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PubMed1995

Apolipoprotein E genotypes in Parkinson's disease with and without dementia.

The apolipoprotein E gene (Apo E) type 4 allele is a genetic risk factor influencing the development and age of onset of Alzheimer's disease. Because Parkinson's disease shares many characteristics of Alzheimer's disease, we studied the frequencies of Apo E genotypes in a cohort of 52 Parkinson's disease patients with dementia and 61 patients without dementia. Dementia was determined per National Institute of Neurological and Communicative Disorders and Stroke criteria and Mattis Dementia Rating Scale (DRS) < 126. Normal cognition was defined as DRS > 132. Apo E genotype and allele frequencies did not differ between demented and nondemented parkinsonian patients. Neither group's genotype and allele frequencies differed from that of a nondemented population of 78 controls. We conclude that the Apo E epsilon 4 allele influences neither the development of Parkinson's disease nor the dementia associated with Parkinson's disease.

Age of Onset↗
PubMed2006

Validation of a memory inventory for the assessment of awareness of memory deficits in Alzheimer's disease in Chinese elderly.

BACKGROUND: This paper describes the development and validation of the Memory Inventory for Chinese (MIC), for measuring the awareness of memory deficits in the Chinese population with Alzheimer's disease (AD). METHODS: A combination of qualitative and quantitative approaches was adopted. The MIC was developed with focus group discussion and pilot testing. It has a patient and a caregiver version. A consecutive series of 79 new out-patients with the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorder Association (NINCDS-ADRDA) criteria of probable and possible AD and 20 non-demented elderly subjects were recruited. RESULTS: A high internal consistency was found, with Cronbach alpha of 0.89 for the patient version and 0.90 for the caregiver version of MIC. The inter-rater reliability was satisfactory. For validity assessment, the caregiver score of the MIC correlated significantly with cognitive score of the subject as assessed by the Mini-Mental State Examination (rp = -0.37; p < 0.01). The Memory Deficit Awareness Score, calculated by subtracting the patient score from the caregiver score, correlated significantly with clinician ratings of awareness of memory impairment (rs = -0.67; p < 0.01). CONCLUSIONS: The MIC appears to be a culturally appropriate and valid instrument for the measurement of awareness of memory deficits in Chinese patients with AD. Potential applications of the MIC should be further explored in other subtypes of dementia and in prospective studies.

Aged↗
PubMed1990

Some historical and methodological developments in early clinical trials at the National Institutes of Health.

This paper reviews five randomized clinical trials with unusual design or analysis features from institutes other than the National Cancer Institute or the National Heart, Lung and Blood Institute. These are: a Cooperative Study of Retrolental Fibroplasia sponsored by the National Institute of Neurological Diseases and Blindness; the Diabetic Retinopathy Study sponsored by the National Eye Institute; the University Group Diabetes Program sponsored by the National Institute of Arthritis and Metabolic Diseases; a Clinical Trial of the Extracranial to Intracranial Arterial Anastomosis (EC/IC bypass) by the National Institute of Neurological and Communicative Disorders and Stroke; and the Clinical Trial of Hereditary Angioedema by the National Institute of Allergy and Infectious Diseases.

Anastomosis, Surgical↗
PubMed1990

Symmetry and quasi-symmetry: an example in modelling pairs of sounds from children's early speech.

The models of symmetry and quasi-symmetry may have use in the analysis of categorical data in square tables where the row and column classifications are the same. Under the model of symmetry, the probability that an observation falls in, say, the (i,j) cell of a square table is the same as the probability that it falls in the (j,i) cell. The model of symmetry requires that the expected marginal total for any one row of the table be the same as the expected marginal total for the corresponding column. The model of quasi-symmetry, which is a generalization of the model of symmetry, does not require this equality of expected row and column totals. In this paper, we illustrate the use of the models of symmetry and quasi-symmetry in the analysis of categorical data that summarize pairs of sounds in infants' and toddlers' early utterances. In the past, the complexity of infants' early vocalizations has been difficult to measure because of a lack of methods to quantify prelinguistic phonetic variables. We use the models of symmetry and quasi-symmetry as a first attempt to quantify the complexity of infants' early speech. We hope that the discovery of models appropriate for quantification of language development in normal children will have later use in identification of children at risk for communicative disorders.

