Restrictions in social participation of young adults with spina bifida.

PURPOSE: To determine participation restrictions of young adults with spina bifida (SB) in relation to health condition and activity limitations. METHOD: A total of 179 persons aged 16-25 years and born with SB participated in a cross-sectional study. The main outcome on four domains of participation (independent living, employment, education and partner relationships) was assessed using a structured questionnaire. RESULTS: At the mean age of 21 years only 16% were living independently, more than one-third of the participants went to special secondary education, 53% of those who finished education did not have a regular job and 71% did not have a partner. Health condition variables (type of SB, hydrocephalus and level of lesion) and to a lesser extent activity limitations (wheelchair dependence and incontinence) were significant determinants for having participation restrictions. Perceived hindrances in participation included long-distance transportation (19-36%), accessibility (10-42%), physical impairments (22-40%), emotional barriers (20-32%) and financial limits (3-17%). More severe SB, defined as hydrocephalus, high level of lesion and wheelchair dependence, was related with more experienced hindrances due to long-distance transportation accessibility of buildings. CONCLUSIONS: Many young adults with spina bifida experience participation restrictions. Severity of SB was negatively related to participation. Social integration should be a major focus in the professional guidance of youngsters with physical disabilities.

The reliability and validity of the PALOC-s: a post-acute level of consciousness scale for assessment of young patients with prolonged disturbed consciousness after brain injury.

The objective of the study was the validation of the Post-Acute Level of Consciousness scale (PALOC-s) for use in assessing levels of consciousness of severe brain injured patients in a vegetative state or in a minimally conscious state. A cohort of 44 successively admitted patients (between 2 and 25 years of age), who were treated in an early intensive neurorehabilitation programme, were included in the study. Each patient was examined, using the Western Neuro Sensory Stimulation Profile (WNSSP) and the Disability Rating Scale (DRS), once every two weeks resulting in 327 examinations (all videotaped). To determine the reliability of the PALOC-s, six observers rated one videotape of each patient. One of the observers rated the same tapes a second time, 3-4 months later. Validity was determined by correlating 100 ratings of one observer with the scores on the WNSSP and the DRS. To determine the responsiveness of the PALOC-s, the size of change between the scores of the first and last examinations was calculated. The inter-observer correlations and agreement scores varied between .82 and .95. The intra-observer correlation and agreement scores varied between .94 and .96. Correlations with the WNSSP varied between .88 and .93, and with the DRS between .75 and .88. The responsiveness was significantly high (t=8.2), with a standardised effect size of 1.30. It is concluded that the PALOC-s is a reliable, valid, and responsive observation instrument provided it is administered after a structured assessment by an experienced and trained clinician. The PALOC-s is feasible for use in clinical management, as well as in outcome research.

Life satisfaction of young adults with spina bifida.

This study concerns life satisfaction and its determinants in Dutch young adults with spina bifida (SB). Data on life satisfaction (Life Satisfaction Questionnaire [LiSat-9]) were related to hydrocephalus, lesion level, disabilities, and demographic variables. In total, 179 young adults with SB participated (41% male, age range 16-25y; 79% SB aperta, 67% hydrocephalus [HC], 39% wheelchair-dependent). Most were satisfied with their life as a whole (24% dissatisfied). No difference was found from a population reference group (28% dissatisfied). Highest proportions of dissatisfaction were found for financial situation (44%), partnership relations (49%), and sex life (55%). Least dissatisfaction was found for contact with friends (17%) and families (15%). Young adults with SB and HC were more satisfied with their financial situation and family life but were less satisfied with self-care ability and partnership relations than those without HC and the reference group. However, except for self-care ability, relationships between life satisfaction and having SB were weak. In conclusion, self-care ability and partnership relations were rated least favourable and may need more attention from care providers. Overall, SB does not seem to be an important determinant of life satisfaction.

Perceived health in young adults with spina bifida.

The aims of this study were to compare the perceived health of young adults with spina bifida with a population without disability, and to determine the effect of the disease characteristics and resulting impairments on perceived health. This cross-sectional study is part of the Adolescents with Spina Bifida in the Netherlands study. Data were collected by physical examination and a questionnaire. In total, 179 patients (age range 16-25y) participated in the study and perceived health data were completed for 164 participants (92 females, 72 males; mean age 20y 7mo [SD 2y 9mo]). Twenty-six participants had spina bifida occulta and 138 had spina bifida aperta, of whom 115 also had hydrocephalus. Perceived health was measured with the Medical Outcome Study 36-item Short-form Health Survey (SF-36), a generic health status measure. SF-36 scores of young adults with spina bifida were below those of an age-matched population group for six of the eight domains. This difference was largest for the physical functioning domain. Although these differences were statistically significant they were small. Findings for the emotional health domains (vitality, mental health, role problems due to emotional problems) did not differ at all from the population group.

