Neuro-orthopaedic assessment and management in patients with prolonged disorders of consciousness: A review.

BACKGROUND: Following a severe acquired brain injury, neuro-orthopaedic disorders are commonplace. While these disorders can impact patients' functional recovery and quality of life, little is known regarding the assessment, management and treatment of neuro-orthopaedic disorders in patients with disorders of consciousness (DoC). OBJECTIVE: To describe neuro-orthopaedic disorders in the context of DoC and provide insights on their management and treatment. METHODS: A review of the literature was conducted focusing on neuro-orthopaedic disorders in patients with prolonged DoC. RESULTS: Few studies have investigated the prevalence of spastic paresis in patients with prolonged DoC, which is extremely high, as well as its correlation with pain. Pilot studies exploring the effects of pharmacological treatments and physical therapy show encouraging results yet have limited efficacy. Other neuro-orthopaedic disorders, such as heterotopic ossification, are still poorly investigated. CONCLUSION: The literature of neuro-orthopaedic disorders in patients with prolonged DoC remains scarce, mainly focusing on spastic paresis. We recommend treating neuro-orthopaedic disorders in their early phases to prevent complications such as pain and improve patients' recovery. Additionally, this approach could enhance patients' ability to behaviourally demonstrate signs of consciousness, especially in the context of covert awareness.

Self-Awareness Multilevel Assessment Scale (SAMAS): psychometric analysis of inter-rater reliability.

BACKGROUND: Severe acquired brain injury (sABI) frequently causes impairment in self-awareness (ISA), leading to reduced patients' compliance to treatment, worse functional outcome, and high caregiver distress. Self-awareness (SA) is a multilevel and complex function that, as such, requires a specific and effective assessment. To date, many tools are available to evaluate the declarative, but not emergent and anticipatory levels of awareness, therefore the Self-Awareness Multilevel Assessment Scale (SAMAS) was recently proposed. The new tool proved to be useful to assess SA at different levels across all domains of functioning (motor, cognitive, psycho-behavioural, etc.) because it measures not only the declarative SA, but also emergent and anticipatory levels of SA, thus overcoming some important limits of other current assessment methods. AIM: This study evaluated the inter-rater reliability (IRR) of the SAMAS. METHODS: Four professionals blind to each other evaluated 12 patients with sABI. Each patient was rated by two professionals. RESULTS: Inter-rater reliability was moderate-to-excellent, adding evidence in support of the use of SAMAS to specifically diagnose ISA after sABI. CONCLUSIONS: The SAMAS can help to better address neurorehabilitation, as it allows assessing ISA as early as possible, at all possible levels of awareness and functional domains.

The Effect of Dual-Task Motor-Cognitive Training in Adults with Neurological Diseases Who Are at Risk of Falling.

Falls are common in patients with neurological diseases and can be very problematic. Recently, there has been an increase in fall prevention research in people with neurological diseases; however, these studies are usually condition-specific (e.g., only MS, PD or stroke). Here, our aim was to evaluate and compare the efficacy of an advanced and innovative dual-task, motor-cognitive rehabilitation program in individuals with different neurological diseases who are at risk of falling. We recruited 95 consecutive adults with neurological diseases who are at risk of falling and divided them into four groups: 31 with cerebrovascular disease (CVD), 20 with Parkinson's disease (PD), 23 with traumatic brain injury (TBI) and 21 with other neurological diseases (OND). Each patient completed a dual-task, motor-cognitive training program and underwent two test evaluations to assess balance, gait, fear of falling and walking performance at the pre-and post-intervention. We found that our experimental motor-cognitive, dual-task rehabilitation program was an effective method for improving walking balance, gait, walking endurance and speed, and fear of falling, and that it reduced the risk of falls in patients with different neurological diseases. This study presents an alternative approach for people with chronic neurological diseases and provides innovative data for managing this population.

Pain in Persons with Disorders of Consciousness.

Pain and suffering in persons with disorders of consciousness (DoC) remain poorly understood, frequently unaddressed or inadequately addressed, and controversial on numerous levels. This narrative literature review will address a number of critical issues germane to pain and suffering in this challenging group of patients, providing an introductory overview of the topic, perspectives on current knowledge regarding pain pathoanatomy and pathophysiology, and a review of common pain generators and factors that can lead to the chronifcation of pain. Caveats on bedside pain assessment challenges, as well as electrophysiologic and neuroimaging findings in these patients, will also be explored. Pain management techniques, including non-pharmacological and pharmacological, will be reviewed. Ethical considerations in the context of pain and suffering in persons with disorders of consciousness will round out the review prior to our concluding comments.

Reduced Priming Effect for Visual-Spatial Perspective Taking in Patients With Severe Acquired Brain Injury.

