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Practice parameter: diagnosis of dementia (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology.

OBJECTIVE: To update the 1994 practice parameter for the diagnosis of dementia in the elderly. BACKGROUND: The AAN previously published a practice parameter on dementia in 1994. New research and clinical developments warrant an update of some aspects of diagnosis. METHODS: Studies published in English from 1985 through 1999 were identified that addressed four questions: 1) Are the current criteria for the diagnosis of dementia reliable? 2) Are the current diagnostic criteria able to establish a diagnosis for the prevalent dementias in the elderly? 3) Do laboratory tests improve the accuracy of the clinical diagnosis of dementing illness? 4) What comorbidities should be evaluated in elderly patients undergoing an initial assessment for dementia? RECOMMENDATIONS: Based on evidence in the literature, the following recommendations are made. 1) The DSM-III-R definition of dementia is reliable and should be used (Guideline). 2) The National Institute of Neurologic, Communicative Disorders and Stroke--AD and Related Disorders Association (NINCDS-ADRDA) or the Diagnostic and Statistical Manual, 3rd edition, revised (DSM-IIIR) diagnostic criteria for AD and clinical criteria for Creutzfeldt--Jakob disease (CJD) have sufficient reliability and validity and should be used (Guideline). Diagnostic criteria for vascular dementia, dementia with Lewy bodies, and frontotemporal dementia may be of use in clinical practice (Option) but have imperfect reliability and validity. 3) Structural neuroimaging with either a noncontrast CT or MR scan in the initial evaluation of patients with dementia is appropriate. Because of insufficient data on validity, no other imaging procedure is recommended (Guideline). There are currently no genetic markers recommended for routine diagnostic purposes (Guideline). The CSF 14-3-3 protein is useful for confirming or rejecting the diagnosis of CJD (Guideline). 4) Screening for depression, B(12) deficiency, and hypothyroidism should be performed (Guideline). Screening for syphilis in patients with dementia is not justified unless clinical suspicion for neurosyphilis is present (Guideline). CONCLUSIONS: Diagnostic criteria for dementia have improved since the 1994 practice parameter. Further research is needed to improve clinical definitions of dementia and its subtypes, as well as to determine the utility of various instruments of neuroimaging, biomarkers, and genetic testing in increasing diagnostic accuracy.

Dementia↗

A 1-year, placebo-controlled preservation of function survival study of donepezil in AD patients.

OBJECTIVE: To examine the effects of donepezil compared with placebo on the preservation of function in patients with AD over a 1-year period. METHODS: This was a prospective, 54-week, double-blind, placebo-controlled, survival to endpoint study. Patients were required to have at entry: a diagnosis of probable AD (National Institute of Neurological and Communicative Disorders and Stroke criteria); Mini-Mental State Examination score of 12 to 20; Clinical Dementia Rating of 1 or 2; modified Hachinski ischemia score < or =4; and capability of performing 8 of 10 instrumental activities of daily living and 5 of 6 basic activities of daily living. Patients (n = 431) were randomized to placebo or donepezil (5 mg/day for 28 days, 10 mg/day thereafter). Outcome measures were the AD Functional Assessment and Change Scale, the Mini-Mental State Examination, and Clinical Dementia Rating scale. At each visit, investigators determined whether predefined criteria for clinically evident decline in functional status had been met. Patients who met the endpoint criteria were discontinued per protocol. RESULTS: Donepezil extended the median time to clinically evident functional decline by 5 months versus placebo. The probability of patients treated with donepezil remaining in the study with no clinically evident functional loss was 51% at 48 weeks, compared with 35% for placebo. The Kaplan-Meier survival curves for the two treatment groups were different (p = 0.002, log-rank test). CONCLUSIONS: Patients with AD continue to show detectable disease progression over time, but treatment with donepezil for 1 year was associated with a 38% reduction in the risk of functional decline compared with placebo.

Aged↗

CSF filtration is an effective treatment of Guillain-Barré syndrome: a randomized clinical trial.

