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  • 1
    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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  • 2
    Electronic Resource
    Electronic Resource
    Desmoplastic ganglioglioma: report of two non-infantile cases (1993)
    Springer
    Acta neuropathologica 85 (1993), S. 199-204 
    Details
    ISSN: 1432-0533
    Keywords: Desmoplastic infantile ganglioglioma ; Ganglioglioma ; Desmoplasia ; Gliofibroma ; Neurofilament polypeptides
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two supratentorial desmoplastic gangliogliomas arising in a 15-year-old boy and a 25-year-old man are reported. Both tumors reached the brain surface and exhibited large cysts. They showed intense desmoplasia and tumor cells of astrocytic and ganglionic differentiation. In one case the ganglionic nature was only demonstrable by immunohistochemistry. Such neoplasms can no longer be regarded as exclusively infantile brain tumors.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00227768
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  • 3
    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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  • 4
    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: Infantile multiple system atrophy ; Ubiquitin ; Neuronal intraunclear 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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  • 5
    Electronic Resource
    Electronic Resource
    Colloid cysts of the third ventricle: an immunohistochemical study (1992)
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 21 (1992), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Eleven colloid cysts of the third ventricle were investigated by immunohistochemistry using 16 mono- and polyclonal antibodies. Colloid cyst epithelium, choroid plexus epithelium and ependyma showed different immunohistochemical profiles. In particular, antibodies to different cytokeratins and to transthyretin revealed considerable and constant differences of immunoreactivity. Therefore, common derivation of the three tissues seems unlikely. Our findings are interpreted in favour of the non-neuroepithelial origin of third ventricular colloid cysts.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2559.1992.tb00340.x
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  • 6
    Electronic Resource
    Electronic Resource
    Ki-67 and biological behaviour in meningeal haemangiopericytomas (1996)
    Oxford, U.K. and Cambridge, USA : Blackwell Science Ltd
    Histopathology 29 (1996), S. 0 
    Details
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The biological behaviour of meningeal haemangiopericytomas was retrospectively studied using immunohistochemical staining with MIB1, a monoclonal antibody against the Ki-67 antigen, a nuclear protein related to cell proliferation. Paraffin-embedded material from 62 tumours from 40 patients were investigated. The proliferating compartment of the tumours was estimated by evaluating the MIB1 staining index, i.e. the percentage of MIB1 positive nuclei in at least 1000 counted tumour cells in representative areas. The staining index ranged from 1.24% to 39.01%. Statistical analysis revealed no significant correlation between the staining index and recurrence-free survival (χ2 = 0.3922, P = 0.5311). Long-term observation (〉100 months), however, revealed a tendency to longer survival in the group with a staining index less than 5%. According to our results, the MIB1 staining index does not contribute to the accuracy of predicting the clinical outcome of meningeal haemangiopericytomas.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2559.1996.d01-479.x
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  • 7
    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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  • 8
    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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  • 9
    Electronic Resource
    Electronic Resource
    Axillarisparese nach Squash-Spielen¶Ein Fallbericht (1999)
    Springer
    Der Nervenarzt 70 (1999), S. 750-753 
    Details
    ISSN: 1433-0407
    Keywords: Schlüsselwörter Axillarisparese ; Armplexusläsion ; Traumatische Nervenläsion ; Sportverletzung ; Zerrungstrauma ; Key words Axillary nerve palsy ; Brachial plexus lesion ; Traumatic nerve lesion ; Sports injury ; Traction injury
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A 27-year-old otherwise healthy male presented with an isolated but complete axillary nerve palsy after excessive squash playing. When repeated electromyographic investigations showed no signs of reinnervation in the deltoid muscle, surgery was performed in order to restore nerve function. Intraoperatively, the nerve showed a short segment of thinning about 2 cm distally the nerve's origin from the posterior fascicle. As intraoperative electrophysiological testing was also negative (no electrically evoked nerve action potentials across the lesion) the suspicious nerve segment was resected and nerve continuity restored by sural grafts. Histologically, no intact nerve structures could be found at the site of the thinning. Most likely the lesion was caused by traction forces. Follow-up studies showed reinnervation of deltoid function over time.
    Notes: Zusammenfassung Nach exzessivem Squash-Spielen entwickelte ein 27jähriger, sonst gesunder Mann eine isolierte, vollständige Axillarisparese. Als sich nach 3 Monaten weder klinisch noch elektrophysiologisch Zeichen der Erholung der Nervenfunktion fanden, wurde der Nerv operativ freigelegt. Dabei zeigte sich nach der Epineurotomie eine sanduhrförmige Ausdünnung des N. axillaris kurz nach dessen Abgang aus dem Fasciculus posterior. Da auch die intraoperative, elektrophysiologische Testung negativ war (kein Nervenaktionspotential über die Verletzungsstelle ableitbar), wurde das auffällige Nervensegment reseziert und die Nervenkontinuität mittels autologer Suralisinterponate wiederhergestellt. Die histologische Aufarbeitung ergab den vollständigen Verlust nervaler Strukturen im Bereich der Ausdünnung. Als Ursache dieser Nervenläsion kommt am ehesten eine Überdehnung in Frage. Postoperative Verlaufsuntersuchungen zeigten eine zunehmende Reinnervation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001150050506
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  • 10
    Electronic Resource
    Electronic Resource
    Different variants of frontotemporal dementia: a neuropathological and immunohistochemical study (1996)
    Springer
    Acta neuropathologica 92 (1996), S. 170-179 
    Details
    ISSN: 1432-0533
    Keywords: Key words Frontotemporal dementia ; Dementia of ; frontal lobe type ; Pick’s disease ; Motor neuron disease ; with dementia ; Pick bodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Histological and immunohistochemical findings in 20 cases of frontotemporal dementias – 8 cases of dementia of frontal lobe type (DFT), 7 cases of Pick’s disease (PD), and 5 cases of motor neuron disease with dementia (MND/D) – are presented. Common features of all three syndromes were: frontotemporal atrophy, involvement of subcortical nuclei, and swollen chromatolytic cells. Ubiquitin (Ub)-positive and tau-negative inclusions in cortical, hippocampal, and motor neurons were found in MND/D and DFT cases, suggesting a common pathogenesis of MND/D and DFT. MND/D showed the same cytoskeletal alterations in motor nuclei as MND without dementia: Bunina bodies and skein-like, Ub-positive inclusions. DFT differed from PD in the preponderance of histopathological changes in upper cortical layers, the sparseness of chromatolytic cells, and the absence of tau-positive Pick bodies (PBs). There were, however, two transitional cases showing Pick-type histology but no PBs, thus linking DFT and PD. PBs expressed chromogranin B and secretoneurin strongly, but chromogranin A only weakly. They were negative for the 70-kDa heat-shock protein, metallothionein, and glutathione-S-transferase.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050505
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