Mobilization under anaesthesia for correction of knee flexion deformity in haemophilia.

INTRODUCTION: Haemophilia patients not treated with primary or secondary prophylaxis, often present with recurrent haemarthrosis. Knee is the most frequently involved joint leading to disabling knee flexion deformity (KFD). Here, we present a retrospective study of our experience on the role of mobilization under general anaesthesia in the correction of KFD. AIM: To study the effectiveness of mobilization under anaesthesia (MUA) for correction of knee flexion deformity (KFD) in persons with haemophilia (PWH). METHODS: Outcome of all patients managed with MUA in our multidisciplinary haemophilia clinic from 2008 to 2019 were included for analysis. PWH with KFD > 20 degree who underwent MUA were included in the study. Under general anaesthesia and cover of clotting factor replacement, gentle joint mobilization was done to achieve maximal correction in flexion deformity, followed by above knee casting in this position. The outcome measures assessed were reduction in knee flexion deformity following MUA and complications, if any. RESULTS: Thirty patients (34 knees) with knee flexion deformity were included in the study. Mean age of the study population was 14.23 years ± 8.3. Study population was analysed in two groups, Group 1 included patients who underwent single MUA and Group 2, patients who underwent two or more MUA. There was significant improvement in KFD correction in both groups. [Group 1; Mean difference: 22 ± 13.7, p value -0.01, 95% CI (16.4-27.5) and Group 2; Mean difference 48.8 ± 19.8, p value -0.00, 95% CI (34.2-64.5)]. CONCLUSION: MUA can be effective in the short-term correction of KFD in PWH particularly those below 15 years of age. It should be done judiciously when target correction is not achieved with other physical methods.

Orthotic walking outcome of persons with motor complete low thoracic spinal cord injury-a retrospective study.

STUDY DESIGN: Retrospective study. OBJECTIVE: To analyse the orthotic walking outcome of patients with Low Thoracic Spinal Cord Injury (LT-SCI). SETTING: The Rehabilitation Institute at Christian Medical College, Vellore, India. METHODS: Data between January 2005 and June 2015 were retrospectively collected from electronic medical reports of patients with motor complete LT- SCI who were admitted for the comprehensive rehabilitation program. The orthotic walking outcome of these patients was measured by the Walking index for SCI version II (WISCI II). Demographical and clinical parameters were measured and their association with the walking outcome was analyzed using regression analysis. RESULTS: A total of 430 patients were identified within the study period. Eighty-five percent of people (n = 365) achieved walking at the time of discharge (WISCI II level 12 = 260 and level 9 = 105). Out of 11 demographical and clinical parameters considered, eight of them were found to be significant predictors of walking in the univariate analysis. Age less than 30 years had the highest odds of predicting WISCI II level 9 and level 12 than those older in the multivariate analysis (OR 17.58; 95% CI 7.35-42.03). Single neurological level T12 increased the chance of achieving WISCI II level 12 by 10 times (OR 10.2; 95% CI 3.8-27.36). CONCLUSIONS: Orthotic walking for persons with motor complete low thoracic spinal cord injury is an achievable goal through a comprehensive rehabilitation program. The factors identified in this study will help rehabilitation professionals strategically select the ideal candidate for orthotic gait training.

Standard Clinical Screening Tests, Sural Radial Amplitude Ratio and F Wave Latency Compared to Conventional Nerve Conduction Studies in the Assessment of Sensorimotor Polyneuropathy in Patients with Type 2 Diabetes Mellitus.

Background and Aims: The measuring tools used for assessment of neuropathy include various questionnaires, monofilament testing, Biothesiometry and the gold standard test, nerve conduction studies (NCS). This study aims to evaluate the diagnostic accuracies of Michigan Neuropathy Screening Instrument (MNSI), Biothesiometry, Semmes Weinstein Monofilament (SWMF), Sural Radial Amplitude Ratio (SRAR) and minimal F wave latency as compared to conventional NCS and arrive at a simple diagnostic algorithm for early detection of Diabetic Peripheral Neuropathy (DPN). Methods: In a cross-sectional observational study on 48 Type 2 diabetes mellitus patients, MNSI, Biothesiometry, SWMF and NCS including F waves and SRAR were done and diagnostic accuracies (sensitivity, specificity, positive and negative predictive values) calculated taking NCS as gold standard. Results: MNSI, Biothesiometry, SWMF, SRAR and minimal F wave latency had a sensitivity of 64.3%, 78.6%, 14.3%, 100% and 78.6% and specificity of 67.7%, 52.9%, 94.1%, 23.53% and 76.47% respectively, with reference to NCS. Based on combined sensitivities and specificities, we arrived at a simple algorithm for early diagnosis of DPN, which showed that DPN could either be diagnosed or ruled out in 75% of the patients by a combination of the Biothesiometry, SRAR and left lower limb minimal F wave latency results. Conclusions: In the setting of an outpatient, multidisciplinary diabetic clinic, simple tests such as questionnaires, monofilament testing and biosthesiometer could be performed with greater ease while considering NCS as the gold standard. This algorithm, combining Biothesiometry, SRAR and left lower limb minimal F wave latency would be less time consuming and help in early diagnosis of DPN.

