Number processing and acalculia in dementia

In this review, studies on number processing and calculation in demented patients are summarized. Single cases, group studies and our own examinations all show that acalculia is a frequent symptom of dementia and correlates with severity of dementia. While calculation and numeral transcoding deficits are often prominent in early courses of the disease, deficits in semantic processing and basic number processing are less severe. Still, single cases demonstrate that very specific dysfunctions in number processing and calculation can occur in dementia. In number transcoding, a characteristic error type occurs in demented patients that has not (or rarely) been described in other brain damaged people so far. In these "shift errors" or "intrusion errors" the wrong number code is used or the number codes are mixed (e.g. patients write "2hindert11" instead of zweihundertelf (two hundred eleven)). Due to the frequent occurence of acalculia in dementia and its specific features, it is suggested that acalculia should be integrated into the criteria for dementia and that number processing and calculation tests are used in the diagnosis of dementia.     

Impact of adult age and Alzheimer's disease on levels of neural noise for letter matching.

We tested healthy young and older adults as well as higher-scoring (Mini-Mental State Exam, MMSE, scores between 14-20) and lower-scoring patients diagnosed with probable Alzheimer's disease (AD) on a letter-matching task. Subjects were instructed to respond "same" if two simultaneously presented letters were identical, or "different" if the letters did not match. Healthy older adults showed a larger "fast-same" effect than healthy young adults. Also, higher-scoring AD patients showed a large "false-different" effect for errors, but lower-scoring AD patients showed a large "false-same" effect. These data indicate that older adults exhibit higher neural noise levels than younger adults. The cross-over error pattern for AD suggests that moderately demented AD patients show evidence of forming degraded visual percepts whereas more severely demented AD patients show evidence of forming incomplete percepts.     

Perceptual attention in aging and dementia measured by Gottschaldt's Hidden Figure Test

Gottschaldt's Hidden Figure Test (GHFT) calls for figure-ground discrimination abilities. The aim of this study was to provide a perceptual attention test for the cognitive assessment of demented patients. In Experiment 1, GHFT normal baselines on 190 healthy controls are achieved, and age-education score correction as well as transformation of the original scores into "Equivalent Scores" is established. On the inferential percentile allocation of 45 mildly-moderately deteriorated DAT patients, 65% of them performed in the critically low range of the score distribution. In Experiment 2, the discriminant power of GHFT between DAT patients and healthy controls matched by age and Experiment 2, the discriminant power of GHFT between DAT patients and healthy controls matched by age and education is assessed. Overall correct classification reached 90%. This experiment shows that GHFT in demented patients works chiefly as an attentional task. The discussion is based largely on methodological and statistical issues.     

Patterns of neuropsychological impairment in dementia of the Alzheimer type and multi-infarct dementia

Patterns of performance on 22 neuropsychological tests were examined in 58 patients with presumed dementia of the Alzheimer type (DAT) and 58 patients with presumed multi-infarct dementia (MID). Few differences between patterns of performance in DAT and MID were found when overall level of performance was taken into account. Within the DAT group, there were some age-related differences in performance that in themselves might support other studies suggesting clinical and neuropathological differences between subtypes of DAT that are related in part to the age at which the condition occurs. However, similar age-related patterns were found in the MID group and the DAT and MID groups did not differ significantly in this respect. Considerable inter-individual variability in pattern of performance was apparent within each group. A proportion of patients were re-assessed 10 months after initial assessment: the extent of decline over time was not predictable from patients' characteristics or test performances at initial assessment. 'De-differentiation' in patterns of impairment, over time or with increasing severity of impairment, was not observed.     

Dementia of the Alzheimer's type and depression

This retrospective review of medical records was designed to address three questions: 1) Can the depression seen in some patients with Dementia of the Alzheimer's Type (DAT) be successfully treated? 2) Does this treatment lead to any long-term improvement in the patient's cognitive status? and 3) Do patients with coexisting DAT and depression have a different long-term clinical course than nondepressed DAT patients? In the authors' sample of 131 DAT subjects, 41 (31%) also met DMS-III criteria for a major affective disorder. Of those DAT plus depression patients whose records reflected treatment (usually with a tricyclic antidepressant), 85% (17 of 20) showed clear evidence of improvement in mood, vegetative signs, or activities of daily living (ADLs) based on review of the medical record. An analysis of change in cognitive function (measured by the Folstein Mini-Mental State) and five global measures failed to reveal any differences between the depressed and nondepressed groups after a mean interval of 17 months. The depression that occurs in approximately one-quarter to one-third of DAT patients appears to respond to appropriate therapy. These patients often show improvement in their mood and ADLs but remain demented.     