Children and young adults in a prolonged unconscious state after severe brain injury: long-term functional outcome as measured by the DRS and the GOSE after early intensive neurorehabilitation.

OBJECTIVE: To investigate the long-term (2-15 years) functional outcome of children and young adults who received an early intensive neurorehabilitation programme (EINP) after a prolonged period of unconsciousness due to severe brain injury; to differentiate between traumatic brain injury (TBI) and non-traumatic brain injury (nTBI); and to compare the results on two different outcome scales: the Disability Rating Scale (DRS) and the Glasgow Outcome Scale Extended (GOSE). SUBJECTS: One hundred and forty-five patients, who were admitted to EINP between December 1987 and January 2001. OUTCOME MEASURES: The Post-Acute Level of Consciousness scale (PALOC-s), the DRS, including categorized scores (DRScat), and the GOSE. RESULTS: The long-term functional level of 90 patients could be determined, of whom 25 were deceased. The mean DRS-score of the surviving patients was 6.8 (SD = 6.6); the mean score on the GOSE was 4.5 (SD = 1.7). There was a significant difference in the outcome amongst traumatic and non-traumatic patients (t88 = 4.21; p < 0.01). The correlation between the DRS and the GOSE was high (Spearman rho = 0.85; p < 0.01), as well as the correlation between the categorized scores of the DRS and the GOSE (Spearman rho = 0.81; p < 0.01). The distribution of outcome scores on the DRScat is more diverse than on the GOSE. Especially item 7 of the DRS, measuring functional independence, showed considerable variance in discriminating between different outcome levels. CONCLUSIONS: More patients with TBI than expected reached a (semi-) independent level of functioning, indicating a possible effect of EINP. Patients suffering from nTBI did not demonstrate these outcome levels. Only a few patients stayed in a vegetative state for more than a couple of years. In this cohort of severe brain-injured young people, the DRS offered the best investigative possibilities for long-term level of functioning.

Predictive value of ischemic lesion volume assessed with magnetic resonance imaging for neurological deficits and functional outcome poststroke: A critical review of the literature.

OBJECTIVE: Ischemic lesion volume is assumed to be an important predictor of poststroke neurological deficits and functional outcome. This critical review examines the methodological quality of MRI studies and the predictive value of hemispheric infarct volume for neurological deficits (at body function level) and functional outcome (at activities level). METHODS: Using Medline, PiCarta, and Embase to identify studies, 13 of the 747 identified studies met the authors' inclusion criteria. Subsequently, studies were tested for adherence to the key methodological criteria for internal, statistical, and external validity. Each criterion was weighted binary, and studies with 6 points or more were judged to be valid for assessing the predictive value of MRI for outcome. RESULTS: The 13 included studies had several methodological weaknesses with respect to internal validity, and none of them took lesion location into account. Only a few used outcome measures according to the International Classification of Functioning, Disability and Health and followed patients beyond 6 months. Correlation coefficients between MRI lesion volume and outcomes were higher for outcomes defined at body function level (National Institutes of Health Stroke Scale; median 0.67; range: 0.57-0.91) than for those defined at the level of activities (Barthel Index; median -0.49; range: -0.33 to -0.74). CONCLUSIONS: Methodological shortcomings of most studies confound the prognostic value of MRI in predicting stroke outcome, and few studies have focused on functional outcome. Future studies should investigate the added value of MRI volume over clinical neurological variables in predicting functional outcome beyond 6 months poststroke.

Body weight-supported treadmill training in chronic incomplete spinal cord injury: a pilot study evaluating functional health status and quality of life.

