Kathleen Kalmar

Evidence-Based Cognitive Rehabilitation: Updated Review of the Literature From 2003 Through 2008

OBJECTIVE To update our clinical recommendations for cognitive rehabilitation of people with traumatic brain injury (TBI) and stroke, based on a systematic review of the literature from 2003 through 2008. DATA SOURCES PubMed and Infotrieve literature searches were conducted using the terms attention, awareness, cognitive, communication, executive, language, memory, perception, problem solving, and/or reasoning combined with each of the following terms: rehabilitation, remediation, and training for articles published between 2003 and 2008. The task force initially identified citations for 198 published articles. STUDY SELECTION One hundred forty-one articles were selected for inclusion after our initial screening. Twenty-nine studies were excluded after further detailed review. Excluded articles included 4 descriptive studies without data, 6 nontreatment studies, 7 experimental manipulations, 6 reviews, 1 single case study not related to TBI or stroke, 2 articles where the intervention was provided to caretakers, 1 article redacted by the journal, and 2 reanalyses of prior publications. We fully reviewed and evaluated 112 studies. DATA EXTRACTION Articles were assigned to 1 of 6 categories reflecting the primary area of intervention: attention; vision and visuospatial functioning; language and communication skills; memory; executive functioning, problem solving and awareness; and comprehensive-holistic cognitive rehabilitation. Articles were abstracted and levels of evidence determined using specific criteria. DATA SYNTHESIS Of the 112 studies, 14 were rated as class I, 5 as class Ia, 11 as class II, and 82 as class III. Evidence within each area of intervention was synthesized and recommendations for Practice Standards, Practice Guidelines, and Practice Options were made. CONCLUSIONS There is substantial evidence to support interventions for attention, memory, social communication skills, executive function, and for comprehensive-holistic neuropsychologic rehabilitation after TBI. Evidence supports visuospatial rehabilitation after right hemisphere stroke, and interventions for aphasia and apraxia after left hemisphere stroke. Together with our prior reviews, we have evaluated a total of 370 interventions, including 65 class I or Ia studies. There is now sufficient information to support evidence-based protocols and implement empirically-supported treatments for cognitive disability after TBI and stroke.

Behavioural improvements with thalamic stimulation after severe traumatic brain injury

Widespread loss of cerebral connectivity is assumed to underlie the failure of brain mechanisms that support communication and goal-directed behaviour following severe traumatic brain injury. Disorders of consciousness that persist for longer than 12 months after severe traumatic brain injury are generally considered to be immutable; no treatment has been shown to accelerate recovery or improve functional outcome in such cases. Recent studies have shown unexpected preservation of large-scale cerebral networks in patients in the minimally conscious state (MCS), a condition that is characterized by intermittent evidence of awareness of self or the environment. These findings indicate that there might be residual functional capacity in some patients that could be supported by therapeutic interventions. We hypothesize that further recovery in some patients in the MCS is limited by chronic underactivation of potentially recruitable large-scale networks. Here, in a 6-month double-blind alternating crossover study, we show that bilateral deep brain electrical stimulation (DBS) of the central thalamus modulates behavioural responsiveness in a patient who remained in MCS for 6 yr following traumatic brain injury before the intervention. The frequency of specific cognitively mediated behaviours (primary outcome measures) and functional limb control and oral feeding (secondary outcome measures) increased during periods in which DBS was on as compared with periods in which it was off. Logistic regression modelling shows a statistical linkage between the observed functional improvements and recent stimulation history. We interpret the DBS effects as compensating for a loss of arousal regulation that is normally controlled by the frontal lobe in the intact brain. These findings provide evidence that DBS can promote significant late functional recovery from severe traumatic brain injury. Our observations, years after the injury occurred, challenge the existing practice of early treatment discontinuation for patients with only inconsistent interactive behaviours and motivate further research to develop therapeutic interventions.

Persistent postconcussion syndrome: The structure of subjective complaints after mild traumatic brain injury.

