RESUMEN
Stiff-person syndrome (SPS) usually manifests as an autoimmune neuromuscular disorder characterised by pronounced and advancing rigidity, primarily affecting the trunk and proximal muscles. There are various clinical subtypes like classic SPS (truncal stiffness, generalised rigidity and muscle spasms), partial SPS (stiff-limb syndrome) and uncommon forms including progressive encephalomyelitis with rigidity and myoclonus. Camptocormia, defined as forward flexion of the spine in the upright position that disappears in the supine position, without fixed deformity, has been described only in two cases as an initial presentation of Anti glutamic acid decarboxylase (GAD) autoimmunity. We encountered a young male presenting with a progressive forward-leaning posture and involuntary rhythmic movements in the lower limb. Diagnostic workup included MRI, blood routines, autoimmune screening, genetic testing, lumbar puncture and electromyography. Elevated serum anti-GAD antibody levels, inflammatory CSF and certain other clinical features supported the diagnosis of SPS. Treatment involved benzodiazepines, muscle relaxants and immunotherapy with intravenous immunoglobulin. This case underscores the importance of considering immune-mediated causes, such as SPS, in patients presenting with camptocormia.
Asunto(s)
Glutamato Descarboxilasa , Atrofia Muscular Espinal , Curvaturas de la Columna Vertebral , Síndrome de la Persona Rígida , Humanos , Masculino , Síndrome de la Persona Rígida/inmunología , Síndrome de la Persona Rígida/diagnóstico , Síndrome de la Persona Rígida/complicaciones , Curvaturas de la Columna Vertebral/inmunología , Curvaturas de la Columna Vertebral/complicaciones , Curvaturas de la Columna Vertebral/etiología , Glutamato Descarboxilasa/inmunología , Atrofia Muscular Espinal/diagnóstico , Atrofia Muscular Espinal/complicaciones , Atrofia Muscular Espinal/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/inmunología , Inmunoglobulinas Intravenosas/uso terapéutico , Adulto , Rigidez Muscular/inmunología , Rigidez Muscular/tratamiento farmacológico , Rigidez Muscular/diagnóstico , Electromiografía , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND: 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. OBJECTIVE: To investigate whether these changes can be detected using muscle ultrasonography (mUSG). METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Asunto(s)
Atrofia Muscular Espinal , Enfermedad de Parkinson , Curvaturas de la Columna Vertebral , Humanos , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/diagnóstico por imagen , Reproducibilidad de los Resultados , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/complicaciones , Atrofia Muscular Espinal/complicaciones , Atrofia Muscular Espinal/diagnóstico por imagen , Músculo Esquelético/diagnóstico por imagenAsunto(s)
Atrofia Muscular Espinal , Enfermedades Musculares , Distrofia Miotónica , Curvaturas de la Columna Vertebral , Humanos , Distrofia Miotónica/complicaciones , Enfermedades Musculares/complicaciones , Curvaturas de la Columna Vertebral/diagnóstico , Curvaturas de la Columna Vertebral/etiología , Atrofia Muscular Espinal/complicacionesRESUMEN
BACKGROUND AND PURPOSE: Axial postural abnormalities, mainly involving the spinal deformities, are disabling symptoms of Parkinson's disease (PD). However, the prevalence of axial postural abnormalities in PD and their clinical correlates remain unclear. The present study aimed to conduct a systematic review and meta-analysis of the prevalence of overall and subtypes of axial postural abnormalities in PD. METHODS: PubMed, Embase, Web of Science and Cochrane databases were searched up to 31st March, 2022. We identified studies that reported the prevalence of axial postural abnormalities in PD. The pooled estimate of prevalence was calculated using a random effect model. Subgroup analysis and meta-regression were performed. RESULTS: There were 19 studies met the inclusion criteria. The overall prevalence of axial postural abnormalities in PD was 22.1% (95% CI 19.7-24.5%). The prevalence of each subtype of axial postural abnormalities was 19.6% for scoliosis (95% CI 10.6-28.7%), 10.2% for camptocormia (95% CI 7.7-12.7%), 8% for Pisa syndrome (95% CI 4.7-11.4%), and 7.9% for antecollis (95% CI 3.9-11.9%). Subgroup analysis showed that the measuring method of axial postural abnormalities exerted significant effects on prevalence estimates. Axial postural abnormalities in PD were associated with older age, longer disease duration, higher H-Y stage, greater levodopa equivalent daily dose, more severe motor symptoms, motor fluctuations, and akinetic-rigid subtype. CONCLUSIONS: Axial postural abnormalities, which include scoliosis, camptocormia, Pisa syndrome, and antecollis, are not uncommon in patients with PD. Future research on axial postural abnormalities should be based on uniform diagnostic criteria and measuring methods.