Affordable passive 3D-printed prosthesis for persons with partial hand amputation.

BACKGROUND AND AIM: Partial hand amputations are common in developing countries and have a negative impact on patients and their families' quality of life. The uniqueness of each partial hand amputation, coupled with the relatively high costs of prostheses, makes it challenging to provide suitable prosthetic solutions in developing countries. Current solutions often have long lead times and require a high level of expertise to produce. The aim of this study was to design and develop an affordable patient-specific partial hand prosthesis for developing countries. TECHNIQUE: The prosthesis was designed for a patient with transmetacarpal amputation (i.e. three amputated fingers and partial palm). The final design was passive, controlled by the contralateral hand, and utilized the advanced flexibility properties of thermoplastic polyurethane in a glove-like design that costs approximately 20 USD to fabricate. Quantitative and qualitative tests were conducted to assess performance of the device after the patient used the final design. A qualitative assessment was performed to gather the patient's feedback following a series of tests of grasp taxonomy. A quantitative assessment was performed through a grasp and lift test to measure the prosthesis' maximum load capacity. DISCUSSION: This study showed that the prosthesis enhanced the patient's manual handling capabilities, mainly in the form of grasp stability. The prosthesis was light weight and could be donned and doffed by the patient independently. Limitations include the need to use the contralateral hand to achieve grasping and low grasp strength. CLINICAL RELEVANCE: Persons with partial hand amputation in developing countries lack access to affordable functional prostheses, hindering their ability to participate in the community. 3D-printed prostheses can provide a low-cost solution that is adaptable to different amputation configurations.

Rehabilitation in South India.

The article describes the rehabilitation services provided at Christian Medical College Vellore, a tertiary care medical college hospital in South India. The department was started by Dr Mary Verghese, who on completion of her medical training sustained spinal cord injury with resulting paraplegia. Following a section on the initial beginnings of the department, the current status of the department offering comprehensive rehabilitation by the multidisciplinary team is highlighted. The article ends with the challenges faced, including limitations in providing affordable solutions, architectural and attitudinal barriers, and inadequate number of rehabilitation physicians and comprehensive rehabilitation centers in the country.

A descriptive pilot study of mitochondrial mutations & clinical phenotype in fibromyalgia syndrome.

BACKGROUND & OBJECTIVES: : Fibromyalgia syndrome (FMS) is one of the most common chronic pain conditions of unknown aetiology. Mitochondrial dysfunction has been reported in FMS with some studies reporting the presence of mitochondrial mutation namely A3243G, which also causes mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes. This pilot study was conducted to assess this mutation and also detect large deletions in mitochondrial DNA (mtDNA) in patients with FMS. METHODS: : Thirty female patients with FMS participated and 30 matched controls were included. Genomic DNA was subjected to polymerase chain reaction (PCR) amplification using specific primers followed by restriction digestion with Apa I enzyme to detect the specific A3243G mtDNA mutation. Long-range PCR was done in two sets to detect the large deletions in the mtDNA. Biochemical parameters including thyroid-stimulating hormone and vitamin D levels were also looked at. RESULTS: : None of the patients were found to carry the common mutation or large deletions. Low vitamin D level was a common finding. Hypothyroidism was found in a few patients. INTERPRETATION & CONCLUSIONS: : Although the common mutation or large mtDNA deletions were not detected in blood mtDNA in the FMS patients, mutations in the muscle and sequence variation in mtDNA remained a possibility. Future studies in both blood and muscle tissue including mtDNA sequencing are warranted in such patients to determine if a subset of FMS patients have mitochondrial myopathy.