OBJECTIVE: To compare CSF filtration (CSFF) and plasma exchange (PE) in the treatment of patients with Guillain-Barré syndrome (GBS). METHODS: In a prospective controlled clinical trial, 37 patients with acute GBS were randomized to receive either CSFF or PE. Inclusion criteria were fulfillment of National Institute of Neurological and Communicative Disorders and Stroke criteria and disability to walk >5 m unassisted. RESULTS: With similar baseline features in both groups (initial disability grades on the six-point grading scale of the GBS Study Group) the primary outcome variable (improvement within 28 days after randomization) was almost identical (test for equivalence p = 0.0014), the mean grade values being 0.82 in the CSFF group and 0.80 in the PE group. After 56 days, 56% (9 of 16 patients) of the CSFF group and 37% (7 of 19 patients) of the PE group had reached grade 2 (i.e., ability of unassisted walking >5 m). After 6 months, the probability to reach grade 2 was about 80% in both groups. In the CSFF group, transient pleocytosis occurred without apparent clinical complications. Clinically relevant complications were higher in the PE-treated group. CONCLUSIONS: Although the number of patients was small, the authors found that the treatment of GBS with CSFF is at least as effective as with PE. CSFF might work by removing from the CSF inflammatory mediators, autoantibodies, or other factors.

Adult↗

Apolipoprotein E epsilon 4 and the pattern of regional brain atrophy in Alzheimer's disease.

BACKGROUND: Although the APOE epsilon 4 allele increases the risk of developing AD, the effects of the epsilon 4 allele on brain atrophy in clinical AD patients are controversial. OBJECTIVE: To investigate a possible relationship between the genetic variants of APOE and brain atrophy in patients with AD. METHODS: Using MRI-based volumetry techniques, the authors compared the volumes of the hippocampal formation, amygdaloid complex, and whole brain in probable AD patients (based on criteria of the National Institute for Neurological and Communicative Disorders and Stroke/Alzheimer's Disease and Related Disorders Association) with different APOE alleles. One group (n = 46) had the epsilon 3/3 allele, one group (n = 46) had the epsilon 3/4 allele, and one group (n = 46) had the epsilon 4/4 allele. The three groups were matched for age, sex, disease duration, education level, and severity of dementia represented by their score of the Mini-Mental State Examination. A possible difference in pattern of cognitive deficits with dose of the APOE epsilon 4 allele was also examined. RESULTS: The normalized hippocampal volume was correlated with the number of APOE epsilon 4 alleles (r = -0.285, p = 0.0007). The amygdalar volume was also correlated with the number of APOE epsilon 4 alleles (r = -0.178, p = 0.037). The number of APOE epsilon 4 alleles was positively correlated with the whole-brain volume (r = 0.185, p = 0.030). It was also correlated with Wechsler Adult Intelligence Scale-Revised performance IQ (r = 0.203, p = 0.017) and with Wechsler Memory Scale-Revised attention/concentration score (r = 0.191, p = 0.025). CONCLUSIONS: Different patterns of regional brain atrophy were found among patients of different APOE genotypes. The effect of APOE epsilon 4 allele on the brains of AD patients may have regional specificity.

Aged↗

Controlled trial of N-acetylcysteine for patients with probable Alzheimer's disease.

The antioxidant N-acetylcysteine (NAC) or placebo was administered in a double-blind fashion to patients who met National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria for probable AD. Testing for efficacy occurred after 3 and 6 months of treatment. Comparison of interval change favored NAC treatment on nearly every outcome measure, although significant differences were obtained only for a subset of cognitive tasks.

Acetylcysteine↗

Infections and course of disease in mild forms of Guillain-Barré syndrome.

