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H C Schoonderwaldt

Publications and source records attributed to H C Schoonderwaldt.

17 recordsLinked to original sources

Improved discrimination of AD patients using beta-amyloid(1-42) and tau levels in CSF.

OBJECTIVE: To evaluate CSF levels of beta-amyloid(1-42) (Abeta42) alone and in combination with CSF tau for distinguishing AD from other conditions. METHODS: At 10 centers in Europe and the United States, 150 CSF samples from AD patients were analyzed and compared with 100 CSF samples from healthy volunteers or patients with disorders not associated with pathologic conditions of the brain (CON), 84 patients with other neurologic disorders (ND), and 79 patients with non-Alzheimer types of dementia (NAD). Sandwich ELISA techniques were used on site for measuring Abeta42 and tau. RESULTS: Median levels of Abeta42 in CSF were significantly lower in AD (487 pg/mL) than in CON (849 pg/mL; p = 0.001), ND (643 pg/mL; p = 0.001), and NAD (603 pg/mL; p = 0.001). Discrimination of AD from CON and ND was significantly improved by the combined assessment of Abeta42 and tau. At 85% sensitivity, specificity of the combined test was 86% (95% CI: 81% to 91%) compared with 55% (95% CI: 47% to 62%) for Abeta42 alone and 65% (95% CI: 58% to 72%) for tau. The combined test at 85% sensitivity was 58% (95% CI: 47% to 69%) specific for NAD. The APOE e4 gene load was negatively correlated with Abeta42 levels not only in AD but also in NAD. CONCLUSIONS: The combined measure of CSF Abeta42 and tau meets the requirements for clinical use in discriminating AD from normal aging and specific neurologic disorders.

Adult↗

Flunarizine in stroke treatment (FIST): a double-blind, placebo-controlled trial in Scandinavia and the Netherlands.

INTRODUCTION: An international, multicenter trial was conducted in 331 patients to determine the effect of a large dose of flunarizine (a calcium entry blocker) in the treatment of acute ischemic stroke in the territory of the Middle cerebral artery. METHODS: The administration of the trial medication should start within 24 h after the initial symptoms of stroke. According to a random schedule, the patients were assigned to a 4-weeks double-blind treatment with either flunarizine (n = 166) or placebo (n = 165): one week intravenous administration (50 mg daily), followed by 3 weeks oral treatment (week 2, 21 mg daily; week 3-4, 7 mg daily). All patients had to be investigated by computerized tomography (CT) within 7 days after stroke onset; 36 patients were secundarily excluded because the CT showed another pathology. During the treatment period, other "stroke therapies" were not allowed. Patients were followed up for 24 weeks. RESULTS: After the 24 weeks trial period, the percentage of patients who were dead or pendent (modified Rankin score 3-5) was similar in both treatment groups (flunarizine 67%, placebo 65%). During the trial, the scores for handicap severity (modified Rankin scale), neurological status (Orgogozo) and activities of daily living (modified Barthel index) strongly improved in both treatment groups, but no differences were found between the treatment groups. In this trial, the administration of trial treatment started relatively late after stroke onset (flunarizine group: mean time interval 13.5 h; placebo 12.3 h). A subgroup of patients received trial medication within 6 h after stroke onset (flunarizine n = 31; placebo n = 29). Also in this subgroup, no differences were found between the flunarizine and placebo group. CONCLUSION: Flunarizine did not improve neurologic and functional outcome in patients with acute ischemic stroke.

Activities of Daily Living↗

MRI in patients with suspected vascular parkinsonism.

To determine whether MRI can reveal more vascular lesions in patients clinically suspected of having vascular parkinsonism, we compared 15 such patients with 15 patients who had idiopathic Parkinson's disease and 10 hypertensive controls. Patients with suspected vascular parkinsonism had significantly more subcortical lesions than those with Parkinson's disease or hypertension. The cutoff point that best distinguished patients with suspected vascular parkinsonism from patients with Parkinson's disease was a 0.6% level of lesioned brain tissue volume. There were two types of vascular parkinsonism: one had an acute onset and lesions located in the subcortical gray nuclei (striatum, globus pallidus, thalamus); the other had an insidious onset and lesions diffusely distributed in the watershed areas.

Aged↗

Vitamin B12 and folate concentrations in serum and cerebrospinal fluid of neurological patients with special reference to multiple sclerosis and dementia.

Vitamin B12 and folate concentrations were measured in serum and cerebrospinal fluid (CSF) in 293 neurological patients. Serum and CSF vitamin B12 concentrations showed a positive correlation. In individual patients CSF B12 concentrations varied considerably for a given serum concentration. The median serum vitamin B12 concentration of the Alzheimer's type dementia group was significantly lower compared with that of a control group. Lower median CSF vitamin B12 concentrations were found in groups of patients with multiple sclerosis and Alzheimer's type dementia. Five patients with heterogeneous clinical pictures had unexplained low serum and CSF B12 concentrations without macrocytosis. Two patients had very high serum B12 and low-normal CSF concentrations which could be explained by a blood-brain barrier transport defect. Serum and CSF folate concentrations did not show significant differences between the various groups.

Adolescent↗

Mannosidosis: a study of two patients, presenting clinical heterogeneity.

Two unrelated patients suffering from mannosidosis, aged 9 and 33 years, are reported, presenting two extremes in the phenotypic expression of this lysosomal storage disorder. Clinical, radiological, biochemical and therapeutical aspects of the disease are discussed. Our patients support the concept that mannosidosis is not a homogeneous syndrome but manifests wide clinical heterogeneity.

Adult↗

The effects of acute cerebrovascular disease on serum and cerebrospinal fluid parameters.

