The perception of disability in cerebral palsy: a cross-sectional study using the WHODAS 2.0.

This observational study aims to describe the level of perceived disability in Cerebral Palsy (CP). We described the perception of adults by using the interviewer-administered version of the WHO disability assessment schedule (WHODAS 2.0). In case of intellectual disability (ID), the proxy-administered version was used, and a caregiver was asked to report the difficulties experienced by the patient; 199 patients were enrolled. The level of perceived disability was higher when referred to patients with ID (proxy report) than when referred to patients without ID (p < .001). For all patients, the level of perceived disability varied depending on the severity and the localization of motor impairment (both p < .001). No differences were observed based on the type of motor impairment. The perceived disability was correlated with age only for patients with no ID (p < .05). The WHODAS 2.0 may be a useful tool to explore the perception of disability in CP.

The perceived impact of Covid-19 pandemic on the children with cerebral palsy: the parents' perspective explored within the "6-F words" framework.

BACKGROUND: In 2020 the world faced the spread of the coronavirus infection disease (Covid-19). This was a general public health emergency but many people with disabilities might have been particularly affected. OBJECTIVE: This paper aims to investigate the impact of the Covid-19 pandemic on children with Cerebral Palsy (CP) and their families. METHODS: 110 parents of children with CP (aged 2 to 19) who completed a questionnaire were included. These children were under the care of one of the Italian Children Rehabilitation Centers. Socio-demographic and clinical information about patients and their families were collected. In addition, difficulties on adopting protective measures and in respecting lockdown rules by children were explored. We adopted the ICF (International Classification of Functioning, Disability and Health) framework to create multiple choice questions. Descriptive statistics were reported and logistic regression analyses were run in order to identify the predictors of perceived impairment in motor, speech, manual and behavioral abilities. RESULTS: Daily activities of children, as well as rehabilitation and fitness sessions, underwent a change during the pandemic. Spending more time with family due to lockdown measures, has had, in some cases a positive effect however there was a perceived decrease in rehabilitation support and school activities. The age range (between 7 and 12 years) and difficulty in respecting rules emerged as significant predictors of the perceived impairment due to Covid-19 pandemic. CONCLUSIONS: The pandemic has had different impacts on children and their families on the basis of children's characteristics. Rehabilitation activities during a hypothetic lockdown should consider these characteristics.

Psychiatric symptoms in adult patients with cerebral palsy: A cohort study.

Background: Patients with cerebral palsy (CP) have an increased risk of developing mental health disorders. Aims: This paper is aimed to investigate the occurrence of psychiatric symptoms in adults with CP and to explore the relation between clinical and psychosocial variables. Methods and procedures: We included 199 adults with a diagnosis of CP. The chi-square and the Mann-Whitney U tests were used to compare clinical and psychosocial variables, the level of perceived disability, and the type of observed parental style in patients with and without psychiatric symptoms. Logistic regression analysis was used to identify variables that could predict the occurrence of mental health disorders. Outcome and results: Anxiety and psychosis were the most represented disorders. Age, living status, assumption of drugs, motor, manual, and global impairment were significantly different between patients with and without psychiatric symptoms. Similarly, a different parental style was observed between the two groups. Logistic regression indicated that living status, prescribed drugs, parental style, and the perceived disability in getting along with others predicted the occurrence of psychiatric symptoms. Conclusions and implications: Results suggest that patients with and without psychiatric symptoms have different clinical and psychosocial characteristics. Some variables should be considered as potentially affecting the mental health of patients with CP.

Trends observed in bilateral cerebral palsy during a thirty-year period: A cohort study with an ICF-based overview.

BACKGROUND: To describe trends observed across thirty years in demographic and clinical characteristics and rehabilitation of patients with bilateral cerebral palsy. METHODS: This retrospective study includes 464 (261 M and 203 F) inpatients with bilateral cerebral palsy, born from 1967 to 1997 and discharged from an outpatient rehabilitative facility from 1985 to 2015. Data concerning the health profile were collected from medical reports and organized in the domains of Body Functions and Structure; Activity and participation and Personal and Environmental factors as proposed in the International Classification of Functioning Disability and Health (ICF). The trend observed over the three birth decades was discussed. RESULTS: The duration of the rehabilitative treatment decreased across decades approximately by two years per decade (from an initial 16.2 yrs to 12.3 yrs). Across the decades the rate of quadriplegia decreased, whereas rates of diplegia increased; spasticity was the prevalent observed motor type for all decades. The most frequent musculoskeletal disorder involved the middle inferior part of the body; among comorbidities a steady decrease in psychiatric disorders was found. With respect to the first decade a slight improvement was observed in the gross motor functioning and in the hand dexterity. No particular trend was observed concerning communication abilities. An increase in the use of pharmacological and surgical treatments for motor symptoms was observed. CONCLUSION: This study presents and describes the functioning of a large sample of Italian patients with bilateral CP on the basis of the ICF framework and it discussed the trend observed across decades.

