OBJECTIVE: To compare postural stability, spinal alignment, mobility, and postural competency in women with unilateral lower extremity lymphedema after radical hysterectomy following gynecologic cancer with a matched control group.
METHODS: Twenty-seven women with unilateral lower extremity lymphedema (lymphedema group, age: 54.14 ± 5.80 years) and 30 healthy women (control group, age: 51.90 ± 6.54 years) were included. The lymphedema severity was evaluated with circumferential measurements. Postural stability with the Biodex Balance System SD and the spinal alignment, mobility, and postural competency with the Spinal Mouse device were assessed.
RESULTS: In the lymphedema group, it was found that 3.7% of the women had mild lymphedema, 7.4% had moderate lymphedema, and 88.9% had severe lymphedema. Static eyes open (EO) (overall, medio-lateral and antero-posterior) and eyes closed (EC) (antero-posterior) stability scores and dynamic EO and EC stability scores (overall and antero-posterior) were detected to be higher in the lymphedema group than in the controls (p < 0.05). Spinal mobility and postural competency scores were lower in the lymphedema group than in the control group (p < 0.05). In other parameters, there were no significant differences between the groups (p > 0.05).
CONCLUSIONS: Decreased postural stability, spinal mobility, and postural competency were detected in women with unilateral lower extremity lymphedema; however, no difference was seen in spinal alignment. These changes should be taken into account in the assessment and the treatment of unilateral lower extremity lymphedema.

The objective of this paper is to explore the postural effect of galvanic vestibular stimulation (GVS) in camptocormia. It is a retrospective case series of 7 Parkinson disease and camptocormia patients. Binaural monopolar GVS was administered. Main outcome variable was trunk forward flexion (TFF), assessed pre and immediately post GVS, and one month after. The TFF was assessed in the standing position, with open and closed eyes. The Berg balance scale was assessed before and 1 month after GVS. The TFF immediately after the GVS showed a reduction of 2.3 cm (p = 0.091) in the first reading, and after a 1-min stance (p = 0.025). Four of 5 patients maintained or even improved this effect a month after (p = 0.082). No changes in Berg balance scale were observed. Our results suggest an acute improvement in posture after one session of GVS, which significantly decreased TFF. Most of the results had a marginal significance due to small sample size.

BACKGROUND Filamin C (FLNC) is an actin crosslinking protein that provides structural support for the sarcomere. The exact function of FLNC is unknown; however, mutations have been reported in myopathies and cardiomyopathies, but rarely both. In this paper, we describe a case of adult-onset camptocormia, proximal myopathy, and cardiomyopathy and an intronic FLNC mutation. CASE REPORT A 56-year-old man was referred to the neurology clinic for truncal weakness. The patient reported having curvature of his spine, which he said his mother also had prior to her dying suddenly due to a \"cardiac issue.\" The patient was found to have fatty infiltration of the periscapular and paraspinal muscles. Additionally, electromyography revealed irritable myopathy of the paraspinal muscles, and an echocardiogram revealed an ejection fraction of 40%. A genetic panel conducted through PerkinElmer Genomics revealed a heterozygous mutation c.1210+3A>G in the intron region of FLNC. Due to his low ejection fraction and family history of sudden cardiac death, he received an implantable cardioverter-defibrillator and began carvedilol. The patient received physical therapy for camptocormia. CONCLUSIONS The variability in genotypic-phenotypic relationships of FLNC mutations is a growing area of research. It is important to increase awareness to further the development of gene-targeted therapies. We hope this unique clinical presentation of co-occurring skeletal and cardiomyopathy secondary to an intronic mutation will increase awareness of the broad phenotypic spectrum of FLNC mutations.

