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F C Stam

Publications and source records attributed to F C Stam.

At least 19 recordsLinked to original sources
PubMed1995

'Lewy body disease': clinico-pathological correlations in 18 consecutive cases of Parkinson's disease with and without dementia.

One of the characteristic histological features of Parkinson's disease (PD), with or without dementia, is the presence of Lewy bodies (LBs) in the brainstem and neocortical and limbic structures. They are often accompanied by Alzheimer type pathology (ATP). In the present retrospective study the clinical features and post-mortem findings of 18 consecutive and unselected PD patients were compared, with special reference to the frequent but not exclusive association of LBs with ATP in Lewy body disease (LBD). LBD is the term applied to a particular pattern of neuronal degeneration associated with LBs. In this study of idiopathic PD patients ATP seems to be the major determinant of the cognitive decline in most patients. Cortical Lewy Bodies (CLBs) were present in all patients reviewed, whether or not dementia was present. It was not possible to distinguish a specific pattern in the cognitive or psychopathological symptoms of dementia that would differentiate LBD from Alzheimer's disease (AD). Although in most cases hippocampal CA2-3 ubiquitin immunoreactive neurites were observed, here again there was no correlation with the presence of dementia.

Aged↗
PubMed1994

Increased numbers of corticotropin-releasing hormone expressing neurons in the hypothalamic paraventricular nucleus of depressed patients.

The hypothalamo-pituitary-adrenal (HPA) axis is known to be activated in depressed patients. Although direct evidence is lacking, this activation is hypothesized to be due to hyperactivity of corticotropin-releasing hormone (CRH) neurons of the hypothalamic paraventricular nucleus (PVN). Recent immunocytochemical studies in experimental animals and in humans showed that the number of CRH-expressing neurons correlated with the activity of these neurons. In addition, colocalization of AVP in CRH neurons has been shown to be an index for the secretory activity. Therefore, we estimated the total number of CRH-immunoreactive neurons and their fraction showing colocalization with AVP in the PVN of 10 control subjects and of 6 depressed patients who were diagnosed to be suffering from a major depression or a bipolar disorder. The mean total number of CRH-expressing neurons of the 6 depressed patients was four times higher, and the number of CRH neurons co-expressing AVP was almost three times higher than those in the control group. We also determined the two activity parameters of CRH neurons in the PVN of 2 subjects with a depressive organic mood syndrome or a depressive disorder not otherwise specified. In these two 'non-major depressed' subjects, the activity parameters of CRH neurons were comparable to those of control subjects. Our observations strongly support the hypothesis that CRH neurons in the PVN are hyperactivated in major depressed patients. This hyperactivity might be causally related to at least part of the symptomatology of depression.

Adult↗
PubMed1992

[Alzheimer's disease: variety without unity].

The diagnosis of Alzheimer's Disease is quite common these days. Nonetheless, a clearcut definition of the concept of Alzheimer's Disease is still beyond scientific reach. This discrepancy makes it particularly relevant to explore the meanings of the concept, and to ask some questions concerning how diseases are defined. Alzheimer's Disease has been introduced into nosology by Kraepelin, at the beginning of the 20th century. Kraepelin's claim that Alzheimer's Disease is a disease sui generis, was contested promptly: by Alzheimer himself, for instance. This initiated a history of intensive debate concerning the distinction between Alzheimer's Disease and Senile Dementia on the one hand, and the distinction between Senile Dementia and ageing on the other. During the 1970s and 1980s, several important changes took place, which elucidated the use of clinical and neuropathological criteria for the diagnosis of dementia and Alzheimer's Disease. This, however, did not end the debate. A definite criterion--a gold standard--for the diagnosis of Alzheimer's Disease is still lacking. Recent developments in the field of molecular biology furthered the insights into the pathological processes in Alzheimer's Disease. These developments show that a unitary definition of Alzheimer's Disease (including the presenile and senile forms) is not to be expected. There are therefore insufficient reasons to accept a disease entity called 'Alzheimer's Disease'. In this sense we are not far removed from Alzheimer's and Kraepelin's days. The interpretation--both in- and outside science--of Alzheimer's Disease as an entity is therefore not supported by sound scientific arguments.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1991

Distribution pattern and functional state of alpha 1-antichymotrypsin in plaques and vascular amyloid in Alzheimer's disease. A immunohistochemical study with monoclonal antibodies against native and inactivated alpha 1-antichymotrypsin.