Objective: To examine the structure of persistent postconcussive symptoms in a sample of patients with mild traumatic brain injury. Design: Multivariate discriminant analysis in a series of 50 consecutive clinical referrals evaluated for postconcussive symptoms, neuropsychological functioning, and personality and emotional functioning at least 3 months after injury; follow‐up information regarding level of disability was obtained for 37 patients at least 1 year after injury. Setting: Neuropsychology clinic affiliated with a comprehensive brain trauma rehabilitation center. Patients: 50 consecutively referred patients with a diagnosis of mild traumatic brain injury referred by physicians, rehabilitation nurses, or attorneys because of persistent deficits or subjective complaints consistent with a postconcussion syndrome. Main Outcome Measure: Postconcussive symptoms endorsed on the Post Mild Traumatic Brain Injury Symptom Checklist. Results: Four factors consisting of multiple symptoms were identified: cognitive factor, affective factor, somatic factor, and sensory factor. Using these four factors, K‐means cluster analysis of subjects was applied to classify patients. Patient clusters consisted of those with minimal symptoms, those with primarily cognitive‐affective symptoms, those with prominent somatic symptoms, and those with severe global symptoms (P = .000). Patient symptom clusters were largely unrelated to neurological or neuropsychological functioning. The presence of chronic disability and resumption of productive functioning differed significantly among groups (P = .003). Conclusions: Subjective complaints provide clinically meaningful information and are strongly related to the nature and extent of disability after mild traumatic brain injury. Characterization of a single postconcussive syndrome may be misleading, and it may be more meaningful to define a number of postconcussive syndromes with differing symptom profiles and recovery.

Assessment Scales for Disorders of Consciousness: Evidence-Based Recommendations for Clinical Practice and Research

OBJECTIVES To conduct a systematic review of behavioral assessment scales for disorders of consciousness (DOC); provide evidence-based recommendations for clinical use based on their content validity, reliability, diagnostic validity, and ability to predict functional outcomes; and provide research recommendations on DOC scale development and validation. DATA SOURCES Articles published through March 31, 2009, using MEDLINE, CINAHL, Psychology and Behavioral Sciences Collection, Cochrane Database of Systematic Reviews, Database of Abstracts of Reviews of Effects, Cochrane Central Register of Controlled Trials, Biomedical Reference Collection, and PsycINFO. Thirteen primary terms that defined DOC were paired with 30 secondary terms that defined aspects of measurement. Scale names, abbreviations, and authors were also used as search terms. Task force members identified additional articles by using personal knowledge and examination of references in reviewed articles. STUDY SELECTION Primary criteria included the following: (1) provided reliability, diagnostic validity, and/or prognostic validity data; (2) examined a cohort, case control, or case series sample of persons with DOC who were age older than or equal to 18 years; and (3) assessed in an acute care or rehabilitation setting. Articles were excluded if peer review was not conducted, original data were not reported, or an English language article was not available. The initial search yielded 580 articles. After paired rater review of study abstracts, guideline development was based on 37 articles representing 13 DOC scales. DATA EXTRACTION Rater pairs classified studies addressing diagnostic and prognostic validity by using the American Academy of Neurology 4-tier level of evidence scheme, and reliability by using a task force-developed 3-tier evidence scheme. An independent quality review of ratings was conducted, and corrections were made. DATA SYNTHESIS The Coma Recovery Scale-Revised (CRS-R), Sensory Stimulation Assessment Measure (SSAM), Wessex Head Injury Matrix (WHIM), Western Neuro Sensory Stimulation Profile (WNSSP), Sensory Modality Assessment Technique (SMART), Disorders of Consciousness Scale (DOCS), and Coma/Near-Coma Scale (CNC) have acceptable standardized administration and scoring procedures. The CRS-R has excellent content validity and is the only scale to address all Aspen Workgroup criteria. The SMART, SSAM, WHIM, and WNSSP demonstrate good content validity, containing items that could distinguish persons who are in a vegetative state, are in a minimally conscious state (MCS), or have emerged from MCS. The Full Outline of UnResponsiveness Score (FOUR), WNSSP, CRS-R, Comprehensive Levels of Consciousness Scale (CLOCS), and Innsbruck Coma Scale (INNS) showed substantial evidence of internal consistency. The FOUR and the CRS-R showed substantial evidence of good interrater reliability. Evidence of diagnostic validity and prognostic validity in brain injury survivor samples had very high levels of potential bias because of methodologic issues such as lack of rater masking. CONCLUSIONS The CRS-R may be used to assess DOC with minor reservations, and the SMART, WNSSP, SSAM, WHIM, and DOCS may be used to assess DOC with moderate reservations. The CNC may be used to assess DOC with major reservations. The FOUR, INNS, Glasgow-Liege Coma Scale, Swedish Reaction Level Scale-1985, Loewenstein Communication Scale, and CLOCS are not recommended at this time for bedside behavioral assessment of DOC because of a lack of content validity, lack of standardization, and/or unproven reliability.

