Cognitive profile in a young woman with Susac syndrome: a case report.

Susac syndrome is a rare immune-mediated endotheliopathy affecting the microvasculature. It presents three main symptoms: encephalopathy, branch retinal artery occlusions, and hearing loss . Here we present a new case report focusing on the evolution of the cognitive profile. The patient underwent two neuropsychological examinations. The first, one month after the onset of the symptomatology, highlighted a prevalent involvement of verbal executive functions. The second, conducted six months later, revealed a global improvement in most previously deficient areas, although with the persistence of a difficulty in cognitive estimation. . This case illustrates the importance of a comprehensive analysis of patients with Susac syndrome to appreciate the whole range of cognitive deficits and reliably evaluate symptom evolution.

Trunk Control Test as a Main Predictor of the Modified Barthel Index Score at Discharge From Intensive Post-acute Stroke Rehabilitation: Results From a Multicenter Italian Study.

OBJECTIVES: To verify whether trunk control test (TCT) upon admission to intensive inpatient post-stroke rehabilitation, combined with other confounding variables, is independently associated with discharge mBI. DESIGN: Multicentric retrospective observational cohort study. SETTING: Two Italian inpatient rehabilitation units. PARTICIPANTS: A total of 220 post-stroke adult patients, within 30 days from the acute event, were consecutively enrolled. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: The outcome measure considered was the modified Barthel Index (mBI), one of the most widely recommended tools for assessing stroke rehabilitation functional outcomes. RESULTS: All variables collected at admission and significantly associated with mBI at discharge in the univariate analysis (TCT, mBI at admission, pre-stroke modified Rankin Scale [mRS], sex, age, communication ability, time from the event, Cumulative Illness Rating Scale, bladder catheter, and pressure ulcers) entered the multivariate analysis. TCT, mBI at admission, premorbid disability (mRS), communication ability and pressure ulcers (P<.001) independently predicted discharge mBI (adjusted R2=68.5%). Concerning the role of TCT, the model with all covariates and without TCT presented an R2 of 65.1%. On the other side, the model with the TCT only presented an R2 of 53.1%. Finally, with the inclusion of both TCT and all covariates, the model showed an R2 increase up to 68.5%. CONCLUSIONS: TCT, with other features suggesting functional/clinical complexity, collected upon admission to post-acute intensive inpatient stroke rehabilitation, independently predicted discharge mBI.

Cognitive and psychological outcomes and follow-up in severely affected COVID-19 survivors admitted to a rehabilitation hospital.

BACKGROUND: The severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) represents the most recent severe pandemic resulting in coronavirus disease 2019 (COVID-19). COVID-19 can damage the central nervous system, requiring admission to intensive care units (ICU) and aggressive treatments (long-term ventilatory assistance and sedation) to stabilize vitals. Most post-COVID-19 patients experience cognitive impairments and mood or stress disorders. We aimed to study the frequency of cognitive deficits in COVID-19 survivors, the relationship between clinical factors in the acute phase and cognitive outcomes, affective states, and quality of life. We explored cognitive reserve (CR) role, as a post-COVID-19 resilience factor. METHODS: Twenty-nine COVID-19 inpatients were assessed using a neuropsychological battery, mood scales, quality of life, and social integration questionnaires. Twenty-five were retained through telephone follow-up to monitor cognitive sequelae, affective states, and reintegration levels roughly 8 months after hospital discharge. We administered the Cognitive Reserve Index questionnaire. RESULTS: We found most patients display no cognitive deficits. When they did, multi-domain impairment occurred most frequently, especially involving executive functions. Results revealed a significant correlation between depression levels and the interval between ICU admission and tracheal tube removal. We found increased levels of depression and anxiety at follow-up, a significant relationship between resuming daily life activities, high CR, and executive functions. CONCLUSIONS: These findings suggest the importance of psychological support in the long term and the modulating role of cognitive reserve in quality of life after infection.

Cognitive Profile in Patients Admitted to Intensive Rehabilitation after Stroke Is Associated with the Recovery of Dysphagia: Preliminary Results from the RIPS (Intensive Post-Stroke Rehabilitation) Study.

