocormia are not permanent, which means that the patient is able to straighten his posture. A postural brace is used to treat this disorder which enables the patient to redress and maintain the correct upright posture of the trunk.

Subthalamic deep brain stimulation and trunk posture in Parkinson's disease.

We sought to assess the efficacy of subthalamic nucleus deep brain stimulation (STN-DBS) in Parkinson's disease (PD)-associated trunk posture abnormalities retrospectively analyzing data from 101 patients reporting mild-to-severe trunk posture abnormalities of a cohort of 216 PD patients treated with STN-DBS at our center. Abnormal trunk posture was rated on a scale of 0 (normal) to 4 (marked flexion with an extreme abnormality of posture) as per the grading score reported in the Unified Parkinson's Disease Rating Scale. The independent effect of STN-DBS on trunk posture was assessed comparing Medication-Off (presurgery) vs Stimulation-On/Medication-Off (post-surgery). The combined effect of STN-DBS plus levodopa was evaluated comparing Medication-On (presurgery) vs Stimulation-On/Medication-On (post-surgery). Analyses were conducted considering both the entire cohort of patients and the subgroup with camptocormia (CMC) and Pisa syndrome (PS). The independent effect of STN-DBS resulted in a 41.4% improvement in abnormal trunk posture severity (P<.001), with 78.2% of patients (n=79) reporting an improvement of at least 1 point. The combined effect of STN-DBS and levodopa resulted in a 30.9% improvement (P=.061), with 54.5% of patients (n=55) reporting an improvement of at least 1 point. The subanalysis of patients with CMC (n=23) and PS (n=5) showed a 42.7% improvement in abnormal posture severity when considering the independent effect of STN-DBS (P<.001) and 30.5% when considering the combined effect of STN-DBS and levodopa (P<.001). STN-DBS may have the potential for improving posture in patients with advanced PD.

Gait dynamics in Pisa syndrome and Camptocormia: The role of stride length and hip kinematics

This is an observational cross-sectional study evaluating gait dynamics in patients with Parkinson’s Disease (PD) and severe postural deformities, PD without axial deviations and healthy subjects. Ten PS individuals with Pisa syndrome (PS) and nine subjects with Camptocormia (CC) performed 3-D Gait Analysis and were evaluated with walking and balance scales. Correlations with clinical and functional scales were investigated. Spatio-temporal and kinematic data were compared to ten PD subjects without postural deformities (PP) and ten healthy matched individuals (CG). Data obtained showed decreased walking velocity, stride and step length in PP, PS and CC groups compared to controls. The correlation analysis showed that stride and step length were associated with reduced functional abilities and disease severity in PS and CC groups. Kinematic data revealed marked reduction in range of movements (ROMs) at all lower-extremity joints in PS group. While, in CC group the main differences were pronounced in hip and knee joints. PS and CC groups presented a more pronounced reduction in hip articular excursion compared to PP subjects, revealing an increased hip flexion pattern during gait cycle. Moreover, the increased hip and knee flexion pattern adversely affected functional performance during walking tests. Results obtained provide evidence that step length, along with stride length, can be proposed as simple and clear indicators of disease severity and reduced functional abilities. The reduction of ROMs at hip joint represented an important mechanism contributing to decreased walking velocity, balance impairment and reduced gait performance in PD patients with postural deformities.

Bent spine syndrome as the initial symptom of late-onset Pompe disease.

Late-onset Pompe disease (LOPD) is a rare disorder characterized by progressive proximal muscle weakness and early respiratory insufficiency, for which enzyme replacement therapy (ERT) is available. Having diagnosed a case of LOPD presenting with bent spine syndrome, we conducted a brief survey in the French centers involved in management of Pompe disease, from which we collected data on 3 other cases. The patients (3 women and 1 man) had a mean age of 64 years (range 51-77 years) and a delay in diagnosis of approximately 10 years (range 8-42 years). At diagnosis, 3 patients already had respiratory symptoms. All had normal or very mildly raised creatine kinase levels and magnetic resonance imaging abnormalities in the paraspinal muscles. They exhibited the most frequent mutation in Pompe disease (c.-32-13 T>G). Clinicians should be aware of this atypical presentation of LOPD to enable earlier diagnosis and treatment. Muscle Nerve 56: 167-170, 2017.

Camptocormia in Parkinson's Disease: A Muscle Disease Due to Dysregulated Proprioceptive Polysynaptic Reflex Arch.

Camptocormia (from the Greek “kamptein” = to bend and “kormos” = trunk) is an anterior flexion of the thoracolumbar spine while standing, walking, or sitting that disappears in the supine position. The syndrome, also known as “bent spine syndrome,” occurs in nearly 10% of idiopathic Parkinson's disease (iPD) patients (Yoritaka et al., 2013), but also in other neurodegenerative diseases and may occur in myopathies with axial involvement, in all forms of myositis, in dystonia, as a pharmacological side effect or as functional disorder. It causes marked impairment in quality of life and often leads to social isolation. The pathophysiology of camptocormia is as heterogenous as the causes of underlying diseases are, but recent work has provided some insights into the pathophysiology of PD-associated camptocormia and opened options for treatement. These findings show, how important it is to understand the interaction between muscle innervation, Golgi tendon organ and the central nervous system in regulating muscle tone.

A Case of Polymyositis Presenting as Bent Spine Syndrome

Polymyositis (PM) is a subset of idiopathic inflammatory myopathies. The muscles involved with PM are typically proximal and distal limb muscles, but paraspinal muscles are rarely affected. The primary PM clinical symptom is gradual proximal muscle weakness but unusually abnormal trunk posture. Bent spine syndrome (BSS), also referred to camptocormia, is defined as an abnormal flexion of the trunk, appearing in standing position. An idiopathic axial myopathy is the most common cause of primary BSS. A few cases of inflammatory myopathy, a secondary BSS, have been reported. We describe a 59-year–old polymyositis patient with normal finding on an magnetic resonance imaging femur scan who presented with BSS only, myopathic findings on electromyography and elevation of muscle enzymes. (J Rheum Dis 2016;23:261-265)

Camptocormia and deep brain stimulation: The interesting overlapping etiologies and the therapeutic role of subthalamic nucleus-deep brain stimulation in Parkinson disease with camptocormia

Background:Camptocormia is known as “bent spine syndrome” and defined as a forward hyperflexion. The most common etiologic factor is related with the movement disorders, mainly in Parkinson's disease (PD).Case Description:We present the case of a 51-year-old woman who has been followed with PD for the last 10 years, and also under the therapy for PD. An unappreciated correlation low back pain with camptocormia developed. She underwent deep brain stimulation (DBS) in the subthalamic nucleus bilaterally and improved her bending posture.Conclusion:The relationship between the DBS and camptocormia is discussed in this unique condition.

Characterization of morphological trunk changes in camptocormia patients

Camptocormia (kamptes-‘flexion’ + kormos-‘trunk’), or bent spine syndrome as it is also described in the literature, is characterized by anteflexion of the thoraco-lumbar spine in standing, walking...