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Biomedical subjects

J Haan

Publications and source records attributed to J Haan.

At least 19 recordsLinked to original sources
PubMed1992

Hereditary cerebral hemorrhage with amyloidosis-Dutch type: a study of fibrinolysis.

In view of reported associations between increased bleeding tendency and systemically decreased alpha 2-antiplasmin in patients with systemic amyloid deposition we studied alpha 2-antiplasmin, fibrinogen, C-reactive protein and blood levels of locally produced endothelial hemostasis factors in the acute and quiescent phase in 16 patients with hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D). None of the factors measured in the quiescent phase of the disease was abnormal. In the acute phase, shortly after a stroke, only factor VIII:Ag was evidently elevated. We concluded that systemic abnormalities in the part of the fibrinolysis system studied are not likely to be responsible for multifocal and recurrent cerebral hemorrhages in HCHWA-D. The role of an elevated factor VIII:Ag level in the acute phase is unclear.

Amyloidosis↗
PubMed1992

Alzheimer's disease and hereditary cerebral hemorrhage with amyloidosis-Dutch type share a decrease in cerebrospinal fluid levels of amyloid beta-protein precursor.

The amyloid beta-protein is a 39-42 amino acid peptide that is deposited in senile plaques and in cerebral vessel walls in individuals with Alzheimer's disease, Down's syndrome, hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D), and, to a much lesser extent, normal aging. It is derived from abnormal proteolytic processing of its parent protein, the amyloid beta-protein precursor. Here we show that individuals with the HCHWA-D mutation and clinically manifesting the disease have markedly decreased cerebrospinal fluid levels of soluble amyloid beta-protein precursor (0.7 +/- 0.4 micrograms/ml) compared with age-matched normal subjects (3.0 +/- 0.2 micrograms/ml) as determined by quantitative immunoblotting and enzyme-linked immunosorbent assays. Similarly, age-matched patients diagnosed with probable Alzheimer's disease also have decreased cerebrospinal fluid levels of soluble amyloid beta-protein precursor (1.0 +/- 0.3 micrograms/ml). These parallel findings suggest a common biochemical marker for these two diseases and further establish the pathogenic relatedness of HCHWA-D and Alzheimer's disease.

Alzheimer Disease↗
PubMed1992

Morphology of cerebral plaque-like lesions in hereditary cerebral hemorrhage with amyloidosis (Dutch).

We studied the presence and morphology of plaque-like lesions in the frontal cortex of six patients, aged 40 to 76 years, with hereditary cerebral hemorrhage with amyloidosis--Dutch type (HCHWA-D), using beta/A4 immuno-, silver, Congo red and thioflavin S staining. Two types of beta/A4 immunoreactive and Congo red-negative plaques were detected. The first type was composed of argyrophilic fibrous material in periodic acid-methenamine silver (PAM) and modified Bielschowsky staining and lacked silver-stained degenerating neurites. Therefore, this type of plaque has the same staining properties as the diffuse plaque described in Alzheimer's disease, Down's syndrome and nondemented elderly. The second type of plaque, occurring only in the three oldest patients and numerically increasing with age, consisted of a spherical non-argyrophilic area of granular texture with a rim of PAM-positive material. The PAM-positive fibrous material of both types of plaques was mingled with coarser and compact, irregular-shaped argyrophilic structures in the oldest patient. The described plaques did not show bright fluorescence with thioflavin S staining. These results indicate, that the morphology of plaques, encountered in HCHWA-D, is diverse and changes with age.

Adult↗
PubMed1992

Comparison between the Icelandic and Dutch forms of hereditary cerebral amyloid angiopathy.

Hereditary cerebral amyloid angiopathy has been described in Icelandic and Dutch families. Although the clinical manifestations show similarities, biochemical characterization revealed that amyloid in the Icelandic patients consists of cystatin C and in the Dutch patients of beta-protein. Both diseases are caused by a single base mutation leading to the same amino acid (viz. glutamine). Furthermore, both cystatin C and the beta-protein precursor are protease inhibitors. Therefore, the mechanism of amyloidogenesis may be similar in both diseases. A comparison of clinical, pathological, genetic, and biochemical aspects of these two types of hereditary cerebral amyloid angiopathy is presented.

