Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology.

Corticobasal syndrome and dementia with Lewy bodies are clinical presentations with unique and overlapping features but distinct pathological substrates. We report the case of an 80 year-old man who presented with apraxia, rigidity, slowness, right arm myoclonus, a 10-year history of probable REM-sleep behavior disorder, and later developed visual hallucinations. At autopsy, he had pathological features of corticobasal degeneration, and Lewy body disease confined to the brainstem. This report highlights the importance of considering co-existing pathologies when a clinical presentation defies categorization, and demonstrates that salient features of dementia with Lewy bodies may result from pathology limited to the brainstem.

Non-pharmacological Management of Behavioral Symptoms in Frontotemporal and Other Dementias.

Worldwide prevalence of dementia is predicted to double every 20 years. The most common cause in individuals over 65 is Alzheimer's disease (AD), but in those under 65, frontotemporal dementia (FTD) is as frequent. The physical and cognitive decline that characterizes these diseases is commonly accompanied by troublesome behavioral symptoms. These behavioral symptoms contribute to significant morbidity and mortality among both patients and caregivers. Medications have been largely ineffective in managing these symptoms and carry significant adverse effects. Non-pharmacological interventions have been recommended to precede the utilization of pharmacological treatments. This article reviews the research about these interventions with special attention to the variations by etiology, especially FTD. The authors offer recommendations for improving utilization of these strategies and future research recommendations.

The experience of caregiving: differences between behavioral variant of frontotemporal dementia and Alzheimer disease.

OBJECTIVE: To examine caregiver strain, depression, perceived sense of control, and distress from patient neuropsychiatric symptoms in family caregivers of persons with Alzheimer disease (AD) and behavioral variant of frontotemporal dementia (bvFTD) and determine whether group differences exist. METHODS: Family caregivers were recruited from the Memory and Aging Center in San Francisco, California. Analyses of cross-sectional data on 53 family caregivers (AD = 31, bvFTD = 22) were performed. The Mann-Whitney U test was used to contrast groups. RESULTS: There were statistically significant differences between the AD and bvFTD caregivers in strain, distress, and perceived control but not in depression. On average, bvFTD caregivers experienced greater strain and distress, more depressive symptoms, and lower perceived control. CONCLUSIONS: Findings support that experiences of AD and bvFTD caregivers may differ. Further study is needed to identify possible explanatory factors for these group differences.

Functional deficits among patients with mild cognitive impairment.

BACKGROUND: Diagnostic criteria for mild cognitive impairment (MCI) include no significant functional decline, but recent studies have suggested that subtle deficits often exist. It is not known whether these differ by MCI type. We investigated the level and type of functional impairment among patients with MCI. METHODS: We studied 498 patients, evaluated at the Alzheimer's Disease Research Centers of California between 2006 and 2009, who had multidisciplinary evaluations by experts, including neurologic examination and neuropsychological testing. Patients were diagnosed with MCI and subtype was determined using cognitive domain scores. In a cross-sectional descriptive study, we examined whether functional impairment differed by MCI subtype, using the Blessed Roth Dementia Rating Scale (range: 0-17, higher scores indicating more impairment). RESULTS: Among the participants, the mean age was 75.4 years, 50.7% were women, and 81.7% were white. Patients with amnestic- (n = 392, 78.7%) and nonamnestic-type (n = 106, 21.3%) MCI had similar total Blessed Roth Dementia Rating Scale (1.6 and 1.5, respectively; P = .84) and Mini-Mental State Examination (26.5 and 26.7, respectively; P = .60) scores. Patients with amnestic MCI were more likely to have difficulty in remembering lists and recalling recent events (P < .05 for both) and less likely to have difficulty in eating and with continence (P = .01 for both), as compared with those with nonamnestic MCI. CONCLUSIONS: Despite the MCI diagnostic criteria suggesting no functional impairment, our results indicate that patients with MCI experience mild functional deficits that vary according to the type of MCI.