OBJECTIVE: Twenty-eight percent of patients with the Guillain-Barré syndrome remain able to walk unaided. Studying patients with the mild form of Guillain-Barré syndrome can further contribute to knowledge of the spectrum of the syndrome and explore whether this subgroup may need treatment with IV immunoglobulin. METHODS: Patients fulfilling the National Institute of Neurologic and Communicative Disorders and Stroke criteria for Guillain--Barré syndrome were included in a nationwide survey over a 2-year period. Clinical characteristics and serum samples were collected prospectively. In addition, a questionnaire was completed concerning the course and outcome of the disease. RESULTS: A total of 139 patients were included. Nineteen of the patients (14%) included were mildly affected, and 120 (86%) were severely affected. Infections with Epstein-Barr virus were found more frequently in mildly affected patients (p = 0.02). Antiganglioside antibodies were less frequently found in the mildly affected patients (p = 0.03). The degree of severity of the disease between mildly and severely affected patients was different on the day of admission (p < 0.01). Thereafter, the groups showed a remarkably similar rate of progression. Thirty-eight percent of mildly affected patients report problems in hand function and an inability to run at 3 and 6 months (all women, p = 0.02). CONCLUSION: The difference in severity of Guillain--Barré syndrome seems to be determined in an early phase of the disease. Preceding infections and antiganglioside antibodies may influence the initial immune attack, determining the severity of the disease. The presence of residual signs in patients with mild disease may advocate the use of early treatment in mildly affected patients.

Bacterial Infections↗

Diagnostic utility of abbreviated fluency measures in Alzheimer disease and vascular dementia.

BACKGROUND: Several studies indicate semantic fluency more sensitively discriminates patients with Alzheimer disease (AD) from normal elderly persons, with disproportionate impairment of semantic over phonemic fluency. OBJECTIVE: To determine the ability of abbreviated fluency measures in the clinic setting (1-minute letter F and animal fluency tests) to detect AD, and to assess whether difference scores between these measures discriminate patients with AD and vascular dementia (VaD) from normal elderly persons. METHODS: The authors studied patients with AD (n = 98) meeting National Institute of Neurological Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria, VaD patients (n = 18) meeting National Institute of Neurological Disorders and Stroke-Association Internationale pour la Recherche et l'Enseignement en Neurosciences criteria, cognitively impaired but not demented patients (CIND; n = 25), vascular CIND patients (VCIND; n = 24), and normal control subjects (NCs; n = 46). RESULTS: Analysis of covariance controlling for age, education, and overall impairment indicated all groups generated fewer animal names compared with NCs, whereas only VaD patients generated fewer letter F words compared with NCs. On standardized scores, patients with AD and CIND, unlike those with VCIND and VaD, scored significantly worse on the animal fluency test than on the letter F fluency test. The animal fluency test was superior in discriminating all patient groups from NCs. Positive likelihood ratios (PLRs) revealed animal fluency scores <15 were 20 times more likely in a patient with AD than in an NC (sensitivity = 0.88; specificity = 0.96). Letter F scores <4 discriminated VaD from AD patients (PLR = 4.0; sensitivity = 0.44; specificity = 0.90). Difference scores <0 (i.e., fewer animal than letter F words) discriminated patients with VCIND from those with CIND (PLR = 2.5; sensitivity = 0.32; specificity = 1.00). CONCLUSIONS: A 1-minute semantic fluency test can assist in early detection of dementia in the memory clinic setting.

Aged↗

Telehealth in speech pathology: applications to the treatment of stuttering.

The potential value of telecommunications systems in treating communication disorders is being increasingly recognized. This mode of service delivery shows particular promise in giving patients access to specialist services for problems like stuttering, which are difficult to manage and often require long-term follow-up support. The Institute for Stuttering Treatment and Research at the University of Alberta has used videoconferencing to provide follow-up support to geographically remote adults who have undergone intensive treatment on site.

Alberta↗

Low-bandwidth, Internet-based videoconferencing for physical rehabilitation consultations.

Low-bandwidth, Internet-based videoconferencing was used to provide physical rehabilitation consultation services for eight community hospitals. Videoconferencing and file transmission used a PC and modem. A separate telephone line was used for voice. Over 21 months, 47 physical rehabilitation consultations were completed for communication disorders, foot care, gait problems, orthotics, prosthetics, arm weakness and wheelchair prescription. Consultations were approximately 40 min long. Clinician questionnaires were completed by 47 individuals. While more than 80% of the questionnaire responses supported the telemedicine approach, remote clinicians rated their satisfaction higher than did the specialists. Client questionnaires were completed by 24 individuals (a response rate of 51%). All clients were comfortable with and had confidence in the teleconsultations.

Attitude of Health Personnel↗

Research into telehealth applications in speech-language pathology.

