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  • 1995-1999  (1)
  • 1990-1994  (1)
  • Agyria  (1)
  • Colitis ulcerosa  (1)
  • 1
    ISSN: 1434-5196
    Keywords: Key words Internal cerebral vein thrombosis ; Sinovenous thrombosis ; Colitis ulcerosa ; Trauma ; Schlüsselwörter Hirnvenenthrombose ; Sinusthrombose ; Trauma ; Colitis ulcerosa
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine , Law
    Description / Table of Contents: Zusammenfassung Venöse Abflußstörungen des Gehirns werden mit unterschiedlicher Häufigkeit im Obduktionsgut aufgeführt. Neben entzündlichen Prozessen, hämorheologischen Störungen und Neoplasien kommen bei der forensischen Begutachtung traumatische Ursachen für die Genese von Thrombosen in Frage. Berichtet wird über eine 14 Jahre alt gewordene Schülerin, die 2 1/2 Tage nach einer tätlichen Auseinandersetzung verstarb. Anamnestisch wurde von einer Colitis ulcerosa berichtet. Initial waren weder äußere Verletzungen noch neurologische Auffälligkeiten erkennbar. In der Nacht wurde sie komatös in der Klinik aufgenommen. Die kranielle computertomographische Untersuchung zeigte eine zunehmende intraventrikuläre und intrazerebrale Blutung, Zeichen der venösen Stauung und ein Hirnödem. Die Patientin verstarb im dissoziierten Hirntod. Neuropathologisch fand sich eine in Organisation stehende Thrombose des Sinus sagittalis superior, die sich in die inneren Hirnvenen fortsetzte und die durch die entzündliche Darmerkrankung verursacht war. Eine traumatische Genese war somit auszuschließen.
    Notes: Abstract Thromboses of the cerebral veins which occur at a frequency of 3.75 to 9.3% in autopsy material are caused by local or systemic inflammatory processes, hematological disorders, neoplasms or traumatic brain damage. We report on a 14-year-old girl who suffered from colitis ulcerosa during the last 4 years of life. Immediately after a brawl with a schoolboy she complained of headache and vertigo, but no physical abnormality was detected by the physician. She was admitted to the hospital after becoming comatose during the following night. On neurological examination she was comatous with a decerebrate state, the pupils were small and non-reactive to light, divergent ocular bulbi and bilateral extensor plantar responses. A cerebral computer tomogramm showed a cerebral edema and a hemorrhage in the left basal ganglia and both cerebral ventricles which subsequently increased to the right cerebral ganglia, the corpus callosum and the right cerebral hemisphere. Despite antiedematous therapy the girl died on the second day after the brawl. A neuropathology examination revealed thrombosis of the superior sagittal sinus extending to the internal cerebral veins and a bilateral hemorrhagic infarction of the thalamus and the basal ganglia with intraventricular hemorrhage. As the thrombus in the sagittal sinus showed signs of organisation, a postraumatic origin alone is improbable and a hemorrheological disorder due to colitis ulcerosa seemed to be the main cause.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 394-399 
    ISSN: 1433-0350
    Keywords: Lissencephaly ; Agyria ; Neuronal migration ; Miller-Dieker syndrome ; Walker-Warburg syndrome ; Congenital muscular dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The neuropathological findings at autopsy in four cases of type I and three of type II lissencephaly are presented. Type I lissencephaly is characterized by agyriapachygyria with a markedly thickened cerebral cortex with four coarse histological layers. The normally myelinated white matter, often with neuronal heterotopias, is very narrow, and the gray-to-white matter ratio is inverted (about 4:1); there are no white-gray interdigitations. Claustrum and capsula extrema are absent. Ventricular dilatation is present, especially of the occipital horns. In the hypoplastic brain stem large olivary heterotopias can often be observed. Severe cerebellar malformations, obstructive hydrocephalus, severe eye abnormalities, and congenital muscular dystrophy are not seen. Clinically, type I lissencephaly presents as “isolated lissencephaly sequence” or as “Miller-Dieker syndrome” with characteristic facial dysmorphism. The long survival of 20 years achieved by one of our patients is very uncommon. Type II lissencephaly is characterized by widespread agyria. Usually, obstructive hydrocephalus is present with a thin cerebral mantle showing a slightly thickened cortex and a narrow, hypomyelinated white matter often with neuronal heterotopias (gray-to-white matter ratio about 1:1). The border between gray and white matter is blurred. Claustrum and capsula extrema are absent. Histologically, the cortex appears disorganized without layering; widespread leptomeningeal gliomesenchymal proliferations and glioneuronal heterotopias are present. Hydrocephalus is caused by obstruction of the subarachnoid space, aqueductal stenosis, and Dandy-Walker malformation. The latter is a typical finding in type II lissencephaly together with severe cerebellar cortical dysplasias. Aplasia of the olfactory nerves, gliotic fusion of the frontal lobes, and an occipital cephalocele may be found. Type II lissencephaly occurs in clinical syndromes with severe eye abnormalities and congenital muscular dystrophy (the so-called “Walker-Warburg syndrome”). The congenital muscular dystrophies have no dystrophin deficiency, as we were able to show immunohistochemically in the muscle biopsies of two further patients with this syndrome.
    Type of Medium: Electronic Resource
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