Video-telemedicine in a memory disorders clinic: evaluation and management of rural elders with cognitive impairment.

OBJECTIVE: Telemedicine is increasingly being used to provide consultation for healthcare in rural areas. Little work has been done with dementia although preliminary research suggests that clinical diagnosis performed via telemedicine consultation is valid. We implemented a program to provide multidisciplinary, state-of-the-art diagnosis of cognitive impairment by video-telemedicine (VTM) integrated into a clinical setting. METHODS: Patients at a rural veteran's community clinic were referred by their local provider for evaluation of memory complaints by the multidisciplinary team of the San Francisco Veterans Administration (SFVA) Memory Disorders Clinic (MDC). The evaluation was integrated into the usual clinic structure and included a neurological evaluation and neuropsychological testing by the MDC team via video assisted by a remote clinician at the community clinic. RESULTS: We evaluated 15 new patients referred to our multidisciplinary clinic. In each case, the VTM format permitted the MDC team to arrive at a working diagnosis; 12 patients with dementia, two with mild cognitive impairment, and one cognitively normal. Relevant treatment recommendations were made to the patients and caregivers. The evaluation results were discussed with providers who joined the MDC postclinic conference via VTM. In the majority of cases, recommendations were followed and there was satisfaction with VTM by providers and patients. CONCLUSIONS: VTM is emerging as an effective way to provide consultation and care to rural residents who may not have access to specialty services and can be integrated into current clinical settings.

Contraindicated medication use among patients in a memory disorders clinic.

BACKGROUND: Inappropriate or contraindicated use of medications in elderly patients is common and associated with poor outcomes. An important risk factor for adverse drug events is the increased sensitivity to drug effects on the central nervous system (CNS). There is a high rate of use of CNS-active drugs in patients with cognitive impairment, despite the fact that these medications may worsen cognition and be a possible "reversible" cause of memory loss. OBJECTIVES: The goals of this study were to establish the prevalence of these contraindicated medications in a population of elderly patients referred to a memory disorders clinic for evaluation and to determine if those individuals receiving contraindicated medications had specific characteristics. This included determining how many patients were concurrently being prescribed a cholinesterase inhibitor. METHODS: The review included new patients consecutively evaluated for cognitive complaints in a memory disorders clinic between June 2003 and August 2004. Each patient underwent a comprehensive evaluation by a multi-disciplinary team during a 3-hour clinic appointment. A thorough history of cognitive deficits and associated symptoms was obtained by the physician, who also performed a comprehensive neurologic examination. All patients underwent neuropsychologic testing with an extensive cognitive battery. In addition, patients' electronic medical records were reviewed to determine a list of prescribed and over-the-counter medications at the time of the initial referral. Contraindicated medications were identified using the updated Beers criteria of medications that should be avoided in older patients with cognitive impairment or that have high CNS adverse effects. RESULTS: A total of 100 patients (91 men, 9 women; mean [SD] age, 75.8 [9.7] years; 73% white) were included in the study. Eighty-six patients were determined at the time of evaluation to have some kind of cognitive impairment. They were mildly impaired, with a mean (SD) Mini-Mental State Examination score of 22.9 (5.1), based on a scale of 0 to 30. Twenty-two patients were taking > or =1 contraindicated medication that could potentially affect their cognition; the most frequently prescribed were benzodiazepines, oxybutynin, amitriptyline, fluoxetine, and diphenhydramine. Twenty-eight of the 100 patients were being treated with a cholinesterase inhibitor at the time of their evaluation; of these, 4 (14%) were also taking > or =1 medication with anticholinergic properties. CONCLUSIONS: Despite research evidence and recommendations to avoid these CNS-active medications because of their adverse effects, they continue to be prescribed in elderly patients with cognitive impairments. Further research is needed to determine strategies that will help reduce their administration in this population.

