Chordoid Meningioma. Case Report and Review of the Literature.

Chordoid meningioma is an uncommon histopathological type of meningioma, frequently associated with Castleman's syndrome. Histologically, chordoid meningiomas are similar to chordomas. Because of their high proliferative index, they present aggressive biological behavior and high risk of postoperative recurrence. We report a case of chordoid meningioma in an adult patient without Castleman's syndrome manifestation. As its chordoid feature is related with a rapid recurrence after incomplete removal, meticulous histopathological examination is crucial for the adequate postoperative treatment plan.

The development of cerebral amyloid angiopathy in cerebral vessels. A review with illustrations based upon own investigated post mortem cases.

The process of ß-amyloid accumulation in cerebral vessels is presented. Cerebral amyloid angiopathy (CAA) was confirmed during an autopsy. It was diagnosed according to the Boston criteria. Cerebral amyloid angiopathy can involve all kinds of cerebral vessels (cortical and leptomeningeal arterioles, capillaries and veins). The development of CAA is a progressive process. ß-amyloid appears first in the tunica media, surrounding smooth muscle cells, and in the adventitia. ß-amyloid is progressively accumulated, causing a gradual loss of smooth muscle cells in the vessel wall and finally replacing them. Then, the detachment and delamination of the outer part of the tunica media results in the "double barrel" appearance, fibrinoid necrosis, and microaneurysm formation. Microbleeding with perivascular deposition of erythrocytes and blood breakdown products can also occur. ß-amyloid can also be deposited in the surrounding of the affected vessels of the brain parenchyma, known as "dysphoric CAA". Ultrastructurally, when deposits of amyloid fibers were localized in or outside the arteriolar wall, the degenerating vascular smooth muscle cells were observed. In the Institute of Psychiatry and Neurology the study was carried out in a group of 48 patients who died due to intracerebral hemorrhage caused by sporadic CAA.

Pentylenetetrazol-kindling of seizures selectively decreases [3H]-citalopram binding in the CA-3 area of rat hippocampus.

The present study was aimed at determining the changes in the 5-HT transporter activity, in different brain structures after pentylenetetrazol induced kindling of seizures. We examined [3H]-citalopram binding in the rat brain structures, and the neurodegenerative effects in the hippocampal formation using autoradiographic and immunohistochemical methods. A statistically significant and selective reduction in the binding of [3H]-citalopram was found in the CA3 field of the hippocampus (P=0.009), and a similar tendency, close to the significance level, in the dentate gyrus (P=0.05). This effect was accompanied by a loss of neurons and activation of microglia in the hippocampal formation. The present data suggest the important role for CA3- serotonergic innervation in pentylenetetrazol induced kindling of seizures.

Report on the first polish case of the Gerstmann-Sträussler-Scheinker syndrome.

In the course of epidemiological studies on Creutzfeldt-Jakob Disease in Poland, the authors found a male patient aged 54 years with dementia rapidly progressing for a year and ataxia of the extremities. EEG tracings were abnormal but without features typical of CJD. About six months after hospitalisation the patient died. Neuropathological examination of his brain demonstrated spongiform lesions of medium intensity present mainly in the cortex of frontal and occipital lobes, with slight proliferation of astroglia. In the cerebellar cortex numerous deposits of PAS-positive substance amorphous or in the shape of kuru plaques were disclosed. A smaller number of these plaques were found in the cortex of occipital and temporal lobes, and in the putamen. All deposits stained strongly with monoclonal 3F4 antibody to human prion protein (PrP). Genetic studies disclosed in the 20th chromosome, in the PrP gene, mutation at codon 102 (P102L). Codon 129 was homozygous for methionine (M129M). It was established, moreover, that patient's father had at the same age a similar disease and died after one year and patient's sister died after a six-year-long neurological disease diagnosed as multiple sclerosis. On the basis of clinical, genetic and neuropathological findings the authors diagnosed the Gerstmann-Sträussler-Scheinker syndrome, a familial prion disease with autosomal dominant character. This is the first report on this syndrome in Poland.

Major histocompatibility complex class II (MHC II) expression in the normal and pathological human foetal spinal cord.

