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PubMed1985

Disproportionate intra-uterine head growth and developmental outcome.

The authors examined the relationship between disproportionate intra-uterine head growth, or a relatively small head, and later development, using data collected as part of the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke. Within the population of infants who are normal by conventional criteria (term infants with appropriate weight and head-circumference for gestational age), a group with relatively small heads was defined by each of four different methods. Developmental outcome measures included the Bayley Mental and Motor Scales at eight months, Stanford-Binet IQ at four years and Wechsler Intelligence Scales IQ at seven years. No clinically meaningful differences in developmental outcome were found between the infants with relatively small heads and the remainder of the infants.

Birth Weight↗
PubMedDate not supplied

Epidemiology of severe myoclonic epilepsy of infancy.

Severe myoclonic epilepsy of infancy (SMEI) is a newly recognized epileptic syndrome. It is characterized by multiple febrile seizures, often prolonged, subsequent development of uncontrollable mixed-myoclonic seizures, and, eventually, psychomotor retardation. Drugs for myoclonic epilepsy--valproate (VPA), the suximides, and the benzodiazepines--have been shown to be useful in SMEI. Among children with seizures in the National Institute of Neurological and Communicative Disorders and Stroke Collaborative Perinatal Project (NCPP), one individual with SMEI was identified. This finding from the NCPP suggests that the incidence of SMEI is approximately 1 in 40,000 children. Such an incidence is supported by observations at the Texas Tech University Health Sciences Center.

Brain↗
PubMed1994

Alcohol-related dementia in the institutionalized elderly.

This study examined the distribution of alcohol-related and other dementias in a sample of 130 cognitively impaired residents of long-term care facilities in a Northern Ontario community. Study procedures entailed standardized psychiatric, neurological, and neuropsychological evaluations. Diagnoses of dementia of the Alzheimer type (DAT) and vascular dementia were based on criteria of the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association. The diagnosis of alcohol-related dementia (ARD) was based on extensive review of medical history to assess before alcohol abuse and stabilization or improvement in cognitive functioning following institutionalization in conjunction with no other identifiable cause of dementia. ARD comprised 24% of this population compared with DAT (35%), vascular dementia (19%), and other causes (22%). The ARD group was, on average, 10 years younger than the other groups. It had nearly twice the average length of institutionalization and had milder cognitive impairment on both clinical ratings and neuropsychological tests. A diagnosis of ARD was present in the medical records for only 25% of patients in this group. These findings suggest that ARD may be more common than previously suspected in the distribution of dementias in long-term care facilities.

Aged↗
PubMed1978

The social behavior of elderly aphasic versus nonaphasic long-term patients.

A study was made of the social consequences of communicative disorders among 51 elderly hospitalized long-term patients. Independent variables used to account for variations in social behavior were: 1) the presence or absence of aphasia, an impairment resulting in varying degrees of loss of verbal recognition, comprehension and language production; 2) sex; and 3) the level of nursing-care needs as an indicator of overall physical independence. A rating scale for measuring "Social Involvement" was constructed. It involved four analytically-derived factors - Interpersonal Behavior, Familial and Social Contacts, Group Participation, and Extent of Social Contacts. Since a comparison of the 15 aphasic and 36 nonaphasic subjects on the basis of these four factors revealed no substantial main effect, it would seem that social isolation was not attributable to aphasia per se. Rather, a number of significant interactions indicated that differences in the levels of social involvement were attributable to the combined influences of sex, presence or absence of aphasia, and varying levels of physical independence. The relationship between social involvement and social isolation has implications for the overall management and rehibilitation of long-term patients.

Aged↗
PubMed1991

Progression of cognitive impairment in Alzheimer's disease.