Functional MRI Studies in Friedreich's Ataxia: A Systematic Review.

Friedreich's ataxia (FRDA) is an inherited neurodegenerative movement disorder with early onset, widespread cerebral and cerebellar pathology, and no cure still available. Functional MRI (fMRI) studies, although currently limited in number, have provided a better understanding of brain changes in people with FRDA. This systematic review aimed to provide a critical overview of the findings and methodologies of all fMRI studies conducted in genetically confirmed FRDA so far, and to offer recommendations for future study designs. About 12 cross-sectional and longitudinal fMRI studies, included 198 FRDA children and young adult patients and, 205 healthy controls (HCs), according to the inclusion criteria. Details regarding GAA triplet expansion and demographic and clinical severity measures were widely reported. fMRI designs included motor and cognitive task paradigms, and resting-state studies, with widespread changes in functionally activated areas and extensive variability in study methodologies. These studies highlight a mixed picture of both hypoactivation and hyperactivation in different cerebral and cerebellar brain regions depending on fMRI design and cohort characteristics. Functional changes often correlate with clinical variables. In aggregate, the findings provide support for cerebro-cerebellar loop damage and the compensatory mechanism hypothesis. Current literature indicates that fMRI is a valuable tool for gaining in vivo insights into FRDA pathology, but addressing that its limitations would be a key to improving the design, interpretation, and generalizability of studies in the future.

Brain Magnetic Spectroscopy Imaging and Hereditary Spastic Paraplegia: A Focused Systematic Review on Current Landmarks and Future Perspectives.

Magnetic resonance spectroscopy (MRS) is a non-invasive neuroimaging technique used to investigate in vivo brain metabolites. MRS could provide a sensitive tool for the study of hereditary spastic paraplegia (HSP) by helping to unveil the underlying biochemical mechanisms and monitoring response to treatment. This focused systematic review aimed to summarize the brain metabolite findings in studies performed in genetically determined HSP. The second aim was to provide a critical analysis and recommendations for well-designed protocols for future studies. Fourteen MRS studies have been analyzed with overall 61 HSP patients, falling within a wide range of age at onset, disease duration, and age at the MRS scan, including children and adults. The genetic diagnosis included several subtypes (SPG2/3/4/5/10/11/28/31/54). SPG11 and SPG54 have been more frequently investigated. The MRS methodology included different MR field strength, not easily comparable spectra areas varying from whole brain to various cortical areas, brain stem and cerebellum sampling. No consistency in disease severity and other outcome measures was observed. The main MRS findings corresponded to the white matter metabolite abnormalities in the corticospinal tracts. In summary, this focused review provides insights on the current knowledge of brain metabolites in HSP and, in particular, in SPG11 and SPG54. Despite the inhomogeneity of the studies to date reported, brain metabolites as assessed by MRS could represent potentially useful diagnostic markers and prognostic indicators of disease progression in HSP. Specific recommendations regarding the MRS technical protocol, CNS area sampling, study design, and applicability of findings are given.

Psychosocial impact of sport activity in neuromuscular disorders.

Previous studies demonstrated the benefits of motor exercise and physical activity in neuromuscular disorders. However, very few papers assessed the effects of sport practise. The aim of this multicentre study was to assess the impact of sport activity on self-esteem and emotional regulation in a cohort of athletes with neuromuscular disorders. The 38 patients with Duchenne, Becker or other types of muscular dystrophy or spinal muscular atrophy practising sport (aged 13-49 years) and 39 age-, gender-, disability- and disease-matched patients not practising sport were enrolled. Testing procedures to assess self-esteem, anxiety and depression disorder, personality trait and quality of life (QoL) were used. Patients practising sport had a significantly higher self-esteem, lower level of depression, greater social own identity and adherence and QoL. Frequency of sport activity may represent a complementary therapy in neuromuscular disorders to improve mental and social well-being.

Becoming a young adult with cerebral palsy.

