Mild Cognitive Deficits Research Articles

Cost of malingering mild traumatic brain injury-related cognitive deficits during compensation and pension evaluations in the veterans benefits administration

ABSTRACTGiven the high rates of exaggeration in those claiming long-term cognitive deficits as a result of mild traumatic brain injury (mTBI), the aim of this study was to evaluate the rates of malingering in those seeking disability through the Veterans Benefits Administration and estimate the financial burden of disability payments for those receiving compensation despite exaggerated mTBI-related cognitive deficits. Retrospective review included 74 veterans seen for Compensation and Pension evaluations for mTBI. Rates of malingering were based on failure of the Medical Symptom Validity Test (MSVT) and/or the Test of Memory Malingering (TOMM) trial 1 ≤ 40. Total estimated compensation was based on the level of disability awarded and the number of individuals found to be malingering cognitive deficits. Overall, 33–52% of the sample was found to be malingering mTBI-related cognitive deficits. The malingering groups were receiving approximately $71,000–$121,000/year ($6,390–$7,063 per year, per veteran on average). Estimated nationwide disability payments for those possibly malingering mTBI-related cognitive deficits would be $136–$235 million/year (projected costs from 2015–2020 = $700 million–$1.2 billion). It is critical that providers and administrative officials identify those exaggerating disability claims attributed to mTBI. The cost of malingering impacts society in general as well as veterans themselves, as it diverts needed funds/resources away from those legitimately impaired by their military service.

Apathy in Patients with Parkinson's Disease Correlates with Alteration of Left Fronto-Polar Electroencephalographic Connectivity.

Introduction: Quantitative electroencephalography (QEEG) brain frequency and network analyses are known to differentiate between disease stages in Parkinson's disease (PD) and are possible biomarkers. They correlate with cognitive decline. Little is known about changes in brain networks in relation to apathy.Objective/Aims: To analyze changes in brain network connectivities related to apathy.Methods: 40 PD patients (14 PD with mild cognitive deficits and 26 PD with normal cognition) were included. All patients had extensive neuropsychological testing; apathy was evaluated using the apathy evaluation score (AES, median 24.5, range 18–39). Resting state EEG was recorded with 256 electrodes and analyzed using fully automated Matlab® code (TAPEEG). For estimation of the connectivities between brain regions, PLI (phase lag index) was used, enhanced by a microstates segmentation.Results: After correction for multiple comparisons, significant correlations were found for single alpha2-band connectivities with the AES (p-values < 0.05). Lower connectivities, mainly involving the left fronto-polar region, were related to higher apathy scores.Conclusions: In our sample of patients with PD, apathy correlates with a network alteration mainly involving the left fronto-polar region. This might be due to dysfunction of the cortico-basal loop, modulating motivation.

Early childhood myoclonic epilepsy: An independent genetic generalized epilepsy with myoclonic seizures as the main seizure type.

To elucidate the characteristics of the myoclonic seizures alone, or predominant myoclonus combined with generalized tonic-clonic seizures (GTCS) and/or absences, in early childhood, and discuss its classification. Forty-two children were retrospectively recruited between January 2006 and June 2015. The mean age of seizure onset was 40.5months. They were divided into 4 groups: myoclonic seizures alone; predominant myoclonus combined with GTCS; predominant myoclonus combined with absences; predominant myoclonus combined with both GTCS and absences. Interictal EEG showed generalized spike- or polyspike-wave discharges at 2-4Hz. Seizures were controlled in 22 patients at a mean age of 60.5months. The psychomotor development was normal (30/37) or mildly delayed (7/37). We reported a cohort of patients with early childhood myoclonic epilepsy (ECME), with the following characteristics: Seizures started below 5years old in otherwise normal children; Seizure types included myoclonic seizures alone or combined with GTCS and/or absences; Febrile or afebrile GTCS might appear firstly; Interictal EEG showed generalized spike- or polyspike-wave; Seizures usually were in remission before adolescence with normal development or mild cognitive or behavioral deficits in most. ECME might be an independent epileptic syndrome not established by International League Against Epilepsy (ILAE) previously.