Change in cognitive function was assessed over 12 months in 110 patients over the age of 65 satisfying National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS/ADRDA) criteria for "probable" Alzheimer's Disease. A highly significant deterioration in cognitive function was observed. Decline in cognitive scores was relatively normally distributed. Patients who died during the follow-up had more apraxia at entry to the study than survivors. A greater rate of decline was seen in patients whose parents suffered from dementia (but not in those where a sibling or other relative was affected), in subjects who had moderate dementia, and those who had been ill for less than 24 months. Age, age of onset, and the presence or absence of aphasia or apraxia had no influence on rate of progression. A cluster analysis revealed three patterns of decline.

Age Factors↗
PubMed1996

Affective disturbances in Alzheimer's disease.

OBJECTIVE: To evaluate the emotional disturbances in patients with Alzheimer's disease (AD) using both a categorical and a dimensional approach. DESIGN: Prospective study. SETTING: Outpatient clinic in a neurological department from a general University Teaching Hospital. MEASURES: A semi-structured interview was used to fill in the Hamilton Depression Rating Scale, the Retardation Rating Scale for depression, the Tyrer and Covi scales for anxiety, and the Depressive Mood Scale for emotional disturbances. The cognitive status was assessed by the Mini-Mental State Examination and the Mattis Dementia Rating Scale. PATIENTS: One hundred eighteen consecutive AD outpatients fitting the criteria for probable or possible AD, according to the National Institute of Neurological and Communication Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association, were compared with 34 community-dwelling healthy older controls and with 20 inpatients meeting the diagnostic criteria for depression according to the Diagnostic and Statistical Manual of Mental Disorders, 3rd Edition-Revised. RESULTS: No AD patient met the criteria for Major Depressive Episode or Generalized Anxiety Disorder, but 10 (8%) met the criteria for Dysthymic Disorder. AD patients scored significantly higher than the control group but lower than the depressed group for depressive and anxious symptomatology. Depressive symptomatology was correlated negatively to the cognitive status and positively to anxious symptomatology. Two main dimensions in emotional disturbances were described using the Depressive Mood Scale: Emotional Deficit (anhedonia, hypoexpressiveness) and Loss of Control (felt irritability, hyperexpressiveness). Emotional Deficit was correlated positively to the depressive symptomatology and correlated negatively to the cognitive status and the Loss of Control dimension. Loss of Control was correlated positively to the severity of the depressive and anxious symptomatology and weakly to cognitive performance. CONCLUSION: Affective changes were found frequently in AD patients, but no major affective disorder was found. The dimensional approach seems to be more appropriate than the categorical approach to describe the emotional disturbances in these patients.

Affect↗
PubMed1999

Clinico-neuropathological correlation of Alzheimer's disease in a community-based case series.

OBJECTIVES: Most clinico-neuropathological correlative studies of Alzheimer's Disease (AD) are based on research cohorts that are not necessarily generalizable to patients seen in the general medical community. In this study, we examine the accuracy of the criteria used in diagnosing AD in a community-based case series of patients with memory complaints. DESIGN AND PARTICIPANTS: Clinical and neuropathological diagnoses were obtained from 134 patients evaluated for dementia who subsequently underwent autopsy. SETTING: Subjects who exhibited new symptoms of dementia and were enrolled in the University of Washington/Group Health Cooperative Alzheimer's Disease Patient Registry were eligible for this study. MEASUREMENTS: Clinico-pathological correlation was performed using NINCDS-ADRDA (National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association) and CERAD (Consortium to Establish a Registry for Alzheimer's Disease) criteria. RESULTS: Ninety-five of the 134 cases studied met CERAD neuropathological criteria for AD. The sensitivity of NINCDS-ADRDA "probable AD" was 83% (diagnosing AD correctly) and overall clinical diagnostic accuracy was 75%. However, there was a high rate of additional neuropathological findings. Only 34 of the 94 cases had pure AD on neuropathology, whereas the remainder frequently had coexisting vascular or Parkinson's disease lesions. CONCLUSIONS: This study of a large series of community-based incident dementia cases provides a way of judging the adequacy of currently available clinical diagnostic criteria. It also shows that co-existing neuropathological findings are common in community-based AD.