Improved evaluation and management of cognitive impairment: results of a comprehensive intervention in long-term care.

OBJECTIVES: Although as many as 50% of patients in long-term care have dementia, it is often not diagnosed and therefore, undertreated. We determined whether an intervention could improve the diagnosis and management of patients with cognitive impairment (CI) in long-term care. DESIGN, SETTING, AND PARTICIPANTS: The assessment phase consisted of a record review of 60 consecutively admitted patients to the San Francisco VA Nursing Home Care Unit (NHCU). Cognitive impairment was determined by admission MMSE < or = 24, indication of cognitive problem on MDS, or chart diagnosis of dementia. The evaluation consisted of a repeat chart review of 60 additional consecutively admitted patients. INTERVENTION: The intervention consisted of low-cost and easy-to-implement educational activities (training, focus groups), strategies to document cognitive status, and consultation with dementia experts. MEASUREMENTS/RESULTS: The 2 cohorts of 60 patients did not differ on demographics or on other characteristics (P > .25 for all). Prior to the intervention, of the 23 patients with CI, 52% had an identified etiology, 35% had a physician management plan, and 22% had a multidisciplinary care plan. Postintervention, of the 22 patients with CI, 91% had an identified etiology (P = .007), 86% had a physician plan (P = .001), and 59% had a multidisciplinary plan (P = .016). CONCLUSIONS: Initial results confirmed the underdiagnosis and undertreatment of CI in our long-term care facility. Our intervention with educational programs, increased documentation of cognitive status, and consultation resulted in increased identification of etiology and improved plans for management of patients with CI.

Non-pharmacological Management of Behavioral Symptoms in Frontotemporal and Other Dementias.

(Reprinted with permission from Current Neurology and Neuroscience Reports 2016; 16:14).

Implementation of advanced practice nurse clinic for management of behavioral symptoms in dementia: a dyadic intervention (innovative practice).

Behavioral symptoms are common in all types of dementia and often result in significant caregiver stress and illness, institutionalization of the patient, and reduced quality of life for the patient and caregiver. Health care practitioners often lack the expertise or time to adequately assess behavioral symptoms or counsel caregivers about interventions. Our goal was to implement a specialty clinic managed by advanced practice nurses to assess and manage behavioral symptoms associated with dementia. The clinic evaluations consisted of an assessment of the patient by the Nurse Practitioner during the time that the family caregiver(s) was interviewed by the Clinical Nurse Specialist and focused on an assessment of the cognitive and functional abilities of the patient, identification of triggers for the problematic behaviors, and assessment of caregiver coping. We evaluated 66 dyads since implementation in February 2010. The patients were primarily female, Caucasian, 74.3 years of age with Alzheimer's disease. The majority of caregivers were spouses (n = 44) followed by adult children (n = 20) and then siblings (n = 2). Targeted interventions were developed and caregiver counseling, support, and education were an integral part of the consultation and included written information, video instruction, and internet resources. Evaluations indicated caregivers and referring providers found the appointment helpful in managing behavioral symptoms and caregiver stress.

Demonstrating "collect once, use many"--assimilating public health secondary data use requirements into an existing Domain Analysis Model.

The healthcare industry has an increasing need for clinical data content standards to support patient care and data re-use in areas such as research, quality and public health. The Diabetes Data Strategy (Diabe-DS) project was formed in 2009 by the HL7 EHR Working Group to demonstrate a repeatable process that identifies disease-specific Common Data Elements (CDEs) for clinical care and secondary use. The Diabe-DS project previously developed a set of important CDEs and supporting data models for clinical care, quality and research uses of diabetes data. This paper will describe the process for identifying the data elements and activities required for public health use of clinical data, and mapping them to Diabe-DS CDEs, use case and data models. The result is a model for consideration which provides data needed in the immediate clinical environment of care, and supports the use of data for multiple uses.

Potentially inappropriate medication use in older adults with mild cognitive impairment.