Child, Preschool↗
PubMed2003

Restoration of locomotion in paraplegics with aid of autologous bypass grafts for direct neurotisation of muscles by upper motor neurons--the future: surgery of the spinal cord?

OBJECTIVES: Paraplegia means a lifelong sentence of sensory loss, paralysis and dependence. Complete spinal cord lesions cannot heal up to now despite intensive experimental research, remarkable efforts and recent achievements in bio-technology and re-engineering. Traumatic paraplegia due to spinal cord injury (SCI) is a quite frequent condition and related to the socio-economical situation of the population. It is experienced disproportionately by young people. The rise in gunshot wounds is dramatic. SCI has appeared refractory to treatment. PATIENTS AND METHODS: Since 1980 G.A.B. had tried surgical repair of the spinal cord (SC) after experimental bisection in rats, and since 1993 research was done on monkeys (macaca fascicularis) to be closer to human physiology. The sciatic nerve was removed and used as an autologous graft from the lateral bundle of the spinal cord (tractus corticospinalis ventro lateralis) to the three muscles of both legs being known to be most important for locomotion: M. gluteus maximus, M. gluteus medius and M. quadriceps femoris. The first fruitful transplantation in a human being was performed in July 2000. RESULTS: The results in rats were promising and fulfilled the requirements of the American Task Force of the National Institute of Neurological and Communicative Disorders and Stroke of the US. The results in monkeys confirmed the paradigm so that we performed the first operation in a young lady suffering for four months from complete SC lesion T9 after approval by the ethical committee. First voluntary movements of the connected muscles after 17 months. 27 months after op she was able to walk up to 60 steps with the help of a walker and to climb steps in the water. Improvement is still continuing. DISCUSSION: SCI has appeared refractory to any kind of treatment. Compensatory strategies are still experimental in human beings. Autologous nerve grafts from the spinal cord tissue (the lateral spinal bundle) connected to peripheral muscle nerves seem promising in paraplegics. But the physiology is still unclear when the glutamatergic upper motor neuron connected to motor end-plates (cholinergic) does work like in our patient. CONCLUSION: Further studies in primates and paraplegic patients are necessary to clarify the bypass grafting of the SC to muscle groups distal to the complete SCI to restore locomotion.

Adult↗
PubMed1981

Psychosocial effects of an intensive summer communication program for cleft palate children.

The preventative psychosocial effects of an intensive summer residential program for children with communication disorders related to cleft lip and palate are evaluated. Twelve children completed the five-week program. Following the program, the children increased their social interaction rates during observed interactions with nonhandicapped peers and according to parent ratings. Less difference existed between the children's perceived reinforcement value of peer interactions, and their expectancies for initiating such interactions. The limitations of these findings due to the small sample size and the lack of a control group are discussed. The value of multimethod measurement systems are also discussed.

Child↗
PubMed1991

The influence of parenting stress and child behavior problems on parental estimates of expressive and receptive language development.

This study examined the relative contributions of parental reports of child-related and parent-related stress to their estimates of expressive and receptive language skills on the Minnesota Child Development Inventory in 152 children referred to a communicative disorders clinic. Correlational analysis showed moderately high agreement between parental estimates and objective measures of expressive and receptive language ability. Multiple-regression analyses revealed that independent measures of language acquisition accounted for the greatest amount of variance in parental estimates of expressive and receptive language skills. Stressful child behavior accounted for a small but statistically significant proportion of the variance in estimates of expressive and receptive language skills. Parental stress, in contrast, did not contribute significantly to language estimates. These findings suggest that parents are able to formulate estimates of language development which are relatively independent of personal or child-related stressors.

