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  • 1
    Electronic Resource
    Electronic Resource
    Morphological and immunohistochemical characterization of granular cells in non-hypophyseal tumours of the central nervous system (1997)
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 30 (1997), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report 14 biopsy cases of granular cell tumours (GCT) of the central nervous system (CNS) outside the pituitary gland. In six cases the granular cells determined the morphology (actual GCT), the other eight consisted of different CNS tumours with a varying granular cell component. Pronounced immunoreactivity for ubiquitin and alpha-1-antichymotrypsin could be found in all investigated tumours, while GFAP, neuron specific enolase, von Willebrand factor, vimentin, S-100 protein, alpha-1-antitrypsin, actin, and the neurofilaments 68 kDa and 160 kDa showed mostly weak positivity in some cases. Four out of eight GCT showed no immunoreactivity for MIB1; the other four cases had a proliferation index between 0.5% and 15%. Six out of nine cases were positive for p53. We consider granular cells to originate from different cell types. Thus, although morphologically identical, GCT are actually biologically heterogeneous. GCT of the CNS may represent gliomas of mostly astrocytic origin with a metabolically induced transformation of some tumour cells into granular cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2559.1997.5360788.x
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  • 2
    Electronic Resource
    Electronic Resource
    Neurozystizerkose (1995)
    Springer
    Der Pathologe 16 (1995), S. 348-353 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Neurozystizerkose ; Zerebrale Verkalkungen ; Immuntoleranz ; Hydrozephalus ; Hirninfarkt ; Key words Neurocysticercosis ; Cerebral calcification ; Immuntolerance ; Hydrocephalus ; Cerebral infarction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 54-year-old female patient with a 10-year history of ventriculoperitoneal shunt resulting from communicating hydrocephalus of undetermined aetiology is reported. Transient gait disturbances and cerebral infarction at the age of 46 did not lead to further insights into the nature of the disease. After many years with only occasional disturbances, a distinct organic brain syndrome developed. Thorough examination led to a tentative diagnosis of neurocysticercosis; this was based on the history, liquor diagnosis and cerebral microcalcifications in CT. Despite the initiation of specific therapy, the patient died of the sequelae of the disease. At autopsy, characteristic cicatricial residues of mainly basal leptomeningitis were found with collapsed parasitic cysts. Additional intracerebral mesenchymal-glial reactions were less conspicuous. Residual ependymitis had caused aqueductal stenosis. Death was due to cachexia, bronchopneumonia and a lung abscess. The clinical course and morphology of neurocysticercosis are discussed. The disease has become rare in our country, but is globally the most important parasitic disease of the central nervous system.
    Notes: Zusammenfassung 54 jährige Patientin mit bereits 10 Jahre altem ventrikuloperitonealem Shunt wegen eines ätiologisch unklaren Hydrocephalus communicans. Eine passagere Gangstörung und ein Hirninfarkt mit 46 Jahren führten zu keiner weiteren Klärung der Krankheitsgenese. Nach langjähriger Zwischenphase mit gelegentlicher Verwirrung der Patientin trat ein ausgeprägtes hirnorganisches Psychosyndrom auf. Eingehende Diagnostik ergab aufgrund der Anamnese, der Liquor- und der CT-Befunde mit nachweisbaren kleinen zerebralen Verkalkungen den Verdacht auf eine Neurozystizerkose. Die Patientin starb trotz eingeleiteter spezifischer Therapie an den Folgen der Erkrankung. Bei der Obduktion zeigen sich charakteristische narbige Residuen einer v. a. basalen Leptomeningitis mit kollabierten Parasitenzysten. Zusätzliche intrazerebrale mesenchymal-gliöse Residuen waren weniger auffällig. Eine abgelaufene Ependymitis war Ursache einer Aquäduktstenose. Todesursache waren eine Kachexie, Bronchopneumonie und ein Lungenabszeß. Diskutiert werden Klinik, Verlauf und Morphologie der Neurozystizerkose, die – bei uns selten geworden – weltweit die wichtigste parasitäre Erkrankung des ZNS darstellt.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050113
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  • 3
    Electronic Resource
    Electronic Resource
    Paraneoplastische limbische Enzephalitis (1997)
    Springer
    Der Pathologe 18 (1997), S. 406-410 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Paraneoplastische neurologische Syndrome ; Limbische Enzephalitis ; Anti-Hu-Syndrom ; Kleinzelliges Bronchialkarzinom ; Key words Paraneoplastic neurological syndromes ; Limbic encephalitis ; Anti-Hu-syndrome ; Small cell lung cancer
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a 69-year old male patient who presented with vague depressive symptoms and loss of weight accompanied by lack of appetite, ageusia and anosmia. The patient died of bronchial pneumonia, and neuropathological examination revealed a so-called limbic encephalitis. The paraneoplastic origin of this case was later confirmed by discovery of a clinically inapparent, indolently growing small cell lung cancer. This case illustrates the difficult clinical diagnosis of paraneoplastic limbic encephalitis and the need for a close cooperation of pathologists and neuropathologists in the clarification of such cases.