PRE-SCREENING FOR EPISODIC MEMORY DEFICITS CAN ENRICH FOR ELIGIBLE SUBJECTS IN EARLY ALZHEIMER’S DISEASE CLINICAL TRIALS

Positive biomarkers and a mild cognitive deficit define Prodromal Alzheimer's disease (AD). A key challenge of clinical trials is identifying subjects to meet screening criteria for both biomarker and cognitive deficits. Scores on the CANTAB Paired Associates Learning (PAL) task of episodic memory correlate with AD biomarkers. Pre-screening with PAL is easy to implement and is time as well as cost effective. Here we assess the use of PAL to enhance samples for biomarker positive subjects and deficits in other cognitive screening tasks; RBANS total score, delayed memory index, Clinical Dementia Rating Scale (CDR), and Mini-mental state examination (MMSE). During screening for an early Alzheimer's disease trial, data was collected on cognitive function and CSF biomarkers (AB1–42,Tau) in adults aged over 50 (n= 56). Estimates for improving sample selection were based on the age-specific probability of biomarker positivity in the general population together with the sensitivity and specificity of episodic memory tasks (PAL and RBANS). Ability of PAL to detect a deficit in RBANS, CDR and MMSE was based on demographically adjusted logistic regression, and subject scores relative to quantile bands of normative (PAL) data. The correlation between PAL and the RBANS delayed memory index was -0.81, and PAL predicted a deficit in RBANS (>-1sd) with an accuracy of 89%; the AUC was 0.96. Participants with a predicted deficit on RBANS also showed a lower average MMSE score (24.8 vs 28.6) and a higher CDR-SOB score (1.85 vs 0.48). Performance on PAL (-1sd) worse than age adjusted norms (∼1700) displayed high specificity for identifying deficits in the RBANS and CDR. The number of subjects screened to identify those who are AB biomarker positive is reduced with increasing impairment on PAL, in accordance with the sensitivity and specificity. PAL can validily identify subjects’ with episodic memory deficits, early in the recruitment process, to enrich samples for biomarker positive subjects, and identify those likely to show deficits in other cognitive tasks. As such, PAL has potential for reducing trial costs, screen failure rates, site and patient burden, by targeting subjects with a high probability of meeting screening criteria.

Incongruence of subjective memory impairment ratings and the experience of memory problems in older adults without dementia: a mixed methods study

ABSTRACTObjectives: The objective of this study was to describe the experiences of older adults living with subjective memory impairment (SMI) and examine the extent to which SMI severity was associated with impact of SMI on daily life.Method: A mixed methods convergent design was utilized. Participants with SMI (n = 19, mean age 80.7 years) were recruited from community settings. Semi-structured interviews were analyzed using thematic analysis; these findings were integrated with descriptive statistics from questionnaire and cognitive status data.Results: The impact of SMI varied depending on the personal meaning individuals attributed to the experience. Older adults with normal cognition reported episodic memory problem compared to more pervasive problems reported by participants with mild cognitive deficits. The impact of memory problems ranged from frustration/embarrassment to avoidance of social activities, but the degree of emotional impact was not reflected in SMI severity or cognitive status.Conclusion: SMI is common in older adults without dementia but the impact on function and well-being is variable and does not seem to be associated with objective cognition. Future research is needed to validate these associations and to inform the development of SMI measures that accurately reflect older adults’ experiences.

Region specific oligodendrocyte transcription factor expression in a model of neonatal hypoxic injury

White matter injury (WMI) of prematurity is associated with a spectrum of neurological disorders ranging from mild cognitive and behavioral deficits to cerebral palsy. Translational studies have implicated impaired oligodendrocyte development after hypoxia as the primary cause of WMI, but the underlying mechanisms remain poorly understood. The goal of this study was to identify alterations in the expression of oligodendrocyte precursor cell transcription factors in a mouse model of transient mild global hypoxia.Postnatal day (P) 7 mouse pups were exposed to hypoxia (7.5% O2) for 60minutes. We compared oligodendrocyte differentiation and subsequent myelin formation between hypoxia and sham animals at P9, P14 and P28 by examining the expression of key transcription factor regulators of oligodendrocyte differentiation (Ascl1, Olig1, Olig2, and Nkx2.2), as well as APC, a mature oligodendrocyte marker, in the major white matter regions including the corpus callosum, external capsule and anterior commissure. We also examined the effect on myelin formation by examining two myelin specific protein constituents, myelin associated glycoprotein (MAG) and myelin basic protein (MBP), in white matter tracts and whole brain lysate respectively.We found that transient hypoxia at P7 altered the expression of Ascl1, Olig1 and Nkx2.2, resulting in delayed myelination in the external capsule. In addition, our study showed that oligodendrocyte progenitor cells specified several days prior to a hypoxic event are more susceptible to maturation arrest than those specified shortly prior to hypoxia. Our results suggest that alterations of Ascl1, Olig1 and Nkx2.2 underlie impaired oligodendrocyte differentiation and deficient myelination in WMI. These transcription factors are potential therapeutic targets for the treatment of WMI in preterm infants.