The ability of the specific immune response of organisms is determined by the possibility of synthesis, transport and presentation of the major histocompatibility complex class II (MHC II) molecules on the surface of antigen-presenting cells. MHC II molecules are responsible for the binding, transport and presentation of a foreign antigen to helper T lymphocytes. They also stimulate the multiplication of specific B lymphocytes and determine the type of antibodies produced. The expression of MHC II molecules on the cellular surface of the spinal cord in cervical, thoracic, lumbar and sacral regions was studied on 30 normal human foetuses between 11 and 22 weeks of gestation (GW) and 9 foetuses with genetic defects (Down syndrome, mucopolysaccharidosis, mucoviscidosis or Nori's syndrome) between 17 and 22 GW. The immunocytochemical presence of MHC II molecules was found in all regions of the spinal cord in both groups of foetuses, normal and pathological, during the whole interval under study. The molecules were dispersed in the grey and white matter of the spinal cord and located most frequently on the surface of cells, near the central canal and blood vessels. These cells corresponded morphologically and immunocytochemically with amoeboid and ramified microglia. No differences in MHC II expression between the spinal cord regions or between normal foetuses and those with genetic defects were noted. It seems likely that such an early occurrence of MHC II expression in the spinal cord of foetuses with normal development, as well as the absence of abnormalities in this expression in foetuses with genetic defects, may indicate the significant role of these molecules in the immunological protection of the foetus and thus ensuring normal embryogenesis.

Lewy body variant of Alzheimer's disease and Alzheimer's disease: a comparative immunohistochemical study.

Immunohistochemistry (IHC) and ultrastructural study were performed on 19 demented autopsy cases of sporadic Alzheimer's disease (AD). Semiquantitative IHC assessment of the pathological changes, according to the criteria of the Consortium to Establish a Registry of Alzheimer's Disease (CERAD) and the Consortium on Dementia with Lewy Bodies, showed morphological hallmarks of AD in 18 demented patients. It was found that 11 of these cases fulfilled criteria for "pure" AD, whereas the remaining 7 cases, with mixed findings, Lewy bodies (LBs) and Lewy-related dystrophic neurites, neuritic plaques (NP) and sometimes neurofibrillary tangles (NFT), met the criteria for Lewy body variant of Alzheimer's disease (LBV). One case with brain stem and cortical LBs but without NP and NFT was finally diagnosed as a pure form of dementia with Lewy bodies (DLB). Regional distribution and semiquantitative assessment frequency of alpha-synuclein-immunoreactive LBs, tau-immunoreactive NFT and beta-amyloid immunoreactive senile plaques, were compared between LBVand AD. Ultrastructural examination confirmed the filamental structure of cortical LBs. In conclusion, IHC study including antibody to alpha-synuclein, the sensitive marker for Lewy bodies, revealed the coexistence of brain stem and cortical LBs and pathological features of AD in a great part of dementia cases. Patients with mixed, LBs, NP and sometimes NFT pathology, fulfilled neuropathological CERAD criteria for LBV. Semiquantitative comparative IHC study, according to LBs- and NFT-scores and CERAD NP-scores showed in the LBV group a significantly lower frequency of NFT coexisting with neocortical LBs than in the group with pure form of AD.

Neurones and microglia in central nervous system immune response to degenerative processes. Part 1: Alzheimer's disease and Lewy body variant of Alzheimer's disease. Quantitative study.

The quantitative correlation between neurone loss and brain immune response, assessed by intensity of microglia inflammatory reaction in cortical association area and limbic cortex, was investigated and compared in previously immunohistochemistry (IHC) and ultrastructural confirmed 11 cases of Alzheimer's disease (AD), 7 cases mixed form of Dementia with AD findings and Lewy bodies (AD/DLB) reported, in accordance with Consortium on Dementia, as Lewy body variant of AD (LBV) and 6 non-demented autopsy control cases from 63 to 86 years old. In the present work we investigated association and limbic cortical areas linked with memory mechanisms; there are regions characterised by early distribution of IHC confirmed AD and DLB/AD (LBV) markers, as well as a substantial physiological stability of neurone pool regardless of age. The results indicated that AD and LBV differ in their neurone loss intensity and inflammatory reaction, with much higher intensity in AD. In Alzheimer's disease, neurone loss in association temporal cortex made up 51% of control values with simultaneous 8-fold increase in the density of MHC II-positive activated microglia, whereas in LBV, both the loss of neurone density and the increase in activated microglia density, was not so high (up to 41% and 4-5-fold, respectively). Changes in the limbic cortex were less pronounced. A strong correlation in the clinical material between neurone loss and microglia activation in both processes, especially in AD (r = 0.73), speaks in favour of the hypothesis on the neuronal immune surveillance and arousal of immune brain response in conditions of declining control, due to significant neurone loss in the neurodegenerative process. The inflammatory reaction of MHC II-immunoreactive microglia, concomitant with neurodegenerative process, seems to be a consequence of increased immune response due to loss of neurones and weakening of their control upon immunosurveillance in central nervous system.

Disseminating histologically benign multiple papilloma of the choroid plexus: case report.

