Moyamoya disease in Down's syndrome: a report of two cases.
The case of two children with Down's syndrome and multiple cerebral infarction on CT scan are described. Angiography revealed advanced moyamoya disease.
Carotid Artery, Internal↗
Biomedical subjects
S Vicari
Publications and source records attributed to S Vicari.
Electrocortical mapping, MRI, and neuropsychological measures: evidence of Alzheimer's disease in the presence of vascular lesions.
We report here a case study of a 76-year-old woman with a high school education, whose presenting psychiatric symptomatology indicated dementia of unknown etiology. Neuropsychological test results were consistent with AD, but diagnosis was complicated by an MRI showing a large right hemisphere cortical infarct and scattered subcortical changes leading to a diagnosis of possible AD. Electrocortical mapping showed the right hemisphere infarct, and gave independent evidence suggestive of AD in the relatively intact left hemisphere. This case demonstrates the utility of multidimensional assessment as an aid to differential diagnosis.
Clinical Conference↗
Cognitive functions in adult Down's syndrome.
Cognitive profiles of performances were obtained from a selected group of adult Down syndrome (DS) subjects (n = 20; mean age: 34.5 years; s.d.: 7.7) by means of an ad hoc neuropsychological battery. With the aim of examining, from a neuropsychological point of view, the modifications that increasing age produces in this group of patients, cognitive performances of younger DS subjects (mean age: 28 years; s.d.: 4.77) were compared with analogous performances obtained by the older ones (mean age: 39.8 years; s.d.: 5.11). Subsequently, to clarify qualitative aspects of cognitive patterns in the subgroups of young and old DS subjects, two different groups of control patients were utilised. Neuropsychological data collected from a group of adult mentally retarded subjects were compared with cognitive performances demonstrated by young DS subjects, while the old group of DS subjects was analyzed in comparison with a group of patients affected by initial form of Alzheimer disease (AD). Altogether, the results of our study do not seem to support, from a neuropsychological point of view, the hypothesis that mental decline observed in DS subjects reproduces the cognitive patterns of impairment observed in AD patients.
Adult↗
Defective concept formation in parkinsonians is independent from mental deterioration.
An extensive neuropsychological battery (the Mental Deterioration Battery) was utilized to distinguish, within a sample of 24 idiopathic Parkinsonians, those showing signs of diffuse mental deterioration (n = 9) from those without deterioration (n = 15). Performances of control subjects on a wide range of tests exploring mnesic, visuo-constructive, linguistic and general intellectual functions (n = 21) did not differ from analogous performances of Parkinsonians without signs of diffuse mental deterioration. The Wisconsin Card Sorting Test was then used to verify the hypothesis that a selective impairment of cognitive functions subsumed by the integrity of frontal lobes could be demonstrated in Parkinsonian patients. Our results provide evidence that in this task, defective performances are obtained by Parkinsonians and even by patients without signs of diffuse cognitive impairment. These findings seem to confirm that a deficit in concept formation, maintenance and shifting is largely independent of the dementia frequently noticed in Parkinson's disease.
Aged↗
Differential aspects of cognitive impairment in patients suffering from Parkinson's and Alzheimer's disease: a neuropsychological evaluation.
Neuropsychological profiles of cognitive impairment demonstrated by a group of Parkinson patients and by a group of Alzheimer patients both affected by a comparable level of mental deterioration were obtained by means of a neuropsychological battery. The results pointed out differential patterns of cognitive impairment exhibited by the two pathological groups. Particularly, Alzheimer's patients appear to be mainly impaired in mnesic functions, while Parkinsonian subjects show a pattern of performances indicative of a prevalent impairment of the functions underlied by frontal lobes. These data are in agreement with the hypothesis that different anatomical-functional cerebral structures are mainly involved in determining cognitive deterioration in the two diseases.
Alzheimer Disease↗
[Treatment of paralysis of intrinsic muscles of the hand of leprous origin].
Explore the source record for details and available documents.
Adolescent↗
[TEffect of trihydroxyethylrutoside in diabetic retinopathy].
Explore the source record for details and available documents.
Adolescent↗
The recovery of cerebrospinal fluid acetylcholinesterase activity in Alzheimer's disease patients after treatment with metrifonate.
We investigated the relationship between peripheral and central cholinesterase (ChE) inhibition levels after chronic treatment of Alzheimer's disease (AD) patients with metrifonate (MTF). In a 6-month, double-blind, placebo-controlled trial in AD patients treated with a weekly 2.9 mg/kg MTF dose, we observed 17.15 +/- 23.43, 66.92 +/- 7.30 and 60.80 +/- 12.20% inhibition (n = 6) of cerebrospinal fluid (CSF) and red blood cell (RBC) acetylcholinesterase (AChE) and plasma butyrylcholinesterase (BuChE), respectively. In another study, AD patients were treated with daily MTF to achieve RBC AChE inhibition levels of 85.90%. The CSF AChE inhibition was 67.93 +/- 13.69% (n = 3) at 3-4 h after the last treatment and 6.62 +/- 9.36% (n = 2) at 8 days after dosing. The recovery half time of CSF AChE was 2.21 +/- 1.22 days. These data show that CSF AChE recovers faster than the peripheral plasma and RBC enzymes. Under conditions of chronic weekly dosing with MTF, RBC AChE inhibition does not reflect CSF, and arguably, brain AChE inhibition. Our data do not support continuous central neuronal AChE inhibition as the mechanism for the long-term efficacy of metrifonate for the treatment of AD.
Acetylcholinesterase↗
[Alzheimer's disease and Down's syndrome: a review].
In the last few years a number of studies highlighted striking similarities between Alzheimer's Disease and Down's Syndrome. More specifically, neurochemical, pathologic, genetic and clinical features of the Alzheimer's Disease were described also in the Down's Syndrome, but with some particular differences. The most significant literature reports investigating these aspects are reviewed.
Alzheimer Disease↗
[Evaluation of cognitive functions in patients with chronic renal insufficiency].
Fifteen undialyzed and thirteen dialyzed patients affected by chronic renal failure were studied by means of an extensive neuropsychological battery with the aim of assessing the incidence of a deterioration of cognitive functions. Mean performances scores obtained by the two groups of patients were compared with analogous scores obtained by a group of control subjects similar as for literacy and age. The comparisons among the three groups did not reveal any difference as for performance scores obtained to various tasks nor for the global assessment derived for the entire battery. Furthermore our data do not point out any significative correlation between global performances on neuropsychological battery and blood levels of creatinine and urea nitrogen or duration of dialytic treatment of renal subjects. In conclusion, the results of our study do not show evidence of cognitive impairment in subjects affected by chronic renal failure and do not confirm the hypothesis of a detrimental effect of prolonged periods of dialytic treatment on cognitive functions.
Blood Urea Nitrogen↗