Association Between 2 Measures of Cognitive Instrumental Activities of Daily Living and Their Relation to the Montreal Cognitive Assessment in Persons With Stroke

ObjectivesTo explore the relation between a computer adaptive functional cognitive questionnaire and a performance-based measure of cognitive instrumental activities of daily living (C-IADL) and to determine whether the Montreal Cognitive Assessment (MoCA) at admission can identify those with C-IADL difficulties at discharge. DesignProspective cohort study. SettingAcute inpatient rehabilitation unit of an academic medical center. ParticipantsInpatients (N=148) with a diagnosis of stroke (mean age, 68y; median, 13d poststroke) who had mild cognitive and neurological deficits. InterventionsNot applicable. Main Outcome MeasuresAdmission cognitive status was assessed by the MoCA. C-IADL at discharge was assessed by the Executive Function Performance Test (EFPT) bill paying task and Activity Measure of Post-Acute Care (AM-PAC) Applied Cognition scale. ResultsGreater cognitive impairment on the MoCA was associated with more assistance on the EFPT bill paying task (ρ=−.63; P<.01) and AM-PAC Applied Cognition scale (ρ=-.43; P<.01). This relation was nonsignificant for higher MoCA scores and EFPT bill paying task scores. The AM-PAC Applied Cognition scale and the EFPT bill paying task had low agreement in classifying functional performance (Cohen's κ=.20). A receiver operating characteristic curve identified optimal MoCA cutoff scores of 20 and 21 for classifying EFPT bill paying task status and AM-PAC Applied Cognition scale status, respectively. For values above 20 and 21, sensitivity increased whereas specificity decreased for classifying functional deficits. Approximately one third of the participants demonstrated C-IADL deficits on at least 1 C-IADL measure at discharge despite having a MoCA score of ≥26 at admission. ConclusionsQuestionnaire and performance-based methods of assessment appear to yield different estimates of C-IADL. Low MoCA scores (<20) are more likely to identify those with C-IADL deficits on the EFPT bill paying task. The results suggest that C-IADL should be assessed in those who have mild or no cognitive difficulties at admission.

Accelerated Age-Dependent Hippocampal Volume Loss in Parkinson Disease With Mild Cognitive Impairment.

Patients with Parkinson disease are at high risk of developing dementia. During the course of the disease, a substantial number of patients will experience a cognitive decline, indicating the dynamics of the underlying neuropathology. Magnetic resonance imaging (MRI) has become increasingly useful for identifying structural characteristics in radiological brain anatomy existing prior to clinical symptoms. Whether these changes reflect pathology, whether they are aging related, or both often remains unclear. We hypothesized that aging-associated brain structural changes would be more pronounced in the hippocampal region among patients with Parkinson disease having mild cognitive deficits relative to cognitively unimpaired patients. Using MRI, we investigated 30 cognitively healthy patients with Parkinson disease and 33 patients with nondemented Parkinson disease having mild cognitive impairment. All participants underwent structural MRI scanning and extensive clinical and neuropsychological assessments. Irrespective of the study participants' cognitive status, older age was associated with reduced cortical thickness in various neocortical regions. Having mild cognitive impairment was not associated with an increased rate of cortical thinning or volume loss in these regions, except in the hippocampus bilaterally. Patients with Parkinson disease having mild cognitive impairment show an accelerated age-dependent hippocampal volume loss when compared with cognitively healthy patients with Parkinson disease. This may indicate pathological processes in a key region for memory functioning in patients with Parkinson disease at risk of developing dementia. Structural MRI of the hippocampal region could potentially contribute to identifying patients who should receive early treatment aimed at delaying the clinical onset of dementia.

