Clinical Approach to Cognitive and Neurobehavioral Symptoms.

PURPOSE OF REVIEW: This article provides a framework for the approach to patients with cognitive or neurobehavioral concerns. RECENT FINDINGS: Recent advances in structural neuroimaging, functional neuroimaging, and disease biomarkers have greatly expanded knowledge of brain-behavior relationships, neural networks and functional connectivity, and pathophysiologic processes leading to cognitive and neurobehavioral disorders. However, any one of these studies is subject to misinterpretation if not applied in the appropriate clinical context. SUMMARY: A systematic approach to the history and examination in patients with cognitive and neurobehavioral symptoms is important in marrying clinical assessments with contemporary diagnostic studies and treatments.

When Is My Care Team Rounding? A Simple Signage Intervention to Increase Awareness of Rounding Times.

Patient and caregiver awareness of multidisciplinary rounds (MDR) times, and their subsequent involvement in MDR, aids in decreasing adverse health outcomes, reducing average length of stay, and increasing satisfaction. The objective of this study was to increase patient and caregiver awareness of MDR times using signage interventions and to assess the state of rounding processes with patient and caregiver satisfaction pre- and post-intervention. We administered survey questions to assess MDR interaction and awareness regarding MDR times. Patient and caregiver awareness of rounding times increased significantly by 25.87% (P = .0043) post-intervention. Although patients' confidence in the physician remained largely unchanged after the intervention due to high initial confidence levels, MDR satisfaction metrics increased slightly post-intervention. Thus, our signage intervention increased rounding time awareness in the MDR process.

Neurocognitive profile of a man with Dandy-Walker malformation: Evidence of subtle cerebellar cognitive affective syndrome.

Background: The Dandy-Walker Malformation (DWM) is a congenital birth malformation that is characterized by a triad of features: cerebellar dysgenesis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa that displaces the dural sinuses and the tentorium. Despite this defining triad, clinical presentation can be highly heterogeneous in part due to severity of structural changes. To date, there been limited consideration of cognitive-behavioral symptoms of DWM in relation to nonmotor functions of the cerebellum, specifically cerebellar cognitive affective syndrome (CCAS).Method: In this case study, we describe the neuropsychological and behavioral profile of a 48-year-old man with DWM who was seen due to concerns, expressed solely by the patient's father, about his son's atypical housing, employment and social skills.Results: Neuropsychological test findings revealed high average intellect on standard intellectual measures (WAIS-IV), with stronger verbal (superior) than perceptual reasoning (average) skills. Across all cognitive domains, performance was generally within expectations, although bilateral fine motor skills were impaired. In contrast, he exhibited weaknesses on nontraditional neuropsychological measures assessing orbitofrontal-limbic circuitry, including reward sensitivity decision making and indices of threat-related emotional physiology.Conclusions: Through the use of traditional and nontraditional neuropsychological measures, subtle cognitive weaknesses in fronto-executive and affective regulation were illuminated and likely explain the patient's functional difficulties. Etiologically, these findings are consistent with the nonmotor functions of the cerebellum as described by CCAS.

Visual Object Discrimination Impairment as an Early Predictor of Mild Cognitive Impairment and Alzheimer's Disease.

OBJECTIVE: Detection of cognitive impairment suggestive of risk for Alzheimer's disease (AD) progression is crucial to the prevention of incipient dementia. This study was performed to determine if performance on a novel object discrimination task improved identification of earlier deficits in older adults at risk for AD. METHOD: In total, 135 participants from the 1Florida Alzheimer's Disease Research Center [cognitively normal (CN), Pre-mild cognitive impairment (PreMCI), amnestic mild cognitive impairment (aMCI), and dementia] completed a test of object discrimination and traditional memory measures in the context of a larger neuropsychological and clinical evaluation. RESULTS: The Object Recognition and Discrimination Task (ORDT) revealed significant differences between the PreMCI, aMCI, and dementia groups versus CN individuals. Moreover, relative risk of being classified as PreMCI rather than CN increased as an inverse function of ORDT score. DISCUSSION: Overall, the obtained results suggest that a novel object discrimination task improves the detection of very early AD-related cognitive impairment, increasing the window for therapeutic intervention. (JINS, 2019, 25, 688-698).

Shared decision making in mild cognitive impairment.