AIMS: This paper aims to describe the functioning profile of a clinical sample of patients with Cerebral Palsy at the time of transition. METHODS AND PROCEDURE: For this retrospective observational study, we considered data concerning 389 patients born from 1967 to 1997 with a diagnosis of CP and discharged at the age of 18 ±â€¯3 from "La Nostra Famiglia" Children Care Centres. We reported data concerning: identifiable risk factors, the type of CP, the level of motor, manual and communication abilities, the occurrence of associated impairments and environmental factors, and examine the trends over the decades. OUTCOME AND RESULTS: The disorder was mainly bilateral (86%): 57% of patients had quadriplegia and 43% had diplegia. Most of patients had a spastic disorder (86%). Comorbidities were frequent, mainly intellectual developmental disorder (63.5%). One fifth of patients showed a severe impairment of motor, manual and communicative skills in addition to a severe intellectual development disorder. Over decades, the outcome as expressed by the motor functioning profile improved but the outcome as expressed by the global functioning profile worsened because of the growing number of severe multi-disabilities. CONCLUSION AND IMPLICATION: Knowledge about the functioning profile of young adults with cerebral palsy can support health services in the provision of developmentally appropriate care.

Functional and Structural Brain Damage in Friedreich's Ataxia.

Friedreich's ataxia (FRDA) is a rare hereditary neurodegenerative disorder caused by a GAA repeat expansion in the FXN gene. There is still no cure or quantitative biomarkers reliaby correlating with the progression rate and disease severity. Investigation of functional and structural alterations characterizing white (WM) and gray matter (GM) in FRDA are needed prerequisite to monitor progression and response to treatment. Here we report the results of a multimodal cross-sectional MRI study of FRDA including Voxel-Based Morphometry (VBM), diffusion-tensor imaging (DTI), functional MRI (fMRI), and a correlation analysis with clinical severity scores. Twenty-one early-onset FRDA patients and 18 age-matched healthy controls (HCs) were imaged at 3T. All patients underwent a complete cognitive and clinical assessment with ataxia scales. VBM analysis showed GM volume reduction in FRDA compared to HCs bilaterally in lobules V, VI, VIII (L>R), as well as in the crus of cerebellum, posterior lobe of the vermis, in the flocculi and in the left tonsil. Voxel-wise DTI analysis showed a diffuse fractional anisotropy reduction and mean, radial, axial (AD) diffusivity increase in both infratentorial and supratentorial WM. ROI-based analysis confirmed the results showing differences of the same DTI metrics in cortico-spinal-tracts, forceps major, corpus callosum, posterior thalamic radiations, cerebellar penduncles. Additionally, we observed increased AD in superior (SCP) and middle cerebellar peduncles. The WM findings correlated with age at onset (AAO), short-allelle GAA, and disease severity. The intragroup analysis of fMRI data from right-handed 14 FRDA and 15 HCs showed similar findings in both groups, including activation in M1, insula and superior cerebellar hemisphere (lobules V-VIII). Significant differences emerged only during the non-dominant hand movement, with HCs showing a stronger activation in the left superior cerebellar hemisphere compared to FRDA. Significant correlations were found between AAO and the fMRI activation in cerebellar anterior and posterior lobes, insula and temporal lobe. Our multimodal neuroimaging protocol suggests that MRI is a useful tool to document the extension of the neurological impairment in FRDA.

Benefits of Centralized Scheduling in a Postacute Residential Rehabilitation Program for People With Acquired Brain Lesions: A Pilot Study.

OBJECTIVE: To determine whether the use of a designated staff person to coordinate and schedule therapy services in a postacute residential rehabilitation program for people with acquired brain lesions results in (1) a higher-intensity treatment and a reduced length of stay; (2) reduced rehabilitation costs; and (3) increased patient and staff satisfaction. DESIGN: This nonrandomized retrospective study from 2009 through 2012 uses data collected relative to 2 different methods of scheduling: (1) self-planning, in which each single team member makes appointments that are then displayed on a shared board; and (2) managed planning, in which appointments are made by a designated staff person. SETTING: A residential center for people with postacute acquired brain lesions. PARTICIPANTS: Patients (N=20) with acquired brain lesions who had similar clinical and demographic characteristics comprised the managed planning group (n=10) and the self-planning group (n=10). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Organizational measures (length of stay in rehabilitation, number of treatment hours, total cost of rehabilitation), clinical outcome scales (Disability Rating Scale, FIM, and Barthel Index), perceived quality of treatment by patients (questionnaire), and perceived satisfaction of team members (Job Descriptive Index). RESULTS: All patients improved on all clinical rating scales at the time of discharge (all effect sizes are large). In the managed planning group, the number of treatment hours increased (Cohen's d=2.15), resulting in reduced length of stay (Cohen's d=.95) and cost of rehabilitation (Cohen's d=1.22). In addition, the quality of treatment perceived by the patients and their families increased, while team member satisfaction did not change. CONCLUSIONS: The use of a designated staff person to manage therapy services improves efficiency and efficacy of a patient-centered health care system. The proposed scheduling system results in a remarkable cost saving for the National Health System.