Monoclonal antibodies (mAbs) were raised against inactivated alpha 1-antichymotrypsin (ACT) to study the presence and functional state of the serine protease inhibitor alpha 1-antichymotrypsin in cerebral amyloid deposits in Alzheimer's disease. A panel of seven different mAbs was obtained; six of them were directed against neoepitopes that are expressed on ACT after interaction with proteases (inactivated ACT) and one mAb was directed against an epitope that is exposed both on native and inactivated ACT. The mAbs against neoepitopes could discriminate native ACT from complexed and inactivated ACT in vitro as shown in binding experiments in the presence of either native or inactivated ACT. With the mAbs against ACT we found that: (a) besides classical congophilic plaques, amorphous noncongophilic beta/A4-positive plaques were stained; (b) amorphous and classical plaques reacted with both types of mAbs against ACT indicating that this ACT was either complexed to a protease or proteolytically inactivated; (c) vascular amyloid was not stained for ACT. The presence of ACT in amorphous and classical plaques and its absence in vascular amyloid may indicate differences in the proteolytic degradation of preamyloid into amyloid fibrils. Our study strongly suggests that ACT is biologically active in amyloid plaques from an early stage.

Aged↗
PubMed1991

Cerebral amyloid plaques in Alzheimer's disease but not in scrapie-affected mice are closely associated with a local inflammatory process.

Complement proteins of the classical pathway can be immunohistochemically identified in cerebral amyloid plaques in Alzheimer's disease. Microglial cells in and around amyloid plaques express class II major histocompatibility (MHC) antigens and complement receptors CR3 and CR4. Negative immunostaining for immunoglobulins and for T-cell subsets in the brain parenchyma demonstrates a lack of evidence for the involvement of specific immune responses (such as an immune complex-mediated complement activation or a cell-mediated immune response) in cerebral amyloid deposits in Alzheimer's disease. Cerebral amyloid plaques in scrapie-affected mice (slow-virus induced encephalopathy) do not contain complement factors C1q and C3c and are not clustered with microglial cells expressing MHC class II molecules or complement receptor CR3. The data presented suggest the induction of a reactive inflammatory process by beta/A4 amyloid in the human brain, but not by scrapie-induced PrP amyloid in mice. Our findings do not support the hypothesis that the immune system is involved in the generation of amyloid plaques in Alzheimer's disease.

Aged↗
PubMed1990

Acute phase proteins are present in amorphous plaques in the cerebral but not in the cerebellar cortex of patients with Alzheimer's disease.

Using immunohistochemical staining for beta-amyloid proteins in the brains of Alzheimer patients two basic types of plaques can be found: 'amorphous' or 'diffuse' non-congophilic plaques and classical plaques. Acute phase proteins alpha 1-antichymotrypsin, complement factors and P-component ('plaque-associated molecules') have been reported in both types of plaques in the cerebral cortex. In the present study we could not find immunoreactivity for these acute phase proteins in the amorphous plaques in the cerebellar cortex. These proteins seem to be essential for formation of classical plaques.

Acute-Phase Proteins↗
PubMed1990

Inherited syndrome of microcephaly, dyskinesia and pontocerebellar hypoplasia: a systemic atrophy with early onset.