STUDY DESIGN: A controlled single-case design: A1 (baseline: 6 weeks), B (intervention: 12 weeks of treadmill training (TT), maximally five times a week/30 min a day), A2 (wash-out: 6 weeks), follow-up measurement: 6 months. OBJECTIVE: To investigate the effects of TT on functional health status (FHS) and quality of life (QoL) in subjects with a chronic incomplete spinal cord injury (ISCI). SETTING: Rehabilitation Department, University Medical Centre Utrecht, The Netherlands. METHODS: Three male subjects with a stable (>48 months postinjury) ISCI, American Spinal Injury Association (ASIA) class C (n=2) and D (n=1). Performance-based walking, subject's perception concerning quality of life (SEIQoL) and activities of daily living Canadian Occupational Performance Measure (COPM). RESULTS: The results of the three subjects were variable. Changes in QoL were relatively small and diverse. After 6 months' follow-up, QoL was unchanged in subjects 1 and 2, and improved in subject 3. In subject 2, performance of activities of daily living (ADL) was significantly improved, consistent with his perception of improvement (P<0.05), and this improvement was sustained throughout the follow-up period. Walking ability improved in subject 3 (P<0.05) but performance of other activities remained stable. Performance of ADL decreased slightly in subject 1 whereas his walking speed and Get up and Go performance improved (P<0.05). CONCLUSIONS: This study demonstrates positive effects of TT on FHS. A randomised clinical trial should be executed before definite conclusions about the effect of TT on FHS and QoL can be drawn. SPONSORSHIP: KF Hein Foundation and Rehabilitation Centre De Hoogstraat Scientific Foundation.

Ischemic lesion volume correlates with long-term functional outcome and quality of life of middle cerebral artery stroke survivors.

PURPOSE: Previous studies investigating relationships between stroke lesion volume and outcome were restricted to short follow-up periods (3-6 months) and outcome measures of stroke severity and activities only, whereas functional improvement has been found to extend far beyond six months. Therefore, this study investigated relationships between infarct volume and a broad range of outcomes of stroke survivors at a long follow-up period. METHODS: Correlations between lesion volumes (determined by conventional MRI scans in the second week post-stroke) and outcomes after one year of 75 first-ever ischemic stroke survivors were investigated. RESULTS: Moderate Spearman Rank correlation coefficients were found between lesion volume and motor impairment (Motricity Index (MI): -0.43, p < 0.01; Fugl Meyer Motor Assessment Scale (FM): -0.43; p < 0.01). Correlation coefficients with activities of daily living were moderate but low associated with Barthel Index (r(s) = 0.30; p < 0.01), modified Rankin Scale (r(s) = 0.39; p < 0.01) and Frenchay Activities Index (r(s) = -0.35; p < 0.01). Lesion volume had a significant but low association (r(s) = 0.27; p = 0.02) with patient's health status measured with Sickness Impact Profile 68 (SIP68)) and a moderate correlation with well-being assessed with Life Satisfaction Questionnaire (LSQ; r(s) = -0.45; p < 0.01). Found correlation coefficients were slightly stronger after correction for mixed (cortical/subcortical) and purely subcortical lesion location. CONCLUSIONS: It can be concluded that infarct volume moderately correlates with long-term motor impairment, functional outcome and quality of life of patients surviving stroke.

Poststroke hand swelling and oedema: prevalence and relationship with impairment and disability.

OBJECTIVE: To examine the prevalence of swelling and oedema of the hand in stroke patients and relationships with impairments and disability. DESIGN: Cross-sectional. SETTING: Stroke unit at Rehabilitation Centre De Hoogstraat, The Netherlands. SUBJECTS: Eighty-eight adult stroke patients who were admitted for clinical rehabilitation during one year (2000). MEASUREMENTS AND METHODS: Assessment took place two weeks after admission to the rehabilitation centre. Degree of swelling was measured with a hand volumeter and oedema was defined as a volumeter score deviating more than two standard deviations from the expected score derived from population data. Further assessment included tonus (Modified Ashworth Score), sensibility, tactile inattention, carefulness, and motor function (Utrecht Arm/Hand Test). Arm disability was measured with Frenchay Arm Test and Nine-hole Peg Test. RESULTS: Some degree of hand swelling was present in 72.7% and oedema in 33.0% of our patients. Swelling and oedema were significantly more often seen in patients with hypertonic fingers and impaired sensibility. Patients with hand oedema had significantly worse Frenchay Arm Test and Nine-hole Peg Test scores. CONCLUSIONS: Swelling and oedema of the hand are common among stroke patients in clinical rehabilitation.

Children and young adults in a prolonged unconscious state due to severe brain injury: outcome after an early intensive neurorehabilitation programme.

