Cognitive and Behavioral Neurology最新文献

Here we report the case of an individual who developed proper- and common-name anomia with no category specificity, alexia with agraphia for kanji (Japanese morphograms), and mild verbal and semantic memory impairment after unilateral herpes simplex encephalitis. Although their common-name anomia, alexia with agraphia, and semantic memory impairment resolved within 2 years, this individual continued to experience proper-name anomia and verbal memory impairment. Encephalitic damage was limited to the left anterior temporal lobe (ATL), amygdala, hippocampus, and parahippocampal gyrus, sparing the mid-fusiform and posterior inferior temporal gyri. Although ATL lesions are not typically associated with semantic memory impairment, we suggest that damage to the left ATL can result in both proper- and common-name anomia, as was evident in the current case. In these cases, proper-name anomia may be more severe and persistent than common-name anomia, which may be mild and significantly improved within several years. In cases of semantic memory deficits, persistent common-name anomia, and severe alexia with agraphia, there is typically more extensive involvement of the temporal lobe than seen in the current case, including the mid-fusiform and posterior inferior temporal gyri.

CSF1R-related disorder, a catastrophic neurodegenerative disease, arises from genetic mutations in the colony-stimulating CSF1R. Initial misdiagnosis is common, as demonstrated by this case involving a 52-year-old female who presented with symptoms of limb numbness and weakness. Differential diagnosis first indicated Parkinsonism, lacunar infarction, and cervical spondylosis. Subsequently, however, this patient's clinical presentation evolved to include bradykinesia, cognitive decline, and a spectrum of neurological manifestations. A Pan-V2 assay revealed a heterozygous mutation in the CSF1R gene. Craniocerebral MRI showed cerebral infarctions, lacunar infarctions, and leukoaraiosis. Despite symptomatic treatments, our patient's clinical status continued to decline until her family chose to discontinue further medical interventions. This case underscores the diagnostic complexities of early detection of CSF1R-related disorders. It emphasizes the importance of including leukodystrophy in such differential diagnoses and the need for prompt genetic screening in patients who present with progressive leukoencephalopathy, especially when cerebrospinal fluid analysis is unremarkable.

The dorsomedial prefrontal cortex plays a critical role in movement initiation, and damage to this area can impair this function. Here we present the case of an individual who had difficulty with voluntary initiation of liquid swallowing after surgical removal of a glioblastoma from the right dorsomedial prefrontal cortex. This individual had no difficulty swallowing solids, perhaps because of the additional external movement triggers (eg, chewing) involved. Liquid swallowing involves fewer movement triggers and requires a quicker application of force during the oral propulsive phase when liquids are transferred from the oral cavity to the oropharynx. This individual did not have buccofacial apraxia or apraxia of speech, which are often associated with swallowing apraxia linked to damage in the precentral, premotor, and inferior frontal gyri. To our knowledge, few studies have focused on movement initiation impairments affecting the upper extremities and speech, and cases involving swallowing are notably rare.

Background: Evaluation of sensory functions in chronic stroke survivors is essential to plan and implement effective treatment and rehabilitation.

Objective: To investigate the validity and reliability of the Turkish version of the Revised Nottingham Sensory Assessment (rNSA-T) in chronic stroke survivors.

Methods: We applied the World Health Organization's translation protocols to develop the rNAS-T. We then tested its validity and reliability in 85 chronic stroke survivors using criterion validity and consistency for demographic variables, as well as test-retest and inter-rater reliability analyses.

Results: The criterion validity of the rNSA-T was supported by significant correlation between participants' scores on the rNSA-T, the Katz Index of Independence in Activities of Daily Living (Katz-AD) (r = 0.430-0.674, P < 0.05), and the Rivermead Motor Assessment (RMA) (r = 0.528-0.773, P < 0.05). rNSA-T results remained consistent across variables of sex and side affected by stroke (P > 0.05). The test-retest reliability of the rNSA-T was excellent in all subdimensions (ICC = 0.865-1.000), as was the inter-rater reliability (κ = 0.875-1.000).

Conclusion: The rNSA-T is a valid and reliable tool for evaluation of sensory functions in chronic stroke survivors.

Background: Although episodic memory is the primary concern in individuals with mild cognitive impairment (MCI), other cognitive functions may also be affected, including language. Language impairment in individuals with MCI has been attributed primarily to the breakdown of semantic representations, difficulties in accessing semantic information, and the weakening of executive functions. However, in most prior studies of word processing in individuals with MCI, researchers have used measures focused on noun production.

Objective: To investigate how verb production tasks might aid in detecting cognitive impairment in individuals with MCI.

Methods: We compared the performance of 45 individuals with MCI and 45 healthy controls on action naming and action fluency tasks.

Results: In the action naming task, the performance of participants with MCI was significantly impaired compared to healthy controls in terms of total score, the number of semantic errors produced, and the use of generic terms. In the action fluency task, participants with MCI produced significantly fewer verbs, fewer clusters, and fewer switches than healthy controls.

Conclusion: The results of our study emphasize the utility of verb production tasks in the identification of cognitive impairment in individuals with MCI and provide evidence of the importance of including action naming and action fluency tasks in the assessment of individuals with MCI.