A literature review was conducted to investigate the extent to which telehealth has been researched within the domain of speech-language pathology and the outcomes of this research. A total of 13 studies were identified. Three early studies demonstrated that telehealth was feasible, although there was no discussion of the cost-effectiveness of this process in terms of patient outcomes. The majority of the subsequent studies indicated positive or encouraging outcomes resulting from telehealth. However, there were a number of shortcomings in the research, including a lack of cost benefit information, failure to evaluate the technology itself, an absence of studies of the educational and informational aspects of telehealth in relation to speech-language pathology, and the use of telehealth in a limited range of communication disorders. Future research into the application of telehealth to speech-language pathology services must adopt a scientific approach, and have a well defined development and evaluation framework that addresses the effectiveness of the technique, patient outcomes and satisfaction, and the cost benefit relationship.

Attitude of Health Personnel↗

Serial brain MRI at 3-6 month intervals as a surrogate marker for Alzheimer's disease.

A surrogate marker is needed for Alzheimer's disease (AD) both to aid diagnosis and to assess interventions. Despite widespread use, brain imaging markers have largely been confounded by overlap with "normal" ageing. 39 elderly subjects completed up to four serial volumetric brain MRI scans with intervals from 2.5 months to 7 months. By National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria, five subjects had probable AD, two possible AD and 32 were negative for AD, although this group included memory-impaired subjects. Total brain and ventricular volumes were measured for each scan, and rates of change for each interval calculated. The rate of change in ventricle-to-brain ratio was 15.6% per year (standard deviation (SD) 2.8%) for probable AD compared with 4.3% per year (SD 1.1%) for negative AD (p<0.001). There was no significant difference between these groups' mean ventricle-to-brain ratios measured at a single time point (p=0.25). Rates of change in brain or ventricular volume over time also differed between the two groups (p<0.001). Power calculations reveal that to detect a 20% reduction in the excess rate of atrophy of a probable AD cohort in just 6 months, with 90% power, 135 subjects would be required in each arm of a randomized placebo controlled trial. For a 30% reduction in the excess rate of atrophy, 61 subjects would be required. Rate of change analysis makes serial brain MRI a valuable surrogate marker for Alzheimer's disease. Since only 6 months or less is required between scans, this procedure has both clinical relevance and potential for monitoring interventions.

Aged↗

Transnasal, transeptal sphenoidal approach to hypophysectomy.

Since 1960 the most useful and widely employed technique for transphenoidal hypophysectomy has generally been via the sublabial, transeptal approach. This procedure has offered excellent exposure, limited blood loss and a direct, midline approach through the sphenoid to the hypophysis, thus preserving certain midline structures as a guide to the appropriate point of entry into the sella turcica. This approach has certain drawbacks, however, not the least of which is the extensive sublabial incision that must be made and the need to remove or otherwise interfere with the nasal spine. This maneuver can result in changes in the vertical dimension of the septal-collumellar area and, more important, often results in permanent numbness of the upper teeth. In patients who wear dentures, it will sometimes produce a bothersome scar which will require refitting or change in wearing patterns for these dentures. It also requires surgical closure. For the past two years at the Cleveland Clinic Foundation a modified transeptal, transphenoidal approach to hypophysectomy has been undertaken conjointly between the Department of Otolaryngology and Communicative Disorders and the Department of Neurosurgery. This approach to hypophysectomy may offer a good alternative to the sublabial approach, and in competent hands avoids some of the complications of the latter procedure.

Humans↗

Research in otolaryngology: NIH support and other considerations.

The National Institutes of Health continue to provide support for a major portion of biomedical research in this country. Research serves as the keystone to the continued health of Otolaryngology, but there are clear indications that otolaryngologists' direct involvement in research is diminishing at a dangerous pace. Some of the causes of this problem are identified. Solutions include, first and foremost, more general recognition of the importance to the speciality of biomedical research; currently available mechanisms offered by the National Institute of Neurological and Communicative Disorders and Stroke for the support of investigator training might be utilized more extensively and collaborative research between clinician and basic science investigators should be encouraged.

Academic Medical Centers↗

Primary squamous cell carcinoma of the parotid gland.

