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  • 1
    ISSN: 1432-0533
    Keywords: Epilepsy ; Pathology ; Tumor ; Malformation ; Inflammation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The surgical treatment of chronic pharmacoresistant epilepsies is increasing rapidly. Although several studies have reported on histopathological findings in temporal lobe epilepsy, little is known about the surgical pathology of other seizure disorders. Here we report the histopathological fingings in 63 consecutive surgical specimens of patients who were operated for chronic pharmacoresistant epileptic seizures other than temporal lobe epilepsy (37 corticectomies, 19 functional hemispherectomies, 5 lobectomies, 1 multilobectomy, and 1 frontal lobe deafferentiation combined with a temporal lobectomy). There were structural lesions in 85.7% of the specimens. In 16 cases (25.4%) the predominant lesions were malformative (focal glioneuronal hamartias and hamartomas, vascular malformations, abundant ectopic neurons in the white matter, microgyria, and arachnoid cyst). Lesions indicating preor perinatal necrosis such as porencephaly, ulegyria, and congenital hemiatrophy were present in 7 cases (11.1%). Twelve specimens (19.0%) contained low-grade neoplasms (7 gangliogliomas, 3 astrocytomas, 1 oligoden-droglioma and 1 oligoastrocytoma). There were 3 cases of Rasuussen encephalitis, 1 specimen with atrophy and gliosis due to previous herpetic encephalitis and 1 case with an old abscess wall. Posttraumatic or postoperative changes were the predominant finding in 7 specimens (11.1%). In 7 patients there were only nonspecific changes such as cortical atrophy and gliosis or old hemorrhage. No structural alterations were identified in 9 specimens (14.3%). The findings suggest that the structural lesions observed in the great majority of the specimens were closely related to the pathogenesis of intractable seizures.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words: Ganglioglioma ; Hamartia ; Proliferation ; Ki-67 ; p53
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Gangliogliomas are tumors composed of intimately admixed neuronal and glial components and account for approximately 1   % of all brain tumors. Here we report the histopathological findings in 61 gangliogliomas. Epilepsy was the most common presenting symptom. Most gangliogliomas were located in the temporal lobes (74 %). Thirteen percent of the gangliogliomas were associated with glioneuronal hamartias. There was considerable variation in neuronal size and density, presence of binucleated neurons, calcifications, desmoplasia, lymphocytic infiltrate, pilocytic differentiation, Rosenthal fibers, location, or histological uniformity. Fifteen percent of the gangliogliomas contained areas of purely astrocytic differentiation. All tumors were examined immunohistochemically for an aberrant p53 tumor suppressor gene product and for the presence of nuclear antigens associated with cell proliferation (Ki-67, Ki-S1, proliferating cell nuclear antigen). In 45 of 61 cases (74   %) labeling indices for Ki-67 were less than 1   %. Nuclear labeling for Ki-67 was observed exclusively in the astrocytic component. Gangliogliomas with very large neurons had higher Ki-67 labeling indices and occurred in younger patients than gangliogliomas with small – or intermediate – sized neurons. None of the tumors had an aberrant expression of p53. The observations suggest that gangliogliomas may arise from glioneuronal hamartias through neoplastic transformation of the astrocytic component.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Epilepsy ; Gamma aminobutyric acid ; Receptor ; Ammon's horn sclerosis ; Hippocampus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Alterations of gamma aminobutyric acid (GABA)-mediated neurotransmission have been implicated in the pathogenesis of epilepsies. Here we examine the distribution of the GABAA receptor in the hippocampus of 78 surgical specimens from patients with chronic pharmacoresistant focal epilepsies. The receptor was localized immunohistochemically with the monoclonal antibody bd-24 which selectively recognizes the α1 subunit of the GABAA receptor. The results were compared with the receptor distribution of 28 normal hippocampal specimens obtained at autopsy. In the great majority of the surgical specimens a loss of GABAA receptor immunoreactivity was present in CA1 (92.3  %), CA4 (78.2  %), the dentate granular cell layer (70.5  %) and the molecular layer of the dentate gyrus (65.4  %). The subiculum revealed a normal staining pattern in all but 4 cases. In no instance did we observe an increase of immunoreactivity in any region or cell population. The decrease of GABAA receptor immunoreactivity was closely related to neuronal loss in the respective specimen and to Ammon's horn sclerosis. There was no correlation between GABAA receptor loss and the patient's age at surgery, duration of seizures, age at onset of seizures and to the presence or absence of secondary generalized tonic clonic seizures. The data suggest that the observed loss of GABAA receptor immunoreactivity is a secondary phenomenon rather than an event that is relevant for the pathogenesis of epileptic seizures.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European radiology 8 (1998), S. 1197-1197 