HIV-associated Hodgkin's disease

Hodgkin's disease (HD) appears to occur more frequently in people with HIV and within this population HD demonstrates a number of differences. In people with HIV, Epstein-Barr virus (EBV) is found more frequently in Reed-Sternberg cells and mixed cellularity and lymphocyte depleted histological subtypes predominate. Patients with HIV present with more advanced stage HD often with extranodal disease. The prognostic variables for HD in patients with HIV relate to the HIV rather than the HD. In people with HIV the response rates are lower, the relapse rates higher, the infectious complications more frequent and the overall survival worse than for HD in the general population.     

Revised versions of the Childhood Health Assessment Questionnaire (CHAQ) are more sensitive and suffer less from a ceiling effect

OBJECTIVE: To determine the relative discriminant validity of 3 new versions of the Childhood Health Assessment Questionnaire (CHAQ) and to determine the relative concordance between children and their parents. METHODS: The parents of 48 children with musculoskeletal disability and 101 nondisabled controls were given the CHAQ and the 3 revised versions in random order. Children older than 5 years also completed the questionnaires. RESULTS: All 3 new versions of the CHAQ were more sensitive at differentiating patients from controls (relative efficiency 1.35-1.65); the versions with 8 added items were even more sensitive (relative efficiency 1.79-2.32). The new versions of the CHAQ suffered less from a ceiling effect and were more normally distributed. Concordance between children and parents was moderate to high for all versions. CONCLUSION: Researchers and clinicians should consider using a revision of the CHAQ. By being more sensitive at differentiating patients, the revised versions of the CHAQ will allow fewer subjects to be studied and will be more able to detect differences between patients in the clinic.     

Vascular dementia versus dementia of Alzheimer's type: do they have differential effects on caregivers' burden?

OBJECTIVES: We investigated homecare patients with dementia of Alzheimer's type (DAT; n = 36) or vascular dementia (VD; n = 36) and their care-providing relatives regarding clinical and psychosocial variables to determine whether DAT and VD impose different burdens on caregivers. METHOD: All patients were diagnosed according to ICD-10 criteria. The diagnoses were confirmed by internal medical, clinical-neurological, and psychiatric parameters. The severity of the dementias was graded according to the Global Deterioration Scale (GDS). Caregiving relatives responded to the Behavioral Pathology in Alzheimer's Disease Rating Scale (BAD), the Blessed Dementia Scale (BDS), and the Screen for Caregiver Burden (SCB). RESULTS: Analyses revealed that caregivers' burden (SCB), disease symptoms and personality changes of patients (BAD), and the patients' inability to cope with everyday tasks (BDS) were sharply higher for DAT than for VD patients in the group with severe dementia. Concerning patients with mild or moderately severe disease, scores in the DAT group were similar or lower than those in the VD group. CONCLUSION: In early stages, VD patients impose a greater burden on relatives than do patients with DAT. In severe stages this relationship undergoes a reversal, with relatives of DAT patients experiencing the burden more adversely than those of VD patients. The differences in the onset and course characteristics, as well as the specific differences between these two types of dementia with respect to caregiver burden factors, call for their diagnostic separation and the development of specific homecare support systems for family caregivers.     

Nephelometry compared with differential antibody titre in routine rheumatoid factor measurements.

The comparative merits of agglutination techniques (differential agglutination titre (DAT)/latex slide test) and nephelometry for the routine measurements of IgM rheumatoid factors have been studied in this 2-part paper. The first part investigates the errors inherent in standard DAT measurements, and the second determines the rate of false positive results in an elderly nonrheumatoid population. It was found that the errors in agglutination techniques are due to differences in setting up the tests rather than in interpreting the results, and that the coefficient of variance was consistently around 20%. Approximately 1 in 5 of DAT results have such a high error as to be clinically valueless. The false positive rate in the elderly nonrheumatoid population was only 2% with nephelometry compared with 9% on the latex slide test. A previous study had demonstrated the advantages of nephelometry over DAT in routine rheumatological use, showing not only greater reproducibility but also a more accurate positive detection rate than the DAT in rheumatoid arthritis. The results also suggest that the traditional spectrum of rheumatoid factors of low titre detectable in the general population is probably an artefact inherent in agglutination techniques and that a definitive cut-off point is more likely. As nephelometers are generally available in biochemistry laboratories there is much to recommended their routine use for the measurement of IgM rheumatoid factors.     