    Notes: Zusammenfassung Berichtet wird über den Fall eines 69jährigen Mannes mit einer unklaren depressiven Symptomatik und Gewichtsabnahme in Verbindung mit Appetitlosigkeit, Ageusie und Anosmie. Der Patient verstarb an einer Bronchopneumonie, und die neuropathologische Untersuchung ergab den Befund einer sog. limbischen Enzephalitis. Die paraneoplastische Genese des Krankheitsbildes wurde dann a posteriori durch den Nachweis eines klinisch unbekannten, lokal indolent wachsenden kleinzelligen Bronchialkarzinoms bestätigt. Der Fall illustriert die schwierige klinische Diagnose einer paraneoplastischen limbischen Enzephalitis und unterstreicht die Notwendigkeit einer engen Zusammenarbeit von Pathologen und Neuropathologen in der Aufklärung derartiger Fälle.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050234
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  • 4
    Electronic Resource
    Electronic Resource
    Paragangliom der Cauda equina (1997)
    Springer
    Der Pathologe 18 (1997), S. 484-487 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Paragangliom ; Ependymom ; Filum terminale ; Cauda equina ; Differentialdiagnose ; Key words Paraganglioma ; Ependymoma ; Filum terminale ; Cauda equina ; Differential diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a case of paraganglioma of the cauda equina in a 48-year-old man. These slow-growing, mostly benign and encapsulated intradural–extramedullar tumours are clinically characterized by lumbago, paraesthesia and motor deficits as well as occasi-onally by incontinence. Paragangliomas of the cauda equina show monomorphous (main) cells arranged in a typical cell cluster pattern, demonstrating a lobular architecture and a fine net of connective tissue formed by so-called sustentacular cells; some tumours show focal ganglionic differentiation. Because of their morphological similarity to the more common ependymomas, paragangliomas of the cauda equina are sometimes misdiagnosed so that their incidence is likely to be higher than previously estimated. Immunohistochemistry findings are positive for neuron-specific enolase, chromogranin and other neuronal markers as well as neuropeptides (main cells) and S-100 protein (sustentacular cells) while widely lacking reactivity for GFAP. Paragangliomas of the cauda equina represent a rare entity, of which only 80 cases have been described in the literature. Because they only occur sporadically, they are often not included in the differential diagnosis of mass lesions of the region of the cauda equina.