Dysphagia represents one of the most frequent symptoms in the post-acute stroke population. Swallowing impairment and cognitive deficits can often co-occur. This study aims to investigate the relationship between cognitive impairment and the recovery of dysphagia in patients attending specific rehabilitation. Patients admitted to intensive rehabilitation units were administered the Functional Oral Intake Scale (FOIS) and Montreal Cognitive Screening Test (MoCA); when screening positive for dysphagia, they entered a rehabilitation program. Their FOIS score at discharge was the primary outcome measure. In the multivariate analysis, younger age (B = - 0.077, p = 0.017), higher MoCA (B = 0.191, p = 0.002), and higher FOIS (B = 1.251, p = 0.032) at admission were associated with higher FOIS at discharge. When executive function (EF) replaced the MoCA total score in the model, younger age (B = - 0.134, p = 0.001), higher admission EF (B = 1.451, p < 0.001), and FOIS (B = 1.348, p = 0.035) were associated with higher FOIS at discharge. Our results confirm the hypothesis that a better cognitive profile upon admission is associated with a higher probability of dysphagia recovery at discharge. EF seems to have a crucial role in dysphagia recovery. These results highlight the importance of considering the cognitive profile when assessing and treating dysphagia after stroke and of using screening tests that include executive functions.

Italian translation and cross-cultural validation of an assessment tool for participation in stroke survivors: the Frenchay Activities Index.

PURPOSE: Participation needs to be assessed objectively, to state accurate rehabilitation objectives. The Frenchay Activities Index (FAI) is a widely used tool to measure participation in stroke patients. To date, no cross-culturally validated Italian version of FAI is available. This study provides a translation and cross-cultural adaptation of FAI into Italian, assessing its validity and reliability in sub-acute stroke patients. METHODS: According to international guidelines, a multistep translation and cultural adaptation protocol of forward and backward translations was conducted by qualified linguists and independent native English translators and revised by a healthcare committee. Patients admitted to intensive inpatient rehabilitation after stroke were recruited. Structural validity, reliability (internal consistency, inter- and intra-rater reliability and measurement error), and construct validity were studied. RESULTS: One hundred and seventy-three patients were included in this study. No significant observations in terms of comprehensibility and conceptual equivalence of the FAI Italian version emerged. The exploratory factorial analysis revealed the presence of two subscales (i.e., domestic chores and work/leisure). The internal consistency resulted good for the first and second subscale (α = 0.821 and 0.716, respectively). Intra- and inter-reliability showed an ICC > 0.90 for both subscales. SEM = 5.75% and 2.33% and MDC = 15.85% and 6.48% were found for the first and second subscale, respectively. Construct validity of first subscale was satisfactory, as 100.0% a priori hypotheses were met, while for the second subscale it was moderate, as 66.6% a priori hypotheses were respected. CONCLUSION: FAI-I provides a tool for professionals to measure participation in Italian stroke patients in health and social care settings.

Cross-validation of predictive models for functional recovery after post-stroke rehabilitation.

BACKGROUND: Rehabilitation treatments and services are essential for the recovery of post-stroke patients' functions; however, the increasing number of available therapies and the lack of consensus among outcome measures compromises the possibility to determine an appropriate level of evidence. Machine learning techniques for prognostic applications offer accurate and interpretable predictions, supporting the clinical decision for personalised treatment. The aim of this study is to develop and cross-validate predictive models for the functional prognosis of patients, highlighting the contributions of each predictor. METHODS: A dataset of 278 post-stroke patients was used for the prediction of the class transition, obtained from the modified Barthel Index. Four classification algorithms were cross-validated and compared. On the best performing model on the validation set, an analysis of predictors contribution was conducted. RESULTS: The Random Forest obtained the best overall results on the accuracy (76.2%), balanced accuracy (74.3%), sensitivity (0.80), and specificity (0.68). The combination of all the classification results on the test set, by weighted voting, reached 80.2% accuracy. The predictors analysis applied on the Support Vector Machine, showed that a good trunk control and communication level, and the absence of bedsores retain the major contribution in the prediction of a good functional outcome. CONCLUSIONS: Despite a more comprehensive assessment of the patients is needed, this work paves the way for the implementation of solutions for clinical decision support in the rehabilitation of post-stroke patients. Indeed, offering good prognostic accuracies for class transition and patient-wise view of the predictors contributions, it might help in a personalised optimisation of the patients' rehabilitation path.