BACKGROUND: Patients with mild cognitive impairment (MCI) may be especially vulnerable to the side effects of potentially inappropriate medications (PIMs), especially those that impair cognition. METHODS: We conducted a cross-sectional study to determine the prevalence of PIM use among 689 patients with MCI. We used the 2003 Beers Criteria for cognitive impairment to identify PIMs. We then determined if certain patients were more likely to use PIMs. RESULTS: There were 143 (20.8%) patients with MCI taking a PIM: 108 (15.7%) patients were taking one PIM and 35 (5.1%) patients were taking two or more PIMs. The most common PIMs were anticholinergics (35.7%) and benzodiazepines (31.5%). Patients were more likely to be taking PIMs if they were women and were taking a greater number of medications and less likely if they had a history of myocardial infarction. CONCLUSIONS: Patients with MCI are frequently taking PIMs that may negatively affect cognition. Future research is needed to assess whether cognitive impairment symptoms are improved if PIM use is reduced.

Treatment practices of mild cognitive impairment in California Alzheimer's Disease Centers.

OBJECTIVES: To examine "real world" treatments for patients with mild cognitive impairment (MCI). DESIGN: Cross-sectional. SETTING: California Department of Public Health Alzheimer's Disease (AD) Research Centers of California. PARTICIPANTS: Five hundred seventy-eight patients diagnosed with MCI. MEASUREMENTS: All patients underwent comprehensive neurological and neuropsychological evaluations. Logistic regression models were used to determine patient characteristics associated with use of anti-AD medications, statins, antioxidants, and folic acid. RESULTS: One hundred sixty-six patients (28.7%) were taking anti-AD medications; use was associated with greater functional impairment, higher education, MCI subtype, and older age (P<.05 for all). Two hundred fifty-two patients (43.6%) were taking statins; use was associated with diabetes mellitus, hypertension, myocardial infarct, male sex, and MCI subtype (P<.05 for all). One hundred fifteen patients (19.9%) were taking antioxidants; use was associated with higher education and diabetes mellitus and varied according to site (P<.05 for all). Thirty-seven patients (6.4%) were taking folic acid; use was associated with nonwhite race, male sex, and greater functional impairment (P<.05 for all). CONCLUSION: This study suggests that patients with MCI are frequently being treated with "off label" cholinesterase inhibitors and memantine, as well as other possible cognition-enhancing drugs. Further investigation of the effect of treatment patterns on the clinical course of MCI is needed.

Evaluation of the diagnosis and management of cognitive impairment in long-term care.

The objective was to determine the prevalence of screening for cognitive impairment and its effect on diagnosis and management of cognitive impairment in a long-term care setting, especially in comparison to depressive and behavioral symptoms. The study was a prospective cohort study of medical records of 60 patients admitted to the 120 bed Veterans Administration Nursing Home in San Francisco. Screening was determined by documented results on the Mini-Mental Status Examination, Geriatric Depression Scale, and Minimum Data Set. Diagnosis and management were determined by review of medical records for provider documentation. Strategies for management were broad, including interventions such as pharmacological therapy, education, behavioral strategies, or caregiver interventions. Screening was conducted routinely for cognitive impairment (95%), depressive symptoms (95%), and behavioral symptoms (85%). Cognitive status was uncertain in 8 patients; 23 (44%) met criteria for cognitive impairment. Twelve (52%) had a differential diagnosis of cognitive impairment and 11 (48%) had evidence of strategies for management. Depressive and behavioral symptoms were equally or almost as common as cognitive impairment (44% and 25%, respectively), but evidence of a management plan was more frequently present (80% and 100%; p = 0.002 vs. with cognitive impairment). Although depressive and behavioral symptoms were frequently diagnosed and interventions initiated, the diagnosis and management of cognitive impairment were often imprecise or absent. Further research is needed to identify barriers to diagnosis and management so that greater numbers of patients can benefit from recommended strategies.