Nonpharmacological treatment procedures for Parkinson's disease

Nonpharmacological treatment strategies in Parkinson' disease include heterogeneous treatment modalities, such as physiotherapy, occupational therapy, speech therapy, cognitive training and deep brain stimulation as well as noninvasive brain stimulation strategies. Even in the early stages of Parkinson's disease nonpharmacological interventions, such as active exercise therapy and speech therapy can be indicated taking the individual symptoms of apatient into account. Mild cognitive deficits are frequently detected in the course of the disease and progression of these disorders to dementia in the advanced stages of the disease is not uncommon. The starting point for acognitive training, training strategy and training frequency is unknown and currently under investigation. Deep brain stimulation is an established treatment modality, which should be considered when motor fluctuations cannot be adequately controlled by pharmacological treatment. This therapeutic option depends on patient-specific needs and has to be managed by amultiprofessional team. Non-invasive neurostimulation techniques, such as transcranial magnetic stimulation and transcranial direct current stimulation are experimental tools and cannot currently be recommended for general use.

Cognitive profiles of patients with early detected and treated congenital hypothyroidism.

Children with congenital hypothyroidism (CH) detected by newborn screening and adequately treated may have mild cognitive deficits. OBJECTIVES">To assess the intelligence quotient of children with CH and identify the presence of specific cognitive deficits. A group of 60 children with CH detected by newborn screening, who were aged 9-10 years old and received adequate treatment since their first month of life was selected and compared to a control group of 60 children without CH in the same age range. Inclusion criteria: children without concurrent diseases, who were attending school in a single shift, and whose parents had at least completed secondary education. The following tests were administered during individual interviews: the Wechsler Intelligence Scale for Children (third edition), the Rey complex figure test, the Woodcock-Muñoz revised test, the Conners Continuous Performance Test II, the Illinois Test of Psycholinguistic Abilities, the verbal fluency test, the Knox Cube Test, the Trail Making Test, the faces test, and the 5 digit test. The statistical analysis was done using Student's t tests (for independent samples) with Bonferroni's correction (p < 0.002). Even within the normal average range, significant differences were observed between both groups in terms of total intelligence quotient and performance intelligence quotient (small and moderate effect sizes, respectively). In terms of performance, children with hypothyroidism had a significantly poorer performance in processing speed, reaction times, attention, cognitive flexibility, visuoconstruction, and long-term memory. No significant differences were found between both groups in the verbal area. Children with congenital hypothyroidism and without mental disability had mild cognitive deficits, which should be taken into account for a comprehensive patient care.

The Efficacy of a Computer-Assisted Cognitive Rehabilitation Program for Patients with Mild Cognitive Deficits: A Pilot Study

Background/Study Context: Whereas computer-assisted cognitive rehabilitation (CR) programs show promise as tools for improving cognition in certain populations, there is not a consensus regarding their efficacy. This study focuses on restorative CR, a treatment designed to improve cognitive functioning affected by progressive brain changes due to disease or aging, through computer-assisted cognitive exercises. The purpose of this study was to investigate the efficacy of a computer-assisted restorative CR intervention for improving cognitive functioning in older rehabilitation patients with relatively mild cognitive deficits.Methods: Older adult residents in a Maryland retirement community (N = 43) who met inclusion criteria were assigned to either the CR treatment or the control group. Treatment group participants completed 3 weeks (nine sessions) of Memory Match, an online CR module designed to improve attention and visual memory, whereas the control group did not complete the CR program after the baseline assessment. Analyses were based on the 38 (n = 20 treatment, n = 18 control) participants (mean age = 78.08 ± 10.31) who completed the post-assessment Brief Cognitive Assessment Tool (BCAT) and a self-rating inventory (SRI) of cognitive ability.Results: Treatment group participants who received the CR treatment obtained significantly higher BCAT scores (medium to large effect size) at post-assessment than control group participants over the same period. Additional evidence for the efficacy of the CR program was found by comparing responses on a SRI of cognitive ability between the two groups.Conclusion: The authors discuss the merits and shortcoming of this pilot study, the utility of the CR program for older rehabilitation patients with relatively mild cognitive deficits, and ideas for future research.

Functional connectivity disruptions correlate with cognitive phenotypes in Parkinson's disease.

