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Author(s): Maurice A. Smith (corresponding author) [1]; Jason Brandt [2]; Reza Shadmehr [1] A steady progression of motor dysfunction takes place in Huntington's disease [1] (HD). The origin of this [...]

Objectives. Executive functions supervise the contents of working memory, where information from long-term memory is integrated with information in the immediate present. This study examined whether executive attentional abilities were uniquely associated with the performance of complex, instrumental activities of daily living (IADLs) in cognitively intact and physically high-functioning older women. Methods. Participants were 406 community-residing, older women aged 70-80 years in the Women's Health and Aging Study (WHAS) II, screened to be physically high functioning and cognitively intact using the Mini-Mental State Exam. Hierarchical regression models, adjusted for demographic and disease variables, were used to evaluate the association of cognitive domains, including executive attention, memory, psychomotor speed, and spatial ability with summary measures of IADL (e.g., looking up and dialing a telephone number) and mobility-based ADL (e.g., walking 4 meters) function. Results. Tests of executive attention were associated with performance on IADLs (6.6%) and, to a lesser degree, mobility-based ADLs (1%), adjusting for demographic and disease variables. In particular, the mental flexibility component of the Trail Making Test accounted for the majority of attentional variance in IADL performance. Older age, lower education, and African American race were also associated with poorer physical test performances. Discussion. Executive difficulties in flexibly planning and initiating a course of action were selectively associated with slower performance of higher-order IADL tests, relative to other domains of cognition, in a high-functioning, community-based older cohort. These results suggest that executive functions may be important in mediating the onset and progression of physical functional declines.

Children with one of several severe neurological conditions resulting in repeated seizures may benefit from a surgical procedure involving the removal of half the brain called a hemispherectomy. Life quality measurements were evaluated in 58 children who had had hemispherectomies between 1968 and 1996. Parents and doctors considered all these operations to have been minimally to very successful in improving the patients' overall condition and reducing the number of seizures. Most operations were done on children with Rasmussen's syndrome or birth defects of the brain's physical or vessel structure. Four children died., Purpose. To report the outcomes of the 58 hemispherectomies performed at Johns Hopkins between 1968 and January 1996. Methods. Charts were reviewed of the 58 hemispherectomies performed at Johns Hopkins Medical Institutions by the Pediatric Epilepsy Group during the years 1968 to 1996. Twenty-seven operations were done for Rasmussen's syndrome, 24 operations for cortical dysplasias/hemimegalencephalies, and 7 for Sturge-Weber syndrome or other congenital vascular problems. Seizure control alone did not seem to adequately describe the outcomes of the procedure. Therefore, a score was constructed that included seizure frequency, motor disability, and intellectual handicap. This burden of illness score better described the child's handicap before and after surgery. Results. Perioperative death occurred in 4 out of 58 children. Of the 54 surviving children, 54% (29/54) are seizure-free, 24% (13/54) have nonhandicapping seizures, and 23% (12/54) have residual seizures that interfere to some extent with function. Reduction in seizures was related to the etiology of the unilateral epilepsy. Eighty-nine percent of children with Rasmussen's, 67% of those with dysplasias, and 67% of the vascular group are seizure-free, or have occasional, nonhandicapping seizures. All operations were considered by the parents and the physicians to have been successful in decreasing the burden of illness. In 44 the procedure was very successful, in 7 it was moderately successful, and in 3 it was minimally successful. Success was related to the etiology, and early surgery was preferable. Conclusion. Hemispherectomy can be a valuable procedure for relieving the burden of seizures, the burden of medication, and the general dysfunction in children with severe or progressive unilateral cortical disease. Early hemispherectomy, although increasing the hemiparesis in children with Rasmussen's syndrome, relieves the burden of constant seizures and allows the child to return to a more normal life. In children with dysplasias, early surgery can allow the resumption of more normal development. Pediatrics 1997;100:163-171; hemispherectomy, burden of illness, epilepsy surgery, quality of life., ABBREVIATIONS. CVA, cerebrovascular accident; MDQ motor developmental quotient; IQ intelligence quotient; DQ development quotient; EEG, electroencephalogram. The term hemispherectomy is used throughout the literature for several different neurosurgical procedures designed [...]

An algorithm has been developed to predict the time a patient with Alzheimer's disease will need to be admitted to a nursing home and the time to death. It consists of tables that allow scores for different predictors to be added up. The predictors include age at onset, length of illness and presence of symptoms such as tremor, rigidity, gait disorders, delusions and hallucinations. The algorithm will give the number of months until nursing home placement and death. The algorithm accurately predicted outcomes in a validation sample., Objective.--To develop and validate an approach that uses clinical features that can be determined in a standard patient visit to estimate the length of time before an individual patient with Alzheimer disease (AD) requires care equivalent to nursing home placement or dies. Design.--Prospective cohort study of 236 patients, followed up semiannually for up to 7 years. A second validation cohort of 105 patients was also followed. Setting.--Three AD research centers. Patients.--All patients met National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) criteria for probable AD and had mild dementia at the initial visit. Intervention.--Predictive features, ascertained at the initial visit, were sex, duration of illness, age at onset, modified Mini-Mental State Examination (mMMS) score, and the presence or absence of extrapyramidal signs or psychotic features. Main Outcome Measures.--(1) Requiring the equivalent of nursing home placement and (2) death. Results.--Prediction algorithms were constructed for the 2 outcomes based on Cox proportional hazard models. For each algorithm, a predictor index is calculated based on the status of each predictive feature at the initial visit. A table that specifies the number of months in which 25%, 50%, and 75% of patients with any specific predictor index value are likely to reach the end point is then consulted. Survival curves for time to need for care equivalent to nursing home placement and for time to death derived from the algorithms for selected predictor indexes fell within the 95% confidence bands of actual survival curves for patients.When the predictor variables from the initial visit for the validation cohort patients were entered into the algorithm, the predicted survival curves for time to death fell within the 95% confidence bands of actual survival curves for the patients. Conclusions.--The prediction algorithms are a first but promising step toward providing specific prognoses to patients, families, and practitioners. This approach also has clear implications for the design and interpretation of clinical trials in patients with AD. JAMA. 1997;277:806-812

Background. Studies using clinical measures to track AD progression often assume linear declines over the entire course of the disease, which may not be justified. The objective of this study was to model change in measures of the clinical severity of Alzheimer's disease (AD) over time. Methods. We developed a method to apply growth curve models to prospective data and characterize AD patients' functional change over time. Data from the modified Mini-Mental State Examination (mMMSE) and measures of basic and instrumental ADL, administered semiannually for up to 5 years to 236 patients with probable AD, were modeled. Results. The rate of decline in mMMS scores per 6-month interval gradually increased as scores dropped from the maximum of 57 to 20. The rate of decline then decreased as scores approached 0, resulting in an inverse 'S' curve. The rate of increase in instrumental ADL scores per interval attenuated as the scores increased, while that for basic ADL scores across intervals was constant. Conclusions. Differences in the pattern of progression of the three measures is in part a function of their psychometric properties. The progression curves may also reflect content-specific features of the instruments. Superimposition of the modeled decline in these three content areas suggests a hypothetical model of the relative timing of cognitive and functional changes in AD.