Asunto(s)
Enfermedad de Parkinson , Escoliosis , Curvaturas de la Columna Vertebral , Tortícolis , Humanos , Enfermedad de Parkinson/complicaciones , Escoliosis/complicaciones , Prevalencia , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/complicaciones , Tortícolis/complicaciones , SíndromeRESUMEN
OBJECTIVES: The aim of this review was to specify the potential association between age-related changes in sagittal spinal curvature and risk of pelvic organ prolapse in women. METHODS: A systematic review based on the PRISMA statement was performed. Keywords were chosen according to the eligibility criteria in line with the PICO model. For inclusion, studies had to be based on observational cohorts, case controls and cross sectional studies. The quality of the articles was assessed using the STROBE scale. RESULTS: Five papers from 1996 to 2021 were included in the present review with conflicting results. Four studies reported a positive association between anatomical pelvic organ prolapse at clinical examination, a loss of lumbar lordosis and an increase in thoracic kyphosis. The remaining study, using a questionnaire-based assessment of symptoms, found no association between prolapse-related symptoms and age-related changes in sagittal spinal curvatures. CONCLUSION: Based on the available evidence, this systematic review suggested a very moderate evidence of association between the development of anatomical pelvic organ prolapse and age-related changes in sagittal spinal curvature in women. This review remains very limited by the small number and the heterogeneity of the studies included. Further studies in the lifetime continunm, with high quality methodology are needed to better understand the physiopathology of pelvic organ prolapse.
Asunto(s)
Cifosis , Lordosis , Prolapso de Órgano Pélvico , Curvaturas de la Columna Vertebral , Anciano , Estudios Transversales , Femenino , Humanos , Lordosis/etiología , Prolapso de Órgano Pélvico/etiología , Curvaturas de la Columna Vertebral/etiologíaAsunto(s)
Antipsicóticos/efectos adversos , Distonía/inducido químicamente , Atrofia Muscular Espinal/etiología , Recto del Abdomen/efectos de los fármacos , Esquizofrenia/tratamiento farmacológico , Curvaturas de la Columna Vertebral/etiología , Distonía/complicaciones , Distonía/diagnóstico por imagen , Femenino , Humanos , Persona de Mediana Edad , Atrofia Muscular Espinal/diagnóstico por imagen , Recto del Abdomen/diagnóstico por imagen , Curvaturas de la Columna Vertebral/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
This study aimed to investigate whether fat infiltration in lumbar paravertebral muscles assessed by magnetic resonance imaging (MRI) could be related to dynamic sagittal spino-pelvic balance during gait in adult spinal deformity (ASD). This is a retrospective analysis of 28 patients with ASD. The fat infiltration rate of lumbar erector spinae muscles, multifidus muscles and psoas major muscles was measured by T2 weighted axial MRI at L1-2 and L4-5. Dynamic sagittal spinal and pelvic angles during gait were evaluated using 3D motion analysis. The correlation between fat infiltration rate of those muscles with variations in dynamic kinematic variables while walking and static radiological parameters was analyzed. Spinal kyphosis and pelvic anteversion significantly increased during gait. Fat infiltration rate of erector spinae muscles at L1-2 was positively correlated with thoracic kyphosis (r = 0.392, p = 0.039) and pelvic tilt (r = 0.415, p = 0.028). Increase of spinal kyphosis during walking was positively correlated with fat infiltration rate of erector spinae muscles both at L1-2 (r = 0.394, p = 0.038) and L4-5 (r = 0.428, p = 0.023). Qualitative evaluation of lumbar erector spinae muscles assessed by fat infiltration rate has the potential to reflect dynamic spino-pelvic balance during gait.