Psychotropic polypharmacy presents a diagnostic challenge that may be further complicated by inadequate medication history and underappreciation of the cognitive effects of such polypharmacy. Here we present the case of a 57-year-old man who presented to our memory clinic with progressive cognitive decline and a prior neuropsychological evaluation supporting the diagnosis of a neurodegenerative disorder. He was taking multiple psychotropic medications at the time, but the exact dosages were unclear due to a lack of collateral history. He was also taking prescribed opioids and a combination of buprenorphine and naloxone for pain relief, again with unclear dosages at the time of presentation. Brain imaging and cerebrospinal spinal fluid biomarker testing were negative for Alzheimer pathophysiologic processes. Months later, the patient was taken to the emergency room after an overdose caused by overuse of opioid medications. Once he was taken off all psychoactive medications, the patient's cognitive impairment completely reversed, and he became independent in activities of daily living. Psychotropic polypharmacy can have a myriad of cognitive manifestations which need to be better recognized by clinicians. Deprescription of such medications should be attempted whenever clinically appropriate.

The emergence of new-onset neuropsychiatric symptoms in middle age presents a diagnostic challenge, particularly when differentiating between a primary psychiatric disorder and an early neurodegenerative disease. The discrepancy between bedside clinical diagnosis and subsequent neuropathological findings in such cases further highlights the difficulty of accurately predicting pathology, especially when there are no evident focal lesions or changes in brain volume. Here we present the case of a 59-year-old woman with inconclusive neuroimaging who exhibited pronounced neuropsychiatric and behavioral symptoms initially suggestive of a mood disorder, then of behavioral variant frontotemporal dementia. However, upon autopsy, we identified coexisting Lewy body disease pathology and tau-related changes, including argyrophilic grain disease and primary age-related tauopathy. This case illustrates the challenges encountered when diagnosing late-onset neuropsychiatric symptoms, emphasizes the link between such symptoms and early-onset dementia and argyrophilic grain disease, and contributes to our understanding of the impact of mixed neuropathology in this population. Accurate diagnosis is essential for the development of molecular-specific therapies and, as well as for accurate prognosis and enrollment in clinical trials.

Background: Impulsivity resulting in unrestrained eating has been implicated as a contributing factor for obesity. Delay discounting (DD) tasks where individuals choose between a smaller immediate reward and a larger delayed reward provide useful data to describe impulsive decision-making and to determine the extent to which delayed rewards are discounted.

Objective: To study the association between body mass index(BMI) and delay discounting for food and money in adult women.

Methods: We used a DD task with real food rewards to investigate impulsive decision-making as related to BMI in participants who self-identified as women. Participants in group A had a mean BMI of 21.4 (n = 14), and participants in group B had a mean BMI of 32.2 (n = 14). Each group was tested in a hungry state during a single session. We performed fMRI during a DD task requiring participants to choose between a food item (one sandwich) constituting a smaller immediate reward and multiple food items (two, three, or four sandwiches) constituting a series of larger delayed rewards available at different intervals. The steepness of the discounting curve for food was determined from these decisions. Participants then completed a monetary discounting task to facilitate a comparison of the discounting of food and monetary rewards.

Results: Participants in group B discounted food rewards more steeply than monetary rewards. Decisions for delayed rewards led to increased activations of brain areas related to executive control on fMRI, such as the head of the caudate nucleus and the anterior cingulate cortex (ACC) in group A, but not group B participants.

Conclusion: Our findings suggest that group B had difficulty deciding against the immediate food rewards due to insufficient recruitment of cortical control areas. Therefore, impulsivity is an important target for behavioral interventions in individuals with obesity.

Background: Antisocial behaviors occur in up to 91% of individuals with behavioral variant frontotemporal dementia (bvFTD). Prior work has shown that antisocial behaviors can be differentiated into aggressive and nonaggressive rule-breaking behavioral subtypes. Socioemotional dysfunction is common in bvFTD and unique compared to other types of dementia.

Objective: To determine whether socioemotional dysfunction relates to general antisocial behaviors in individuals with bvFTD, or whether different types of socioemotional dysfunction relate to aggressive versus rule-breaking behaviors.

Methods: Informants for 28 participants with bvFTD and 21 participants with Alzheimer disease (AD) completed the Social Behavior Questionnaire (SBQ) and the Interpersonal Reactivity Index (IRI). The SBQ measures the presence and severity of 26 antisocial behaviors, including subscales for aggressive behaviors (SBQ-AGG) and nonaggressive rule-breaking behaviors (SBQ-RB). The IRI measures cognitive and emotional empathy capabilities, including subscales for Empathic Concern (IRI-EC) and Perspective-taking (IRI-PT).

Results: As expected, participants with bvFTD had higher scores on the SBQ in total than participants with AD, as well as on the SBQ-AGG and SBQ-RB separately. Participants with bvFTD had lower scores on the IRI-EC and IRI-PT than participants with AD (P < 0.0001 for all measures). Lower scores on the IRI-PT correlated with higher scores on the SBQ-AGG-but not with higher scores on the SBQ-RB-across the combined group of participants (P = 0.007), and within participants in the bvFTD group (P = 0.01) specifically, after controlling for covariates of age, sex, dementia severity, and IRI-EC scores. Lower scores on the IRI-EC correlated with higher scores on the SBQ-AGG-but not with higher scores on the SBQ-RB-across the combined group of participants (P = 0.02) after controlling for covariates of age, sex, dementia severity, and IRI-PT scores.

Conclusion: Our results suggest that socioemotional dysfunction relates to antisocial behaviors in individuals with bvFTD, but that the mechanisms leading to aggressive and rule-breaking behaviors are differentiable, providing meaningful implications for distinct approaches to treatment and prevention.