Primary squamous cell carcinoma of the parotid gland is an uncommon malignancy. It can be diagnosed only after squamous cell carcinoma metastatic to the parotid gland has been excluded. Histologic evaluation must differentiate primary squamous cell carcinoma from high-grade mucoepidermoid carcinoma or adenocarcinoma. Retrospective review of parotid gland neoplasms seen in the Department of Otolaryngology and Communicative Disorders between 1972 and 1987 identified eight cases for an incidence of 1.8%. The majority of these patients had advanced disease and were treated with both surgery and radiation therapy. Fifty percent of the cases demonstrated no evidence of disease at an average follow-up of 29 months.

Adult↗

Behavioral and emotional disturbance in individuals with Williams syndrome.

Behavioral and emotional disturbance was assessed in 70 children and adolescents with Williams syndrome. They were compared with an epidemiological control population, which was statistically controlled for age, gender, and level of mental retardation. Those with Williams syndrome were more likely to be diagnosed as suffering psychiatric disorder. The disorder was characterized by anxiety, hyperactivity, preoccupations, and inappropriate interpersonal relating. Significantly increased rates of other individual symptoms were also found, including sleep disturbance and hyperacusis. These results, considered with earlier findings, suggest that there is a valid behavior phenotype of Williams syndrome. This is frequently associated with sufficient impairment to consider inclusion of the behavior phenotype in future official taxonomies of mental disorders.

Analysis of Variance↗

Emotion recognition by children with Down syndrome: investigation of specific impairments and error patterns.

The ability of children with Down syndrome to recognize expressions of emotion was compared to performance in typically developing and nonspecific intellectual disability groups matched on either MA or a performance-related measure. Our goal was to (a) resolve whether specific emotions present recognition difficulties; (b) investigate patterns of errors; and (c) explore the relationships among emotion-recognition ability and cognitive, linguistic, and adaptive behavior levels. Emotion-recognition ability in the Down syndrome group was significantly poorer than in the typically developing group overall, particularly for fearful expressions. Error patterns and relationships between task performance and assessment measures also differed across groups. Findings are consistent with a neurological explanation of specific deficits in sociocognitive functioning in children with Down syndrome.

Adolescent↗

The development and analysis of interactive videodisc technology to teach speechreading.

Members of the Department of Communication Disorders and Special Education and the Institute for Interactive Technologies at Bloomsburg University developed a computer-based interactive videodisc instructional program to teach speechreading skills. Speechreading Challenges on Videodisc provides users with practice in speechreading words, sentences, and stories presented by over 150 people in a user friendly environment. Unlike traditional videotapes, videodisc technology provides immediate feedback to the user. To determine the program's effectiveness in teaching speechreading, a comprehensive evaluation of the program was undertaken. Seventy-four college students participated in a study using the program to learn speechreading. Results indicated a statistically significant improvement in speechreading ability from pretest to posttest measures on both the videodisc program evaluation as well as on the Costello Test of Speechreading.

Deafness↗

Twins early development study (TEDS): a multivariate, longitudinal genetic investigation of language, cognition and behavior problems in childhood.

The Twins Early Development Study (TEDS) focuses on the early development of the three most common psychological problems in childhood: communication disorders, mild mental impairment and behavior problems. The TEDS twins were assessed longitudinally at 2, 3, 4 and 7 years of age in order to investigate genetic and environmental contributions to change and continuity in language and cognitive development; it is multivariate in order to examine the origins of comorbidity; and it uses a large sample in order to study abnormal development in the context of normal development. The twins were identified from birth records of twins born in the UK in 1994-96. More than 15,000 pairs of twins have been enrolled in TEDS and the participating families are representative of the UK. The measures at 2, 3 and 4 years are administered by parents. At 7 years, children are assessed for language and cognitive development using telephone testing, parents and children are interviewed about behavior problems, and teachers also assess behavior problems as well as academic achievement. One set of findings is that the same genes largely contribute to both language and cognitive problems and the same genes affect normal and abnormal development, a result that suggests that general impairment may be a better target for genetic research than specific language impairment independent of nonverbal cognitive problems. DNA has been obtained so far for more than 4000 pairs and is being used initially in molecular genetic studies of language problems and hyperactivity.

Birth Certificates↗