Direct-antiglobulin-test-negative immune haemolytic anaemia and thrombocytopenia in a patient with Hodgkin's disease

A case of direct-antiglobulin-test (DAT)-negative auto-immune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP) associated with Hodgkin's disease (HD) is reported. A 52-year-old male was admitted with anaemia, thrombocytopenia, and lymphadenopathy. The patient was DAT negative, although he exhibited the clinical features of warm-type AIHA and elevated levels of red-blood-cell-associated IgG (RBC-IgG). The serum level of platelet-associated IgG (PA-IgG) was markedly increased. A biopsy specimen of the inguinal lymph nodes showed HD of mixed cellularity. Marked improvement of subjective symptoms, normalization of haematological values and a decrease in the level of both RBC- and PA-IgG were observed after the start of combination chemotherapy for HD. Although the association of HD, ITP, and/or AIHA has been infrequently reported, the measurement of RBC-IgG is recommended in cases of HD with anaemia even though DAT is negative, since HD is known to be associated with various protean immunological abnormalities.     

Are there clinical and epidemiological differences between familial and non-familial Alzheimer's disease?

To determine whether there are clinical and epidemiological differences between familial and non-familial Alzheimer's disease, we studied 151 patients with the clinical diagnosis of Dementia of the Alzheimer Type (DAT). Eighty-four index patients (56%) had at least one relative with reported DAT-type memory loss; the remaining 67 did not. For comparison, patients with a positive family history were grouped into a suspected familial DAT (FDAT), and those with a negative family history were grouped into a suspected non-familial DAT (NFDAT). There were no significant differences between FDAT and NFDAT patients with respect to age of symptom onset, survival time, rate of cognitive decline, motor function, behavioral signs or symptoms, head trauma, thyroid dysfunction, maternal age, or birth order. There were no significant differences between pedigrees of FDAT and NFDAT patients in respect to the prevalence of Down's syndrome or age of mortality. To examine a more "familial" set of FDAT patients, an FDAT subgroup (F2DAT) consisting of 33 index patients with two or more affected relatives with DAT-type memory loss was compared to the 67 NFDAT patients in a similar analysis. No significant differences were found. However, NFDAT index patients had a higher reported prevalence of head trauma than those patients in the F2DAT subgroup (NFDAT 22%, F2DAT 7%, OR = 4.2, CI = 0.9-20.0, P = 0.08). Thus severe head trauma may be a more important etiological factor in the non-familial form of DAT. To better examine this tendency, future studies should be conducted utilizing a larger number of pedigrees and familial criteria which account for the number of persons at risk and the age at onset of disease.     

End-of-life care of persons with dementia

Many clinicians with different training and practice are involved in the care of persons with dementia. Whereas neurologists and psychiatrists focus their attention on the early phase of dementia, geriatricians and palliative care specialists are particularly involved at the end of demented patients' lives. To summarize the progress of knowledge in this field, it seems possible to answer four fundamental questions. When? Several longitudinal studies of cohorts of demented and nondemented patients showed clearly that dementia is a risk factor for early death. There are no survival differences between Alzheimer's and Lewy body disease patients. Patients with vascular dementia have the worst prognosis. These results need to be analyzed with consideration of associated comorbidity, types and intensity of care, and dementia treatment. Why? Studies conducted on the basis of death certificates appear to be biased. A large autopsy study performed in the geriatric department of Geneva University Hospital showed no difference existed in immediate causes of death between demented and nondemented hospitalized old patients. On the other hand, cardiac causes are significantly more frequent in vascular dementia than in Alzheimer's disease or mixed dementia patients. How? Deaths of demented patients raise a lot of ethical considerations. It is always difficult to know demented patients' awareness of the end of life. It is really difficult to accompany these patients, with whom communication is essentially nonverbal. During this delicate phase of the end of life, how can formal health professionals help the family members who are afraid of both death and dementia? And after? Suffering of family members and caregivers has to be strongly considered. This goal includes the improvement of our communication skills with the patient, and the facilitation of interdisciplinary exchanges with the caregiver's team and with the family members to allow acceptance of the death.     

