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Upper limb function for people with Friedreich ataxia determines capacity to participate in daily activities. Current upper limb measures available do not fully capture impairments related to Friedreich ataxia. We have developed an objective measure, the Ataxia Instrumented Measure-Spoon (AIM-S), which consists of a spoon equipped with a BioKin wireless motion capture device, and algorithms that analyse these signals, to measure ataxia of the upper limb during the pre-oral phase of eating. The aim of this study was to evaluate the AIM-S as a sensitive and functionally relevant clinical outcome for use in clinical trials. A prospective longitudinal study evaluated the capacity of the AIM-S to detect change in upper limb function over 48 weeks. Friedreich ataxia clinical severity, performance on the Nine-Hole Peg Test and Box and Block Test and responses to a purpose-designed questionnaire regarding acceptability of AIM-S were recorded. Forty individuals with Friedreich ataxia and 20 control participants completed the baseline assessment. Thirty individuals with Friedreich ataxia completed the second assessment. The sensitivity of the AIM-S to detect deterioration in upper limb function was greater than other measures. Patient-reported outcomes indicated the AIM-S reflected a daily activity and was more enjoyable to complete than other assessments. The AIM-S is a more accurate, less variable measure of upper limb function in Friedreich ataxia than existing measures. The AIM-S is perceived by individuals with Friedreich ataxia to be related to everyday life and will permit individuals who are non-ambulant to be included in future clinical trials.

Descriptive, retrospective. Scientific level of evidence IV.

The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine.

A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed.

Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5years and 2months (6months to 13years and 3months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3years and 5months (range 2-12years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment.

Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.

The selection of lowest instrumented vertebra (LIV) in adolescent idiopathic scoliosis (AIS) is determined by coronal and sagittal plane indicators. Failure to properly select the LIV can lead to suboptimal outcomes and the need for revision surgery. A subset of patients have discordant coronal last touched vertebra (cLTV) and stable sagittal vertebra (SSV) which complicates the choice of LIV. The purpose of this study is to report the incidence of discordant cLTV and SSV when choosing LIV and investigate the association between length of fusion and patient-reported outcomes (PROs) and distal junctional kyphosis (DJK).

This retrospective multicenter cohort study included AIS patients with discordant pre-operative cLTV and SSV. Patients fused only to include the proximal cLTV were compared to patients fused to include the distal SSV. Primary outcomes included DJK and PROs measured by SRS-22.

Eight hundred and fifty-six patients were identified of which 114 (13.3%) had discordant SSV and cLTV. The DJK incort of the SSV, respectively. selleck chemical Lenke Modifier type B and C patients with fusions short of the SSV had a 9.2 times increased risk of developing DJK at 2 years compared to patients with fusions including the SSV (95% CI 2.8, 29.7; p  less then  0.001). However, patients with fusions short of the SSV and no evidence of DJK were 9.2 times more likely to have improvement in the SRS-22 pain domain compared to patients with fusions including the distal SSV (95% CI 1.1, 77.4; p = 0.042) CONCLUSION Patients fused short of the SSV are at significant risk for the development of DJK at 2 years post-operatively. However, patients with shorter fusions were more likely to have an improvement in their pain as measured by patient-reported outcomes than patients with longer fusions.

Descriptive survey.

Compare radiographic parameters measured using surface topography (ST) with those obtained radiographically to determine the validity of ST for scoliotic assessment.

While anterior-posterior radiography is the gold standard for diagnosing scoliosis, repeated radiographic use is associated with increased carcinogenicity. Studies have thus focused on radiation-free systems, including ST, to calculate the scoliotic angle. Seventeen patients ages 25-76 were included. Each patient received one AP radiograph and three repeated ST measurements over two months. Values were analyzed by two raters to determine comparability between ST and radiographic measurements. Interobserver reliability (ICC) was calculated and statistical significance was determined by the p-value of a paired two-tailed t-test.

ICC showed excellent reliability (> 0.90). There was no significant difference (p > 0.40) in apical vertebral deviation/translation between conventional radiography (0.9 ± 0.8) and ST (1.2 ± 1.