Cognitive deficits in Parkinson's disease are thought to be related to altered functional brain connectivity. To date, cognitive-related changes in Parkinson's disease have never been explored with dense-EEG with the aim of establishing a relationship between the degree of cognitive impairment, on the one hand, and alterations in the functional connectivity of brain networks, on the other hand.This study was aimed at identifying altered brain networks associated with cognitive phenotypes in Parkinson's disease using dense-EEG data recorded during rest with eyes closed. Three groups of Parkinson's disease patients (N = 124) with different cognitive phenotypes coming from a data-driven cluster analysis, were studied: G1) cognitively intact patients (63), G2) patients with mild cognitive deficits (46) and G3) patients with severe cognitive deficits (15). Functional brain networks were identified using a dense-EEG source connectivity method. Pairwise functional connectivity was computed for 68 brain regions in different EEG frequency bands. Network statistics were assessed at both global (network topology) and local (inter-regional connections) level.Results revealed progressive disruptions in functional connectivity between the three patient groups, typically in the alpha band. Differences between G1 and G2 (p < 0.001, corrected using permutation test) were mainly frontotemporal alterations. A statistically significant correlation (ρ = 0.49, p < 0.001) was also obtained between a proposed network-based index and the patients' cognitive score. Global properties of network topology in patients were relatively intact.These findings indicate that functional connectivity decreases with the worsening of cognitive performance and loss of frontotemporal connectivity may be a promising neuromarker of cognitive impairment in Parkinson's disease.

EFFECT OF EDUCATIONAL GUIDELINE ON NURSES' KNOWLEDGE REGARDING PATIENTS WITH MINIMAL HEPATIC ENCEPHALOPATHY

Background: Minimal hepatic encephalopathy (MHE) is the mildest type of spectrum of hepatic encephalopathy(HE). Patients with MHE have no recognizable clinical side effects of HE, but have mild cognitive and psychomotor deficits .The prevalence of MHE is high in patients with cirrhosis of liver and varies between 30% and 84%. Aim of this study was to study the effect of educational guideline on nurses' knowledge regarding patients with minimal hepatic encephalopathy. materials and method: A Quasi experimental study was used in carrying out this study a convenience sample of all available nurses at the hepatology word at specialized medical hospital. one tool was used to collect data consisted of 2 parts demographic data and nurses' knowledge questionnaire sheet and three tests were used in this study (pre test, post test, after 3 months test). Results: the study results showed that there were no statistical significance between demographic data of nurses and total knowledge of nurses about hepatic encephalopathy in post test and3 months after educational guideline test. Conclusion: there was a beneficial effects of educational guideline on nurses' knowledge and there was an improvement in nurses knowledge about minimal hepatic encephalopathy after educational guideline. Recommendation: Continuous educational guideline based on the assessment of nurses' knowledge can be carried out periodically with emphasizing follow up.

Fahr’s Disease Presenting as Bipolar Affective Disorder - Case Report

We present a case of a 59-year-old woman with bipolar affective disorder and idiopathic calcium accumulation in the basal ganglia (lenticular nuclei). Her physical and neurological examinations were without clinically significant abnormalities. Neuropsychological assessment shows mild cognitive deficit. Clinical and laboratory data meet the diagnostic criteria for Fahr’s disease. Discussions were held about the relationship between calcification of basal ganglia and the occurrence of the bipolar disorder.

Personality and global cognitive decline in Japanese community-dwelling elderly people: A 10-year longitudinal study

ObjectivesTo examine the longitudinal associations between the big five personality and changes in global cognitive function among community-dwelling elderly people involved in the National Institute for Longevity Sciences - Longitudinal Study of Aging. MethodsThe participants were 594 individuals (age range 60–81years) and followed for 10years and tested six times. Personality was assessed by the Japanese version of NEO five factor inventory at baseline. Cognitive function was assessed by the Japanese version of Mini Mental State Examination (MMSE) at all visits. For participants with a baseline MMSE score≥28, logistic generalized estimating equation models estimated the odds ratio (OR) and 95% confidence interval (CI) for MMSE score≤27 at each follow-up visit, according to a 1-SD increase of the baseline personality score. Post hoc analyses were performed for mild cognitive deficits, baseline MMSE score≥24 and ≤27, to estimate the OR and CI for MMSE score≤23. ResultsThe adjusted OR for MMSE score≤27 was 0.78 (95% CI, 0.69–0.88), with a 1-SD increase in Openness to Experience score. In post hoc analyses, the adjusted OR for MMSE score≤23 was 0.50 (95% CI, 0.35–0.72) with a 1-SD increase in Conscientiousness score. Relationships between other personality traits and the decline in MMSE score were not significant. ConclusionsHigher Openness to Experience was associated with a reduction in risk for cognitive decline in community-dwelling older adults. Higher Conscientiousness might also predict lower risk for severe cognitive decline, especially for individuals with mild cognitive deficits.

