Dissociation between preserved body structural description and impaired body image following a pediatric spinal trauma.

In adult patients, Spinal Cord Injury (SCI) may influence the mental Body Representation (BR). Currently, there is no evidence on the modulation of SCI on BR during early stages of cognitive development. Here, we investigated BR in a 3-year-old child with complete SCI. The patient was administered with a specific battery assessing different BR components. We found evidence for putative classical neuropsychological dissociation between a preserved topological map with impaired semantic knowledge of the body. This finding sheds new light on the impact of SCI on BR in childhood, as well as on the level of interdependence between BR's components..

Tolerability and efficacy of erythropoietin (EPO) treatment in traumatic spinal cord injury: a preliminary randomized comparative trial vs. methylprednisolone (MP).

The only available treatment of traumatic spinal cord injury (TSCI) is high-dose methylprednisolone (MP) administered acutely after injury. However, as the efficacy of MP is controversial, we assessed the superiority of erythropoietin (EPO) versus MP in improving clinical outcome of acute TSCI. Patients aged 18 to 65 years after C5-T12 injury, and grade A or B of the ASIA Impairment Scale (AIS), admitted within 8 h, hemodynamically stable, were randomized to MP according to the NASCIS III protocol or EPO iv (500 UI/kg, repeated at 24 and 48 h). Patients were assessed by an investigator blind to treatment assignment at baseline and at day 3, 7, 14, 30, 60 and 90. Primary end point: number of responders (reduction of at least one AIS grade). Secondary end points: treatment safety and the effects of drugs on a number of disability measures. Frequentistic and post hoc Bayesian analyses were performed. Eight patients were randomized to MP and 11 to EPO. Three patients (27.3 %) on EPO and no patients on MP reached the primary end point (p = 0.17). No significant differences were found for the other disability measures. No adverse events or serious adverse events were reported in both groups. The Bayesian analysis detected a 91.8 % chance of achieving higher success rates on the primary end point with EPO in the intention-to-treat population with a 95 % chance the difference between EPO and MP falling in the range (-0.10, 0.51) and a median value of 0.2. The results of Bayesian analysis favored the experimental treatment.

A noninvasive neuroprosthesis augments hand grasp force in individuals with cervical spinal cord injury: the functional and therapeutic effects.

OBJECTIVES: The primary purpose of this study was to evaluate myoelectrically controlled functional electrical stimulation (MeCFES) for enhancing the tenodesis grip in people with tetraplegia. The second aim was to estimate the potential number of candidates for the MeCFES device. The application of MeCFES provides the user with direct control of the grasp force as opposed to triggered FES systems. METHODS: Screening 253 medical records of C5 to C7 spinal cord injury resulted in 27 participants who trained activities of daily living for 12 × 2 hours, using the MeCFES. Hand function was evaluated by the Action Research Arm Test (ARAT). Primary outcome was the ARAT change score with/without the device, before/after the intervention period. Secondary outcome was the number of positive or clinically relevant change scores with respect to the cohort. RESULTS: The MeCFES improved hand test score in 63% of the subjects at first application. Training resulted in a significant therapeutic effect, which resulted in an overall increase of hand function in 89% of the participants and 30% experienced a clinically relevant change (6 points or more). CONCLUSIONS: Clinical relevance was found both as an assistive aid and as a therapeutic tool in rehabilitation. The therapeutic effect deserves further investigation in clinical studies.

Affordances after spinal cord injury.

Spinal cord injury can cause cognitive impairments even when no cerebral lesion is appreciable. As patients are forced to explore the environment in a non-canonical position (i.e., seated on a wheelchair), a modified relation with space can explain motor-related cognitive differences compared to non-injured individuals. Peripersonal space is encoded in motor terms, that is, in relation to the representation of action abilities and is strictly related to the affordance of reachability. In turn, affordances, the action possibilities suggested by relevant properties of the environment, are related to the perceiver's peripersonal space and motor abilities. One might suppose that these motor-related cognitive abilities are compromised when an individual loses the ability to move. We shed light on this issue in 10 patients with paraplegia and 20 matched controls. All have been administered an affordances-related reachability judgement task adapted from Costantini, Ambrosini, Tieri, Sinigaglia, and Committeri (2010, Experimental Brain Research, 207, 95) and neuropsychological tests. Our findings demonstrate that patients and controls show the same level of accuracy in estimating the location of their peripersonal space boundaries, but only controls show the typical overestimation of reaching range. Secondly, patients show a higher variability in their judgements than controls. Importantly, this finding is related to the patients' ability to perform everyday tasks. Finally, patients are not faster in making their judgements on reachability in peripersonal space, while controls are. Our results suggest that not moving freely or as usual in the environment impact decoding of action-related properties even when the upper limbs are not compromised.