The vegetative and minimally conscious states: A comparison of clinical features and functional outcome

Objective:To compare the vegetative (VS) and minimally conscious states (MCS) with regard to the incidence of specific clinical features and differences in functional outcome across the first year post injury. It was hypothesized that patients diagnosed with MCS on admission to rehabilitation would have more favorable outcomes at 1 year post injury relative to patients diagnosed with VS and that this difference would be more pronounced for traumatic (TBI) versus nontraumatic brain injury (NTB1). Design: Retrospective analysis of clinical findings collected using a standardized methodology (ie, Coma Recovery Scale). Findings were utilized to determine diagnosis and functional outcome at 1, 3, 6, and 12 months post injury. Setting: Acute and extended care brain injury rehabilitation hospital. Patients: 104 patients (62 male, 42 female; mean age = 37 years; mean weeks post injury to admission = 10) with severe TBI or NTB1 (VS = 55; MCS = 49) admitted to a rehabilitation-based coma intervention program. Results: Functional outcome at 3,6, and 12 months post Injury was significantly more favorable for patients diagnosed with MCS relative to those with an admitting diagnosis of VS, and this difference was greatest for patients in MCS following TBI. Visual tracking and motor agitation were found to occur in VS but were of low frequency in comparison to MCS. Conclusions: In patients with severe disorders of consciousness, those diagnosed with MCS show more continuous improvement and attain significantly more favorable outcomes by 1 year post injury than those diagnosed with VS. The presence of specific clinical features, particularly visual tracking, may contribute additional information to differential diagnosis in patients whose level of behavioral responsiveness is severely diminished.

The JFK coma recovery scale—revised

The JFK Coma Recovery Scale (CRS) was developed to help characterise and monitor patients functioning at Rancho Levels I–IV and has been used widely in both clinical and research settings within the US and Europe. The CRS was recently revised to address a number of concerns emanating from our own clinical experience with the scale, feedback from users and researchers as well as the results of Rasch analyses. Additionally, the CRS did not include all of the behavioural criteria necessary to diagnose the minimally conscious state (MCS), thereby limiting diagnostic utility. The revised JFK Coma Recovery Scale (CRS-R) includes addition of new items, merging of items found to be statistically similar, deletion or modification of items showing poor fit with the scales underlying construct, renaming of items, more stringent scoring criteria, and quantification of elicited behaviours to improve accuracy of rating. Psychometric properties of the CRS-R appear to meet standards for measurement and evaluation tools for use in clinical and research settings, and diagnostic application suggests that the scale is capable of discriminating patients in the minimally conscious state from those in the vegetative state. Individuals interested in obtaining a copy of the JFK CRS-R and administration and scoring procedures are referred to the authors.

Diagnostic and prognostic guidelines for the vegetative and minimally conscious states

Many individuals who sustain severe brain injury experience prolonged or permanent disorders of consciousness. While these disorders may appear homogeneous, important distinctions exist in prognosis and clinical management. Studies suggest, however, that the incidence of diagnostic inaccuracy is high in both acute care and rehabilitation settings. In this paper, we review consensus-based diagnostic and prognostic criteria for the vegetative and minimally conscious states. We also discuss recent developments and future directions for research in this area.