Survival in persons with traumatic spinal cord injury receiving structured follow-up in South India.

OBJECTIVE: To assess the survival in persons with traumatic spinal cord injury (SCI) receiving structured follow-up in South India. DESIGN: Retrospective study. SETTING: Rehabilitation center. PARTICIPANTS: Persons with traumatic SCI (N=490) residing within a 100-km radius of the institute who were managed and regularly followed up by the rehabilitation center between the years 1981 and 2011. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Survival rates and mortality risk factors. Measures were estimated using the product limit (Kaplan-Meier) method and the Cox model. RESULTS: The survival rate after SCI was 86% after 5 years, 71% after 15 years, and 58% after 25 years. Survival of persons with complete high cervical injury is substantially low compared with other levels of SCI. Level of injury and extent of lesion (Frankel classification and/or American Spinal Injury Association Impairment Scale) play a significant role in predicting survival of this population. CONCLUSIONS: Survival rates of regularly followed-up persons with SCI from this study show promising results, though survival rates are lesser when compared with studies from developed countries. Better understanding of the predictors, causes of deaths, comprehensive rehabilitation, community integration, and regular follow-up could possibly assist in improving survival rates.

A rare presentation of subacute progressive ascending myelopathy secondary to cement leakage in percutaneous vertebroplasty.

Percutaneous vertebroplasty is used to manage osteoporotic vertebral body compression fractures. Although it is relatively safe, complications after vertebroplasty ranging from minor to devastatingly major ones have been described. Cement leakage into the spinal canal is one such complication. Subacute progressive ascending myelopathy is an infrequent neurologic complication after spinal cord injury, typically presenting as ascending neurologic deficit within weeks after the initial insult. The precise cause of subacute progressive ascending myelopathy still remains an enigma, considering the rarity of this disorder. The authors present the case of a 62-yr-old woman with osteoporotic vertebral fracture who underwent percutaneous vertebroplasty and developed T6 complete paraplegia because of cement leakage. A few weeks later, the neurologic level ascended to higher cervical level (C3). To date, no case of subacute progressive ascending myelopathy secondary to cement leakage after percutaneous vertebroplasty has been reported. Literature is reviewed regarding subacute progressive ascending myelopathy, and the rehabilitation challenges in the management of this patient are discussed.

Gorham's disease of the spine.

BACKGROUND: Gorham's disease of the spine is very rare and usually associated with poor prognosis. Till date very few cases have been reported in the literature. It is characterized by osseous invasion by angiomatous vascular mass without skip areas, eventually causing lysis of affected bone. Morbidity and mortality are high in those with spinal and/or visceral involvement. Neurological complications increase the mortality to 33% whereas with chylothorax, mortality increases to more than 50%. OBJECTIVES: To describe the clinical course and the challenges in planning and implementing effective rehabilitation services for management of patients with Gorham's disease of the spine. METHODS: We report two patients with Gorham's disease who had neurological deficits secondary to spinal cord involvement, and their rehabilitation perspectives. RESULTS: Both patients achieved satisfactory ambulation and functional outcomes. CONCLUSION: Multiple revision surgeries may be required to ensure spinal stability. When working with these patients, one must remain vigilant about spinal stability as well as about possible serious pulmonary complications, and be prepared to make appropriate management decisions when necessary.

Traumatic brachial plexus injury: electrodiagnostic findings from 111 patients in a tertiary care hospital in India.

OBJECTIVE: The study aims to characterise the electrodiagnostic findings of patients with traumatic brachial plexus injuries (BPIs) in India and to analyse the association between aetiologies and levels of injuries. METHODS: A total of 111 consecutive electrodiagnostic studies done between January 2009 and June 2011 on persons with traumatic BPI were retrospectively analysed. SETTING: Electrodiagnostic Laboratory, Department of Physical Medicine and Rehabilitation in a tertiary care university teaching hospital in South India. MAIN OUTCOME MEASURES: Nerve conduction velocities and electromyography (EMG) to locate the level of BPI, Dumitru and Wilbourne scale to assess the severity of BPI. RESULTS: We studied 106 males and five females, ranging from 11 to 59 years of age. All but one had unilateral BPI. Motorcycle crashes were the most frequent cause (n=64, 58%). Isolated supraclavicular injury was found in 98 arms (88%) and infraclavicular injury in seven arms (6%). Root-level injuries were more common in motorcycle crashes and occupation-related trauma, while trunk-level injuries were more often found in automobile crashes, falls, bicycle-related trauma and penetrating wounds. Pan root (C5-T1) involvement was more common in the motorcycle trauma group (74%). There was no significant association between aetiologies and levels of BPIs. A total of 73 (65%) plexus injuries were of 'severe' category as per Dumitru and Wilbourn scale. CONCLUSIONS: Motorcycle crash is the most common cause of traumatic BPIs. Supraclavicular injury is the rule in most cases. Proper attention needs to be given to differentiate the mild to moderate injuries from the severe injuries with EMG techniques since most of the cases are severe. There was no significant association found between aetiologies and levels of injury.

