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H Chui

Publications and source records attributed to H Chui.

25 records · Page 2Linked to original sources

Studies of the enteric nervous system in Alzheimer disease and other dementias of the elderly: enteric neurons in Alzheimer disease.

Studies of the myenteric (Auerbach) plexus of esophagus, stomach, small intestine, colon, and rectum, by microdissection and pointcount morphometry, for 18 patients with Alzheimer disease (AD), eight with other types of dementia of the elderly, non-demented elderly patients, and younger control patients, show a normal loss of enteric neurons and plexus mass with age, comparable to that reported by others for rats and guinea pigs. Values for patients with AD or other non-AD dementias did not differ from those for elderly controls. Enteric neurons in AD or the other dementias studied showed no definite stain with ALZ-50, a monoclonal antibody to a derivative of the microtubule-associated protein tau, which stains degenerating cerebral neurons in AD. Although the processes in the central nervous system in AD affect some neurons derived from the neural plate, the results of this study suggest that the enteric neurons, which are of neural crest origin, are not affected in AD. Enteric neurons, at least by the methods of this study, do not provide a useful peripheral marker for AD.

Age Factors↗

Temporal and hippocampal metabolic rate during an olfactory memory task assessed by positron emission tomography in patients with dementia of the Alzheimer type and controls. Preliminary studies.

Positron emission tomography with fludeoxyglucose F 18 was used to assess cortical metabolic rate during an olfactory memory task in six patients with dementia of the Alzheimer type. Metabolic activity was compared with that of both age-matched controls performing the olfactory task and controls resting with their eyes closed. Patients had lower metabolic rates in the anterior portion of the medial-temporal cortex than did controls, and the difference was greatest between patients and the controls performing the memory task. This region is known to receive a large olfactory input and has been implicated in the encoding of human memory. Normal controls resting with their eyes closed had significantly lower metabolic rates in this area when compared with normal controls performing the task. Our results are consistent with those of earlier reports of temporal lobe decreases in metabolic rate and extend them through the examination of areas salient to the behavioral loss.

Aged↗

Phenotypic heterogeneity in familial Alzheimer's disease: a study of 24 kindreds.

We report the clinical and neuropathological characteristics occurring in 180 demented individuals from 24 kindreds with familial Alzheimer's disease (FAD). Each family had at least two affected generations, and at least one autopsy or brain biopsy was compatible with the diagnosis of AD. Forty-nine neuropathological specimens or reports were reviewed. Mean age of onset for the total group was 54.7 years +/- 11.5, with a large range of 30 to 84 years. Mean age at death was 63.5 years +/- 12.2, with a range of 46 to 85. Mean duration of disease was 8.8 years +/- 4.4, with a range of 1 to 23 years. Six findings suggested phenotypic heterogeneity in FAD. (1) Five families represented an early age of onset group with mean onset at 42 years (range 30 to 51 years) and mean disease duration of 6.7 years. (2) Eight families represented a late onset group with mean onset at 68 years (range 59 to 78 years) and a mean duration of 8.5 years. (3) Seven families were of Volga German ancestry, all originating from the same two villages in Russia. Mean age of onset was 55.9 years (range 40 to 72 years), with a mean disease duration of 10 years. This group probably represents the genetic founder effect of an autosomal dominant gene for AD. (4) One family had the unusual characteristics of neurofibrillary tangles and granulovacuolar change but no amyloid plaques, a mean disease duration of more than 11 years, and a "schizophrenia-like" onset. (5) One family with late onset also had clinical and pathological evidence for anterior horn cell disease. (6) Two autopsies in 1 family both showed remarkable rarefaction of myelin and expansion of perivascular spaces in centrum semiovale (état criblé), with marked leptomeningeal and cortical amyloid angiopathy, distinct from the other FAD brains. It remains to be determined whether the clinical and pathological differences between these families represent genetic heterogeneity at the biochemical or molecular level.

Adult↗

Functional recovery. A major risk factor for the development of postpoliomyelitis muscular atrophy.

A retrospective study was undertaken to identify potential risk factors for the development of progressive postpoliomyelitis muscular atrophy (PPMA). Patients with PPMA (n = 57) were compared with patients with a history of poliomyelitis but without a history of progressive weakness (n = 49). Patients who later developed PPMA had histories of more widespread acute paralysis, but relatively greater functional recovery. They were less disabled, and reported higher recent activity levels. Seventy-nine percent of the total variance between the PPMA and control groups could be accounted for by recovery alone (ie, severity minus disability). Functional recovery is generally attributed to reinnervation of sarcomeres by collateral sprouting from surviving lower motor neurons. Since degree of recovery predicts the risk of developing PPMA, our findings suggest that enlarged motor units may carry an increased susceptibility for dysfunction and/or degeneration.

Adult↗

Churg-Strauss syndrome (allergic granulomatous angiitis). Neuro-ophthalmologic manifestations.

Two patients with severe bronchial asthma, hypereosinophilia, and peripheral neuropathy were initially observed with neuro-ophthalmologic signs and symptoms that included amaurosis fugax, superior oblique palsy, ischemic optic neuropathy, and scattered areas of retinal infarction. Clinical investigation led to the diagnosis of Churg-Strauss syndrome (allergic granulomatous angiitis). As in other collagen vascular diseases, ocular signs or symptoms may occasionally be the most prominent manifestation of the disease. Recognition of this systemic disorder by the ophthalmologist may minimize systemic and ocular complications.

Asthma↗

Natural history of vascular dementia.

Knowledge about the natural history of vascular dementia (VaD) provides one context for assessing efficacy in pharmacological drug trials. We reviewed the literature for original studies reporting either (a) duration of survival or (b) cognitive status following a diagnosis of multi-infarct dementia (MID). Among 13 papers (combined sample size = 470; mean duration of follow-up = 4.3 years), survival was significantly shorter following a clinical diagnosis of MID compared with Alzheimer disease (AD). Nine studies (combined sample size = 175; mean duration of follow-up = 2.9 years) reported at least two mental status scores separated by at least 1 year. Among four studies using the Mini-Mental State Examination, no consistent differences in annualized rate of decline could be observed for MID compared with AD. Also, no factors were identified that predict rate of decline. This review reveals many limitations in our current knowledge about the natural history of VaD. Understanding would be enhanced by multinational harmonization of definitions for study entry point, diagnosis of VaD, classification of subtypes, as well as cognitive, functional, and behavioral endpoints.

Cognition Disorders↗