The Predictive Validity of a Brief Inpatient Neuropsychologic Battery for Persons With Traumatic Brain Injury

OBJECTIVE To examine the predictive validity of a brief neuropsychologic test battery consisting of the Galveston Orientation and Amnesia Test, the California Verbal Learning Test-II, Trail-Making Test (TMT), Symbol Digit Modalities Test, grooved pegboard, phonemic and categorical word generation tasks, the Wechsler Test of Adult Reading (WTAR), and the Wisconsin Card Sorting Test-64 relative to functional outcome at 1 year in persons with traumatic brain injury. DESIGN Inception cohort study. Follow-up period of 12 months. SETTING Seven Traumatic Brain Injury Model System centers. Neuropsychologic testing was conducted during the acute inpatient rehabilitation stay and functional outcome measures were obtained at 1-year outpatient follow-up. PARTICIPANTS Adults (N=174) who met criteria for admission to inpatient brain injury rehabilitation. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURES FIM instrument, Disability Rating Scale, Supervision Rating Scale, Satisfaction With Life Scale (SWLS), and Glasgow Outcome Scale-Extended. RESULTS Multiple regression analyses revealed that performance on the neuropsychologic test battery was predictive of outcome at 1 year postinjury for all outcome measures, except FIM motor scores and the SWLS. Cognitive performance using this battery was found to predict 1-year outcomes above and beyond functional variables and injury severity variables collected during inpatient rehabilitation, thereby indicating incremental validity for this test battery. Individual tests that were found to be significant predictors of 1-year outcomes included the WTAR and TMT part B. CONCLUSIONS These findings support the clinical utility and ecological validity of this battery with respect to level of disability, functional independence, and supervision required.

Longitudinal outcome of patients with disordered consciousness in the NIDRR TBI model systems programs

Few studies address the course of recovery from prolonged disorders of consciousness (DOC) after severe traumatic brain injury (TBI). This study examined acute and long-term outcomes of persons with DOC admitted to acute inpatient rehabilitation within the National Institute on Disability and Rehabilitation Research (NIDRR) TBI Model Systems Programs (TBIMS). Of 9028 persons enrolled from 1988 to 2009, 396 from 20 centers met study criteria. Participants were primarily male (73%), Caucasian (67%), injured in motor vehicle collision (66%), with a median age of 28, and emergency department Glasgow Coma Scale (GCS) score of 3. Participant status was evaluated at acute rehabilitation admission and discharge and at 1, 2, and 5 years post-injury. During inpatient rehabilitation, 268 of 396 (68%) regained consciousness and 91 (23%) emerged from post-traumatic amnesia (PTA). Participants demonstrated significant improvements on GCS (z=16.135, p≤0.001) and Functional Independence Measure (FIM) (z=15.584, p≤0.001) from rehabilitation admission (median GCS=9; FIM=18) to discharge (median GCS=14; FIM=43). Of 337 with at least one follow-up visit, 28 (8%) had died by 2.1 years (mean) after discharge. Among survivors, 66 (21%) improved to become capable of living without in-house supervision, and 63 demonstrated employment potential using the Disability Rating Scale (DRS). Participants with follow-up data at 1, 2, and 5 years post-injury (n=108) demonstrated significant improvement across all follow-up evaluations on the FIM Cognitive and Supervision Rating Scale (p<0.01). Significant improvements were observed on the DRS and FIM Motor at 1 and 2 years post-injury (p<0.01). Persons with DOC at the time of admission to inpatient rehabilitation showed functional improvement throughout early recovery and in years post-injury.

Does the FOUR score correctly diagnose the vegetative and minimally conscious states