    ISSN: 1432-1084
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 91 (1996), S. 376-384 
    ISSN: 1432-0533
    Keywords: Key words Epilepsy ; Immunohistochemistry ; Neurotransmitter ; Pathology ; Tumor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Circumscribed cortical lesions are frequently encountered in patients with chronic focal epilepsies. However, the pathogenesis of seizures is poorly understood. To determine whether the perilesional cortex shows evidence for abnormal excitatory or inhibitory neurochemical activity, we immunohistochemically examined the distribution of the α1 subunit of the GABAA receptor (GABAR), the N-methyl-d-aspartate receptor subunit 1 (NR1), and glutamate decarboxylase (GAD) in 30 surgical specimens of neocortical epilepsy-associated lesions. These comprised 7 low-grade gliomas, 2 gangliogliomas, 2 dysembryoplastic neuroepithelial tumors, 4 glioneuronal malformations, 5 vascular malformations, and 10 glial or gliomesodermal scars. All specimens originated from patients with chronic pharmacoresistant epilepsy. In 73% of the cases there was a distinct difference in immunoreactivity for GABAR, GAD or NR1 between the perilesional zone and the normal cortex. With each of the markers there was reduced perilesional immunoreactivity in 30% of the specimens. Increased staining for GAD was seen in 17%, for GABAR in 7%, and for NR1 in 13% of the cases. The age at surgery, onset of seizures, epilepsy duration, and maximal seizure frequency did not differ significantly between patients with normal and those with altered perilesional immunoreactivity patterns. Although the perilesional changes for GAD, GABAR or NR1 were heterogeneous, they suggest a disturbed balance between excitatory and inhibitory synaptic transmission which may contribute to the pathogenesis of focal seizures.
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  • 6
    ISSN: 1432-0533
    Keywords: Key words Chromosome 9 ; Chromosome 16 ; Epilepsy ; Hamartoma ; Pathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Glioneuronal malformations with a striking histological resemblance to cortical tubers of tuberous sclerosis, but no extracerebral stigmata of this phacomatosis, are frequently encountered in patients with chronic pharmacoresistant epilepsies. It is controversial as to whether these lesion represent a forme fruste of tuberous sclerosis or a distinct entity. The recently reported loss of heterozygosity (LOH) at the regions of the TSC1 or TSC2 locus in hamartomas obtained from different organs of patients with established tuberous sclerosis, including cortical tubers, stimulated us to examine epilepsy-associated tuberous sclerosis-like glioneuronal malformations with respect to LOH at the TSC1 and TSC2 loci of chromosomes 9q34 and 16p13.3, respectively. The analysis was carried out on DNA derived from paraffin-embedded brain tissues of 11 patients. For 5 patients, peripheral blood leukocytes were also available for DNA extraction. We performed microsatellite analysis with five markers on chromosome 9 and four markers on chromosome 16. In addition, polymerase chain reaction-restriction fragment length polymorphism (RFLP) analysis was performed using a polymorphic EcoRV restriction site in exon 40 of the TSC2 gene. No LOH was identified in any of the cases. These findings do not support a relationship between the epilepsy-associated glioneuronal lesions and tuberous sclerosis. However, tuberous sclerosis is genetically heterogeneous and microsatellite and RFLP analysis cannot exclude small deletions or point mutations. Thus, given the histopathological similarity of glioneuronal malformations in epilepsy patients to cortical tubers, further molecular genetic studies will be needed as our understanding of the molecular basis of tuberous sclerosis increases to completely clarify the relationship of these lesions to tuberous sclerosis.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1920
    Keywords: Key words Creutzfeldt-Jakob disease ; Prion disease ; Magnetic resonance imaging ; Dementia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To ascertain whether increased grey matter signal intensity on T2-weighted images in patients with sporadic Creutzfeldt-Jakob disease (CJD) corresponds to the stage and severity of this disease, we correlated MRI findings in four of our own and previously reported patients with sporadic CJD with the clinical variants, neuropathological changes at autopsy, duration of the disease and survival time after MRI examination. Of 15 patients with the extrapyramidal type of CJD, 10 showed increased signal in the basal ganglia on T2-weighted images. One of seven patients with the Heidenhain variant had increased signal in the occipital cortex. Patients without increased grey matter signal intensity had a longer overall duration of CJD (P = 0.035). Although the interval between onset of neurological symptoms and MRI was not different, patients without increased grey matter signal also survived longer after MRI examination (P = 0.022).