Evaluation of posture and pain in persons with benign joint hypermobility syndrome.

The objective of the present study is to compare and quantify the postural differences and joint pain distribution between subjects with benign joint hypermobility syndrome (BJHS) and the normal population. This observational, non-randomized, and controlled study was conducted at Rheumatology and Physical Medicine and Rehabilitation Medicine Departments of a tertiary care teaching hospital. Subjects comprise 35 persons with diagnosis of BJHS, and the control group was matched for age and sex. Reedco's Posture score (RPS) and visual analogue scale (VAS) were the outcome measures. The subjects were assessed for pain in ten major joints and rated on a VAS. A standard posture assessment was conducted using the Reedco's Posture score. The same procedure was executed for an age- and sex-matched control group. Mean RPS for the BJHS group was 55.29 ± 8.15 and for the normal group it was 67 ± 11.94. The most common postural deviances in subjects with BJHS were identified in the following areas of head, hip (Sagittal plane), upper back, trunk, and lower back (Coronal plane). Intensity of pain was found to be more in BJHS persons than that of the normal persons, and the knee joints were the most affected. The present study compared and quantified the postural abnormalities and the pain in BJHS persons. The need for postural re-education and specific assessment and training for the most affected joints are discussed. There is a significant difference in posture between subjects with BJHS and the normal population. BJHS persons need special attention to their posture re-education during physiotherapy sessions to reduce long-term detrimental effects on the musculoskeletal system.

Life expectancy for people with disabilities.

This article reviews the literature on life expectancy in the disabled population. The literature is surveyed with regard to the main elements that reduce life expectancy for people with disabilities. These are immobility, cognitive and intellectual impairment, swallowing problems and tube feeding, epilepsy and incontinence. The article discusses various improvements in survival in the last few years and discusses the possibility of further improvements in the future. The difficulties are discussed with regard to early prediction of life expectancy both in childhood and after an acute neurological event in adulthood. The literature is then reviewed with regard to the cohort studies on quantification of life expectancy in disabled people. The literature is discussed and pragmatic clinical conclusions are drawn.

The effectiveness of intravesical oxybutynin, propantheline, and capsaicin in the management of neuropathic bladder following spinal cord injury.

The objective of this study was to compare the therapeutic response of intravesical oxybutynin, propantheline, and capsaicin in the treatment of neurogenic detrusor overactivity. Carried out in the Department of Physical Medicine and Rehabilitation at a university teaching hospital in India, patients acted as their own controls. Oxybutynin 5 mg in solution or propantheline 15 mg in solution and capsaicin were instilled intravesically in each patient. Urodynamic studies were done before and after the intravesical instillation of each drug. The nonparametric tests were used for statistical analysis. The efficacy of intravesical capsaicin in the treatment of neurogenic detrusor overactivity was statistically significant for reflex volume (RV) (p = 0.018), cystometric capacity (CC) (p = 0.0440), leak volume (LV) (p = 0.000), and leak frequency (LF) (p = 0.009). The Kruskal-Wallis test for paired sample comparing pre- and post-LV and LF for intravesical capsaicin was significant at 2nd week (p = 0.002 and 0.054, respectively). There was a significant difference in therapeutic response between intravesical oxybutynin, propantheline, and capsaicin in the treatment of detrusor overactivity for LV and LF at 2nd week (p = 0.017 and 0.003, respectively). When comparing responses of oxybutynin and propantheline, more subjects demonstrated improvement with intravesical propantheline than oxybutynin for RV, detrusor leak point pressure (LPP), clean intermittent catheterization volume (CICV), and LV. This study suggests that intravesical agents may be used as effective adjuvants in the management of incontinence due to neurogenic detrusor overactivity following spinal cord injury.