Brief Screening of Vascular Cognitive Impairment in Patients With Cerebral Autosomal-Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy Without Dementia.

Background and Purpose—Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a monogenic form of cerebral small vessel disease leading to early-onset stroke and dementia, with younger patients frequently showing subclinical deficits in cognition. At present, there are no targeted cognitive screening measures for this population. However, the Brief Memory and Executive Test (BMET) and the Montreal Cognitive Assessment (MoCA) have shown utility in detecting cognitive impairment in sporadic small vessel disease. This study assesses the BMET and the MoCA as clinical tools for detecting mild cognitive deficits in CADASIL.Methods—Sixty-six prospectively recruited patients with CADASIL, and 66 matched controls completed the BMET, with a subset of these also completing the MoCA. Receiver operating characteristic curves were calculated to examine the sensitivity and specificity of clinical cutoffs for the detection of vascular cognitive impairment and reduced activities of daily living.Results—Patients with CADASIL showed more cognitive impairment overall and were poorer on both executive/processing and memory indices of the BMET relative to controls. The BMET showed good accuracy in predicting vascular cognitive impairment (85% sensitivity and 84% specificity) and impaired instrumental activities of daily living (92% sensitivity and 77% specificity). The MoCA also showed good predictive validity for vascular cognitive impairment (80% sensitivity and 78% specificity) and instrumental activities of daily living (75% sensitivity and 76% specificity). The most important background predictor of vascular cognitive impairment was a history of stroke.Conclusions—The results indicate that the BMET and the MoCA are clinically useful and sensitive screening measures for early cognitive impairment in patients with CADASIL.

Structural Covariance Networks and Their Association with Age, Features of Cerebral Small-Vessel Disease, and Cognitive Functioning in Older Persons.

Recently, cerebral structural covariance networks (SCNs) have been shown to partially overlap with functional networks. However, although for some of these SCNs a strong association with age is reported, less is known about the association of individual SCNs with separate cognition domains and the potential mediation effect in this of cerebral small vessel disease (SVD). In 219 participants (aged 75-96 years) with mild cognitive deficits, 8 SCNs were defined based on structural covariance of gray matter intensity with independent component analysis on 3DT1-weighted magnetic resonance imaging (MRI). Features of SVD included volume of white matter hyperintensities (WMH), lacunar infarcts, and microbleeds. Associations with SCNs were examined with multiple linear regression analyses, adjusted for age and/or gender. In addition to higher age, which was associated with decreased expression of subcortical, premotor, temporal, and occipital-precuneus networks, the presence of SVD and especially higher WMH volume was associated with a decreased expression in the occipital, cerebellar, subcortical, and anterior cingulate network. The temporal network was associated with memory (p = 0.005), whereas the cerebellar-occipital and occipital-precuneus networks were associated with psychomotor speed (p = 0.002 and p < 0.001). Our data show that a decreased expression of specific networks, including the temporal and occipital lobe and cerebellum, was related to decreased cognitive functioning, independently of age and SVD. This indicates the potential of SCNs in substantiating cognitive functioning in older persons.

Anti-LGI1 encephalitis: Clinical syndrome and long-term follow-up.