OBJECTIVE: Social cognition can be impaired after a severe acquired brain injury (sABI), but mechanisms potentially underlying these difficulties remain to be clarified. This study aimed at investigating perspective taking ability in individuals with sABI. METHOD: Twenty individuals with sABI and 20 healthy controls (HCs) have been enrolled in this case-control study. All participants were submitted to an experimental visual-spatial priming memory procedure and a self-report assessment of perspective taking (i.e., the Interpersonal Reactivity Index [IRI]). Individuals with sABI were submitted to neuropsychological tests to assess executive subcomponents, working memory, and visual attention. RESULTS: The analysis on self-report scales data documents a significant between groups difference in the IRI-Fantasy subscale, with HCs showing a higher tendency to imaginatively transpose oneself into fictional situations than individuals with sABI. Analysis of performance on the experimental procedure revealed the priming effect in HCs but not in sABI individuals. Moreover, individuals with sABI performed significantly poorer than HCs on the indices of the experimental procedure. CONCLUSIONS: Our data preliminarily demonstrated that visual-spatial perspective taking is reduced after sABI. Findings above could give some clues for the rehabilitative intervention in sABI and suggest the possible application of the procedure here used in assessing perspective taking after sABI.

Disorders of consciousness terminology: history, evolution and future directions.

There has been a significant evolution of nomenclature with regards to classification of persons with disorders of consciousness (DoC) over the last 100 years. This paper provides a review of the evolution of this terminology with discussion of the advantages and disadvantages of historical and current terms. Recommendations for how this evolution should continue moving forward in the best interest of patients, their families, society, clinical care, and research will also be addressed. The taxonomy we choose, hopefully by international consensus, has multifaceted implications that go well beyond just a debate on nomenclature.

Late recovery of responsiveness after intra-thecal baclofen pump implantation and the role of diffuse pain and severe spasticity: a case report.

Patients with a prolonged disorder of consciousness (DoC) may present with severe spasticity and diffuse pain, which might impair motor output, thus preventing any possible behavioral responsiveness. A 26-year-old man affected by frontoparietal hemorrhage was operated by hematoma evacuation and decompressive craniectomy; coma persisted for 1 month; cranioplasty and ventriculo-peritoneal shunting was performed after 4 months. At admission in rehabilitation, he was diagnosed as vegetative state/unresponsive wakefulness syndrome (VS/UWS). The implantation of intrathecal baclofen (ITB) pump (Medtronic SynchroMed™ II), 14 months after, (60 µg/daily), dramatically improved behavioral responsiveness according to Coma Recovery Scale-Revised (CRS-R) from 6 to 12 (1 month after ITB). Nociception Coma Scale-Revised (NCS-R) also changed from 4 to 8 at the same time points. This case report may be an example of covert cognition that should have been diagnosed as a functional locked-in syndrome or motor-cognitive dissociation, rather than as VS/UWS.

An International survey on diagnostic and prognostic protocols in patients with disorder of consciousness.

To date, no international guidelines or recommendations for diagnosis or prognosis of patients with disorders of consciousness (DoC) have been established. The International Brain Injury Association's (IBIA) Special Interest Group on Disorders of Consciousness (DoC-SIG) launched an international multicenter survey to compare diagnostic and prognostic procedures across countries and clinical settings. Objectives: To explore which specific diagnostic protocols and prognostic indices were utilized in the care for persons with DoC in different countries and to determine the usage, if any, of national guidelines in the care of such patients. Methods: The questionnaire included 17 questions in two distinct sections (I - clinical and instrumental tools and involvement of caregivers and II - clinical, anamnestic and instrumental markers). Results: Physicians composed 50% of the survey respondents (120) and were all involved in post-acute rehabilitation care. In the majority of countries, respondents reported that there were no national guidelines or recommendations for DoC care. The Glasgow Coma Scale (GCS) and the Coma Recovery Scale-Revised (CRS-R) were the most frequently used clinical scales for diagnostic purposes. The majority of respondents reported the involvement of caregivers in the evaluation of behavioral responsiveness of patient with DoC. The survey indicated that only a few centers performed neurophysiological investigations routinely as diagnostic instrumental procedures. Our results suggest that international guidelines and recommendations for the care of persons with DoC still need to be formulated and ideally agreed to by consensus.

Coma Recovery Scale-Revised With and Without the Emotional Stimulation of Caregivers.

The Coma Recovery Scale-Revised (CRS-R) is the gold standard of responsiveness assessment in patients with disorder of consciousness. The purpose of this study is to search for the efficacy of the caregivers' involvement in the evaluation of responsiveness in these patients. Responsiveness assessment was performed in 15 patients with CRS-R. The CRS-R was administered with and without the emotional stimulation of the primary caregiver at different times. Our preliminary findings seem to suggest that, including also the caregivers during CRS-R assessment, may obtain better responsiveness scoring than that obtained by professionals and might reduce the misdiagnosis rate.