Shared decision making (SDM) occurs when patients and clinicians consider patients' values and preferences while discussing medical evidence to inform healthcare decisions. SDM enables patients with mild cognitive impairment (MCI) to express values and preferences when making current healthcare decisions and presents a unique opportunity to inform future decision making in the case of further cognitive decline. However, clinicians often fail to facilitate SDM with patients with MCI. This review describes research pertaining to value solicitation, weighing of the medical evidence, and medical decision making for individuals with MCI, explores the role of caregivers, identifies barriers to and facilitators of SDM in MCI, and suggests strategies to optimize SDM for persons with MCI in neurology clinical practice. Further research is needed to identify more strategies for decision support for individuals affected by cognitive impairment.

A Novel Cognitive Stress Test for the Detection of Preclinical Alzheimer Disease: Discriminative Properties and Relation to Amyloid Load.

OBJECTIVE: To examine the utility of a novel "cognitive stress test" to detect subtle cognitive impairments and amyloid load within the brains of neuropsychologically normal community-dwelling elders. METHODS: Participants diagnosed as cognitively normal (CN), subjective memory impairment (SMI), mild cognitive impairment (MCI), and preclinical mild cognitive impairment (PreMCI) were administered the Loewenstein-Acevedo Scale for Semantic Interference and Learning (LASSI-L), a sensitive test of proactive semantic interference (PSI), retroactive semantic interference, and, uniquely, the ability to recover from the effects of PSI. Ninety-three subjects (31 men and 62 women) were recruited from three academic institutions in a research consortium. A subset of these individuals underwent 18F florbetapir positron emission tomography scanning. Relative percentages of impairment for each diagnostic group on the LASSI-L were calculated by χ(2) and Fisher's exact tests. Spearman's rho was used to examine associations between amyloid load and different cognitive measures. RESULTS: LASSI-L deficits were identified among 89% of those with MCI, 47% with PreMCI, 33% with SMI, and 13% classified as CN. CN subjects had no difficulties with recovery from PSI, whereas SMI, preMCI, and MCI participants evidenced deficits in recovery from PSI effects. Among a subgroup of participants with normal scores on traditional neuropsychological tests, the strong associations were between the failure to recover from the effects of PSI and amyloid load in the brain. CONCLUSION: Failure to recover or compensate for the effects of PSI on the LASSI-L distinguishes the LASSI-L from other widely used neuropsychological tests and appears to be sensitive to subtle cognitive impairments and increasing amyloid load.

Proactive Semantic Interference is Associated with Total and Regional Abnormal Amyloid Load in Non-Demented Community-Dwelling Elders: A Preliminary Study.

OBJECTIVE: To evaluate the relationship between susceptibility to proactive semantic interference (PSI) and retroactive semantic interference (RSI) and brain amyloid load in non-demented elders. METHODS: 27 participants (11 cognitively normal [CN] with subjective memory complaints, 8 CN without memory complaints, and 8 with mild cognitive impairment [MCI]) underwent complete neurological and neuropsychological evaluations. Participants also received the Semantic Interference Test (SIT) and AV-45 amyloid PET imaging. RESULTS: High levels of association were present between total amyloid load, regional amyloid levels, and the PSI measure (in the entire sample and a subsample excluding MCI subjects). RSI and other memory measures showed much weaker associations or no associations with total and regional amyloid load. No associations between amyloid levels and non-memory performance were observed. CONCLUSIONS: In non-demented individuals, vulnerability to PSI was highly associated with total and regional beta-amyloid load and may be an early cognitive marker of brain pathology.

Quantitative application of the primary progressive aphasia consensus criteria.

OBJECTIVE: To determine how well the consensus criteria could classify subjects with primary progressive aphasia (PPA) using a quantitative speech and language battery that matches the test descriptions provided by the consensus criteria. METHODS: A total of 105 participants with a neurodegenerative speech and language disorder were prospectively recruited and underwent neurologic, neuropsychological, and speech and language testing and MRI in this case-control study. Twenty-one participants with apraxia of speech without aphasia served as controls. Select tests from the speech and language battery were chosen for application of consensus criteria and cutoffs were employed to determine syndromic classification. Hierarchical cluster analysis was used to examine participants who could not be classified. RESULTS: Of the 84 participants, 58 (69%) could be classified as agrammatic (27%), semantic (7%), or logopenic (35%) variants of PPA. The remaining 31% of participants could not be classified. Of the unclassifiable participants, 2 clusters were identified. The speech and language profile of the first cluster resembled mild logopenic PPA and the second cluster semantic PPA. Gray matter patterns of loss of these 2 clusters of unclassified participants also resembled mild logopenic and semantic variants. CONCLUSIONS: Quantitative application of consensus PPA criteria yields the 3 syndromic variants but leaves a large proportion unclassified. Therefore, the current consensus criteria need to be modified in order to improve sensitivity.