Amyloid beta-Protein Precursor↗
PubMed1992

Progressive dementia, without cerebral hemorrhage, in a patient with hereditary cerebral amyloid angiopathy.

A now 58-year-old female patient, carrier of the point-mutation in the beta-amyloid gene on chromosome 21 which causes hereditary cerebral hemorrhage with amyloidosis - Dutch type, developed progressive dementia after the age of 55 years. She never suffered from a cerebral hemorrhage. Dementia has been described as a feature of hereditary amyloid angiopathy before, but only in patients who also suffer from strokes. The clinical manifestation of the patient described here underlines the relation between the Dutch type of hereditary amyloid angiopathy and (familial) Alzheimer's disease.

Amyloid beta-Peptides↗
PubMed1992

[Coma polyneuropathy after cardiopulmonary resuscitation].

A 60 year old female patient developed an acute polyneuropathy a few days after a successful cardiopulmonary reanimation followed by coma for several days. Recovery was good as demonstrated at one year follow-up. Residual damage consisted in very mild myoclonic jerks (abortive Lance-Adams syndrome).

Biopsy↗
PubMed1991

No confirmation of visual evoked potential diagnostic test for migraine.

We have attempted to replicate the results of studies on a diagnostic test reported to have 90% sensitivity and 89-96% specificity for migraine. The technique is based on peak-to-peak measurements of fast background electroencephalographic activity during a visual evoked potential (VEP) study. VEP latencies and amplitudes did not differ significantly, and showed substantial overlap, between a group of eight migraine patients and ten age-matched healthy controls. We could not recognise previously described fast activity or measure it objectively by peak-to-peak measurements. We cannot confirm that measurement of fast wave activity in the VEP background is useful in diagnosis of migraine.

Adult↗
PubMed1991

Autonomic nervous function in progressive supranuclear palsy.

Autonomic nervous function was assessed in 11 patients with progressive supranuclear palsy, 26 patients with Parkinson's disease, matched for age, medications, disease severity, and disease duration, and 19 age-matched controls. Results of both parasympathetic (heart rate response to deep breathing and Valsalva maneuver) and sympathetic (blood pressure decrease on standing and increase on sustained handgrip) tests were abnormal in both patient groups. Abnormalities in the group of patients with progressive supranuclear palsy were similar to those in the group with Parkinson's disease but were more pronounced. Autonomic dysfunction may have to be considered a feature of progressive supranuclear palsy.

Aged↗
PubMed1991

Autonomic nervous system tests depend on resting heart rate and blood pressure.

In order to study the effects of baseline blood pressure and heart frequency on autonomic function tests, 75 normal subjects (aged 8-96 years) were investigated. Heart rate variability was studied at rest, during deep breathing, following standing up and during a Valsalva manoeuvre. Blood pressure changes were recorded during standing up and during sustained handgrip. Responses were described as ratios and as differences to study the efficacy of both methods. Multiple regression analysis showed that significant relationships with the resting heart rate existed for ratios but not for differences. The blood pressure rise in the sustained handgrip test showed a significant relationship with resting blood pressure regardless of the description method. As expected, relationships with age existed for all four heart rate tests regardless of the description method. The blood pressure responses were not significantly related to age. We advise that heart rate test results should be presented as differences, as this avoids the need for correction for the resting heart rate. Correction for the resting blood pressure improves the accuracy of the standing up blood pressure test. Correction for age remains necessary for heart rate tests but not for blood pressure tests.

Adolescent↗
PubMed1991

Hereditary cerebral hemorrhage with amyloidosis--Dutch type: its importance for Alzheimer research.

Alzheimer's disease is now commonly regarded as a form of 'amyloid encephalopathy'. Amyloid deposits in the cerebral blood vessels and parenchyma consist mainly of a unique protein called amyloid beta protein (A beta P), which has a molecular weight of 4 kDa and is 42 amino acids long. These deposits are thought to be of pathogenetic importance in Alzheimer's disease. Recently, therefore, attention has been focused on the process of turnover of the precursor of A beta P to amyloid fibrils, and the deposition and persistence of A beta P in this disease. The study of several other diseases with cerebral A beta P deposition can be informative in this respect, because they allow the comparison of different pathogenetic mechanisms that lead to this type of deposition. One of these diseases is hereditary cerebral hemorrhage with amyloidosis- Dutch type (HCHWA-D), which is the subject of this review.