Wijdicks and colleagues recently presented the Full Outline of UnResponsiveness (FOUR) scale as an alternative to the Glasgow Coma Scale (GCS) in the evaluation of consciousness in severely brain-damaged patients. They studied 120 patients in an intensive care setting (mainly neuro-intensive care) and claimed that “the FOUR score detects a locked-in syndrome, as well as the presence of a vegetative state.” We fully agree that the FOUR is advantageous in identifying locked-in patients given that it specifically tests for eye movements or blinking on command. This is welcomed given that misdiagnosis of the locked-in syndrome has been shown to occur in more than half of the cases (see Laureys and colleagues for review). As for the diagnosis of the vegetative state, the scale explicitly tests for visual pursuit, and hence can disentangle the vegetative state from the minimally conscious state (MCS). The diagnostic criteria for MCS have been proposed only recently, but Wijdicks and colleagues do not mention the existence of this clinical entity in their article. As for the vegetative state, MCS can be encountered in the acute or subacute setting as a transitional state on the way to further recovery, or it can be a more chronic or even permanent condition. The MCS refers to patients showing inconsistent, albeit clearly discernible, minimal behavioral evidence of consciousness (eg, localization of noxious stimuli, eye fixation or tracking, reproducible movement to command, or nonfunctional verbalization). The FOUR scale does not test for all of the behavioral criteria required to diagnose MCS. It is known from the literature (see Majerus and colleagues for review) that about a third of patients diagnosed with vegetative state are actually in MCS, and this misdiagnosis can lead to major clinical, therapeutic, and ethical consequences. We tested the ability of the newly proposed FOUR scale to correctly diagnose the vegetative state in an acute (intensive care and neurology ward) and chronic (neurorehabilitation) setting. Patients were assessed using the GCS, FOUR scale, and Coma Recovery Scale-Revised (CRS-R) in randomized order. The latter scale was specifically developed to differentiate vegetative patients from MCS and to identify patients that have emerged from MCS. The basic structure of the CRS-R is similar to the GCS and the FOUR scale, but its subscales are much more detailed, targeting more subtle signs of recovery of consciousness. This increased attention to subtle but potentially important clinical signs lengthens the administration time of the CRS-R and makes it more challenging to use in the intensive care setting. Sixty severely brain-injured, postcomatose (ie, GCS 8) patients were prospectively studied (15 in New Jersey and 45 in Liège). Mean age was 50 years (range, 18–86 years); 39 patients were men. Cause was traumatic brain injury (24 patients), postanoxic-ischemic encephalopathy (14 patients), ischemic or hemorrhagic stroke (9 patients), aneurysmal subarachnoid hemorrhage (4 patients), metabolic encephalopathies (3 patients), status epilepticus (3 patients), encephalitis (2 patients), and craniotomy for brain tumor (1 patient). All patients were assessed free of sedative agents or neuromuscular function blockers, and 22 acute patients were intubated. Thirty patients were studied in the acute setting (ie, within 4 weeks after their brain insult; mean, 11 days; range, 1–24 days), and 30 patients were studied in a chronic setting (ie, more than 4 weeks after the insult; mean, 23 months; range, 1 month to 16 years). Overall, 29 patients (16 acute and 13 chronic patients) were considered as being in a vegetative state based on the GCS (ie, GCS subscores showed spontaneous or stimulationinduced eye opening [E 1]; absence of verbalization [V 3]; and absence of localization of pain [M 5]). The FOUR scale identified 4 of these 29 patients (1/16 acute and 3/13 chronic patients) as not being vegetative given that these patients showed visual pursuit (FOUR scale subscore E 4). This finding confirms the authors’ claim that the FOUR scale is superior to the GCS in detecting a vegetative state “where the eyes can spontaneously open but do not track the examiner’s finger.” However, the CRS-R identified an additional seven patients (four acute and three chronic) showing visual fixation (ie, eyes change from initial fixation point and refixate on a new target location for more than 2 seconds on at least two of four trials), and hence meeting the criteria for MCS set forth by the Aspen Workgroup. Therefore, of the 25 patients identified as being in a vegetative state by the FOUR scale, 7 were diagnosed as being in a MCS by the CRS-R (4/15 acute and 3/10 chronic patients). All seven of these patients showed visual fixation, a clinical sign heralding recovery from the vegetative state, but not included in the FOUR eye response score. In conclusion, we welcome this new scale and its effort to more accurately and expeditiously diagnose the locked-in syndrome by specifically assessing voluntary eye movements. The FOUR scale also adds assessment of eye tracking, which allows it to differentiate vegetative from MCS patients, but it should be noted that both acute and chronic patients may solely show visual fixation, an item not evaluated by the FOUR scale.