Critical issue on the extinction and inattention subtest of NIHSS scale: an analysis on post-acute stroke patients attending inpatient rehabilitation.

OBJECTIVES: This study aims to evaluate the diagnostic performance of NIHSS extinction and inattention item, compared to the results of the Oxford Cognitive Screen (OCS) heart subtest. Additionally, the possible role of the NIHSS visual field subtest on the NIHSS extinction and inattention subtest performance is explored and discussed. METHODS: We analysed scores on NIHSS extinction and inattention subtest, NIHSS visual field subtest, and OCS heart subtest on a sample of 118 post-stroke patients. RESULTS: Compared to OCS heart subtest, the results on NIHSS extinction and inattention subtest showed an accuracy of 72.9% and a moderate agreement level (Cohen's kappa = 0.404). Furthermore, a decrease in NIHSS accuracy detecting neglect (61.1%) was observed in patients with pathological scores in NIHSS visual field item. CONCLUSIONS: Extreme caution is recommended for the diagnostic performance of extinction and inattention item of NIHSS. Signs of neglect may not be detected by NIHSS, and may be confused with visual field impairment. TRIAL REGISTRATION: This study refers to an observational study protocol submitted to ClinicalTrials.gov with identifier: NCT03968627 . The name of the registry is "Development of a National Protocol for Stroke Rehabilitation in a Multicenter Italian Institution" and the date of the registration is the 30th May 2019.

Some evidence on Gerstmann's syndrome: A case study on a variant of the clinical disorder.

We describe the case of a bilingual patient with persistent symptoms largely, although not fully, consistent with those that are usually reported in Gerstmann's syndrome. Twenty months after a spontaneous primary intracranial hemorrhage, the patient was evaluated with a series of neuropsychological tasks and underwent an MRI investigation based on Diffusion Tensor Imaging probabilistic tractography. The patient suffered from dysgraphia (difficulty in the access to the graphemic representation of letter forms), autotopoagnosia (difficulties in locating body parts on verbal command), right-left confusion (difficulties in localizing right and left side of symmetrical body parts), and number processing/calculation impairments (predominant difficulties on transcoding tasks). Probabilistic tractography revealed a relatively spared superior longitudinal fasciculus and severe damage to the subcortical white matter connecting the angular gyrus with other parietal regions, such as the intraparietal sulcus and the supramarginal gyrus. Within the framework of the contemporary cognitive accounts of Gerstmann's syndrome, the case supports the assumption of an anatomical intraparietal disconnection more than a functional Grundstörung (core impairment).

'Less is more': validation with Rasch analysis of five short-forms for the Brain Injury Rehabilitation Trust Personality Questionnaires (BIRT-PQs).

BACKGROUND: Previous analyses demonstrated a lack of unidimensionality, item redundancy, and substantial administrative burden for the Brain Injury Rehabilitation Trust Personality Questionnaires (BIRT-PQs). OBJECTIVE: To use Rasch Analysis to calibrate five short-forms of the BIRT-PQs, satisfying the Rasch model requirements. METHODS: BIRT-PQs data from 154 patients with severe Acquired Brain Injury (s-ABI) and their caregivers (total sample = 308) underwent Rasch analysis to examine their internal construct validity and reliability according to the Rasch model. RESULTS: The base Rasch analyses did not show sufficient internal construct validity according to the Rasch model for all five BIRT-PQs. After rescoring 18 items, and deleting 75 of 150 items, adequate internal construct validity was achieved for all five BIRT-PQs short forms (model chi-square p-values ranging from 0.0053 to 0.6675), with reliability values compatible with individual measurements. CONCLUSIONS: After extensive modifications, including a 48% reduction of the item load, we obtained five short forms of the BIRT-PQs satisfying the strict measurement requirements of the Rasch model. The ordinal-to-interval measurement conversion tables allow measuring on the same metric the perception of the neurobehavioral disability for both patients with s-ABI and their caregivers.

The unidimensionality of the five Brain Injury Rehabilitation Trust Personality Questionnaires (BIRT-PQs) may be improved: preliminary evidence from classical psychometrics.