This nationwide study gives a detailed description of the clinical features and long-term outcome of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. We collected patients prospectively from October 2013, and retrospectively from samples sent to our laboratory from January 2007. LGI1 antibodies were confirmed with both cell-based assay and immunohistochemistry. Clinical information was obtained in interviews with patients and their relatives and from medical records. Initial MRI and follow-up MRI were revised blindly. Neuropsychological assessment was performed in those patients with follow-up over 2 years. Annual incidence in the Netherlands was 0.83/million. A total of 34/38 patients had a limbic encephalitis. Subtle focal seizures (66%, autonomic or dyscognitive) and faciobrachial dystonic seizures (FBDS, 47%) mostly occurred before onset of memory disturbance. Later in the disease course, 63% had tonic-clonic seizures. Initial MRI showed hippocampal T2 hyperintensity in 74% of the patients. These lesions evolved regularly into mesial temporal sclerosis (44%). Substantial response to immunotherapy was seen in 80%, with early response of seizures and slow recovery of cognition. At follow-up ≥2 years, most surviving patients reported mild residual cognitive deficit with spatial disorientation. A total of 86% had persistent amnesia for the disease period. Relapses were common (35%) and presented up to 8 years after initial disease. Two-year case fatality rate was 19%. Anti-LGI1 encephalitis is a homogenous clinical syndrome, showing early FBDS and other focal seizures with subtle clinical manifestations, followed by memory disturbances. Better recognition will lead to earlier diagnosis, essential for prompt start of treatment. Long-term outcome of surviving patients is mostly favorable, but relapses are common.

Neurologic Complications of Unprotected Transcatheter Aortic Valve Implantation (from the Neuro-TAVI Trial)

Cerebral embolization during transcatheter aortic valve implantation (TAVI) can lead to a spectrum of clinically relevant manifestations, ranging from overt stroke to mild neurologic or cognitive deficits and subclinical cerebral infarcts. This study sought to determine the frequency of neurologic injury, cerebral ischemic lesions, and cognitive dysfunction in subjects undergoing contemporary commercial TAVI in the United States. Neuro-TAVR is the first prospective, multicenter study to use serial systematic neurologic and cognitive assessments and diffusion-weighted magnetic resonance imaging (at 4 ± 2days after procedure) to investigate the incidence and severity of neurologic injury after contemporary unprotected TAVI in the United States. A total of 44 consecutive patients underwent TAVI at 5 US sites. Diffusion-weighted magnetic resonance imaging lesions were detected in 94%, with a mean of 10.4 ± 15.3 lesions per subject and a median total lesion volume of 295mm3 (interquartile range 71.6 to 799.6mm3). New neurologic impairment (worsening in National Institutes of Health Stroke Scale score from baseline with new cerebral lesions) occurred in 22.6% (7 of 31) of subjects at discharge and 14.8% (4 of 27) at 30days. In addition, cognitive decrements from baseline were identified by the Montreal Cognitive Assessment in 33% (12 of 36) of subjects at discharge and 41% (13 of 32) at 30days. In conclusion, this contemporary cohort of US patients confirms that TAVI results in cerebral infarction in most patients and that 1 in 5 patients have measurable neurologic impairment and 1 in 3 patients have decrease in cognitive measures by Montreal Cognitive Assessment score after TAVI, reinforcing the need for methods to mitigate the risk of brain injury during TAVI.

Cognitive impairment in progressive supranuclear palsy-Richardson’s syndrome is related to white matter damage

IntroductionBeside motor symptoms, patients with progressive supranuclear palsy syndrome (PSPs) commonly present cognitive and behavioral disorders. In this study we aimed to assess the structural brain correlates of cognitive impairment in PSPs. MethodsWe enrolled 23 patients with probable PSP Richardson's syndrome and 15 matched healthy controls. Patients underwent an extensive clinical and neuropsychological evaluation. Cortical thickness measures and diffusion tensor metrics of white matter tracts were obtained. Random forest analysis was used to identify the strongest MRI predictors of cognitive impairment in PSPs at an individual patient level. ResultsPSPs patients were in a moderate stage of the disease showing mild cognitive deficits with prominent executive dysfunction. Relative to controls, PSPs patients had a focal, bilateral cortical thinning mainly located in the prefrontal/precentral cortex and temporal pole. PSPs patients also showed a distributed white matter damage involving the main tracts including the superior cerebellar peduncle, corpus callosum, corticospinal tract, and extramotor tracts, such as the inferior fronto-occipital, superior longitudinal and uncinate fasciculi, and cingulum, bilaterally. Regional cortical thinning measures did not relate with cognitive features, while white matter damage showed a significant impact on cognitive impairment (r values ranging from −0.80 to 0.74). ConclusionsPSPs patients show both focal cortical thinning in dorsolateral anterior regions and a distributed white matter damage involving the main motor and extramotor tracts. White matter measures are highly associated with cognitive deficits. Diffusion tensor MRI metrics are likely to be the most sensitive markers of extramotor deficits in PSPs.