The aim of the study was to analyze the present status of neurologic abnormalities, major orthopaedic deformities and ambulatory status in a large myelomeningocele population. Patients and methods: Cross-sectional study based on the clinical and radiographic records of 322 patients treated and followed-up from 1967 ± 1995. The setting was a multidisciplinary spina bi®da unit within a third-level university hospital, which serves as the referral centre for these patients in Catalonia (Spain). We collected information on diagnosis, central nervous system, musculoskeletal system (spinal and hip deformities) and functional level in each patient. To study relationships among the variables, the Mann-Whitney U and the Chisquared tests were applied. Results were considered to be statistically signi®cant at P levels of 40.05. Results: Mean age was 15.9 years. 78.1% of patients had mid-lumbar, low-lumbar or sacral neurological levels; 97.5% had hydrocephalus and 68.8% were shunted. Prevalence of spine deformities was 45.3%; 38.8% had dislocation of one or both hips. Median age of walking onset was 37.1 months and 74.8% of patients were ambulatory. Median age at which ambulation ceased was 128 months (10 years and 8 months). The bivariate analysis showed statistically signi®cant relationships between neurological level and all the variables studied (P50.001, P50.02) except body mass indexes and intelligence quotient. Conclusions: Neurological level was the main factor that determined neurological abnormalities, major orthopaedic deformities and ambulatory status.
BackgroundThe aim of this study is to validate the questionnaire ECOS-16 (Assessment of health related quality of life in osteoporosis) for the evaluation of health related quality of life (HRQoL) in post-menopausal women with osteoporosis.MethodsAn observational, prospective and multi-centre study was carried out among post-menopausal women with osteoporosis in primary care centres and hospital outpatient clinics. All patients attended 2 visits: at baseline and at 6 months. In addition, the subgroup of outpatients attended another visit a month after the baseline to assess the test-retest reliability. The psychometric properties of the questionnaire were evaluated in terms of feasibility, validity (content validity and construct validity) and internal consistency in baseline, and in terms of test-retest reliability and responsiveness to change in visit at month and visit at 6 months, respectively. In all visits, ECOS-16, EUROQoL-5D (EQ-5D) and four 7-point items about health status (general health status, back pain, limitation in daily activities and emotional status) were administered, whereas only outpatients were given MINI-OQLQ (Mini Osteoporosis Quality of Life Questionnaire), besides all clinical variables; and sociodemographic variables at baseline.Results316 women were consecutively included, 212 from primary care centres and 104 from hospital outpatient clinics. Feasibility: 94.3% of patients answered all items of the questionnaire. The mean administration time was 12.3 minutes. Validity: factor analysis suggested that the questionnaire was unidimensional. In the multivariate analysis, patients with vertebral fractures, co-morbidity and a lower education level showed to have worse HRQoL. Moderate to high correlations were found between the ECOS-16 score and the other health status questionnaires (0.47–0.82). Reliability: internal consistency (Cronbach's α) was 0.92 and test-retest reliability (ICC) was 0.80. Responsiveness to change: ECOS-16 scores increased according to change perceived by the patient, as well as the effect size (ranges between 1.35 to 0.43), the greater the perception of change in patients' general health status, the greater the changes in patients' scores. The Minimal Clinically Important Difference (MCID) suggested a change of 0.5 points in the ECOS-16 score, representing the least improvement in general health status due to their osteoporosis: "slightly better".ConclusionECOS-16 has been proven preliminarily to have good psychometric properties, so that it can be potentially a useful tool to evaluate HRQoL of post-menopausal women with osteoporosis in research and routine clinical practice.
Study design: In some young adults non-shunted myelomeningocele (MMC) patients without symptoms or with unspeci®c clinical ®ndings, moderate or severe stabilised ventriculomegaly are observed in control CT scans. Physicians are then faced with the so-called`Arrested Hydrocephalus' (AH) syndrome. The present study is part of a prospective protocol in which patients with hydrocephalus associated with MMC and long-term clinical diagnosis of AH were included. Objectives: To evaluate myelomeningocele patients never shunted with clinical and/or CT scan criteria compatible with AH. Setting: Multidisciplinary Spina Bi®da Unit in a tertiary university hospital in Barcelona, Spain. Methods: Fourteen MMC patients were selected, in all of them, continuous intracranial pressure (ICP) monitoring was performed. Analysis of the ICP records was done using the method described by Bùrgesen in mmHg. All patients were also studied by intelligence quotient (IQ) testing before and after shunting. Results: The CT in all the study cases showed an Evans ratio 40.30. The majority of patients (11/14) presented continuous high or intermittently raised ICP (active or unstable compensated hydrocephalus group) and in three (3/14) cases we con®rmed the diagnosis of arrested hydrocephalus. There were no complications associated with ICP monitoring. Conclusions: In view of our results, we believe that in all non-shunted cases with clinical and/ or CT scan criteria compatible with AH, the use of an objective method, such as continuous ICP monitoring, is necessary to diagnose arrested hydrocephalus, and a more comprehensive method that includes extensive neuropsychological valoration should be developed for the diagnosis and treatment of arrested hydrocephalus. Sponsorship: Supported in part by Grant number 97-0923 from the Fondo de Investigaciones Sanitarias de la Seguridad Social (FISS). Spinal Cord (2000) 38, 495 ± 497
Epiphysiolysis from a series of 253 patients under regular control in the Spina Bifida Unit at our hospital has been analysed from 1967 to the present time. From that total there have been 18 physeal fractures in 9 of the myelomening ocele patients. The clinical characteristics, diagnostic difficulties and therapeutic criteria for this type of lesion are considered, and we suggest the need to use suitable orthoses whenever the patient is able to walk, and to temporarily increase the level of orthotisation in patients if the physis of the knee is affected.
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