A rare case of extensively disseminating multiple benign choroid plexus papilloma is shown. The patient first reported with high-grade hydrocephalus and two tumours in the 3rd and 4th ventricle was treated by atrioventricular shunt insertion, subtotal resection of the 4th ventricle tumour, and adjuvant 60Co irradiation of the posterior fossa. The dissemination that followed was revealed by computerised tomography and magnetic resonance imaging, and involved both the supra- and infratentorial ventricular systems, spinal canal, and brain parenchyma. Three years after the resection of the 4th ventricle tumour, the patient underwent excision of a temporal lobe lesion for relief of neurological symptoms, but showed no improvement and died 5 years after the primary diagnosis of CNS tumour. An autopsy was not performed. Analysis of the primarily resected mass showed distinct papillary pattern with no anaplasia, mitoses, multinucleation orgiant cell formation, and cytokeratin positivity at the absence of vimentin and glial fibrillary acidic protein. Analysis ofthe temporal lobe tumour again showed definite papillary formation with no signs of malignisation and virtually no mitotic figures, and the presence of cytokeratin, but not vimentin or glial fibrillary acidic protein. On both occasions, the diagnosis was choroid plexus papilloma (WHO grade I).

Major histocompatibility complex class II expression in the frontal and temporal lobes in the human fetus during development.

Antigen-presenting cells (APCs) that show the expression of major histocompatibility complex (MHC) class II molecules in adult persons are related to an early phase of immunological response. These molecules are responsible for the binding, transport, and presentation of a foreign antigen to helper T lymphocytes and determine the type of antibodies produced. They also stimulate the multiplication of specific B lymphocytes and participate in the elimination of autoreactive lymphocytes and maturation of T lymphocytes. Cells with MHC II molecules expressed on their surface were observed in the frontal and temporal lobes of 30 brains of human fetuses with normal development between gestation weeks (GW) 11 and 22. MHC II expression was noted during the whole interval under study. Its immunocytochemical localization was noted on the surface of the cerebral meninges cells, in the choroid plexus of the lateral ventricle and blood vessel lumen, and in ameboid microglia (AM) and ramified microglia (RM) cells in both cerebral lobes of the human fetus. The expression of MHC II that occurred on the cells of the central nervous system (CNS) already in GW 11 may be evidence not only of an early capability of immunological protection of the fetal nervous system, but also of a significant role potentially played by this system in normal embryogenesis. Despite continuous controversy over the cellular lineage of microglia origin, the expression of MHC II on AM and RM cells indicates its mesodermal origin, as in other APCs.

Major histocompatibility complex class II (MHC II) expression during the development of human fetal cerebral occipital lobe, cerebellum, and hematopoietic organs.

In adults, under physiological conditions proteins of the major histocompatibility complex, class II (MHC II) molecules are synthesized and then presented on the surface of the cells known under a common name as antigen presenting cells (APCs). Dendritic cells (DCs), microglia, macrophages, ameboid microglia and lymphocytes B are qualified as APCs. The aim of present study was to evaluate the expression of MHC II molecules in the central nervous system (CNS) and hematopoietic organs during the fetal development. Observations were made on the cerebral occipital lobe, cerebellum, thymus, spleen and liver of 30 normal human fetuses, between 11 and 22 week of gestation (GW). Histological, histochemical and immunohistochemical techniques were used to identify cells with expression of MHC II molecules. In the brain, MHC II molecules were detected on macrophages/ameboid microglia in meninges, choroid plexus and single cells of ramified microglia in deeper layers of the cortex and white matter. In the other organs besides macrophages and dendritic cells, MHC II molecules were also immunopositive in thymic epithelial cells, and in the spleen and liver also in other cells of stroma and lobule. The expression of MHC II molecules on so extensive population of cells, at an early stage of the fetal development, may evidence their significant involvement in histogenesis and morphogenesis. It seems that in adults the complex of MHC II with protein is originated from the foreign antigen. On the contrary, during normal fetal development the complex of MHC II with protein origins most probably from the fetus own structures.

The significance of immunocytochemical markers, synaptophysin and neurofilaments in diagnosis of ganglioglioma.

