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.
Bacterial endotoxin (lipopolysaccharide; LPS) given to animals in large doses results in pronounced, midzonal liver injury. Exposure to smaller, non-injurious doses of LPS augments the toxicity of certain hepatotoxicants. This study was conducted to delineate the development of injury in a rat model of augmentation of aflatoxin B1 (AFB1) hepatotoxicity by LPS. At large doses (i.e., > 1 mg/kg, ip), AFB1 administration resulted in pronounced injury to the periportal regions of the liver. Male, Sprague-Dawley rats (250–350 g) were treated with 1 mg AFB1/kg, ip or its vehicle (0.5% DMSO/saline) and 4 h later with either E. coli LPS (7.4 × 106 EU/kg, iv) or its saline vehicle. Liver injury was assessed 6, 12, 24, 48, 72, or 96 h after AFB1 administration. Hepatic parenchymal cell injury was evaluated as increased alanine aminotransferase (ALT) and aspartate aminotransferase (AST) activities in serum and from histologic examination of liver sections. Biliary tract alterations were evaluated as increased concentration of serum bile acids and activities of γ-glutamyltransferase (GGT), alkaline phosphatase (ALP), and 5′-nucleotidase (5′-ND) in serum. At all times and for all markers, injury in rats treated with either AFB1 or LPS alone was absent or modest. In the AFB1/LPS cotreated group, hepatic parenchymal cell injury was pronounced by 24 h and had returned to control values by 72 h. The injury began in the periportal region and spread midzonally with time. Furthermore, changes in serum markers indicative of biliary tract alterations were evident by 12 h and had returned to control values by 72 h. Thus, the nature of the hepatic lesions suggested that LPS potentiated the effects of AFB1 on both parenchymal and bile duct epithelial cells.
Lake Roosevelt (Franklin D. Roosevelt Lake) is the impoundment of the upper Columbia River behind Grand Coulee Dam, and is the largest reservoir within the Bureau of Reclamation's Columbia Basin Project (CBP). The reservoir is located in northeastern Washington, and stretches 151 miles from Grand Coulee Dam north to the Canadian border. The 15-20 miles of the Columbia River downstream of the border are riverine and are under small backwater effects from the dam. Grand Coulee Dam is located on the mainstem of the Columbia River about 90 miles northwest of Spokane. Since the late 1980s, trace-element contamination has been known to be widely present in Lake Roosevelt. Trace elements of concern include arsenic, cadmium, copper, lead, mercury, and zinc. Contaminated sediment carried by the Columbia River is the primary source of the widespread occurrence of trace-element enrichment present in Lake Roosevelt. In 2001, the U.S. Environmental Protection Agency (EPA) initiated a preliminary assessment of environmental contamination of the Lake Roosevelt area (also referred to as Upper Columbia River, UCR site, or UCR/LR site) and has subsequently begun remedial investigations of the UCR site.
Telemedicine is increasingly being used to provide consultation in rural areas. Little work has been done with dementia although preliminary research suggests that clinical diagnosis performed via telemedicine consultation is valid. We used telemedicine to evaluate patients with cognitive impairment in rural Northern California. Patients at a rural VA community clinic 280 miles north of San Francisco were referred by their local provider for telemedicine evaluation of memory complaints by the multidisciplinary team of the San Francisco Veterans Administration (SFVA) Memory Disorders Clinic (MDC), part of the UCSF state and federal Alzheimer's Disease research center. The telemedicine team consisted of a designated physician who performed a history and limited physical exam and a neuropsychologist who administered selected components of a neuropsychological battery used in the MDC. A liaison clinician at the community clinic was trained by MDC staff in the evaluation of patients with cognitive impairment and was present for the evaluation to facilitate the interview, physical exam and neuropsychological evaluation. On completion, community clinic staff attended the MDC case conference via telemedicine to discuss diagnosis and recommendations. Adjunct to the patient evaluation, educational offerings for primary providers and an interactive caregiver education group were broadcast to the community clinic via telemedicine. We have evaluated 10 new patients and completed one follow-up evaluation. Patients included those unable to travel to the SFVAMC who had an involved caregiver. Patients with significant sensory deficits (eg hearing or vision) or moderate-severe dementia (Mini Mental State Examination < 15) were excluded. In each case, the telemedicine format permitted the MDC team to arrive at a working diagnosis and formulate relevant treatment recommendations (antidepressant therapy, cholinesterase inhibitors, counseling about vascular risk factor reduction and behavioral management strategies). Provider in-services and caregiver education groups have been well attended with positive evaluations. Telemedicine is emerging as an effective way to provide consultation and care to rural residents who may not have access to specialty services. Further research about outcomes is needed.
Abstract 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.