Brain MRI findings in Wernicke encephalopathy.

A 71-year-old woman with myelofibrosis on chemotherapy experienced an acute illness with nausea, vomiting, and diarrhea. Two weeks later, she developed an acute confusional state characterized by disorientation and fluctuating alertness with normal speech and language. Her neurologic examination demonstrated an upper motor neuron pattern of right hemiparesis. She reported double vision though ophthalmoparesis was not appreciated. Her gait was normal. While hospitalized, she developed generalized tonic-clonic seizures. Brain MRI revealed a small area of restricted diffusion of the left precentral gyrus (figure). She was diagnosed with a stroke with secondary seizures; however, as the confusional state resolved, she developed profound retrograde and anterograde amnesia. Review of the brain MRI showed high T2 signal in the medial thalamus and contrast enhancement of the mamillary bodies; a diagnosis of Wernicke-Korsakoff syndrome was entertained and she was started on thiamine replacement. The encephalopathy and hemiparesis resolved though she remains severely amnestic.

Emerging biomarkers in cognition.

Knowledge of aging and dementia is rapidly evolving with the aim of identifying individuals in the earliest stages of disease processes. Biomarkers allow clinicians to show the presence of a pathologic process and resultant synapse dysfunction and neurodegeneration, even in the earliest stages. This article focuses on biomarkers for mild cognitive impairment caused by Alzheimer disease, structural magnetic resonance imaging, fluorodeoxyglucose positron emission tomography (PET) or single-photon emission computed tomography, and PET with dopamine ligands. Although these biomarkers are useful, several limitations exist. Several new biomarkers are emerging and a more biological characterization of underlying pathophysiologic spectra may become possible.

Aphasia with left occipitotemporal hypometabolism: a novel presentation of posterior cortical atrophy?

Alzheimer's disease is a common neurodegenerative disease often characterized by initial episodic memory loss. Atypical focal cortical presentations have been described, including the logopenic variant of primary progressive aphasia (lvPPA) which presents with language impairment, and posterior cortical atrophy (PCA) which presents with prominent visuospatial deficits. Both lvPPA and PCA are characterized by specific patterns of hypometabolism: left temporoparietal in lvPPA and bilateral parietoccipital in PCA. However, not every patient fits neatly into these categories. We retrospectively identified two patients with progressive aphasia and visuospatial deficits from a speech and language based disorders study. The patients were further characterized by MRI, fluorodeoxyglucose F18 and Pittsburgh Compound B (PiB) positron emission tomography. Two women, aged 62 and 69, presented with a history of a few years of progressive aphasia characterized by fluent output with normal grammar and syntax, anomia without loss of word meaning, and relatively spared repetition. They demonstrated striking deficits in visuospatial function for which they were lacking insight. Prominent hypometabolism was noted in the left occipitotemporal region and diffuse retention of PiB was noted. Posterior cortical atrophy may present focally with left occipitotemporal metabolism characterized clinically with a progressive fluent aphasia and prominent ventral visuospatial deficits with loss of insight.

Standardized sign-out improves completeness and perceived accuracy of inpatient neurology handoffs.

OBJECTIVES: As residency programs adjust to new duty hour restrictions, the use of cross-coverage systems requiring handoffs will rise. Handoffs are vulnerable to communication failures when unstructured. Accordingly, we implemented a standardized sign-out process on our inpatient neurology services and assessed its effect on completeness and perceived accuracy of handoffs. METHODS: Residents spent the first half of their rotations utilizing unstructured sign-out. They transitioned to a structured sign-out system (using the situation-background-assessment-recommendation format) during the second half of their rotations. We analyzed survey responses before and after implementation to evaluate for an effect. RESULTS: Residents utilizing structured sign-out were significantly more likely to share test results with patients/families prior to shift changes (p = 0.037), update our electronic service list (p = 0.045), and feel all important data were being transmitted (p = 0.041). Overall satisfaction (scale 1-10) increased from 6.2 ± 1.6 to 7.4 ± 1.3 (p = 0.002). CONCLUSIONS: Our findings demonstrate that standardized sign-out improves the completeness and perceived accuracy of handoffs. Such improvement has the potential to improve patient safety and quality of care.