PRIMARY OBJECTIVE: The Rehabilitation Centre Leijpark in The Netherlands provides an Early Intensive Neurorehabilitation Programme (EINP) to children and young adults in a prolonged unconscious state after severe brain injury. In an extensive research project the effects of EINP were studied. This part of the project focused on the outcome in terms of level of consciousness (LOC) in relation to the specific characteristics of a retrospectively studied cohort. RESEARCH DESIGN: This study was executed according to a one-group archived pre-test-post-test design. SUBJECTS: Subjects were all consecutively admitted patients (n=145, 72% male) between December 1987-January 2001. Inclusion criteria were: age 0-25 years, within 6 months after injury, LOC at admission vegetative state (VS) or minimally conscious state (MCS). One hundred and four patients (72%) suffered a traumatic injury and 41 patients (28%) a non-traumatic injury. METHODS AND PROCEDURES: All patients had received EINP until they reached consciousness or until it was concluded that no progress was achieved during 3 months after the start of EINP. Medical files were investigated to collect the patients' characteristics and injury data, to determine the LOC at admission and at discharge and to determine the discharge destination. RESULTS: Almost two-thirds of the patients reached full consciousness. LOC at admission, aetiology and interval since injury were found to be significant prognostic factors. Traumatic patients had a much better outcome than non-traumatic patients. A comparison with earlier outcome studies showed a more favourable outcome than expected. It is argued that a multi-centre study is needed to confirm possible effects of EINP.

Relationship between ischemic lesion volume and functional status in the 2nd week after middle cerebral artery stroke.

OBJECTIVE: To examine the relationship between the volume of the middle cerebral artery stroke lesion and functional status in the subacute phase of stroke. METHODS: Infarctvolumes of 94 patients with a 1st middle cerebral artery stroke assessed on conventional MRI scans obtained in the 2nd week poststroke were related to a clinical measure of stroke severity (National Institutes of Health Stroke Scale [NIHSS]) and to functional status: motor impairment (Motricity Index [MI]) and limitation in activities (Barthel Index [BI] and modified Rankin Scale). Separate correlations were computed for patients with large (>30 ml) and small (30 ml) lesions, and to investigate the influence of lesion location on the relationship between volume and functional status, correlations were computed for patients with left and right hemisphere lesions and for patients with cortical and subcortical lesions. RESULTS: Lesion volume correlated strongly with NIHSS scores (R = 0.61) and moderately with the patient's functional status (MI [R between -0.42 and - 0.49], BI [R = -0.43], and Modified Rankin Scale [R = 0.45]). Right hemisphere lesions and cortical lesions had a stronger correlation with functional status. In patients with small lesion volumes (0-30 ml), no relationship between lesion volumes and functional status was seen at all. CONCLUSIONS: Lesion volume is moderately to strongly related to the functional status in the 2nd week poststroke.

High prevalence of incontinence among young adults with spina bifida: description, prediction and problem perception.

STUDY DESIGN: Cross-sectional study. OBJECTIVES: To study the prevalence of incontinence, problem perception and determinants of urinary and faecal incontinence in young adults with spina bifida. SETTING: Nation-wide study in the Netherlands. PARTICIPANTS: A total of 179 of 350 invited patients participated, including 37 patients with spina bifida occulta and 142 with spina bifida aperta, of whom 119 had hydrocephalus; 41% were male and mean age was 20.4 (range 16-25 years). METHODS: Data were collected from interviews, physical examination, neuropsychological tests and medical records. RESULTS: Urinary and faecal incontinence was common in young adults with spina bifida (60.9 and 34.1%, respectively), regardless of the bladder and bowel management they used. The majority of urinary and faecal incontinent patients perceived this as a problem (69.7 and 77.0%, respectively). Spina bifida aperta, hydrocephalus and a level of lesion of L5 or above were associated with patients suffering from urinary and/or faecal incontinence. Predictors of perceiving urinary incontinence as a problem were, in addition to being incontinent, not having hydrocephalus and having a level of lesion of L5 or above. The only predictor of perceiving faecal incontinence as a problem was the frequency of incontinence. CONCLUSION: A majority of young adults with spina bifida suffer from urinary and faecal incontinence and most of them perceive their incontinence as a problem. Therefore, further efforts are important to improve urinary and faecal continence.

Secondary impairments in young adults with spina bifida.