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1920
    Keywords: Key words Sarcoidosis ; Magnetic resonance imaging ; Brain neoplasm
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Sarcoidosis may also present as an extra- or intra-axial mass involving the central nervous system. These lesions are sometimes operated upon, because a neoplasm is suspected. We report two cases of unusual tumour-like extra- and intra-axial sarcoidosis. The extra-axial mass was just medial to the jugular foramen. Its morphology and signal characteristics differed from the more common lesions in this area. The intra-axial mass was in the temporal lobe, with only minor leptomeningeal involvement. Extra-axial sarcoidosis can be confused with a meningioma because these lesions can give relatively low signal on T2-weighted images. Intra-axial masses are presumed to represent a propagation and fusion of multiple leptomeningeal granulomas through the Virchow-Robin spaces in the brain; this pattern can be sought on contrast-enhanced T1-weighted images.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Der Pathologe 16 (1995), S. 245-255 
    ISSN: 1432-1963
    Keywords: Schlüsselwörter WHO-Klassifikation der Gehirntumoren ; Gradierung ; Immunhistochemie ; Proliferationsbestimmung ; Molekulargenetik ; Key words WHO classification of brain tumors ; Grading ; Immunohistochemistry ; Proliferation markers ; Molecular genetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary In recent years there has been considerable progress in brain tumor neuropathology. Several new diagnostic entities have been recognized, subclassification schemes have been modified, and new concepts on the histogenesis and cell biology of brain tumors have emerged. In 1993, a revised WHO classification of brain tumors was published by an international committee. This article summarizes the pertinent new aspects. As novel tumor entities, the central neurocytoma, the dysembryoplastic neuroepithelial tumor (DNT), desmoplastic infantile ganglioglioma (DIG) and pleomorphic xanthoastrocytoma (PXA) have been included. Several histopathological variants of meningiomas have been added of which only the papillary meningioma and the atypical meningioma are characterized by an increased rate of recurrence. Meningeal hemangiopericytomas and hemangioblastomas are classified as tumors of non-meningothelial origin. The glioblastoma multiforme, which had previously been listed as an embryonal tumor, is now recognized as an astrocytic glioma. Immunohistochemistry has greatly advanced the practical diagnosis and classification of brain tumors. There are specific markers for all normal and neoplastic cell types except for oligodendroglioma cells. The prognosis of and therapeutic approaches to brain tumors greatly depend on histopathological grading. The WHO proposes four tumor grades, i. e., I, II, III, and IV. As a rule, grades I and II tumors are viewed as benign or semi-benign neoplasms and grades III and IV tumors as malignant. There are attempts to use new biological parameters for the grading of brain tumors. Antibodies to proliferation-associated proteins reflect tumor growth. Molecular genetic approaches to tumor-associated genes and gene loci are particularly promising new tools for the future.