Ganglioglioma is a tumor composed of neoplastic neurons and neoplastic glial cells mixed in different proportions. Astrocytes are the essential glial component. The tumor proliferates mostly in the temporal lobe cortex, scarcely in other areas of the brain. In ganglioglioma the population of ganglionic neurons is very difficult to diagnose. In the last ten years, immunocytochemical reaction with synaptophysin and neurofilament protein (NFP) triad considered as neoplastic neuron markers seemed to differentiate neoplastic nerve cells from normal neurons occurring incidentally within neoplastic proliferation area. In our study, the reaction with synaptophysin and NFP was performed on fragments of normal frontal lobe, pons and cerebellum as well as on fragments of post-operation tumor diagnosed as ganglioglioma. A positive synaptophysin reaction was obtained on the surface of neoplastic neurons in gangliogliomas, as well as on the surface of normal neurons in the encephalon, cerebellum and pons. Both neoplastic and normal neurons and their processes showed the expression of neurofilaments protein triad. Thus, a positive reaction of neurons with synaptophysin and NFP seems to be nonpathognomic in the diagnosis of the neoplastic neuron population in gangliogliomas.

Dysembryoplastic neuroepithelial tumor. A case report.

Dysembryoplastic neuroepithelial tumor (DNT) is a rare, benign tumor encountered in the cortex. It is characterized by the presence of cells of different histogenesis. Due to its mixed nature (glial-neuronal), WHO histological classification of brain tumors included it into the group of neuronal and glial-neuronal mixed tumors. Case of tumor in a 19-year-old woman experiencing for three years seizure of temporal lobe epilepsy is presented. A cranial magnetic resonance imaging (MRI) showed "pseudocystic" tumor in temporal lobe. Histological and immunocytochemical examinations of the tumor fragment removed during surgery revealed large numbers of neuronalglial nodules occurring in the cerebral cortex. Columns of glial-neuronal structures crossing parallely to the cortex surface, surrounded by oligodendrocyte-like cells (OLC) were a characteristic feature of the tumor texture. In the tumor interstitium, "floating" maturated, dysplastic-free ganglionic cells were visible in numerous bright spaces. In addition, numerous lobuliform--structured areas consisted of oligodendrocyte-like cells. Oligodendrocyte-like cells were characterized by positive immunoreaction to the presence of S-100 protein and synaptophysin. Basing on clinical manifestation and histopathological findings dysembryoplastic neuroepithelial tumor was diagnosed.

Electron microscopic and immunohistochemical verification of diagnosis in two archival cases recognized as encephalitis necroticans acuta (ENA) on routine examinations.

Two archival cases diagnosed 20 years ago on routine neuropathological methods as Encephalitis Necroticans Acuta (ENA) were investigated in EM and by immunohistochemical methods. The previous diagnosis was confirmed only in one case because Herpes simplex virus was found. In the second case the intracellular inclusions visible in ME corresponded to Measles Virus thus previous diagnosis was changed to SSPE.

The comparison of microglia maturation in CNS of normal human fetuses and fetuses with Down's syndrome.

The study was performed on the tissues derived from the central nervous system (CNS) of 72 normal human fetuses between 8 and 22 week of gestation (GW) and 30 fetuses with genetically confirmed Down's syndrome between 17 and 22 GW. Histochemical, immunocytochemical and ultrastructural examinations of microglial cells in frontal lobe, mesencephalon and cerebellum were carried out. A quantitative evaluation of developing microglia was performed in comparison with astroglial cells by counting the mean number of cells per 1 mm2. The study indicated that microglial cells emerge at the same time in all structures under study, both in normal fetuses and in those with Down's syndrome. It was also found that ameboid microglia (AM) and ramified microglia (RM) emerge at the same time and show the same morphological structure in both groups of fetuses. It was revealed that in the CNS of fetuses with Down's syndrome, the number of ramified microglial cells increased significantly as compared with in normal fetuses. Astroglial cells outnumbered microglial cells in the normal fetal development. Due to the enhanced number of RM cells in the CNS of fetuses with Down's syndrome the quantitative difference between these cells obliterated, and microglial cells in the frontal lobe cortex even outnumbered astroglial cells.

Border zone neovascularization in cerebral ischemic infarct.

Immunocytochemical and quantitative studies on vascular reaction (angiogenesis) in cortical border zone of infarct were undertaken. Intensity and temporal profile of angiogenesis was assessed in 60 patients aged between 48 and 69 (younger group), and between 70 and 92 (older group), with cerebral infarct in the area of middle cerebral artery vascularization, who died during the first six weeks following the stroke. We have found that angiogenesis was a multistage process in which four stages were distinguished: phase of primary activation of endothelial cells, two consecutive phases of active angiogenesis and final phase of only sporadic proliferation of vessels. The distinction of phases in a multiphase angiogenic cascade helped us to evaluate the correlation with survival time and the age of patients. The most pronounced intensification of angiogenesis and increased density of CD 31 positive capillaries in penumbra were observed in the second phase, especially in younger patients. The duration of the penumbral neovascularization decreased in the older age patient. Our results indicate that sprouting angiogenesis is a quantitatively significant source of vessels in the cerebral infarct border zone. However, non-therapeutically stimulated angiogenesis developed only 3-4 days after the stroke, that is beyond the period of reversible changes in ischemic penumbra recognized as a "therapeutical window" in the human brain. The angiogenic therapy opens a new way towards the revascularization of ischemic brain infarct.