Asunto(s)
Marcha , Región Lumbosacra/patología , Imagen por Resonancia Magnética , Músculos Paraespinales/patología , Enfermedades de la Columna Vertebral/etiología , Enfermedades de la Columna Vertebral/fisiopatología , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Humanos , Cifosis/diagnóstico , Cifosis/etiología , Cifosis/fisiopatología , Pelvis/fisiopatología , Curvaturas de la Columna Vertebral/diagnóstico , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/fisiopatología , Enfermedades de la Columna Vertebral/diagnósticoAsunto(s)
Alargamiento Óseo/métodos , Escoliosis , Fusión Vertebral/métodos , Tracción , Cuidados Posteriores , Niño , Progresión de la Enfermedad , Femenino , Humanos , Fijadores Internos , Limitación de la Movilidad , Osteogénesis Imperfecta/complicaciones , Osteogénesis Imperfecta/diagnóstico , Osteogénesis Imperfecta/fisiopatología , Radiografía Torácica/métodos , Escoliosis/diagnóstico , Escoliosis/etiología , Curvaturas de la Columna Vertebral/diagnóstico por imagen , Curvaturas de la Columna Vertebral/etiología , Tracción/instrumentación , Tracción/métodosRESUMEN
La camptocormia y el sídrome de Pisa (pleurothotonus) se caracteriza por una postura anormal en el tronco, con una flexión de la columna toracolumbar de entre 15-45° durante la bipedestación y la marcha que se corrige completamente con el decúbito supino. Se asocia a la enfermedad de Parkinson y a otras enfermedades neurodegenerativas, siendo su fisiopatología incierta. Se presentan 3 casos de camptocormia secundaria a antipsicóticos atípicos (AU)
Camptocormia is an involuntary flexion between 15-45° of the thoracolumbar spine when standing, walking or sitting, which disappears completely in the supine position. Is associated to Parkinson's disease and other neurodegenerative conditions, being its physiopatology unknown. We report three cases of atypical anti-psychotic induced camptocormia (AU)
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Curvaturas de la Columna Vertebral/diagnóstico por imagen , Curvaturas de la Columna Vertebral/etiología , Antipsicóticos/efectos adversos , Curvaturas de la Columna Vertebral/cirugíaRESUMEN
BACKGROUND: Camptocormia is common in patients with multiple system atrophy (MSA). The current study was aimed at assessing the frequency of camptocormia and its related factors in MSA patients with different disease durations. Also, the impact of camptocormia on disability was evaluated. METHODS: A total of 716 patients were enrolled in the study. They were classified into three groups based on disease duration (≤ 3, 3-5, ≥ 5 years). Specific scales were used to evaluate the motor and non-motor symptoms. Disease severity was assessed using the Unified Multiple System Atrophy Rating Scale (UMSARS). The binary logistic regression model was used to explore the factors related to camptocormia. To analyze the impact of camptocormia on disability in patients with disease duration less than 5 years, propensity score matching (PSM) and stratified Cox regression analysis were used. RESULTS: In the current study, we found that the frequency of camptocormia was 8.9, 19.7 and 19.2% when the disease duration was ≤3, 3-5, ≥ 5 years, respectively. In the disease duration ≤3 years group, we found that MSA-parkinsonian subtype (MSA-P) (OR = 2.043, P = 0.043), higher total UMSARS score (OR = 1.063, P < 0.001), older age of onset (OR = 1.047, P = 0.042), and lower score on the frontal assessment battery (FAB) (OR = 0.899, P = 0.046) were associated with camptocormia. Only greater disease severity was associated with camptocormia in the group of patients with disease duration 3-5 years (OR = 1.494, P = 0.025) and in the group of patients with disease duration ≥5 years (OR = 1.076, P = 0.005). There was no significant impact of camptocormia on disability in patients with a disease duration of < 5 years (HR = 0.687, P = 0.463). CONCLUSION: The frequency of camptocormia increased with prolonged disease duration. Disease severity was related to camptocormia at different stages of the disease. The MSA-P subtype, older age of onset, and lower FAB score were associated with camptocormia in the early stage of the disease.