OBJECTIVE: Pelvic fixation with S2-alar-iliac (S2AI) screws is an established technique in adult deformity surgery. The authors\' objective was to report the incidence and risk factors for an underreported acute failure mechanism of S2AI screws.
METHODS: The authors retrospectively reviewed a consecutive series of ambulatory adults with fusions extending 3 or more levels, and which included S2AI screws. Acute failure of S2AI screws was defined as occurring within 6 months of the index surgery and requiring surgical revision.
RESULTS: Failure occurred in 6 of 125 patients (5%) and consisted of either slippage of the rods or displacement of the set screws from the S2AI tulip head, with resultant kyphotic fracture. All failures occurred within 6 weeks postoperatively. Revision with a minimum of 4 rods connecting to 4 pelvic fixation points was successful. Two of 3 (66%) patients whose revision had less fixation sustained a second failure. Patients who experienced failure were younger (56.5 years vs 65 years, p = 0.03). The magnitude of surgical correction was higher in the failure cohort (number of levels fused, change in lumbar lordosis, change in T1-pelvic angle, and change in coronal C7 vertical axis, each p < 0.05). In the multivariate analysis, younger patient age and change in lumbar lordosis were independently associated with increased failure risk (p < 0.05 for each). There was a trend toward the presence of a transitional S1-2 disc being a risk factor (OR 8.8, 95% CI 0.93-82.6). Failure incidence was the same across implant manufacturers (p = 0.3).
CONCLUSIONS: All failures involved large-magnitude correction and resulted from stresses that exceeded the failure loads of the set plugs in the S2AI tulip, with resultant rod displacement and kyphotic fractures. Patients with large corrections may benefit from 4 total S2AI screws at the time of the index surgery, particularly if a transitional segment is present. Salvage with a minimum of 4 rods and 4 pelvic fixation points can be successful.

暂无摘要。

This study was aimed at describing a case series of brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc), due to its rarity and limited coverage in published data. Another aim was to provide a literature review. We reported four cases of BCIM-SSc from our tertiary center. In addition, we researched the literature and found six articles featuring 17 patients who fit this phenotype. We pooled all cases and reported their features. Most patients were female and had limited SSc, and the median time of BCIM presentation was three years after SSc diagnosis. Asymmetric muscle involvement, scapular winging, dropped head, axial weakness, camptocormia, dysphagia, and dermatomyositis stigmas were common features. All patients had esophageal involvement. Most had positive antinuclear antibody results, a scleroderma pattern in their capillaroscopy images, elevated serum creatine phosphokinase, myopathic electrophysiology, and muscle involvement in magnetic resonance imaging. Muscle histopathological findings varied widely, but in general all showed the presence of lymphoid infiltrates, muscle atrophy, increased MHC-I expression, MAC deposits, vasculopathy, and muscle fiber necrosis. The response to immunosuppressive therapy was highly irregular. BCIM-SSc is a rare disorder that shares many similar phenotypes among the described cases, but has a highly heterogeneous response to treatment. At present, more data on the physiopathology, clinical features, and treatment is still needed.

BACKGROUND: Camptocormia has been reported in a plethora of diseases comprising disorders of the central nervous system, the peripheral nervous system, and the neuromuscular junction as well as hereditary and acquired myopathies. In sporadic late onset nemaline myopathy concomitant axial myopathy is common, but reports about camptocormia as the only presenting symptom in this condition are very rare. Notably, sporadic late onset nemaline myopathy is a potentially treatable condition in particular when associated with monoclonal gammopathy of unknown significance, HIV or rheumatological disorders.
METHODS: We report the case of a 62-year-old female patient, who presented with slowly progressive camptocormia. Comprehensive work-up including neurological work-up, laboratory tests, MR-imaging, muscle biopsy and genetic testing led to the diagnosis of sporadic late onset nemaline myopathy.
CONCLUSIONS: Our case report highlights that sporadic late onset nemaline myopathy has to be considered in patients presenting with isolated camptocormia and comprehensive work-up of camptocormia is mandatory to ascertain the individual diagnosis, especially in consideration of treatable conditions.