Aged↗
PubMed2004

Incidence and prevalence of dementia in the Cardiovascular Health Study.

OBJECTIVES: To estimate the incidence and prevalence of dementia, Alzheimer's disease (AD), and vascular dementia (VaD) in the Cardiovascular Health Study (CHS) cohort. DESIGN: Longitudinal cohort study using prospectively and retrospectively collected data to evaluate dementia. SETTING: Four U.S. communities. PARTICIPANTS: There were 3,602 CHS participants, including 2,865 white and 492 African-American participants free of dementia, who completed a cranial magnetic resonance image between 1992 and 1994 and were followed for an average of 5.4 years. MEASUREMENTS: Dementia was classified by neurologist/psychiatrist committee review using neuropsychological tests, neurological examinations, medical records, physician questionnaires, and proxy/informant interviews. Demographics and apolipoprotein E (APOE) genotype were collected at baseline. Incidence by type of dementia was determined using National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria for AD and Alzheimer's Disease Diagnostic and Treatment Center's State of California criteria for VaD. RESULTS: Classification resulted in 227 persons with prevalent dementia at entry into the study and 480 incident cases during follow-up. Incidence rates of dementia scaled to age 80 were 34.7 per 1,000 person-years for white women, 35.3 for white men, 58.8 for African-American women, and 53.0 for African-American men. Sex differences were not significant within race. Adjusted for age and education, racial differences were only of borderline significance and may have been influenced by ascertainment methodology. Rates differed substantially by educational attainment but were only significant for whites. Those with the APOE epsilon4 allele had an incidence rate at age 80 of 56.4, compared with 29.6 for those without this allele (P<.001). In whites, type-specific incidence at age 80 was 19.2 for AD versus 14.6 for VaD. These rates were 34.7 and 27.2 for African Americans. At termination of observation, women had only a slightly higher prevalence of dementia (16.0%) than men (14.7%). CONCLUSION: Sex and racial differences were not found, and VaD was higher than reported in other studies. These data provide new estimates of dementia incidence in a community sample for projection of future burden.

Black or African American↗
PubMed2006

Deletion/insertion polymorphism of the angiotensin-converting enzyme gene and white matter hyperintensities in dementia: A pilot study.

OBJECTIVES: To examine the association between the angiotensin-converting enzyme (ACE) deletion/insertion (D/I) polymorphism and white matter hyperintensities (WMHs) in patients with dementia. DESIGN: Observational pilot study with adjustment for potential confounders using analysis of covariance. SETTING: Secondary care old-age psychiatry services in greater Manchester, United Kingdom. PARTICIPANTS: Ninety-seven patients with dementia: 49 with Alzheimer's disease (AD, National Institute of Neurological and Communicative Disorders and Stroke/Alzheimer's Disease and Related Disorders Association criteria) and 48 with vascular dementia (VaD, National Institute of Neurological Disorders and Stroke/Association Internationale pour la Recherche et l'Enseignement en Neurosciences criteria). MEASUREMENTS: The ACE D/I polymorphism, WMHs (deep WMHs (DWMHs) and periventricular hyperintensities (PVHs)) on T2-weighted magnetic resonance imaging, and potential cardiovascular confounders. RESULTS: The D/D polymorphism of the ACE genotype was associated with severity of DWMH (P = .005) but not PVH (P = .34), corrected for age, cardiovascular risk factors, and type of dementia. Post hoc analyses were limited by statistical power but suggested an interaction with the apolipoprotein E epsilon4 allele. CONCLUSION: The results support previous observations that genetic factors influence the development of WMHs in dementia. The involvement of the ACE D/I polymorphism in the pathogenesis of DWMHs in dementia (AD and VaD), by a mechanism that is independent of its association with cardiovascular risk factors, should be confirmed in a large population-based sample.

Aged↗
PubMed2006

Four components describe behavioral symptoms in 1,120 individuals with late-onset Alzheimer's disease.