Asunto(s)
Atrofia de Múltiples Sistemas/complicaciones , Atrofia Muscular Espinal/etiología , Curvaturas de la Columna Vertebral/etiología , Edad de Inicio , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Atrofia Muscular Espinal/epidemiología , Factores de Riesgo , Índice de Severidad de la Enfermedad , Curvaturas de la Columna Vertebral/epidemiologíaRESUMEN
ABSTRACT: Trunk pain is a common cause of performance limitation and time away from sport in athletes. However, atraumatic trunk injuries are underrepresented in medical literature and underrecognized clinically. Delays in diagnosis and initiation of appropriate treatment can increase injury morbidity and return-to-play time. Currently, evidence-based guidelines for diagnosis and treatment of trunk pain in athletes are limited. Thus, we provide an overview of atraumatic sport-related injuries to the thoracic spine (disc herniation, scoliosis, kyphosis), ribcage (bone stress injury, costochondritis, Tietze syndrome, slipping rib syndrome, costovertebral or costotransverse joint dysfunction), and chest and abdominal wall musculature (intercostal, serratus anterior, oblique strains, regional myofascial pain), highlighting sport-specific biomechanical considerations. We aim to increase awareness of these causes of trunk pain among sports medicine providers in an effort to guide diagnostic and treatment recommendations that will ultimately improve overall musculoskeletal health in athletes.
Asunto(s)
Traumatismos en Atletas , Torso/lesiones , Traumatismos en Atletas/diagnóstico , Traumatismos en Atletas/etiología , Traumatismos en Atletas/fisiopatología , Traumatismos en Atletas/terapia , Fenómenos Biomecánicos , Humanos , Músculo Esquelético/lesiones , Dolor/etiología , Costillas/anatomía & histología , Costillas/lesiones , Curvaturas de la Columna Vertebral/diagnóstico , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/terapia , Esguinces y Distensiones/diagnóstico , Esguinces y Distensiones/etiología , Esguinces y Distensiones/terapia , Vértebras Torácicas/anatomía & histología , Vértebras Torácicas/lesiones , Torso/fisiopatologíaRESUMEN
BACKGROUND: The sagittal curvature of the spine is hypothesized to play an important role in induction of spinal deformities in adolescent idiopathic scoliosis. We previously showed an S shaped flexible rod, with the same curvature as the pediatric sagittal spinal curve, produces scoliotic-like deformities under physiologic loading. Yet, detailed characteristics of the pediatric sagittal spinal curves associated with higher risk of scoliosis are not well defined. METHODS: A total of 32 patients in a population with a high prevalence of idiopathic-like scoliosis, 22q11.2 deletion syndrome (22q), were included and followed up for at least two-years. We developed a reduced order finite element model (FEM) of the sagittal profile of these 32 patients where the spine was modeled as an S shaped elastic rod. We related the geometrical parameters of the sagittal curves and the deformed FEM of the corresponding S shaped rods to the risk of scoliosis development at two-year follow-up in this cohort. RESULTS: Variations in the sagittal curvature in the cohort of 22q patients resulted in five different deformity patterns shown by finite element analyses. Two sagittal plane deformity pattern groups had high rate of scoliosis development (86% and 100%) whereas the other 3 groups had less than 50% rate of scoliosis development (40%, 33%, and 0%). The pre-scoliotic position of the inflection point (where lordosis turns into kyphosis), the ratio of the spinal curvatures above and below the inflection point, and the length of the spinal curve above and below the inflection point were significantly different between the five deformity patterns groups, p < 0.05. CONCLUSION: Combination of geometrical parameters of the sagittal profile prior to onset of scoliosis can relate to the development of spinal deformity in pediatric population.