Pectus excavatum and scoliosis are associated conditions with a high rate of coincidence. However, there are no reports to guide surgeons on the management of adolescents with moderate scoliosis and pectus excavatum, because there are conflicting conclusions in the literature regarding how the Nuss procedure with substernal bar affects scoliosis.
In 2017, we encountered two patients with moderate scoliosis treated with a spinal orthosis. After undergoing the Nuss procedure for pectus excavatum, their scoliosis acutely progressed into surgical magnitude requiring posterior instrumented spinal fusion. The first patient progressed 26° despite the pre-Nuss radiographs showing him to be Risser 4/5, while the second patient also progressed 26° from the Nuss procedure. Both patients acknowledged noncompliance with brace wear because of discomfort after the Nuss procedure. However, their progression rate still doubles the rate of reported rapid accelerators, indicating that a significant component of curve progression is directly attributed to forces on the spine from the corrective maneuver with substernal bar.
The purpose of this case report is to describe the features of these two patients to help with clinical decision-making in patients with moderate scoliosis (curves >25°) who are contemplating the Nuss procedure for correction of pectus excavatum. We caution patients and providers that spinal deformity could worsen with surgical intervention of the pectus excavatum via the Nuss procedure and necessitate scoliosis surgery.

Camptocormia is a rare and disabling movement disorder resulting in forward bending of the trunk. Camptocormia has many etiologies, although it is frequently observed patients with in Parkinson\'s disease and dystonia. Deep brain stimulation(DBS)of the globus pallidus internus(GPi)and subthalamic nucleus effectively treats camptocormia in Parkinson\'s disease and dystonia patients. Herein, we report a case of dystonic camptocormia induced by repetitive abdominal muscle exercise in which treatment was administered using bilateral GPi-DBS. A 54-year-old woman developed dystonic camptocormia at 53 years of age. Prior to the onset of symptoms, she regularly performed 200 abdominal muscle exercises per day. Oral medications, and botulinum toxin and lidocaine injections, were ineffective. Truncal anterior bending occurred while standing and walking. The patient underwent bilateral GPi-DBS, which instantly and dramatically improved her symptoms. The Burke-Fahn-Marsden dystonia rating scale subscore for the trunk before and after bilateral pallidotomy was 6 and 0, respectively. No perioperative adverse events were observed. Symptomatic relief persisted for 2 years. This case suggest that camptocormia can result from repeated abdominal muscle exercise, and that bilateral GPi-DBS may be a feasible and long-term efficacious procedure for dystonic camptocormia.

BACKGROUND: Desmoid tumors are known to be rare and relatively slow growing with locally invasive histology and high recurrence rates. A rare subset of these tumors occurs adjacent to the spinal column after surgery. Since 1961, only 6 cases of postoperative paraspinal desmoid tumor have been reported. This report is the first to describe the occurrence of a paraspinal desmoid tumor after surgical correction of a spinal deformity.
METHODS: A 56-year-old woman with multiple sclerosis reported 2-3 years of progressively worsening mid and low back pain that was worse with standing and ambulation and almost completely relieved with lying flat. Standing anteroposterior scoliosis radiographs revealed a thoracolumbar spinal deformity, which was surgically corrected with a T4 pelvis spinal fixation and fusion. At her 2-year postoperative clinic visit the patient reported good improvement in her preoperative symptoms but felt a fullness in her right upper back and neck that was nontender. Magnetic resonance imaging revealed a large tumor in this region. The tumor was resected en bloc, and pathology noted a 14.5 cm × 8.7 cm × 4.2 cm mass with spindle cell proliferation and positive staining for β-catenin (mutated gene found in 85% of desmoid tumors).
CONCLUSIONS: This is the first report of a postoperative desmoid tumor after spinal deformity correction and the seventh reported case in the spine literature. All reported cases have been women aged 39-57 years. The patient will be followed closely with magnetic resonance imaging given the known high local recurrence rate of this tumor.