Résultats à l'échelle d'évaluation d'éveil lors d'un coma avec ou sans la stimulation affective de personnes soignantes. L'échelle d'évaluation d'éveil lors d'un coma (Coma Recovery Scale-Revised) demeure la norme de référence en matière d'évaluation de la réactivité de patients aux prises avec des troubles de la conscience. L'objectif de cette étude est d'analyser l'impact de l'implication de personnes soignantes dans l'évaluation de la réactivité de ces patients. Une telle analyse a été effectuée chez quinze patients soumis à l'échelle d'évaluation d'éveil lors d'un coma, et ce, avec ou sans la stimulation affective d'une personne soignante et à différents moments. À cet égard, nos constatations préliminaires semblent indiquer que les scores de réactivité à cette échelle pourraient, en présence de personnes soignantes, dépasser ceux obtenus en compagnie de professionnels et ainsi réduire les taux de diagnostics erronés.

Language-Related Brain Potentials in Patients With Disorders of Consciousness: A Follow-up Study to Detect "Covert" Language Disorders.

Background. Language disorders may occur in patients with disorders of consciousness (DoCs), and they could interfere with the behavioral assessment of consciousness and responsiveness. Objective. In this study, we retrospectively explored whether ERP N400 was eventually associated with the presence of aphasia diagnosed in those patients who had evolved into Exit-Minimally Conscious State (E-MCS) at the clinical follow-up. Methods. In this retrospective cohort study, the ERPs elicited by an auditory sentences task were retrospectively examined in a sample of 15 DoC patients diagnosed according to the Coma Recovery Scale-Revised (CRS-R). All these 15 DoC patients underwent a (at least) 1-year clinical follow-up, which included a neuropsychological evaluation to assess language function among other cognitive functions. Ten healthy individuals also underwent the same paradigm to investigate the variability of ERPs characteristics. Results. The N400 ERP component with centroparietal topography was found in 9 of 10 healthy controls in response to the ill-formed sentences. Analysis of patients' data revealed that (1) a significant N400 component could be detected in 64% (9 of 14 patients) of the DoC patients regardless of the type of DoC; (2) no significant N400 ERP component was retrospectively detected in those E-MCS patients who showed aphasia at the follow-up; and (3) the presence/absence of the N400-ERP component was consistent with the brain lesion side and significantly predict the recovery. Conclusion. These preliminary findings indicate that the absence of N400 was significantly associated with the presence of aphasia diagnosed at the clinical follow-up in E-MCS patients.

A new tool to assess responsiveness in disorders of consciousness (DoC): a preliminary study on the Brief Post-Coma Scale (BPCS).

INTRODUCTION: The Brief Post-Coma Scale (BPCS) is an easy diagnostic tool for individuals with disorders of consciousness (DoC), in a reduced version from a previously Post-Coma Scale, that could distinguish patients in the minimally conscious state (MCS) from those in unresponsive wakefulness syndrome (UWS), formerly defined as vegetative state (VS). OBJECTIVE: Aim of the study was to assess the diagnostic validity of the BPCS in comparison with the Coma Recovery Scale-Revised (CRS-R), in its Italian validated version, the Disability Rating Scale (DRS), the Level of Cognitive Functioning (LCF), and the Glasgow Outcome Scale (GOS). METHODS: In an Italian multicenter study on 545 patients with DoC, 36 post-acute rehabilitation wards, 32 long-term care centers, and 2 family associations participated to data collection. RESULTS: Statistically significant correlations were found between the BPCS and the other clinical scales: R = 0.586 (p < 0.001) with LCF, R = - 0.566 (p < 0.001) with DRS, R = 0.622 (p < 0.001) with CRS-R. The BPCS scores resulted significantly correlated with the time from acute event (R = 0.117, p = 0.006). Patients with GOS score 2 had mean BPCS of 1.84 ± 1.19, whereas those with GOS 3 had significantly higher scores 3.88 ± 1.71 (p < 0.001). Similarly, in patients with vegetative state/UWS (VS/UWS), the mean BPSC score was 1.71 ± 1.09, significantly lower (p < 0.001) than that of patients with minimally conscious state (BPCS = 3.83 ± 1.29). Finally, the agreement of the BPCS and clinical diagnosis was of 84.4%, with an odds ratio OR = 3.781 (95% CI = 3.026-4.725, p < 0.001). CONCLUSIONS: The BPCS has demonstrated statistically significant correlations with the most commonly used scales in persons with DoC.