Asunto(s)
Cifosis , Lordosis , Escoliosis , Curvaturas de la Columna Vertebral , Adolescente , Niño , Humanos , Cifosis/diagnóstico por imagen , Cifosis/etiología , Escoliosis/diagnóstico por imagen , Curvaturas de la Columna Vertebral/diagnóstico por imagen , Curvaturas de la Columna Vertebral/etiología , Columna Vertebral/diagnóstico por imagenRESUMEN
Camptocormia is an involuntary flexion between 15-45° of the thoracolumbar spine when standing, walking or sitting, which disappears completely in the supine position. Is associated to Parkinson's disease and other neurodegenerative conditions, being its physiopatology unknown. We report three cases of atypical anti-psychotic induced camptocormia.
Asunto(s)
Atrofia Muscular Espinal , Monjas , Enfermedad de Parkinson , Curvaturas de la Columna Vertebral , Humanos , Atrofia Muscular Espinal/tratamiento farmacológico , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/tratamiento farmacológico , Curvaturas de la Columna Vertebral/etiologíaRESUMEN
BACKGROUND: Deep brain stimulation of the subthalamic nucleus (STN-DBS) has been reported to be effective for camptocormia in Parkinson's disease (PD). However, the association between clinical effectiveness and the stimulated volumes or structural connectivity remains unexplored. OBJECTIVE: To investigate the effectiveness of STN-DBS for treating camptocormia in PD and its association with volumes of tissue activated (VTA) and structural connectivity. METHODS: We reviewed video recordings of patients who had undergone STN-DBS. The total and upper camptocormia (TCC and UCC) angles were measured to quantify changes in camptocormia. The Movement Disorders Society Unified Parkinson's Disease Rating Scale III (MDS-UPDRS III) was used to assess motor symptoms. Pre- and postoperative brain images were collected for modeling volume of VTA and structural connectivity using Lead-DBS software. RESULTS: Participants included 36 patients with PD (8 with TCC-camptocormia and 2 with UCC-camptocormia) treated with bilateral STN-DBS. After surgery, patients showed a significant improvement in postural alignment at follow-up (mean follow-up duration: 6.0±2.2 months). In the entire sample, higher structural connectivity to the right supplementary motor area (SMA) and right lateral premotor cortex along the dorsal plane (PMd) was associated with larger postsurgical improvements in axial signs and TCC angles after stimulation was turned on. In patients diagnosed with camptocormia, larger improvement in camptocormia angles after STN-DBS was associated with a larger VTA overlap with STN (Râ=â0.75, pâ=â0.032). CONCLUSION: This study suggests that both VTA overlap with STN and structural connectivity to cortical motor regions are associated with the effectiveness of STN-DBS for managing camptocormia in PD.