Objective: To assess the internal construct validity (ICV) of the five Brain Injury Rehabilitation Trust Personality Questionnaires (BIRT-PQ) with Classical Test Theory methods.Methods: Multicenter cross-sectional study involving 11 Italian rehabilitation centers. BIRT-PQs were administered to patients with severe Acquired Brain Injury and their respective caregivers. ICV was assessed by the mean of an internal consistency analysis (ICA) and a Confirmatory Factor Analysis (CFA).Results: Data from 154 patients and their respective caregivers were pooled, giving a total sample of 308 subjects. Despite good overall values (alphas ranging from 0.811 to 0.937), the ICA revealed that several items within each scale did not contribute as expected to the total score. This result was confirmed by the CFA, which showed the misfit of the data to a unidimensional model (RMSEA ranging from 0.077 to 0.097). However, after accounting for local dependency found within the data, fitness to a unidimensional model improved significantly (RMSEA ranging from 0.050 to 0.062).Conclusion: Despite some limitations, our analyses demonstrated the lack of ICV for the BIRT-PQ total scores. It is envisaged that a more comprehensive ICV analysis will be performed with Rasch analysis, aiming to improve both the measurement properties and the administrative burden of each BIRT-PQ.

The assessment of hemineglect syndrome with cancellation tasks: a comparison between the Bells test and the Apples test.

Unilateral spatial neglect (USN) is a frequent consequence of acquired brain injury, especially following right hemisphere damage. Traditionally, unilateral spatial neglect is assessed with cancellation tests such as the Bells test. Recently, a new cancellation test, the Apples test, has been proposed. The present study aims at comparing the accuracy of these two tests in detecting hemispatial neglect, on a sample of 56 right hemisphere stroke patients with a diagnosis of USN. In order to evaluate the agreement between the Apples and Bells tests, Cohen's kappa and McNemar's test were used to assess differences between the two methods of evaluation. Poor agreement and statistically significant differences emerged between the Apples and Bells tests. Overall, the Apples test was significantly more sensitive than the Bells test in detecting USN. Based on these results, the use of the Apples test for peripersonal neglect assessment is therefore highly recommended.

VRT (verbal reasoning test): a new test for assessment of verbal reasoning. Test realization and Italian normative data from a multicentric study.

Verbal reasoning is a complex, multicomponent function, which involves activation of functional processes and neural circuits distributed in both brain hemispheres. Thus, this ability is often impaired after brain injury. The aim of the present study is to describe the construction of a new verbal reasoning test (VRT) for patients with brain injury and to provide normative values in a sample of healthy Italian participants. Three hundred and eighty healthy Italian subjects (193 women and 187 men) of different ages (range 16-75 years) and educational level (primary school to postgraduate degree) underwent the VRT. VRT is composed of seven subtests, investigating seven different domains. Multiple linear regression analysis revealed a significant effect of age and education on the participants' performance in terms of both VRT total score and all seven subtest scores. No gender effect was found. A correction grid for raw scores was built from the linear equation derived from the scores. Inferential cut-off scores were estimated using a non-parametric technique, and equivalent scores were computed. We also provided a grid for the correction of results by z scores.

Long-term cognitive sequelae in a case of Wernicke's encephalopathy after allogeneic stem cell transplantation.

We describe the case of a non-alcoholic patient with chronic myeloid leukemia who developed iatrogenic Wernicke's encephalopathy (WE) following stem cell transplantation. Four years after the WE acute event, the patient's cognitive profile was mainly characterized by moderate memory impairment, and functional and daily-living difficulties. Our report sustains the hypothesis that a iatrogenic form of WE may produce long-term cognitive sequelae even when thiamine therapy is administered in the acute phase until the resolution of the neurological signs.

Recovery of consciousness after 7 years in vegetative state of non-traumatic origin: A single case study.