OBJECTIVES: To investigate behavioral components of Alzheimer's disease (AD) and to analyze behavioral components in relation to disease severity, apolipoprotein E genotype (APOE), sex, years of education, age at onset, and cognitive impairment. DESIGN: Cross-sectional study. SETTING: Data were collected from community-dwelling individuals and those residing in nursing homes. PARTICIPANTS: A total of 1,120 individuals meeting National Institute of Neurological and Communicative Disorders and Stroke/Alzheimer's Disease and Related Disorders Association criteria for late-onset probable AD. MEASUREMENTS: Behavioral symptoms were assessed using the Neuropsychiatric Inventory. First-order polychoric correlations, controlling for disease severity, between the 12 symptom domain scores were estimated, and the resulting matrix underwent principal components analysis. RESULTS: Four interpretable components were identified: behavioral dyscontrol (euphoria, disinhibition, aberrant motor behavior, and sleep and appetite disturbances), psychosis (delusions and hallucinations), mood (depression, anxiety, and apathy), and agitation (aggression and irritability). Scores on the four components were associated with severity of cognitive impairment. Higher behavioral dysfunction, agitation, and mood component scores were associated with lower age at onset. Behavioral dysfunction and mood component scores were associated with sex. None of the components were associated with age at assessment, years of education, or number of APOE epsilon4 alleles. CONCLUSION: Four behavioral components were identified that were comparable with those observed previously. Future analysis of these components will strengthen understanding of the underlying pathology of behavioral symptoms and AD.

Age of Onset↗
PubMed1984

Proposal for summing the incapacity status or environmental status scores.

Factor analysis was performed on the scores of variables of the Incapacity Status (ISS) and the Environmental Status Scale (ESS) in 184 MS patients. The aim of the study was to examine the relation between these variables and to examine the possibilities of using a summation process on scores. It proved that summing scores of the ISS conceals important information on communication disorders and on bladder, bowel and sexual dysfunctions. It was suggested to use three, composed variables of the ISS and the five variables of the ESS in the summation to produce a figure which reflects the handicaps of the patients.

Activities of Daily Living↗
PubMed1988

Guillain-Barré syndrome: diagnostic criteria, epidemiology, clinical course and prognosis.

Thirty-four patients were identified with Guillain-Barré syndrome (GBS) on review of 266 neuropathy cases admitted to a Copenhagen county hospital from June 1977 to January 1984. The age-adjusted incidence rate of GBS is 2.0 x 10(-5) years-1. The natural history of the disease, antecedent events, symptoms and signs, autonomic dysfunction, sequelae, CSF findings and mortality are described. Six cancer patients with GBS differed significantly from the non-cancer patients in a more protracted disease course and failure to improve. The National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for the diagnosis of GBS are discussed, and it is concluded that the criteria, although useful in comparative studies, are too restrictive when used in clinical practice.

Aged↗
PubMed1995

Guillain-Barré syndrome in south-west Stockholm, 1973-1991, 1. Quality of registered hospital diagnoses and incidence.

We describe the incidence and explore the quality of registered diagnosis for Guillain-Barré syndrome (GBS) in a population-based retrospective study in South-West Stockholm (SWS), Sweden, during the period 1973-1991. We used data on registered hospital discharges and open-care visits, and from medical records. Medical records were available for 83 (80.6%) of 103 patients with registered GBS diagnosis at discharge from hospitals. For 69 (83.1%) of such patients, the information from the hospital record fulfilled the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) clinical criteria for GBS. One out-patient presented with suspected GBS. In contrast, none of 40 patients discharged with diagnosis of unspecific polyneuropathy fulfilled the above mentioned criteria. During the period 1973-1991, the mean annual incidence of GBS per 100,000 was 1.74, 95% CI 1.41-2.12, from register data and 1.49, 95% CI 1.19-1.85, after excluding those not fulfilling criteria for GBS. The age-adjusted incidence after validation was higher for men, 1.64, 95% CI 1.19-2.21, than for women, 1.46, 95% CI 1.05-1.99. An increasing incidence with age was found, presenting the age-specific curve a bimodal shape. The results of this study confirm that hospital discharge diagnostic data in Sweden can be used for purposes of epidemiological research and surveillance for GBS, and show that the incidence of GBS in SWS compares to those described in other populations.