A neurodegenerative disease is reported in 5 related families, belonging to a Dutch genetic isolate. Seven children (5 females, 2 males) had microcephaly, spastic pareses, severe extrapyramidal dyskinesia and failure to acquire any voluntary skills. Four died during childhood. Marked pontocerebellar hypoplasia and progressive cerebral atrophy were found by computed tomography of the brain. Autopsy in one case revealed widespread, progressive loss of neurons affecting the olivopontoneocerebellar system more severely than any other part of the brain, accounting for the macroscopic pontocerebellar hypoplasia. A neocortical biopsy from another patient indicated that rough endoplasmic reticulum in neurons as the earliest ultrastructural target of the pathological process. This study confirms the disease as an inherited neuronal degeneration with very early, probably prenatal onset.

Atrophy↗
PubMed1989

Microglial cells around amyloid plaques in Alzheimer's disease express leucocyte adhesion molecules of the LFA-1 family.

Immunostaining for glycoproteins of the LFA-1 family (leucocyte function-associated antigens) was demonstrated on cells in the corona around senile plaques in Alzheimer's disease (AD) and in small glial cells in the subcortex of patients with AD and controls. These cells, which are usually referred to as microglial cells, showed positive immunohistochemical staining with monoclonal antibodies directed against the alpha-chains of all 3 LFA-1 family members, i.e. LFA-1, iC3b-receptor and P150,95, as well as with a monoclonal antibody against the common beta-chain. In the corona a diffuse staining for a ligand of LFA-1, intercellular adhesion molecule (ICAM)-1, was found as well. It is suggested that these molecules of the LFA-1 family may have a function in the dynamics of neuritic degeneration and sprouting.

Alzheimer Disease↗
PubMed1989

Complement activation in amyloid plaques in Alzheimer's dementia.

Amyloid plaques in Alzheimer's dementia contain complement factors C1q, C4 and C3. In the present study we demonstrate complement activation in amyloid plaques using immunoenzymatical techniques and specific antibodies against subunits of individual complement components and activated complement products. Amyloid plaques contain C1q and activated C3 fragments (C3c and C3d, g) but no C1s and C3a. These findings demonstrate that the complement components are not passively bound to the amyloid plaque structures but are the result of an activation process. The role of complement activation in the genesis of senile plaques is discussed.

Aged↗
PubMed1989

A4 protein in Alzheimer's disease: primary and secondary cellular events in extracellular amyloid deposition.

This study was designed to investigate the role of serum proteins, microglia, glial fibrillary acidic protein (GFAP) positive cells and dystrophic neurites in the genesis of cerebral amyloid. Using A4 protein antisera, we found an amorphous non-congophilic, form of plaque, which was not seen in Bielschowsky silver staining or Bodian impregnations. GFAP-positive glial cells, cells immunolabelled for some macrophage markers and dystrophic neurites were detected in congophilic plaques with crystalline amyloid, but not in the amorphous, non-congophilic plaques. The presence of alpha l-antichymotrypsin, complement factors and P component, but not of common serum proteins in both the amorphous and congophilic plaques, indicates that these three proteins may have a pathogenetic role in amyloid formation. Amorphous plaques may be the earlier forms of plaque and consequently, the presence of reactive cells and dystrophic neurites may be secondary phenomena.

Alzheimer Disease↗
PubMed1988

Gliomatosis cerebri in a newborn.

A newborn is reported with diffuse gliomatosis involving the cerebral hemispheres, the brainstem and the cerebellum. The presenting signs were paucity of spontaneous movements except for multifocal clonic seizures, absent response to sensory stimuli and optic atrophy. A CT scan suggested agyria. The child expired on the seventh day. Autopsy disclosed diffuse gliomatosis affecting both cerebral hemispheres, the brainstem and the whole cerebellum, but excluding the spinal cord. Neuronal loss was unusually severe in all the affected areas. The genitals were ambiguous, an association not explained by the cerebral pathology. The karyotype was 46XY (male pseudohermaphroditism). This is probably the first reported instance of gliomatosis cerebri in a newborn.

Brain↗
PubMed1988

The rigid spine syndrome in two sisters.