    Notes: Zusammenfassung Ein internationales Gremium hat 1993 eine revidierte Fassung der WHO-Klassifikation von Tumoren des Zentralnervensystems vorgelegt. Wesentliche Neuerungen sind die Einführung folgender Entitäten: Zentrales Neurozytom, dysembryoplastischer neuroepithelialer Tumor (DNT), desmoplastisches infantiles Gangliogliom (DIG) und pleomorphes Xanthoastrozytom. Bei den Meningeomen wurden eine Reihe von neuen histopathologischen Varianten aufgenommen. Das Glioblastom wird aufgrund seiner immunhistochemisch nachweisbaren GFAP-Expression nun den astrozytären Gliomen zugeordnet. Immunhistochemische Reaktionen haben wesentliche Fortschritte in der Diagnostik und Klassifikation von zentralnervösen Tumoren gebracht. In der Diagnostik zentralnervöser Tumoren spielt die histopathologische Gradierung eine besondere Rolle, da sie als Grundlage für die weitere Behandlung und prognostische Bewertung dient. Die WHO schlägt eine Gradierungsskala vor, welche die 4 Dignitätsgrade I, II, III und IV vorsieht. Als wesentliche histopathologische Parameter fließen Differenzierungsmerkmale der Tumorzellen, die Zelldichte, zelluläre und nukleäre Polymorphie, mitotische Aktivität, pathologische Endothelproliferate und Tumorgewebsnekrosen in die Bewertung ein. Bei der Gradierung zentralnervöser Tumoren wird in zunehmendem Maße versucht, neben histopathologischen Kriterien neue biologische Parameter einzusetzen.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Der Chirurg 70 (1999), S. 1357-1360 
    ISSN: 1433-0385
    Keywords: Key words: Elastofibroma dorsi ; Bilateral ; Literature review. ; Schlüsselwörter: Elastofibroma dorsi ; bilateral ; Literaturübersicht.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung. Ein 75 jähriger Patient stellte sich mit seit 2 Jahren langsam größenprogredienten Raumforderungen unterhalb beider Schulterblätter vor. Anamnestisch bestand ein Zustand nach proximaler Oberarmamputation linksseitig mit folgender prothetischer Versorgung. Klinisch zeigten sich direkt unter den Schulterblattspitzen beidseits 2 faustgroße Tumoren, die fest mit dem tiefer gelegenen Gewebe verwachsen waren. Sowohl magnetresonanztomographisch als auch intraoperativ stellte sich ein infiltratives Wachstum dieser Raumforderungen in die seitliche Thoraxmuskulatur und das Periost der Rippen sowie des Schulterblatts dar, so daß ein maligner Tumor vermutet wurde. Histopathologisch zeigte sich Bindegewebe mit zahlreichen eingelagerten fragmentierten und unregelmäßig konfigurierten elastischen Fasern und unscharfer Begrenzung zum umgebenden Fettgewebe, typisch für ein Elastofibrom. Für Malignität bestand kein Anhalt. Im vorliegenden Fall erscheint es wahrscheinlich, daß eine unphysiologische Belastung der oberen Extremitäten bei dem mit einer linksseitigen Oberarmprothese versorgten Patienten die Entstehung der beidseitigen Elastofibrome begünstigt hat. Das Elastofibrom ist ein seltener, gutartiger, fast immer subscapulär gelegener Weichteiltumor, der bevorzugt bei älteren Frauen auftritt. In etwa 10 % der Fälle findet sich ein bilaterales, meist asynchrones Wachstum. Klinisch und radiologisch stellen Lipome sowie Weichteilsarkome die wichtigsten Differentialdiagnosen dar. Andere seltene Elastofibromlokalisationen an Rumpf, Kopf und Extremitäten werden in der vorliegenden Arbeit aufgeführt.
    Notes: Summary. A 75-year-old man had painless, slowly enlarging tumors in the right and left infrascapular regions. Clinically, the tumors were nontender and semimobile. MR imaging and the operative exploration revealed tumors infiltrating the lateral thoracic muscles and periost of the scapula and underlying ribs. At surgery, the tumors were thought to be malignant, most likely liposarcomas. The histological examination showed the characteristic appearance of elastofibromas with spindle-shaped fibroblasts, which were separated by thick eosinophilic collagenous and elastic fibers. Elastofibromas are benign mesenchymal soft-tissue lesions that mainly affect elderly women. In 10 % of the patients bilateral, often asynchronous tumors are seen. Ninety-nine percent of the lesions are located in the subscapular region. Occasional elastofibromas have been described in the extremities, head, and in the abdominal and thoracic cavities.
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