Improvement rate of patients with severe brain injury during post-acute intensive rehabilitation.

Patients with severe acquired brain injury (SABI) may evolve towards different outcomes. The primary aim was to evaluate the clinical evolution of a large population of patients with SABI admitted to post-acute rehabilitation from 2001 to 2016, diagnosed with severe brain injury (GCS ≤ 8) in the acute phase and a coma duration of at least 24 h. The possible changes between the admission time to a post-acute rehabilitation hospital and the discharge time were measured by means of Glasgow Outcome Scale (GOS), Level of Cognitive Functioning (LCF), and Disability Rating Scale (DRS). We also correlated the improvement rate with some sociodemographic and clinical features of the individuals with SABI enrolled. Data of 890 patients were analyzed (54% TBI, length of stay = 162 ± 186 days, GCS = 7.46 ± 1.28); time interval from the SABI (OR = 0.246, CI 95% = 0.181 - 0.333), scores at admission of LCF (OR = 2.243, CI 95% = 1.492 - 3.73), GOS (OR = 0.138, CI 95% = 0.071 - 0.266), DRS (OR = 0.457, CI 95% = 0.330 - 0.632), and etiology (OR = 2.273, CI 95% = 1.676 - 3.084) played a significant role (p < 0.001, explained variance 69.9%) for improving GOS score. Time interval from the SABI to admission in our post-acute rehabilitation ward (OR = 0.300, CI 95% = 0.179 - 0.501, p < 0.001), length of rehabilitation stay (OR = 2.808, CI 95% = 1.694 - 4.653, p < 0.001), and etiology (OR = 1.769, CI 95% = 1.095 - 2.857, p = 0.020) led to a statistically significant improvement in DRS (explained variance 91%). The most significant predictive factors for the outcome of patients with SABI were etiology, time interval from SABI to admission in rehabilitation, and length of rehabilitation stay.

Retrograde Amnesia for Episodic and Semantic Memories in Amnestic Mild Cognitive Impairment.

Retrograde amnesia (RA), which includes loss of memory for past personal events (autobiographical RA) and for acquired knowledge (semantic RA), has been largely documented in patients with amnestic mild cognitive impairment (aMCI). However, previous studies have produced controversial results particularly concerning the temporal extent of memory impairment. Here we investigated whether, with the onset of hippocampal pathology, age of memory acquisition and retrieval frequency play different roles in modulating the progressive loss of semantic and episodic contents of retrograde memory respectively. For this purpose, aMCI patients and healthy controls were tested for the ability to recall semantic and autobiographical information related to famous public events as a function of both age of acquisition and retrieval frequency. In aMCI patients, we found that the impairment in recollecting past personal incidents was modulated by the combined action of memory age and retrieval frequency, because older and more frequently retrieved episodes are less susceptible to loss than more recent and less frequently retrieved ones. On the other side, we found that the loss of semantic information depended only on memory age, because the remoteness of the trace allows for better preservation of the memory. Our results provide evidence that the loss of the two components of retrograde memory is regulated by different mechanisms. This supports the view that diverse neural mechanisms are involved in episodic and semantic memory trace storage and retrieval, as postulated by the Multiple Trace Theory.

Does retrieval frequency account for the pattern of autobiographical memory loss in early Alzheimer's disease patients?

Episodic autobiographical memory (ABM) has been found to be impaired from the early stage of Alzheimer's disease (AD). Previous works have focused on how ABM decreases over the lifespan, but no study has deeply investigated whether the extent of episodic autobiographical amnesia is mediated by the retrieval frequency of the episodic trace itself. The aim of the present study was to determine whether the frequency of trace retrieval has an effect on the quality of autobiographical incidents recall and whether the extent of this contribution changes over time. For this purpose, the episodic component of ABM was assessed in patients in the early stage of AD through a questionnaire which allowed evaluating memory of past personal incidents as a function of both their age of acquisition and retrieval frequency. We found that both AD patients and healthy controls took advantage of greater retrieval frequency across all time segments, because of their better memory performance on frequently retrieved episodes than less frequently retrieved ones. Although in the AD group the retrieval frequency effect (i.e., higher scores on the episodes rated as more frequently retrieved) was found in all time segments, the extent of its beneficial effect on memory performance was temporally-graded and inversely related to the time course. Our findings provide new evidence that the combined action of both age of memory and retrieval frequency could provide a valuable framework for predicting patterns of ABM loss, at least in early AD patients. In line with the Multiple Trace Theory, we speculated that retrieval frequency protects episodic trace recall against hippocampal damage by reinforcing the neural representation of personal context-rich memories, which consequently are easier to access and recall. Furthermore, the age of memory should change the amplitude of this beneficial effect as a function of the remoteness of the trace.