Preserved cardiac autonomic dynamics during sleep in subjects with spinal cord injuries.

BACKGROUND: Spinal cord injuries (SCI) are associated with altered cardiovascular autonomic control (CAC). Sleep is characterized by modifications of autonomic control across sleep stages; however, no data are available in SCI subjects on CAC during sleep. We aim to assess cardiac autonomic modulation during sleep in subjects with SCI. PATIENTS AND METHODS: 27 participants with a neurological and radiological diagnosis of cervical (Cerv, n = 12, ie, tetraplegic) and thoracic SCI (Thor, n = 15, ie, paraplegic) and healthy subjects (Controls) were enrolled. Overnight polysomnographic (PSG) recordings were obtained in all participants. Electrocardiography and respiration were extracted from PSG, divided into sleep stages [wakefulness (W), non-REM sleep (NREM) and REM] for assessment of CAC, using symbolic analysis (SA) and corrected conditional entropy (CCE). SA identified indices of sympathetic and parasympathetic modulation and CCE evaluated the degree of complexity of the heart period time series. RESULTS: SA revealed a reduction of sympathetic and predominant parasympathetic control during NREM compared to W and REM in SCI patients, independent of the level of the lesion, similar to the Controls. In all three groups, complexity of autonomic regulation was higher in NREM compared to W and REM. CONCLUSIONS: In subjects with SCI, cardiac autonomic control changed across sleep stages, with a reduction of sympathetic and an increase of parasympathetic modulation during NREM compared to W and REM, and a parallel increase of complexity during NREM, which was similar to the Controls. Cardiac autonomic dynamics during sleep are maintained in SCI, independent of the level of the lesion.

Bench Comparative Assessment of Mechanically Assisted Cough Devices.

BACKGROUND: Mechanically assisted cough devices are used in patients with impaired cough to avoid secretion accumulation. We compared 5 mechanically assisted cough devices by bench testing using a breathing simulator and assessed their user-friendliness. METHODS: We measured inspiratory and expiratory airway pressures and peak expiratory flow, the strongest indicator of cough efficacy. We performed 2 bench tests: 1) to ascertain the differences between preset and actual settings in 3 different machines of each mechanically assisted cough device and 2) to assess the effects of varying respiratory impedance and air leaks on performance of the devices. We also evaluated the user-friendliness of the devices by measuring the time required and errors in accomplishing 4 tasks by 10 physicians unfamiliar with mechanically assisted cough devices compared with product specialists from the distributing companies. Physicians also scored the ease of use. RESULTS: Four mechanically assisted cough devices during insufflation and all 5 during exsufflation showed differences between preset and actual airway pressures. All but one device showed uneven actual pressure values between models of the same type. Peak expiratory flow was significantly influenced by the mechanical properties in 2 devices and by air leaks in 4 devices. The median time to accomplish all tasks by the product specialist (10 [interquartile range of 2-29] s) was overall significantly shorter compared with all physicians (from 19 [14-65] to 36 [19-116] s). The number of procedural errors, but not the perceived ease of use, differed significantly between the devices. CONCLUSIONS: The performance of different mechanically assisted cough devices was erratic and included variance between models from the same manufacturer; it was affected by respiratory system impedance and air leaks. Time and rate of errors for performing procedures were elevated. These findings indicate that the devices are not interchangeable and that the settings should be targeted for each patient with the specific machine being used. Improvements in reliability, performance, and user-friendliness are advisable.