Child↗
PubMed1997

Guillain-Barré syndrome in south-west Stockholm, 1973-1991, 2. Clinical epidemiology.

The clinicoepidemiological features of Guillain-Barré syndrome (GBS) were studied using population-based information from medical records of 69 patients in South-West Stockholm, during the period from January 1973 to June 1992. The diagnoses were validated according to the National Institute of Neurological and Communicative Disorders and Stroke criteria. Mean age at onset was 43 +/- 20 years. For 46 patients, events during the 30-day period preceding clinical onset, 74% of them identified as respiratory infections, were recorded. The presence of preceding events was associated with male gender. A more rapid clinical progression was found among women. A CSF/serum albumin ratio > or = 10, denervation potentials and mechanical ventilation required were associated with poor recovery or long duration of hospitalization. Reduced motor conduction velocity was more common and pronounced among older patients and with a high CSF/serum albumin ratio. These results suggest that there is clinical, electrophysiological and epidemiological heterogeneity in GBS, and that clinicoepidemiological subgroups of GBS may exist.

Adolescent↗
PubMed2003

Decreased striatal D2 receptor density associated with severe behavioral abnormality in Alzheimer's disease.

OBJECTIVES: Since patients manifesting behavioral and psychological symptoms of dementia (BPSD) are a burden for their families and caregivers, the underlying neurobiological mechanism of this condition should be clarified. Using positron emission tomography (PET), we previously reported that wandering behavior in dementia was associated with a disturbed dopaminergic neuron system. We herein investigated the relationship between the severity of BPSD and the striatal D2 receptor density in Alzheimer's disease (AD). METHODS: Ten patients with probable AD as per the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) and the AD and Related Disorders Association (ADRDA) criteria and five normal subjects were examined with PET. The tracer used was [11C]raclopride (D2 antagonist). The uptake of [11C] raclopride was calculated as the estimation of binding potential (BP) of the striatum to the cerebellum. The AD patients were institutionalized in multiple nursing homes, and their BPSD were evaluated by the Behavioral Pathology in AD Frequency Weighted Severity Scale (BEHAVE-AD-FW) scale (Reisberg). RESULTS: There was a significant inverse Spearman's correlation between BEHAVE-AD-FW score and the BP, especially between the score of the behavioral domain and the BP values. The BP was found to be lower in severer BPSD patients. CONCLUSIONS: Patients with AD who manifest severe BPSD may have some dysfunction of striatal dopamine metabolism compared with those without BPSD.

Aged↗
PubMed2004

Diagnosis of suspected Alzheimer's disease is improved by automated analysis of regional cerebral blood flow.

PURPOSE: Accurate diagnosis of Alzheimer's disease (AD), the most common form of dementia, remains difficult. In order to assess whether fully automated stereotactic surface projection (3D-SSP) presentation contributes to the diagnosis of AD by single-photon emission computed tomography (SPECT), we investigated the diagnostic accuracy of transaxial display with and without 3D-SSP analysis as well as the correlation between cerebral perfusion in different cortical areas and the mini mental score (MMS). METHODS: Seventy-two patients referred because of cognitive impairment were included in the study. According to the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) and the Alzheimer's disease and Related Disorders Association (ADRDA) criteria, 27 patients were diagnosed as having probable AD while 45 were classified as non-AD patients. 3D-SSP was used to quantify the regional cerebral blood flow (rCBF) acquired from SPECT imaging. RESULTS: Compared with the transaxial section presentation alone, 3D-SSP presentation improved the area under the receiver operating curve (p<0.05) as well as intra-observer (k=0.73 vs 0.88) and inter-observer (k=0.50 vs 0.84) reproducibility. Upon normalisation of regional to thalamic activity, multiple regression analysis revealed a strong correlation between the MMS and rCBF in the right parietal cortex (p=0.002). CONCLUSION: Addition of 3D-SSP to the transaxial section display of ECD-SPECT studies improves the reproducibility and the diagnostic performance in respect of AD in patients with cognitive impairment and provides a valid tool for assessment of the severity of cortical perfusion abnormalities in such patients.