PRIMARY OBJECTIVE: To report consistent recovery of consciousness to a state of severe disability in a young patient after 7 years in vegetative state due to severe acquired brain injury, with reflections on protraction of intensive care and expert follow-up for the purpose of intercepting possible, albeit infrequent, cases of late recovery of consciousness. RESEARCH DESIGN: Single case study Methods: This study describes the 9-year history of a healthy 15-year-old who suffered cerebellar haemorrhage due to rupture of an arteriovenous malformation, followed by a brief period of coma then protracted vegetative state; late stabilization of general condition with resolution of neurosurgical complications. Clinical monitoring employed scales for structured assessment of severe disability and disorders of consciousness. RESULTS: The transition from vegetative state to full consciousness occurred over a period of ~ 3 months, 7 years after onset. In the subsequent 2 years the patient has shown slow but progressive overall improvement in a framework of severe residual motor and cognitive disability. CONCLUSIONS: Cases of recovery of consciousness after periods of vegetative state exceeding 1 year are rare, but remind one that a negative prognosis decided too early may be a self-fulfilling forecast.

The Italian version of the Brain Injury Rehabilitation Trust (BIRT) personality questionnaires: five new measures of personality change after acquired brain injury.

The aim of this study was to describe the translation and adaptation of the BIRT personality questionnaires for the Italian population. This included the replication of validity testing and the collection of normative data. Following translation and adaptation according to cross-cultural guidelines, the questionnaires were administered as a pre-test to a sample of 20 healthy subjects and then to 10 patients. The questionnaires were then administered to 120 healthy subjects equally distributed by sex, education, and age, to collect normative data from an Italian population. The questionnaires were easily administered to both healthy subjects and patients. Statistical analysis on normative data was conducted to find the mean value for each questionnaire. This study lays the foundations for using a new instrument to assess behavioral changes after acquired brain injury on the Italian population.

Impaired reading not due to visual field loss in a patient with a right-hemipsheric lesion.

We describe a right-handed patient (M.B.), who developed left hemianopsia and a severe reading impairment after right occipital-parietal hemorrhage. The pattern of his reading deficit was very similar to that of pure alexia (alexia-without-agraphia): extremely slow reading times with frequent grapheme substitutions and omissions. A test of letter reading while controlling for saccadic eye movements and hemifield of presentation ruled out hemianoptic alexia. Although there have already been reports of reading impairments in right handers following right- hemispheric lesions, ours is, to the best of our knowledge, the first where visual field loss can be definitely excluded as the main cause. Based on a standard neuropsychological assessment and on additional behavioral tests, we argue that M.B.'s difficulties are unlikely to be due to right-hemisphere dominance for language. After considering several candidate explanations, we suggest that M.B.'s symptoms may be related to an impairment in attentional processes related to reading.

Modulation of Corticospinal Excitability during Action Observation in Patients with Disorders of Consciousness.

Brain imaging studies have recently provided some evidence in favor of covert cognitive processes that are ongoing in patients with disorders of consciousness (DoC) (e.g., a minimally conscious state and vegetative state/unresponsive wakefulness syndrome) when engaged in passive sensory stimulation or active tasks such as motor imagery. In this exploratory study, we used transcranial magnetic stimulation (TMS) of the motor cortex to assess modulations of corticospinal excitability induced by action observation in eleven patients with DoC. Action observation is known to facilitate corticospinal excitability in healthy subjects, unveiling how the observer's motor system maps others' actions onto her/his motor repertoire. Additional stimuli were non-biological motion and acoustic startle stimuli, considering that sudden and loud acoustic stimulation is known to lower corticospinal excitability in healthy subjects. The results indicate that some form of motor resonance is spared in a subset of patients with DoC, with some significant difference between biological and non-biological motion stimuli. However, there was no covariation between corticospinal excitability and the type of DoC diagnosis (i.e., whether diagnosed with VS/UWS or MCS). Similarly, no covariation was detected with clinical changes between admission and discharge in clinical outcome measures. Both motor resonance and the difference between the resonance with biological/non-biological motion discrimination correlated with the amplitude of the N20 somatosensory evoked potentials, following the stimulation of the median nerve at the wrist (i.e., the temporal marker signaling the activation of the contralateral primary somatosensory cortex). Moreover, the startle-evoking stimulus produced an anomalous increase in corticospinal excitability, suggesting a functional dissociation between cortical and subcortical circuits in patients with DoC. Further work is needed to better comprehend the conditions in which corticospinal facilitation occurs and whether and how they may relate to individual clinical parameters.