Signs of cardiac autonomic dysfunction during sleep in patients with Alzheimer's disease

Autonomic nervous system (ANS) failure may be an important complication in some neurological diseases. Conventional tests for the assessment of ANS functions during wakefulness cannot be used for many patients with dementia of the Alzheimer's type (DA) because of their poor cooperation. In 6 of 16 patients with presenile DAT we found signs of sympathetic cardiac dysfunction during sleep, measured as the decrease in body movement-related heart rate variation (Rbm). The study of Rbm during sleep seems to be a useful tool for ascertaining ANS function in DAT. The presence or absence of ANS dysfunction could be a more reliable criterion than age of onset for identifying subgroups of this disease.     

Staff interaction with vocally disruptive demented patients compared with demented controls

This study addressed the effect of social isolation on vocally disruptive demented patients (V); the results center on the amount of time patients spent in interactions, and the differences in type and time of staff-patient interaction (IA), compared to controls (C). Semi-structured observations were collected (1100 hours) for 37 vocally disruptive patients and 37 controls, all residents of psychogeriatric wards. Quantitative analysis revealed a low frequency of interactions, elicited mainly for procedural matters and short duration of most interaction episodes. Multiple regression analysis showed a significant difference in the type of interaction: there was more correction of undesired behaviour for vocally disruptive patients than for controls. The results show that social isolation is part of the institutionalized demented patients' life. The dominance of short interaction episodes could contribute to overstimulation. The only significant difference found was the frequency of corrective interactions in the group of vocally disruptive patients. We conclude that this is not the cause of their behaviour, but it may reinforce it. Other factors that might cause and maintain this behaviour require investigation.     

Assessment and treatment of patients with dementia of the Alzheimer type]

Recently more than a dozen clinical trials for dementia of the Alzheimer type (DAT) have been conducted in Japan using almost the same diagnostic criteria and assessment procedures. Cholinomimetic drugs such as AChE or M1 agonist are the most common in the current clinical drug trials. DSM-IIIR and NINCDS-ADRDA are usually employed as diagnostic criteria. In the recent report on the sensitivity and specificity of these diagnostic criteria, it has been indicated that the clinician or researcher who wishes to ensure that patients classified as DAT are more likely DAT should choose DSM-III, whereas the investigator who wishes to include the greatest number or DAT cases, seldom assigning a diagnosis of no DAT to a true case, should choose NINCDS-ADRAD. Also, development of exclusion criteria for DAT would be essential to improve interrater reliability of these diagnostic criteria. In the recent clinical trials outside Japan, a dual assessment procedure consisting of objective psychometric test(s) to assess cognitive impairment and global clinical impression of change is a standard method to evaluate the clinical efficacy of drugs for DAT. In psychometric tests, Alzheimer's Disease Assessment Scale (ADAS) is the most common in the US and Hasegawa's Dementia Scale (HDS) in Japan. A Japanese version of ADAS-cog. has been already developed for use in clinical trials in Japan. Also, HDS has recently been revised to improve the sensitivity of the test. In addition, Clinical Dementia Rating (CDR) that is one of the common measures staging severity of dementia in Japan might be a better alternative to the conventional Clinical Global Impression of Change (CGIC) in the US.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pulmonary hypertension in end-stage renal disease and post renal transplantation patients

Background

Information regarding lung function parameters and functional capacity in renal failure and post renal transplantation patients with pulmonary hypertension (PH) is limited. The purpose of this study was to examine the clinical characteristics of patients with PH who were receiving hemodialysis (HD) or peritoneal dialysis (PD) or who had undergone renal transplantation.

Methods

A prospective study was performed on 116 patients (HD =55, PD =17, and post renal transplantation =44) who underwent Doppler echocardiography. PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mmHg. Demographic information, clinical characteristics, pulmonary function tests (PFTs) and the six-minute walk test (6MWT) were collected and compared between the patients with and without PH.

Results

Twelve (21.8%) patients receiving HD, four (23.5%) patients receiving PD, and eight (18.2%) post renal transplantation patients had PH. In the HD group, the physiological indicators (including pulmonary function test parameters, the final Borg score, and walking distance during the 6MWT) were all significantly lower in the patients with PH compared with those without PH (all P<0.0001). However, in the PD and post renal transplantation groups, no significant differences were noted in the demographic characteristics or in the physiological parameters when the PH patients were compared with those without PH (all P>0.05).