Adult↗
PubMed1999

The frequency of clinical variants of Guillain-Barré syndrome in Ferrara, Italy.

As the available diagnostic criteria (National Institute of Neurological and Communicative Disorders and Stroke, NINCDS) for Guillain-Barré syndrome (GBS) do not permit inclusion of clinical variants (CV) of GBS, there are few data on their occurrence and few reports of the overall incidence of the disease. A population-based study in the local health district of Ferrara, Italy in 1981-1993 selected cases fulfilling both NINCDS criteria (NINCDS GBS cases) and CV. The incidence of CV was 0.35 per 100,000 person-years (95% CI: 0.15-0.68), 0.32 when age-adjusted to the Italian population. No difference was found between CV and NINCDS GBS for male/female ratio, mean age at onset, elevated CSF protein content, seasonal pattern, or mean time delay from first neurological symptom to maximal severity. A higher frequency of antecedent infections for CV and more frequent serious disease at the nadir time for NINCDS GBS were found. A complete recovery was more frequent for CV than NINCDS GBS, but no difference was found regarding good outcome (defined by a satisfactory recovery and resumption of normal functional life). Since most findings were similar for NINCDS GBS and CV cases, they may have similar underlying pathological mechanisms. When diagnostic criteria for GBS include CV, the overall disease incidence in the Ferrara district increases from 1.87 to 2.21 cases per 100,000 person-years (the contribution of CV to the overall incidence of GBS is 15.7%). The currently available diagnostic criteria for GBS, although useful for field studies, may be too restrictive as they can entail the loss of about 15% of cases.

Adolescent↗
PubMed2002

Non-specific markers of neurodevelopmental disorder/delay in selective mutism--a case-control study.

Selective mutism (SM) in children is frequently associated with language disorder/delay suggesting that neurobiological factors may be involved in the development of SM. Motor co-ordination problems, reduced optimality pre- and perinatally and minor physical anomalies represent other markers for neurodevelopmental disorder/delay. The present study explores these markers in referred children with SM (n=54), non-referred matched controls (n =108) and in SM subgroups with and without a communication disorder (CoD). Children with SM differed significantly from controls in parent-reported motor developmental delay, and they obtained a higher pre- and perinatal "reduced optimality score". They scored significantly lower on a motor performance test and showed a higher frequency of minor physical anomalies compared with controls. There were no differences in these respects between SM and CoD compared with SM without CoD. The results confirm that neurobiological factors may be involved in the development of SM. Accordingly, the clinical assessment of referred children with SM should include a thorough history of motor development and an evaluation of present motor skills. The interplay of shyness/social anxiety and motor function problems has to be addressed to reduce misunderstandings of the child's behaviour and to adjust demands for motor skills to the actual level of the child.

Adolescent↗
PubMed2006

A parent-report instrument for identifying one-year-olds at risk for an eventual diagnosis of autism: the first year inventory.

A parent-report instrument, the First Year Inventory (FYI), was developed to assess behaviors in 12-month-old infants that suggest risk for an eventual diagnosis of autism. The target behaviors were identified from retrospective and prospective studies. FYIs were mailed to 5,941 families and 25% (N = 1,496) were returned, with higher return rates for white families and for families with greater educational attainment. Ad hoc groups of questions afforded measurement of eight specific constructs, which were combined to establish a general risk index. Boys had higher risk scores than did girls. Maternal race and education influenced answers. A small percentage of infants appeared to be at notably elevated risk. Large-scale longitudinal research is warranted to determine whether the FYI can predict an eventual diagnosis of autism.

Autistic Disorder↗
PubMed2005

An instrument to assess mental patients' capacity to appraise and report subjective quality of life.