Alzheimer Disease↗
PubMed1991

SPECT in the diagnosis of Alzheimer's disease and multi-infarct-dementia.

SPECT with Tc-99m HM-PAO as a radiopharmaceutical was performed in 17 patients meeting research criteria for Alzheimer's disease (AD), in 10 patients with a clinical diagnosis of multi-infarct-dementia (MID) and in 12 healthy volunteers. Regional tracer uptake was measured in frontal, parietal, and temporoparietal regions. A statistically significant decrease of tracer uptake in the temporoparietal region was found in AD-patients compared with controls. AD-patients showed less activity in this region than MID-patients, but this difference did not reach statistical significance. In both AD- and MID-patients decrease of tracer uptake was not correlated with dementia severity. We conclude that SPECT brain imaging is not yet ready for routine use in the distinction between AD and MID.

Aged↗
PubMed1991

Visual evoked potentials and background EEG activity in migraine.

To investigate whether quantification of the background EEG during a visual evoked potential (VEP) study is of value for the diagnosis of migraine we studied 8 unmedicated migraineurs between attacks, and 10 age-matched controls. Three paradigms were used: the first two concerned pattern-reversal VEPs with different analysis times (500 and 1500 ms), and in the third paradigm the pattern did not reverse. Power spectra were calculated for individual responses, and the delta, theta, alpha and beta areas of the averaged spectra were noted as indicators of background reactivity. Alpha and beta powers were consistently but not significantly higher in the migraine group. The difference was too small to be of value as a diagnostic test. Alpha power was (not significantly) lower in the presence of photic stimulation than in its absence. As this was the case in both groups photic stimulation does not explain the higher alpha powers in the migraine group. We conclude that EEG background activity during the VEP does not distinguish reliably between migraineurs and controls.

Adult↗
PubMed1991

Cognitive function after spinal or general anesthesia for transurethral prostatectomy in elderly men.

Cognitive functions in 53 elderly men who underwent a transurethral prostatectomy were assessed pre-operatively and 4 days and 3 months post-operatively. Thirteen patients had a preference for one particular type of anesthesia, and the remaining 40 were randomly allocated to receive either spinal or general anesthesia. Cognitive function was not different between the groups receiving different types of anesthesia at either time point and did not decrease post-operatively. No pre- or perioperative variable could distinguish the subgroup of patients who had a post-operative decrease of 2 points or more on the Mini-Mental State Examination. No difference in post-operative performance was found in the patient groups with pre-operative Mini-Mental State Examination scores above or under their age-specific norm. It is concluded that neither hospitalization nor the two forms of anesthesia investigated cause a decrease in cognitive function in elderly men.

Aged↗
PubMed1991

Hereditary cerebral hemorrhage with amyloidosis--Dutch type: a congophilic angiopathy. An overview.

Hereditary cerebral hemorrhage with amyloidosis--Dutch type (HCHWA-D) is characterized by recurrent cerebral hemorrhages and dementia at a relatively young age. The symptoms are caused by extensive deposition of amyloid in cerebral arterioles and leptomeningeal arteries. A point-mutation in the beta-protein precursor gene on chromosome 21 is the underlying cause of the disease. This paper summarizes the clinical, radiologic, pathologic, and genetic features of this disease, with special attention to the relation between HCHWA-D and Alzheimer's disease, which is also characterized by beta-protein deposition.

Amyloid beta-Peptides↗
PubMed1991

DNA diagnosis for hereditary cerebral hemorrhage with amyloidosis (Dutch type)

Hereditary cerebral hemorrhage with amyloidosis of the Dutch type (HCHWA-D) is tightly linked to the Alzheimer amyloid precursor protein gene on chromosome 21, which codes for the amyloid beta-protein. A point mutation detected at position 1852 of the amyloid precursor protein gene in four HCHWA-D patients was hypothesized to be the basic defect. This study proves that 22 HCHWA-D patients from three pedigrees all carry this point mutation, whereas the mutation is absent in escapees from the HCHWA-D families as well as in randomly selected Dutch individuals. A mutation-specific oligonucleotide is now available for the confirmation of the HCHWA-D diagnosis. Therefore, presymptomatic testing and prenatal evaluation of individuals at risk in the HCHWA-D families is now feasible.

Aged↗