A Long Term Effects of a New Onset Psychosis after DBS Treated with Quetiapine in a Patient with Parkinson's Disease.

Deep Brain Stimulation represents a therapeutic option for PD patients. In this paper, we present and discuss a case of acute delirium and psychosis manifesting after DBS in a 58-years-old man affected by Parkinson's Disease. We highlight the importance of an exhaustive psychiatric evaluation in candidates for DBS and we underline the severity and non-reversibility of some adverse events associated with the implantation, suggesting the use of Quetiapine in the management of these effects. Acute psychosis may be listed as a potential severe adverse event associated with DBS, even in patients without a clear cut previous history of psychiatric disorders.

Individual and group treatment for patients with acquired brain injury in comprehensive rehabilitation.

PRIMARY OBJECTIVE: The aim of this study was to investigate the hypothesis that group rehabilitation is more effective than individual treatments and provides an improvement in clinical outcomes similar to that achieved by individual treatments alone. RESEARCH DESIGN: Two groups of patients were placed in different rehabilitation settings treated using the same rehabilitation approach. One received only individual treatments and the second group received a combination of both individual and group treatments. The independent variables were measured both pre- and post-treatment and compared between the two groups. METHODS AND PROCEDURES: Seventy-four patients treated with a comprehensive rehabilitation approach were divided into two groups: (a) individual treatment only and, (b) combined treatments (both individual and group). The outcome scales were LCF (Rancho Los Amigos Level of Cognitive Functioning), DRS (Disability Rating Scale) and FIM™ (Functional Independence Measure). RESULTS: The whole sample had obtained statistically significant improvements in all of the outcome scales: LCF (χ(2) = 45.26; p < 0.001), DRS (z = -3.92; p < 0.001) and FIM (z = -4.9; p < 0.001). The comparison between groups did not reveal any pre-treatment difference. Analysis of post-treatment, however, showed a greater improvement in the FIM scale for those in combined individual and group treatment (z = -0.2544, p = 0.01). CONCLUSIONS: Group rehabilitation integrated with individual treatments is more effective than individual treatments alone in improving independence measured by the FIM™ scale. Both groups had obtained statistically significant clinical improvements, the improvement in the FIM™ scale was significantly better in the combined treatment group.

Auditory attention causes visual inattentional blindness.

When engaged in a visual task, we can fail to detect unexpected events that would otherwise be very noticeable. Here we ask whether a common auditory task, such as that of attending to a verbal stream, can also make us blind to the presence of visual objects that we do not anticipate. In two experiments, one hundred and twenty observers watched a dynamic display while performing either a visual or an auditory attention task, or both simultaneously. When observers were listening to verbal material, in order to either understand it or to remember it (auditory task), their probability of detecting an unexpected visual object was no higher than when they were counting bounces of moving items (visual task), although in the former case the observers' eyes and attention could move around the display freely rather than remaining focused on tracked items. Previous research has shown that attending to verbal material does not affect responses to lights flashing at irregular intervals, suggesting that driving performance is not hampered by listening. The lights, however, were expected. Our data imply that listening to the radio while driving, or to a portable audio player while walking or biking, can impair our reactions to objects or events that we do not expect.

The attentional cost of inattentional blindness.

When our attention is engaged in a visual task, we can be blind to events which would otherwise not be missed. In three experiments, 97 out of the 165 observers performing a visual attention task failed to notice an unexpected, irrelevant object moving across the display. Surprisingly, this object significantly lowered accuracy in the primary task when, and only when, it failed to reach awareness. We suggest that an unexpected stimulus causes a state of alert that would normally generate an attentional shift; if this response is prevented by an attention-consuming task, a portion of the attentional resources remains allocated to the object. Such a portion is large enough to disturb performance, but not so large that the object can be recognized as task-irrelevant and accordingly ignored. Our findings have one counterintuitive implication: irrelevant stimuli might hamper some types of performance only when perceived subliminally.