Adolescent↗
PubMed2004

Incidence and clinical features of acute inflammatory polyradiculoneuropathy in Lombardy, Italy, 1996.

OBJECTIVE: To assess the annual incidence of typical Guillain-Barré syndrome (GBS) and its main variants (atypical GBS) in a well-defined population from a large area. MATERIAL AND METHODS: A population-based prospective survey of GBS was undertaken during the calendar year 1996 in Lombardy, Italy (population 8,891,652). Typical and atypical GBS was diagnosed using the National Institutes of Neurological and Communicative Disorders and Stroke (NINCDS) and Ropper criteria. Eligible cases were hospital inpatients traced through a regional registry, the hospital discharge diagnoses, and an ongoing case-control study. Diagnostic and demographic findings were collected for each case. Complete clinical and laboratory features were available for 80% of cases. RESULTS: A total of 138 patients (males 74; females 64) aged 2-91 years fulfilled the diagnostic criteria for typical GBS (128) or atypical GBS (10). GBS variants included Miller-Fisher syndrome (four cases), cranial polyneuritis (three cases), pure motor GBS (two cases), and sensory loss with areflexia (one case). The crude annual incidence of GBS was 1.55 per 100,000 (typical GBS 1.43; atypical GBS 0.11; male 1.67; female 1.43; age <35 years, 0.79; 35-54 years, 1.33; 55-74 years, 3.22; 75+ years, 4.67). The overall rate was 1.58 when age- and sex-adjusted to the 1996 Italian population. Previous infections were reported for 37% of patients. The electrophysiological findings indicated demyelination in 51%, primary axonopathy in 14%, and mixed myelin and axon involvement in 27%. CONCLUSIONS: The incidence of typical GBS is comparable with that in other reports using the NINCDS diagnostic criteria. Atypical GBS accounts for a limited number of cases.

Adolescent↗
PubMed2002

Auditory processing efficiency deficits in children with developmental language impairments.

The "temporal processing hypothesis" suggests that individuals with specific language impairments (SLIs) and dyslexia have severe deficits in processing rapidly presented or brief sensory information, both within the auditory and visual domains. This hypothesis has been supported through evidence that language-impaired individuals have excess auditory backward masking. This paper presents an analysis of masking results from several studies in terms of a model of temporal resolution. Results from this modeling suggest that the masking results can be better explained by an "auditory efficiency" hypothesis. If impaired or immature listeners have a normal temporal window, but require a higher signal-to-noise level (poor processing efficiency), this hypothesis predicts the observed small deficits in the simultaneous masking task, and the much larger deficits in backward and forward masking tasks amongst those listeners. The difference in performance on these masking tasks is predictable from the compressive nonlinearity of the basilar membrane. The model also correctly predicts that backward masking (i) is more prone to training effects, (ii) has greater inter- and intrasubject variability, and (iii) increases less with masker level than do other masking tasks. These findings provide a new perspective on the mechanisms underlying communication disorders and auditory masking.

Adult↗
PubMed1998

Speech articulator measurements using low power EM-wave sensors.

Very low power electromagnetic (EM) wave sensors are being used to measure speech articulator motions as speech is produced. Glottal tissue oscillations, jaw, tongue, soft palate, and other organs have been measured. Previously, microwave imaging (e.g., using radar sensors) appears not to have been considered for such monitoring. Glottal tissue movements detected by radar sensors correlate well with those obtained by established laboratory techniques, and have used to estimate a voiced excitation function for speech processing applications. The noninvasive access, coupled with the small size, low power, and high resolution of these new sensors, permit promising research and development applications in speech production, communication disorders, speech recognition and related topics.

Electromagnetic Phenomena↗