Quality of life (QOL) is increasingly recognized as an important outcome measure in treatment studies and service evaluation. However, patients or service users may sometimes lack the capacity to either evaluate or express their subjective QOL, for example due to cognitive impairment, communication disorders, symptom distress or burden of completing the assessment itself. This paper describes the development of an instrument, the capacity to report subjective quality of life inventory (CapQOL), which evaluates the ability of patients to appraise their subjective QOL and to complete related measures. The CapQOL is a simple and brief screening tool, designed for use in people with a wide range of mental disabilities. It helps researchers to identify individuals who are unable to appraise or report their subjective quality of life. We administered the CapQOL to 442 patients with early psychosis. About 89% of the participants were assessed to be able to complete a subjective QOL measure. The CapQOL demonstrated satisfactory psychometric properties. Further validation studies in people with psychosis as well as other mental disabilities are indicated.

Adult↗
PubMed1993

Cognitive-linguistic assessment of individuals with multiple sclerosis.

Four individuals diagnosed as having multiple sclerosis (MS) and four matched controls were assessed with the Arizona Battery for Communication Disorders (ABCD), a standardized battery of memory and cognitive-linguistic tasks. Subjects with MS and controls performed in an equivalent or nearly equivalent fashion for nine of the ABCD subtests. For five of the subtests (Object Description, Generative Naming, Concept Definition, Generative Writing, and Picture Description), subjects with MS performed substantially lower than the control subjects. Results of this pilot study suggest that portions of the ABCD may be useful in identifying profiles of memory, and cognitive-linguistic impairment among individuals with multiple sclerosis. Further research using a larger sampling of individuals with MS is needed to more fully assess the usefulness of the ABCD with this population.

Adult↗
PubMed1995

Caregiver time use: an outcome measure in clinical trial research on Alzheimer's disease.

OBJECTIVE: To assess whether unpaid caregiver time and paid professional time increase as cognitive impairment associated with Alzheimer's disease increases and to evaluate the utility of caregiver time as an additional outcome measure in clinical trial research of Alzheimer's disease. METHODS: This was a 24-week, double-blind, multicenter, parallel-group, placebo-controlled study conducted at 17 clinical outpatient sites by Hoechst-Roussel Pharmaceuticals Inc. A total of 449 patients older than 40 years with probable Alzheimer's disease of mild to moderate severity (criteria of the National Institute for Neurological and Communicative Disorders and Stroke--Alzheimer's Disease and Related Disorders Association) entered the study, and 284 completed both baseline and week 24 data collection. A total of 160 caregivers completed time allocation surveys at baseline and at 24 weeks. Patients with Alzheimer's disease received 150 mg/day and 225 mg/day Velnacrine maleate (parallel-group treatment) and placebo. Cognitive function was measured with use of cognitive and noncognitive subscales of the Alzheimer's Disease Assessment Scale (ADAS). Unpaid caregiver and paid professional time use were measured with use of the Caregiver Activities Time Survey (CATS). RESULTS: Unpaid caregiver time per day increased significantly with cognitive impairment at baseline as measured by the ADAS cognitive and noncognitive components. Velnacrine therapy significantly improved cognitive function relative to placebo, and this was associated with decreased unpaid caregiving time at trend levels. Specifically, caregivers of patients in the high-dose velnacrine group (225 mg/day) experienced a partial release from their time involvements, especially in the area of patient supervision, by an average of 3.3 hours per day. CONCLUSIONS: To our knowledge, this study represents the first time that the ADAS has been linked to a caregiver outcome. Results suggest that unpaid caregiver time allocation is sensitive to changes in cognitive function and therefore may be useful as an additional outcome measure in clinical trials of pharmaceutical interventions for Alzheimer's disease.

Aged↗
PubMed1980

Case report: the automatic message box as therapeutic aid.

The broad scope of this journal has always included an interest in prosthetic aids for the alleviation of communication disorders. Our recent focus on the field of biological psychiatry adds an intriguing new dimension to this interest. The following case report highlights the relative neglect of some of our most prosaic technology as a valid aspect of "prosthetics."

Adult↗