    Notes: Zusammenfassung Wir berichten über ein Paragangliom der Cauda equina bei einem 48jährigen Patienten. Klinisch imponieren diese langsam intradural-extramedullär wachsenden, meist benignen und von einer bindegewebigen Kapsel umgebenen Geschwülste durch Lumbalgien, Parästhesien und Paresen sowie gelegentlich durch Inkontinenz. Paragangliome der Cauda equina zeichnen sich histomorphologisch durch monomorphe Zellen (Hauptzellen) in typischer „Zellballen”-anordnung mit lobulärer Architektur und feinem Bindegewebsnetz aus Hüllzellen (Sustentakularzellen) aus, z.T. liegt eine ganglionäre Differenzierung vor. Wegen ihrer Ähnlichkeit zum häufigeren Ependymom werden sie mitunter fehldiagnostiziert, so daß ihre Inzidenz wahrscheinlich höher ist als bislang angenommen. Immunhistochemisch exprimieren sie neuronspezifische Enolase, Chromogranin und andere neuronale Marker sowie Neuropeptide (Hauptzellen) und S-100-Protein (Sustentakularzellen) bei weitgehend fehlender Reaktion für GFAP. Das Paragangliom der Cauda equina ist eine seltene Entität, von der bislang erst 80 Fälle in der Literatur beschrieben worden sind und an der gerade aufgrund ihres sporadischen Auftretens bei der Differentialdiagnose von Raumforderungen in diesem Bereich oft nicht gedacht wird.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050247
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  • 5
    Electronic Resource
    Electronic Resource
    Prolaktinbildendes Hypophysenkarzinom Kasuistik eines äußerst seltenen metastasierenden Tumors* (1995)
    Springer
    Der Pathologe 16 (1995), S. 354-358 
    Details
    ISSN: 1432-1963
    Keywords: Schlüsselwörter Hypophysenkarzinom ; Lebermetastase ; Hyperprolaktinämie ; Key words Pituitary adenoma ; Prolactin-secreting pituitary carcinoma ; Metastasizing tumor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 59-year-old male patient was transnasally operated on because of a pituitary adenoma with hypopituitarism. A second operation and X-ray therapy followed a half year later due to recurrent tumor. Both neoplasmas were classified as sparsely granulated prolactin cell adenomas. Immunohistochemical studies revealed strong immunoreactivity for prolactin and FSH in the tumor cells of both the pituitary adenoma and the recurrent tumor. Two years later the prolactin plasma levels were extremely elevated. A tumor in the liver was identified. Biopsy revealed a solid endocrine tumor containing prolactin by immunohistology. Due to structural and immunohistological similarities this tumor could be identified as a metastasis of the pituitary tumor. After 5 months of therapy the patient died from thrombembolism. Post-mortem studies confirmed the diagnosis of a metastasizing prolactin-secreting pituitary carcinoma. Only six similar cases have been reported in the literature. Our case report confirms the experience with 35 definite pituitary carcinomas reparted in the current literature: malignant pituitary tumors develop after pituitary surgery and can be identified not from the pituitary tumor, but only from its metastases.
    Notes: Zusammenfassung Ein 59 jähriger Mann wurde wegen eines zu einer Hypophyseninsuffizienz führenden Hypophysenadenoms transnasal operiert, ein halbes Jahr später wegen residualen Adenomgewebes nachoperiert und anschließend einer Strahlentherapie unterzogen. Der Tumor wurde als gering granuliertes Hypophysenadenom vom Prolaktinzelltyp mit Prolaktin- und FSH-Bildung klassifiziert. Zwei Jahre später wurde bei einer extrem hohen Hyperprolaktinämie ein Lebertumor festgestellt, der sich im Biopsat als solider endokriner Tumor mit immunhistochemisch nachgewiesener Prolaktinbildung herausstellte. Wegen der strukturellen Ähnlichkeit und des weitgehend identischen Markerverhaltens mußte er als Metastase des Hypophysentumors angesehen werden. Nach fünfmonatiger Therapiedauer verstarb der Patient an den Folgen von thrombembolischen Komplikationen. Die Sektion konnte die Diagnose eines metastasierenden prolaktinbildenden Karzinoms bestätigen, da weitere extrahepatische Tumormanifestationen und somit mögliche alternative Primärtumoren nicht darstellbar waren. Der Fall ist mit nur 6 anderen bisher beschriebenen Kasuistiken weitgehend vergleichbar und bestätigt die Erfahrung aus 35 in der moderneren Literatur gesicherten Hypophysenkarzinomen, daß maligne Hypophysentumoren erst nach Hypophysenoperationen manifest werden und nicht am Primärtumor, sondern nur an den Metastasen diagnostiziert werden können.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002920050114
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  • 6
    Electronic Resource
    Electronic Resource
    Summary and conclusions (1993)
    Springer