Sleep apnea and periodic leg movements in the first year after spinal cord injury.

BACKGROUND: Sleep disturbances are frequently reported by patients with spinal cord injury (SCI). Studies have shown an increased incidence of sleep-disordered breathing (SDB) and periodic leg movements during sleep (PLMS) in people with stable long-term SCI. METHODS: This was a prospective observational study in order to evaluate the features and possible predisposing factors of SDB and PLMS in a heterogenic population of consecutive SCI patients admitted at the Spinal Unit of the Niguarda Hospital within the first year after injury. Each patient underwent a clinical assessment, full polysomnography, and arterial blood gas analysis before and immediately after sleep. Multiple logistic regressions were applied in order to evaluate factors associated with SDB and PLMS. RESULTS: Thirty-five (15 tetraplegic and 20 paraplegic) patients were enrolled. Nine patients (25.7%) had an obstructive SDB and 10 (28.6%) had PLMS. The frequency of SDB was higher in tetraplegic with respect to paraplegic patients (Wald statistic: 7.71; P = 0.0055), whereas PLMs were significantly more frequent in patients with an incomplete motor lesion than in subjects with a complete motor lesion (Wald statistic: 6.14; P = 0.013). CONCLUSION: This study confirms a high frequency of SDB and PLMS in SCI patients in the first year following injury. Independently from possible sub-acute and chronic clinical variables, the level and the completeness of the spinal cord lesion are the main factors associated respectively with an early development of SDB and PLMS.

Neurophysiological correlates of sleep leg movements in acute spinal cord injury.

OBJECTIVE: The objective of this study was to analyze the periodicity of leg movement activity emerging during sleep in a group of patients with spinal cord injury and to evaluate their pathophysiological features. METHODS: Twenty patients (16 males, mean age 34.0 years) with traumatic spinal cord lesions were recruited (5 cervical, 15 thoracic; 16 level A and 4 level B at the American Spinal Injury Association impairment scale). Periodicity of sleep leg movements was analyzed; electroencephalographic spectral analysis and heart rate were evaluated for 20s preceding and 30s following the onset of leg movements. RESULTS: Periodic leg movements during sleep (PLMS) index >5/h was found in only 4 patients and only 2 of these had PLMS index >15/h. Eleven patients (group I) did not show any increase in heart rate related to the occurrence of leg movements while the remaining 9 did (group II). Two patients in each group had American Spinal Injury Association impairment level B; 5 patients of group I and none of group II had cervical lesions while 6 patients of group I and all 9 of group II had thoracic lesions. Only 2 patients in group I presented clearly periodic leg movements during sleep and PLMS index >15/h. Electroencephalographic delta, alpha and beta bands around leg movements increased clearly in group II while the changes in group I were very limited or absent. CONCLUSION: Leg movements during sleep are recorded in spinal cord injury patients with completely absent volitional activity in their lower limb but they show clear periodicity only in a small subgroup of them. SIGNIFICANCE: The disconnection from higher nervous structures, in patients with spinal cord injury might favor the appearance of leg movements due to the activity of spinal generators not inhibited by higher influences; correlated autonomic and electroencephalographic changes can be absent. This motor activity might assume the periodic character when a genetic predisposition is present.

Asymptomatic lipomas of the medullary conus: surgical treatment versus conservative management.

OBJECT: The goal of this study was to compare long-term results of surgery with the outcomes of conservative treatment in patients with asymptomatic lipomas of the conus medullaris. METHODS: The parents of 56 consecutive children with a diagnosis of asymptomatic lipoma of the conus medullaris underwent detailed neurosurgical consultation. The pros and cons of both prophylactic surgery and conservative treatment were carefully presented. Both options were offered, and the parents were free to choose the preferred management. A total of 32 children underwent surgical treatment, and 24 were conservatively treated. Afterward, all patients entered the same protocol of serial neurological and urological follow-up at the Centro Spina Bifida. The mean follow-up periods were 9.7 years in the surgical treatment group and 10.4 years in the conservative treatment group. RESULTS: Permanent surgical morbidity was 3.1% (1 patient). During follow-up, tethered cord syndrome occurred in 9.7% of the surgically treated patients (3 of 32 patients) and in 29.1% of the conservatively managed children (7 of 24 patients). This difference did not result in statistical significance, but a clear trend in favor of surgery emerged. Young age at surgery and a cord/sac ratio < 50% appeared to be determining factors in the prevention of subsequent tethered cord syndrome. CONCLUSIONS: The small size of this series does not provide enough statistical evidence that surgical treatment can really improve the natural history of asymptomatic lipomas of the conus medullaris. Nevertheless, surgery appears at least advisable since it reduces by 75% the odds of TCS (p = 0.067), which is quite close to statistical significance.