The comparison of microglia maturation in different structures of the human nervous system.

The aim of the study was to find out whether differences in morphology and time-sequence of microglia appearance in course of development of the phylogenetically different structures of the central nervous system (CNS) in normal human fetus do exist. An attempt was also made to evaluate quantitatively the development of microglial cells in comparison to astroglia, taking into account their role in the structural and immunological maturation of the CNS. The study was performed on CNS tissue of frontal lobes, mesencephalon and cerebellum from 72 fetuses between 8 and 22 week of gestation (GW). Histochemical and immunohistochemical reactions were used as basic study methods. A quantitative evaluation of developing microglia and astroglia in all investigated structures was performed by counting the mean number of cells per 1 mm2. Morphological and ultrastructural patterns of the three basic types of microglia; ameboid, ramified active and ramified resting, were characterized. It was indicated that they emerge at the same time in all structures under study, except the ameboid microglia arising earlier in the mesencephalon. A quantitative evaluation revealed that the number of ameboid microglial cells decreased slightly in an early stage of fetal development. The number of ramified microglial cells between 11 and 22 GW increased in all structures. The highest values of ramified microglia were found in mesencephalon, and the lowest in white matter of cerebellum. The number of astroglial cells exceeded the increase in ramified microglia by several times in all structures.

A case of the subacute brainstem encephalitis.

A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. Basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.

[Morphologic analysis of of microglia in astrocytomas]. / Analiza morfologiczna mikrogleju w guzach pochodzenia astrocytarnego.

Forty human primary brain astrocytoma tumours (anaplastic and non anaplastic) were subjected to correlative light and electron microscopic studies of microglia in tumour tissue and in its surroundings. It was found that a great number of microglia cells were present in the all types of astrocytomas. In the non anaplastic tumours (fibrillary, protoplasmic, gemistocytic) ramified microglia mostly was observed. In glioblastomas and anaplastic astrocytomas the greatest number of ameboid microglia and very rarely ramified microglial cells were found. It is suggested that the differences between the various kinds of astrocytomas determine the difference in the type of microglial reaction. It is assumed that this might be caused by the differences in secreting some factors by these tumour astrocytes.

A morphometric evaluation of morphological types of microglia and astroglia in human fetal mesencephalon.

Quantitative changes associated with the differentiation of microglial and astroglial cells in 48 fetal mesencephalons between 8 and 22 week of gestation (GW) were studies. Using lectin (RCA-1) labelling, two morphological types of RCA-1 positive cells, ameboid microglia (AM) and ramified microglia (RM) were identified on the basis of the cell body shape and the configuration of cytoplasmic processes. Astrocytes (AS) were identified by immunocytochemical labelling of GFAP. Measurements of microglial and astroglial cells were carried out in mesencephalon tectum and tegmentum under the microscope in sequential segments corresponding to equal sized non-overlapping areas. The quantitative data about concerning the number and percentage distribution of AM, RM and AS from each of the grid-quartiles were analyzed. The study revealed a time-sequence of appearance, characteristic pattern of distribution along perpendicular and longitudinal axes originating from aqueduct and changes in percent distribution of both types of microglial cells and astrocytes. Ameboid type of microglia was already present in 8 GW fetus in tectum (22.5 cells/mm2) and in tegmentum (10.2 cells/mm2). During the fetal development the number of AM peaked about 8-9 GW in tectum, accounting for 29 cells/mm2 close to aqueduct, and about 11-12 weeks in tegmentum, reaching 10.2-11 cells/mm2 close to aqueduct. As the fetus development advanced, the number of AM cells both in tectum and tegmentum was slowly dropped down reaching about 1.0 cells/mm2 in 20-22 GW. The ramified microglial cells as well as astroglial cells emerged in the fetal mesencephalon after 11 GW. A quantitative study also revealed a rapid increase in the density of RM in 13-16 GW (6.3 cells/mm2-close to aqueduct) and AS cells in 13-16 GW (36 cells/mm2-close to aqueduct) and later a decrease in cell number was observed. Similarly to AM, changes in the number of RM and AS cells were stabilized to about 20-22 GW. The percentage distribution of each type of microglia and astrocyte cells both in tectum and tegmentum differed markedly during the fetus development depending on the localization along the axes.