Long-standing Dementia Research Articles

Under Reporting of Dementia Deaths on Death Certificates using Data from A Population-Based Study (NEDICES)

Previous studies have shown that dementia is frequently omitted as a cause of death from the death certificate in patients with long-standing dementia. However, most studies exclude those undiagnosed dementia sufferers in the population. In order to overcome this problem, it is necessary to examine all the participants or to screen the population for symptoms of dementia and confirm the diagnosis with a clinical examination (two-phase approach). We used this latter methodology to estimate the proportion of reporting of dementia on death certificates in a prospective population-based study (NEDICES), involving 4,197 elderly people. Community-dwelling subjects with and without dementia were identified and followed during a median of 12.5 years, after which the death certificates of those who deceased were examined. A total of 1,976 (47.1%) died (403 subjects with dementia). Dementia was rarely reported as the primary cause of death, even in known cases of dementia (20.8%). Indeed it was reported in only 13.3% of those with mild dementia and 24.3% of those with moderate or severe dementia; in 24.9% of those with possible or probable Alzheimer's disease; and in 11.9% of those with non-Alzheimer dementia. In a stepwise multiple logistic regression analysis with the dependent variable being presence or absence of dementia on the death certificate, the significant associated independent variables were age at death, severity of dementia, and etiology of dementia. We conclude that reporting of dementia on death certificates remains poor. This suggests a lack of awareness of the importance of dementia as a cause of death.

Long-standing Prion Dementia Manifesting as Posterior Cortical Atrophy

Prion diseases commonly manifest with the phenotype of subacute myoclonic encephalopathy. However, genetic forms of prion disease may have prolonged evolution mimicking neurodegenerative disease. We present the clinical and neuropathological features of a family with an early and long-standing dementia manifesting with posterior cortical atrophy and related to a 120 bp insertional mutation of the prion protein gene. Two cases exhibited mixed prion and Aβ pathology. The differential diagnosis with Alzheimer disease is discussed.

Baylisascaris procyonisInfection in Elderly Person, British Columbia, Canada

<i>Baylisascaris procyonis</i>Infection in Elderly Person, British Columbia, Canada

Apoptotic phenomena are not a major cause of enteric neuronal loss in constipated patients with dementia

Chronic constipation is a frequent symptom in patients with dementia, especially in those institutionalized. However, few data are available on the neuropathological aspects of the colon in such patients. We investigated the enteric neuropathology of the colon in two patients with longstanding dementia and intractable constipation, requiring surgery to alleviate symptoms. The results were compared to those obtained in 10 controls. No abnormalities were found at conventional histological examination, except for the presence of melanosis coli. Immunohistochemical evaluation revealed no important difference between patients and controls, except for a decreased number of enteric neurons in patients. However, this neuronal decrease was not associated to apoptotic phenomena, as observed in patients with severe idiopathic constipation. We concluded that in severely constipated patients with dementia the neuropathological abnormalities might be reconducted to a physiological neuronal decrease as a result of aging, and that the pathophysiological aspects of constipation in these subjects differ from those found in idiopathic constipation.

The frequently low cobalamin levels in dementia usually signify treatable metabolic, neurologic and electrophysiologic abnormalities.

Cobalamin levels are frequently low in patients with dementia, but it is unclear if they represent definable deficiency and what the mechanisms are. Therefore, patients being evaluated for dementia who had low cobalamin levels but no obvious evidence of deficiency were studied hematologically, neurologically and with metabolic tests and were re-evaluated after cobalamin treatment. Abnormalities suggestive of or diagnostic for deficiency were documented in most of the 16 demented and nondemented patients. Metabolic results: 50% of patients tested had abnormal deoxyuridine suppression and 44% had increased serum methylmalonic acid and/or homocysteine levels; these test results correlated with each other. Neurologic results: 73% of patients had clinical abnormalities, primarily mild neuropathies, not attributable to other causes, 75% had electroencephalographic abnormalities, 77% had abnormal visual evoked potentials and 33% had abnormal somatosensory potentials. Metabolic and neurologic dysfunction were present together or absent together in all but 2 cases. Cobalamin therapy improved 50-100% of the various types of abnormalities, although it did not improve cognitive function in the 13 demented patients. Food-cobalamin malabsorption was found in 60% of the patients. Despite the absence of megaloblastic anemia and rarity of traditional malabsorption of free cobalamin, low cobalamin levels in demented patients frequently represent mild cobalamin deficiency and are often associated with food-cobalamin malabsorption. Perhaps most importantly, this is accompanied not only by metabolic changes but by evidence of mild neurologic dysfunction. Their frequent reversibility by cobalamin confirms that these defects indeed arise from cobalamin deficiency. Although the long-standing dementia does not improve, treating such patients with cobalamin has other concrete benefits.

Rheoencephalographic and Other Studies of Betahistine in Humans. III. Improved Methods of Diagnosis and Selection in Arteriosclerotic Dementia

Thirty-six institutionalized geriatric patients with particularly severe and longstanding dementia were selected by chart review and were studied neurologically. Criteria for clinical diagnosis are derived, and it is shown that arteriosclerosis had caused or contributed to their dementias in 33 patients (91.7%) and probably did so in two others (97.2%). Arteriosclerotic dementia may not be rare and may be much more prevalent than may now be considered the case. As 23 patients (63.9%) showed evidence of carotid and/or vertebrobasilar involvement, large-vessel disease also may be more prevalent in geriatric dementia than is now thought and should be considered in all such patients. The results further confirm that careful review of ward records without reference to psychiatric diagnoses of etiology is a simple, rapid, and reliable method of screening a large institutionalized population for possible arteriosclerotic dementia. The selectees should then be surveyed appropriately to confirm the presence of the disease. Since a careful review of the complete hospital records and a thorough neurologic examination proved sufficient, more definitive but more dangerous invasive diagnostic procedures may not be necessary for clinical pharmacologic and similar investigations.