Asunto(s)
Estimulación Encefálica Profunda , Corteza Motora/patología , Atrofia Muscular Espinal/terapia , Red Nerviosa/patología , Evaluación de Resultado en la Atención de Salud , Enfermedad de Parkinson/terapia , Curvaturas de la Columna Vertebral/terapia , Núcleo Subtalámico , Adulto , Anciano , Femenino , Estudios de Seguimiento , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Corteza Motora/diagnóstico por imagen , Atrofia Muscular Espinal/etiología , Atrofia Muscular Espinal/patología , Atrofia Muscular Espinal/fisiopatología , Red Nerviosa/diagnóstico por imagen , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/patología , Enfermedad de Parkinson/fisiopatología , Índice de Severidad de la Enfermedad , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/patología , Curvaturas de la Columna Vertebral/fisiopatologíaRESUMEN
BACKGROUND: Parkinson's disease (PD) is the second most common degenerative neurologic disorder in older adults, and increasing attention has been paid to bone health in PD. Although several studies have shown that patients with PD have a lower bone mineral density (BMD) than do non-PD controls, there have been no systematic reviews in recent years. METHODS: PubMed, Medline, and Web of Science were used to search relevant studies up to May 2020. BMD, BMD T score, and BMD Z score of patients with and without PD were statistically analyzed. Meta-analysis was conducted using Review Manager version 5.3. RESULTS: This meta-analysis included 17 studies comprising 10,289 individuals. In the meta-analysis, adults with PD had lower total body, total hip, total radius, lumbar spine, total femur, femur neck, right-hand, and left-hand BMD than did non-PD controls. The T score of total body BMD, total hip BMD, total radius BMD, lumbar spine BMD, L1-L4 spine BMD, total femur BMD, and femur neck BMD in adults with PD were lower than those in non-PD controls. Futhermore, the Z score of total body BMD, total hip BMD, total radius BMD, lumbar spine BMD, L1-L4 spine BMD, and femur neck BMD was lower in adults with PD than in non-PD controls. CONCLUSIONS: Patients with PD had a lower BMD, BMD T score, and BMD Z score compared with non-PD controls. Therefore, clinicians should routinely monitor BMD of patients with PD to prevent falling and fragility fractures in older adults and optimize BMD before surgical treatment of severe spinal deformity caused by PD.
Asunto(s)
Densidad Ósea , Osteoporosis/diagnóstico por imagen , Enfermedad de Parkinson/fisiopatología , Accidentes por Caídas/prevención & control , Enfermedades Óseas Metabólicas/complicaciones , Enfermedades Óseas Metabólicas/diagnóstico por imagen , Estudios de Casos y Controles , Humanos , Osteoporosis/complicaciones , Fracturas Osteoporóticas/prevención & control , Enfermedad de Parkinson/complicaciones , Curvaturas de la Columna Vertebral/etiología , Curvaturas de la Columna Vertebral/cirugíaRESUMEN
Magnetically controlled growing rods (MCGR) are commonly implanted for the treatment of early-onset scoliosis. While most authors report favorable short-term results, little is known about long-term deformity correction. This prospective cohort study assesses spinal deformity control in a homogeneous spinal muscular atrophy (SMA) patient group treated with MCGR implants, a standardized lengthening protocol and a minimum follow-up of four years. 17 SMA patients with progressive scoliosis were treated with MCGR implanted parallel to the spine with rib-to-pelvis fixation. Radiologic measurements were performed before and after MCGR implantation and during external lengthening procedures. These included measurements of the scoliotic curve, kyphosis, lordosis, pelvic obliquity and the spinal length. Additional clinical data of the complications were also analyzed. 17 children (mean age 7.4 years) were surgically treated and underwent a total of 376 lengthenings. Complication rates were 3.5% in respect to all interventions or 41% of the patients had complications during 3.5% of the lengthening sessions. The initial implantation significantly reduced the main scoliotic curve by 59%, with the correction remaining constant throughout the follow-up. Pelvic obliquity was also significantly and permanently corrected by 72%, whereas kyphosis and lordosis were not influenced. The spinal length could be significantly increased mostly during the first year of treatment. Bilateral implantation of MCGRs for correction of spinal deformity in children with SMA showed no decrease of the lengthening potential during a four-year follow-up. Therefore, the previously described 'law of diminishing returns' could not be applied to this patient population.Level of Evidence/Clinical relevance: Therapeutic Level IV.