Conclusions

Among HD patients, marked aberrations in PFT results or walking distance may identify a subset of patients suffering from PH.     

The power of personality in discriminating between healthy aging and early-stage Alzheimer's disease

This study examined differences in personality in the earliest stages of dementia of the Alzheimer type (DAT) relative to healthy aging, and the power of personality in discriminating healthy aging from early-stage DAT. Four groups of participants (middle-aged controls, older controls, persons with very mild DAT, and persons with mild DAT) and their families were administered Costa and McCrae's NEO Five-Factor Inventory. On the basis of both self-report and informant report, there was an increase in neuroticism and a decrease in conscientiousness in persons with very mild DAT relative to healthy individuals without it, and in persons with mild DAT relative to those with very mild DAT. Moreover, informant reports of neuroticism and conscientiousness capture substantial unique variance in discriminating healthy aging and very mild DAT, above and beyond standard neuropsychological tests. Discussion focuses on the importance of personality traits as a noncognitive indicator of early-stage DAT.     

Critical factors affecting quality of life of adult patients with anorectal malformations or Hirschsprung's disease

OBJECTIVES: The first objective was to compare the quality of life of adult patients with anorectal malformations (ARM) or Hirschsprung's disease (HD) and to compare both groups with healthy people. And the second objective was to examine the factors that affect the quality of life of patients with ARM or HD, using a theoretical model in which patients' background characteristics explain quality of life via mediating disease-specific functioning and psychosocial functioning. METHODS: Three hundred and forty-one patients completed a questionnaire, which assessed sociodemographic characteristics, disease-specific and psychosocial functioning, and quality of life. Clinical factors were extracted from the medical records. RESULTS: Patients with ARM or Hirschsprung's disease did not differ in their quality of life. Compared to healthy people, both patient groups reported more limitations in their "overall" physical quality of life, but only patients with ARM reported impaired quality of life on several specific domains (e.g., physical role-functioning, pain). The model was largely accepted. Most striking were the strong effects of the psychosocial functioning factors in contrast to weak effects of the disease-specific "constipation" and "fecal continence" factors. CONCLUSIONS: The quality of life of patients with ARM or Hirschsprung's disease was found to be comparable. Compared to healthy people, both patient groups encountered "overall" physical health problems, but only patients with ARM reported additional pain and limitations in role functioning due to physical problems. It appeared that psychosocial functioning had the most important effect on the quality of life of patients with ARM or Hirschsprung's disease, while fecal incontinence and constipation had almost no effect on their quality of life.     

Exercise testing in men with significant left main coronary disease

The exercise tests of 26 male patients with significant left main disease were compared with those of 51 patients with three-vessel disease and 38 patients with two-vessel disease. Exercise-induced ischaemia (chest pain and/or greater than 1 mm ST segment change) occurred in 100 per cent of left main, 69 per cent of three-vessel, and 45 per cent of two-vessel disease patients. Though the mean peak work load was significantly higher in the two-vessel disease group than in those with three-vessel of left main disease, there was a wide overlap between groups. No intergroup differences were found in mean peak heart rates. In patients taking propranolol, no differences in mean peak work loads and heart rates were seen. The study showed that the absence of an exercise-induced abnormal electrocardiographic response virtually excludes left main disease. As judged by exercise performance, the presence of left main disease did not correlate with the severity of the patient's symptomatology. Propranolol did not influence the frequency of an ischaemic response in patients with left main or three-vessel disease.     

Working memory and online syntactic processing in Alzheimer's disease: studies with auditory moving window presentation

Twenty patients with dementia of the Alzheimer's type (DAT) and 20 controls were tested on six tests of working memory and a test of online auditory sentence comprehension in which listening times for each phrase in the sentence, as well as the time required for an end-of-sentence plausibility judgment, were measured. The sentences differed in syntactic complexity. Patients had lower working memory scores than controls and performed more poorly on the plausibility judgments. However, patients were not more affected than controls by the syntactic complexity of a sentence in these judgments, and both groups showed similar effects of syntactic structure in the listening-time data. The increase in listening times at syntactically capacity-demanding points in complex sentences, compared with comparable points in matched simpler sentences, did not correlate with measures of working memory. The results indicate that early-stage DAT patients are not impaired in their ability to assign syntactic structure and to use it to determine aspects of sentence meaning, despite their reduced working memories. This provides evidence for a specialization within working memory for syntactic processing.