The aim of this study was to examine the prevalence of secondary impairments in young adults with spina bifida and to relate the prevalence to the type of spina bifida and the level of lesion. This cross-sectional study is part of the ASPINE (Adolescents with Spina Bifida in the Netherlands) study. Data were collected on medical history, hydrocephalus (shunt: yes/no), neurological level of lesion (International Standards for Neurological and Functional Classification of Spinal Cord Injury), visual acuity (Landolt rings), spasticity (Modified Ashworth Scale), contractures (range of motion), scoliosis (deviation from perpendicular), ambulation (Hoffer criteria), pressure sores and blood pressure (physical examination), epilepsy, pain, incontinence and sexuality (questionnaire), and cognitive functioning (Raven Standard Progressive Matrices). In total, 179 patients with spina bifida participated (41% male, age range 16 to 25 years, mean 20 years 9 months, SD 2 years 11 months). These were 37 patients with spina bifida occulta, 119 patients with spina bifida aperta and hydrocephalus (AHC+) and 23 patients with spina bifida aperta without hydrocephalus (AHC-). Of our patient group, 73 had a high-level lesion (L2 and above), 68 a mid-level lesion (L3 to L5), and 38 a low-level lesion (S1 and below). Both subdivisions were strongly related with patients with higher lesions more often having hydrocephalus. Most secondary impairments were found for patients with AHC+, and patients with AHC- were mostly comparable to patients with spina bifida occulta. According to level of lesion, most medical problems were found in the high-level lesion group. However, all subgroups suffered from health problems.

Educational career and predictors of type of education in young adults with spina bifida.

Children with spina bifida (SB) often require special education. To date, little information is available about the educational career of these children. This study focuses on educational career and predictors of attending special education of young adults with SB, using a cross-sectional study including 178 young Dutch adults with SB aged from 16-25. The main outcome was attending regular versus special education. For searching predictive power we selected age, gender, type of SB, level of lesion, hydrocephalus (HC), number of surgical interventions, ambulation, continence and cognitive functioning. Chi-square tests and binary logistic regression were used in the data analysis. Participants with HC attended special primary education more often (59%) than participants without HC (17%). For those participants with HC, the necessity of special primary education was associated with below average intelligence (75% versus 35%), wheelchair dependence (82% versus 39%) and surgical interventions (74% versus 44%). Only half of the participants with HC followed regular secondary education, whereas for participants with SB without HC, the outcome in secondary education was similar to that of the general population (92%). Intelligence was the main predictor of attending special secondary education (odds 5.1:1), but HC (odds 4.3:1) and wheelchair dependence (odds 2.6:1) were also a significant. Other variables were not significant predictors of special secondary education.

Cognitive status of young adults with spina bifida.

The cognitive status of 168 Dutch young adults (103 females, 65 males; mean age 20 years 9 months, age range 16 to 25 years) with spina bifida (SB) was examined. The main purpose was to establish the effect of the type of SB (occulta or aperta) and the effect of hydrocephalus (HC) within the group with SB aperta (AHC+). Results indicated, on average, a lower cognitive status of persons with AHC+ (n=111) than of persons with SB occulta (n=37) and of persons with SB aperta without HC (AHC-; n=20). Almost half the young adults with AHC+ had cognitive impairments of some sort. These included more domain specific impairments (70%) as well as a more general cognitive deficit (30%). Cognitive status of persons with SB occulta and of those with AHC- was similar to that in the healthy population. The presence of associated pathology, rather than SB per se, has a negative effect on cognitive status.

Assessment of oedema in stroke patients: comparison of visual inspection by therapists and volumetric assessment.

PURPOSE: To examine the agreement of assessment of hand oedema in stroke patients by clinical judgement and by use of a hand volumeter. METHOD: A total of 88 rehabilitation patients with stroke received both assessments. Experienced physical therapists classified oedema as 'none', 'minor' or 'severe'. Swelling was independently measured with a hand volumeter. Population data were used to adjust volumeter reading differences for handedness and side of paresis, and to define a cut-off point for oedema of 2 SD of the population distribution. RESULTS: Based on volumetric assessment, 33% of patients had oedema. Physical therapists classified 50% of patients as having minor or severe oedema. Results of both methods were clearly related, but agreement between the assessments was not more than 'fair' (67% agreement; Kappa 0.34). The level of agreement was not substantially affected by the cut-off point used for the volumeter score, the time between both assessments or by the side of paresis. CONCLUSION: Agreement between clinical and volumetric assessment of hand oedema in stroke patients is less than desirable. Volumetric assessment of oedema is recommended for research purposes.