    Child's nervous system 9 (1993), S. 421-421 
    Details
    ISSN: 1433-0350
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00306197
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  • 7
    Electronic Resource
    Electronic Resource
    Ocular findings in Walker-Warburg syndrome (1993)
    Springer
    Child's nervous system 9 (1993), S. 418-420 
    Details
    ISSN: 1433-0350
    Keywords: Walker-Warburg syndrome ; Ophthalmological findings ; Retinal dysplasia ; Rieger's anomaly ; Peters' anomaly ; Pseudoglioma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Ocular symptoms are frequently observed in Walker-Warburg (WWS) and associated syndromes. The majority of patients present with malformations of the anterior segment and severe retinal dysplasia. We report on the findings in a female patient with WWS who died at the age of 9 months. Major ocular findings were: severe iridocorneal malformation, a membran-like structure of the lens and funnel-shaped retinal dysplasia. The retina presented various grades of differentiation with rosettes and atypical sequences of cells, e.g. ganglion cells intermingled between granular layers. The anterior part of the retina presented as a primitive homogeneous layer with a cell-free space that might be interpreted as the primary optic ventricle. This finding suggests that we are dealing with a primary dysplastic non-attachment rather than a real detachment of the retina in WWS. The malformation of the anterior segment was not typical of the Peters' anomaly, as usually described in WWS, but of Rieger's syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00306196
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  • 8
    Electronic Resource
    Electronic Resource
    Infantile multiple system atrophy with cytoplasmic and intranuclear glioneuronal inclusions (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 642-647 
    Details
    ISSN: 1432-0533
    Keywords: KeyWordsInfantile multiple system atrophy Ubiquitin ; Neuronal intranuclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293326
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  • 9
    Electronic Resource
    Electronic Resource
    Motor neuron disease with pallido-luysio-nigral atrophy (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 105-108 
    Details
    ISSN: 1432-0533
    Keywords: Pallido-luysio-nigral atrophy ; Motor neuron disease ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of motor neuron disease (MND) with pallido-luysio-nigral atrophy (PLNA) is reported. The 45-year-old male patient presented with lower motor neuron symptoms and signs of basal ganglia disturbance. He died after a progressive course of 7 months. Neuropathological examination revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in substantia nigra, globus pallidus, and subthalamic nucleus. The presence of ubiquitin-positive inclusions, a hallmark of most variants of MND, confirms this case as an example of MND. At immunoelectron microscopy the granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice previously. The relationship of this syndrome to other forms of MND and its nosological placement are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00454908
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  • 10
    Electronic Resource
    Electronic Resource
    Down's dyndrome and Alzheimer's disease: dendritic spine counts in the hippocampus (1990)
    Springer
    Acta neuropathologica 79 (1990), S. 680-685 
    Details
    ISSN: 1432-0533
    Keywords: Down's syndrome ; Alzheimer's disease ; Hippocampus ; Golgi method ; Dendritic spines
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Samples of the hippocampus of four patients with Down's syndrome [two men aged 35 and 36 years with no evidence of Alzheimer's disease (AD) and two patients aged 47 and 55 years with associated AD] were obtained at post mortem and processed according to the rapid Golgi method. A significant reduction in the number of dendritic spines (DS) was found in the apical (middle, distal and oblique segments) and basilar (thick and thin segments) dendritic arbors of CA1 and CA2–3 pyramidal neurons in patients with Down's syndrome and no AD when compared to age-matched controls. An additional decrease of DS in every segment occurred in Down's patients with associated AD when compared to agematched controls and Down's patients with no AD. In Down's syndrome (either associated or not to AD) thin basilar dendrites were the most severely involved; in AD patients CA1 pyramids were more severely affected than pyramidal neurons of the CA2–3 subfield.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294247
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