Motor imagery in spinal cord injury patients: moving makes the difference.

Both real action control and execution and motor imagery abilities require knowledge of the spatial location of body parts, in other words efference copy information and feedbacks from the sensory system (Frith et al., 2000, Philos. Trans. R. Soc. Lond. B. Biol. Sci., 355, 1771). Spinal cord injuries induce severe motor disability, due to a damage of the descending motor pathways (Cramer et al., 2007, Exp. Brain. Res., 177, 233). Patients' motor imagery competences are variably reported as either normal or defective (Decety & Boisson, 1990, Eur. Arch. Psychiatry Clin. Neurosci., 240, 39; Lacourse et al., 1999, Behav. Brain Sci., 104, 73). We explored biomechanical constraint effects in Spinal Cord Injury (SCI) patients, as they are considered the most reliable indexes of motor imagery abilities (Parsons, 1987b, Cogn. Psychol., 19, 178). Sixteen spinal cord injuries patients and 16 neurologically unimpaired subjects have been administered with (1) the Hand Laterality Task (HLT), in which subjects were asked to judge the laterality of a rotated hand; and (2) the Mirror Letter Discrimination Task (MLD), in which subjects were asked to judge if a rotated character was in its correct upright position or mirror-reversed form. Our patients did not present the effect of stimulus orientation, neither did they show any effect related to biomechanical constraints. Based on these data, the hypothesis is that SCI patients' performance may be ascribed to the use of a different strategy to solve the tasks, based on memory rather than on mental rotation.

A cost-of-illness study of spina bifida in Italy.

INTRODUCTION: Spina bifida (SB) is a congenital malformation of the spinal cord, nerves, and adjacent covering structures, with different levels of severity and functional disability. The economic cost of spina bifida and its prevention using folic acid have never been estimated in Italy. This study was conducted to define the cost of illness of SB in Italy. METHODS: A retrospective multicenter observational study on the social cost of patients with SB was carried out in three SB centers in Italy. Cost data were collected relating to the 12 months preceding the enrollment time (T0), and subsequently 3 months after the T0 time (±20 days) through a case report form designed to collect the relevant information on the costs incurred during the period considered. The data for all patients were analyzed through multivariate analysis on the main parameters. RESULTS: We enrolled 128 patients equally divided between males and females, with a mean age of 13 years (minimum, 0; maximum, 29). Diagnosis was mostly postnatal, with 64 cases diagnosed at birth and 33 cases diagnosed subsequently. The lesion severity levels, as defined in the inclusion criteria, were walking (52 patients); walking with simple orthoses (33 patients); walking with complex orthoses (16 patients); and nonwalking, (25 patients). The anatomic type identified is open SB in most cases (84 patients), followed by closed SB (37 patients) and SB occulta (3 patients). The most significant cost per year was for assistive devices, for a total of 4307.00 €, followed by hospitalization (907.00 €), examinations (592.00 €), and drug therapy (328.00 €). Cost breakdown by age range shows that the highest costs are incurred in the 0-4 age range. The highest cost was for cases of open SB (12,103.00 €). The cost/degree of severity ratio showed that the highest cost was for nonwalking patients (14,323.00 €), followed by patients walking with complex orthoses (13,799.00 €). CONCLUSION: The data from this study show that the mean total cost for a patient with SB was 11,351.00 € per year. Based on data provided by the Italian Institute of Health, we can estimate a total annual social cost of about 60 million Euros per year for SB in Italy. Cost of illness was correlated with age and degree of severity of SB.