Two half-sisters aged 14 and 18 years are described with a rigid spine syndrome as the cardinal clinical feature of an autosomal dominant neuromuscular disorder. Ten years previously, a diagnosis of multicore disease had been made from the clinical signs and muscle biopsy findings. Long term follow-up revealed a non-specific muscular dystrophy with axial predominance and a rigid spine in the younger girl; the older sister presented at the age of 18 with a rigid spine as the only myopathic sign. Computed tomography of the muscles showed severe involvement of the paraspinal musculature, in contrast with either less or no involvement of the other muscles.

Adolescent↗
PubMed1986

Role of microglia in plaque formation in senile dementia of the Alzheimer type. An immunohistochemical study.

Using immunohistochemical and enzyme histochemical methods, we have investigated the presence of mononuclear phagocytic cells around senile plaques in six brains from patients with senile dementia of the Alzheimer type (SDAT). It is generally supposed that reactive microglial cells are involved in amyloid formation "as representatives of the reticuloendothelial system in the brain." We used different monoclonal antibodies directed against cells of the mononuclear phagocyte lineage, antibodies against the macrophage markers alpha 1-antichymotrypsin and lysozyme, and the lectin WGA, in addition to enzyme histochemical staining for nonspecific esterase and acid phosphatase. It was concluded that no macrophages of the mononuclear phagocyte lineage are involved in plaque formation. The role of glial cells in amyloid formation is discussed.

Acid Phosphatase↗
PubMed1986

Age incidence of senile brain amyloidosis.

Neuropathological examination of 1400 successive autopsies in general and mental hospitals revealed that senile plaques and congophilic angiopathy are age related phenomena. There is, however, a remarkable difference between the two types of manifestation of senile amyloidosis. There was a significantly higher incidence of senile plaques in females. Moreover the increase of the incidence with age was also significantly higher in females. Congophilic angiopathy showed no predominance in females. In total 59% of males and 55% of females with senile plaques suffered from Senile Dementia of the Alzheimer Type (SDAT). SDAT appeared to be also an age related phenomenon characterized by a linear increase with age and a predominance in females.

Age Factors↗
PubMed1984

An immunohistochemical study on cerebral vascular and senile plaque amyloid in Alzheimer's dementia.

Senile cerebral amyloidosis has been investigated using immunoperoxidase and enzyme histochemical techniques in six unfixed brains. Our findings do not support the opinion that vascular and senile plaque amyloid are immunoglobulin-derived. In contrast with recent reports we did not detect prealbumin in senile plaques and congophilic angiopathy lesions. All senile plaques contain complement factors C1q, C3 and C4. The highest peroxidase activity was found in the amyloid nucleus but the corona also showed evident peroxidase activity.

Aged↗
PubMed1984

Progressive multifocal leukoencephalopathy presenting as a single mass lesion.

Progressive multifocal leukoencephalopathy (PML) consisting anatomopathologically of multiple foci of white matter demyelination may present both clinically and at CT scanning as a single mass lesion. An additional similar case is reported. As antiviral therapy may prove useful, characteristics of additional examinations including CT scan are described to promote an early diagnosis.

Brain↗
PubMed1984

Interhemispheral neuroepithelial (glio-ependymal) cysts, associated with agenesis of the corpus callosum and neocortical maldevelopment. A case study.

Callosal agenesis and interhemispheral cysts containing ependyma, choroid plexus and glial cells are reported in a male infant. To the authors' knowledge this represents the 4th recorded case. Other findings were: nodular neuronal heterotopias with fully differentiated spiny neocortical neurons, widespread neocortical microgyria, cerebellar hypoplasia and bony abnormalities of the foramen magnum and atlas. This study confirms that glio-ependymal cysts are heterotopias of embryonic ventricular epithelium. The existence of more than one type of heterotopia (i.e. neuronal and ependymal) in 1 individual is significant. This indicates an error of cell generation and cell migration affecting the original structure from which both cell types are derived: the embryonic (sub)ventricular zone.

Agenesis of Corpus Callosum↗