In order to evaluate the effects due to acute cerebrovascular disease on serum and cerebrospinal fluid (CSF) parameters, biochemical data from 312 patients were studied. In serum, CK, LD and alpha 1 protein fraction showed a moderately close relationship to the severity of stroke. In CSF there could not be observed any relation between enzymatic activities (LD, ASAT, CK) and the severity of the disease. The same result was found for CSF protein and the ratio CSF albumin/serum albumin, parameters which are indicative for Blood Brain Barrier (BBB) disturbances. Substances from the intermediate metabolism in the Central Nervous System (CNS) (lactate and pyruvate) were evidently raised in CSF and there was a clear relation between the CSF concentration and the severity of stroke. No indication for IgG immunoglobulin abnormalities in CSF was found. The concentration of neurotransmitter metabolite 5HIAA in CSF was significantly higher in (in)completed stroke than in Transient Ischaemic Attack.

Acute Disease↗

Heterozygosity for homocystinuria in premature peripheral and cerebral occlusive arterial disease.

Premature arteriosclerosis and thromboembolic events are well-known complications of homozygous homocystinuria due to cystathionine synthase deficiency. It is unknown whether heterozygosity for homocystinuria predisposes to premature vascular disease. We explored the frequency of excessive homocysteine accumulation after standardized methionine loading in 75 patients presenting with clinical signs of ischemic disease before the age of 50:25 with occlusive peripheral arterial disease, 25 with occlusive cerebrovascular disease, and 25 with myocardial infarction. In seven patients in each of the first two groups but in none of the patients in the third group, heterozygosity for homocystinuria was established on the basis of pathological homocysteinemia after methionine loading and cystathionine synthase deficiency in skin fibroblast cultures. Because the frequency of heterozygosity for homocystinuria in the normal population is 1 in 70 at the most, we conclude that this condition predisposes to the development of premature occlusive arterial disease, causing intermittent claudication, renovascular hypertension, and ischemic cerebrovascular disease.

Adult↗

The preselection value of the Doppler LP test for shunt-therapy in patients with normal pressure hydrocephalus.

The results of this study confirm the hypothesis that the simple innocuous Doppler LP test may be useful in obtaining information about CSFP-CBF (cerebrospinal fluid pressure-cerebral blood flow) relationships. The value of this test in predicting the clinical outcome of CSF shunting in patients with normal pressure hydrocephalus (NPH) was studied. Fourteen patients with NPH were examined; eight patients showed a positive and six patients a negative Doppler LP test. Seven of the eight patients with a positive test improved following the shunting procedure, but none of the six patients improved after operation. This study suggests that the Doppler LP test is a useful preoperative selection test in patients with NPH. In addition this study supports the hypothesis that CBF autoregulation may be impaired with NPH.

Adolescent↗

Neurologic manifestations of homocystinuria.

Recognition of the disease homocystinuria by the neurologist may be of importance as is illustrated in the dramatic case history of a 20 year old woman. Confusion with Marfan's syndrome frequently occurs. The clinical and pathologic anatomical features are described. A review of the literature is given and the use of contraceptives is discussed.

Adult↗

Two patients with an unusual form of type II fucosidosis.

Two brothers with an unusual form of fucosidosis are presented, providing further evidence for the clinical heterogeneity of the disease. The patients have several characteristics of type II, but from the point of view of progression rate and survival, they resemble more type III. However, the characteristic skin lesions of type III are not seen. In contrast to all other subtypes, a dry, thin skin is observed. The authors tend to classify these patients as a "slow motion" type II variant. Both in leukocyte lysates and plasma, severe alpha-L-fucosidase deficiency was established. In the parents, intermediate alpha-L-fucosidase activities were observed in leukocytes, but normal values in plasma, indicating that plasma is not suitable for carrier detection in this family. It is felt that no conclusions regarding clinical subtypes of fucosidosis can be drawn without careful characterization of the mutant enzyme in the primarily affected tissues.

Adult↗

Changes in carotid flow velocity induced by lowering cerebrospinal fluid pressure in normal pressure hydrocephalus.

This is a report of changes in blood flow velocity in the carotid system induced by lumbar puncture in five patients who had clinical and neuroradiological signs of normal pressure hydrocephalus. After lowering cerebrospinal fluid pressure an increase of carotid flow velocity was found on Doppler hematotachography. These changes of carotid blood flow velocity could not be demonstrated in a control group of four patients with normal cerebrospinal fluid dynamics. This method is easy to perform, does not overtax the patient and seems to be indicated for the diagnosis of NPH. It is presented as a new, simple, reproducible aid to the diagnosis of NPH by Doppler hematotachography.

Adult↗

Neuron-specific enolase, S-100 protein, myelin basic protein and lactate in CSF in dementia.

The pattern of injury of the specific cell structures of the central nervous system (CNS) is different in the various types of the dementia syndrome. We challenged the hypothesis that this could be reflected in specific patterns of brain-specific proteins in the cerebrospinal fluid (CSF). The neuron-specific enolase (NSE), S-100, myelin basic protein (MBP) and lactate levels were retrospectively analyzed in the CSF of 159 patients with various types of dementia. A previous study from our department demonstrated age-related reference values for the brain-specific proteins in the CSF. The present study affirmed the strikingly high NSE and S-100 values in the CSF of patients with autopsydiagnosed Creutzfeld-Jacob disease: NSE, S-100 and MBP levels in the CSF of patients with various other types of dementia, and controls, did not differ significantly. Therefore we concluded that a single determination of CSF concentrations of these brain-specific proteins were of little value in the differential diagnosis of the dementia syndrome. In the diagnosis of normal pressure hydrocephalus increased levels of CSF lactate may be helpful.

Aged↗