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Biomedical subjects

E Tarnowska-Dziduszko

Publications and source records attributed to E Tarnowska-Dziduszko.

At least 19 recordsLinked to original sources
PubMed1996

Major histocompatibility complex class I expression on neurons in subacute sclerosing panencephalitis and experimental subacute measles encephalitis.

Lack of major histocompatibility class I antigens on neurons has been implicated as a possible mechanism for viral persistence in the brain since these antigens are required for cytotoxic T-lymphocyte recognition of infected cells. In subacute sclerosing panencephalitis (SSPE), measles virus (MV) persists in neurons, resulting in a fatal chronic infection. MHC class I mRNA expression was examined in formalin-fixed brain tissue from 6 SSPE patients by in situ hybridization. In addition MHC class I protein expression in MV-infected neurons was examined in experimental Subacute Measles Encephalitis (SME) by double immunohistochemistry. MHC class I mRNA expression was found to be upregulated in SSPE tissues studied, and in 5 out of 6 cases the expression was definitively seen on neurons. The percentage of neurons expressing MHC class I mRNA ranged between 20 to 84% in infected areas. There was no correlation between the degree of infection and expression of MHC class I molecules on neurons. Importantly, the number of neurons co-expressing MHC class I and MV antigens was markedly low, varying between 2 to 8%. Similar results were obtained in SME where 20 to 30% of the neurons expressed MHC class I but <8% co-expressed MHC class I and MV antigens. Perivascular infiltrating cells in the infected regions in SME expressed IFNgamma immunoreactivity. The results suggest that MV may not be directly involved in the induction of MHC class I on neurons and that cytokines such as IFNgamma may play an important role. Furthermore, the paucity of neurons co-expressing MHC class I and MV antigens in SSPE and SME suggests that such cells are either rapidly cleared by cytotoxic T lymphocytes (CTL), or, alternatively, lack of co-expression of MHC class I on MV infected neurons favors MV persistence in these cells by escaping CTL recognition.

Adult↗
PubMed1996

Asymptomatic and late-onset ornithine transcarbamylase (OTC) deficiency in males of a five-generation family, caused by an A208T mutation.

In a large five-generation Polish family, late-onset ornithine transcarbamylase (OTC) deficiency in males segregated with the missense mutation Ala208Thr (A208T), and all heterozygous females were asymptomatic. No other mutations were found in the coding sequences and intron-exon boundaries of the OTC gene. Surprisingly, the mutation originated from the great-grandfather of the index patient who died at age 59 of liver carcinoma. He never had dietary restrictions or hyperammonemic spells throughout life and appears to be the oldest male reported with OTC deficiency. The index patient had a severe OTC deficiency (3% of normal). Eight males died suddenly at ages 4 months to 23 years (average 14 years) after a foudroyant episode triggered by a common infection. The patients remained undiagnosed for 28 years because a metabolic defect was not considered to be the cause of the acute episodes. Recognition of the familial pattern of inheritance was initially unnoticed since the patients were admitted to eight different hospitals. DNA analysis predicted that two 'healthy' boys also had OTC deficiency, which was confirmed by abnormal results of allopurinol challenge tests. Initial suspicion of OTC deficiency in such families is complicated, since symptoms can develop at any age, or even remain absent. This obscures the typical pattern of X-linked inheritance in small families.

Age of Onset↗
PubMed1995

Morphological changes in the corpus callosum in chronic alcoholism.

In a group of 66 patients (age 24 to 78 years) with the clinical diagnosis of chronic alcoholism the changes in the corpus callosum were evaluated. The period of alcohol abuse varied from 3 to over 30 years. In 57 cases atrophy of the corpus callosum was noted. The trunk was involved most frequently. Myelin sheaths exhibited abnormalities from slight pallor to total destruction. In 19 cases the damage of myelin sheaths was restricted to disseminated, perivascular, spongy degenerations. The vessels were sclerotic, especially periventricular ones exhibited degenerative changes. Perivascular gliosis was also seen. Conclusions from the present study indicate that the structural changes observed in the corpus callosum during chronic alcohol abuse are connected with CNS involution and with degenerative changes within vessels walls. The damage of myelin sheaths localized in our material similarly as Marchiafava-Bignami disease, differs from the later by less advanced changes, perivascular spreading of demyelination, and frequent destruction of axons.

Adult↗
PubMed1991

Creutzfeldt-Jakob disease in young people.

Three neuropathologically confirmed cases of Creutzfeldt-Jakob disease in young people (19, 23, and 27 years of age) are described. None had received pituitary hormone therapy. At the onset of illness all patients were suspected of having SSPE or other viral encephalitis, because of the similarity of clinical symptomatology and the shift towards older age of SSPE onsets observed in Poland in recent years.

Adult↗
PubMed1989

[Generalized form of cerebral actinomycosis].

The here presented case was a woman 82 years old suffering for many years of arteriosclerotic Parkinson syndrome. During the last 8 months of her life she exhibited excessive talk flow and states of delirium. Twelve days before death she had a generalized attack of convulsions and the consciousness disorder increased. Moreover, meningeal symptoms were observed, a right side reflex syndrome with Babiński sign and temperature up to 38 degrees C were noted. Death occurred on the 9th day of hospitalization with symptoms of increasing circulatory-respiratory insufficiency. Pathomorphological examination revealed in the myocardium, lungs, liver, kidneys and thyroid multiple purulent foci containing actinomycotic granules. Neuropathological examination revealed both in the cortex and white matter the presence of numerous disseminate bodies staining intensively with HE, PAS- and Gram-Weigert positive and negative in the Ziehl-Nielsen method. Their structure, compact or fine-powdered corresponded to actinomyces colonies. They either showed no reaction or were surrounded by a wall of inflammatory cells such as: neutrophilic leucocytes, lymphocytes, plasmatic cells and histiocytes. The microabscesses were not encapsulated. The case was classified to a diffuse form of brain actinomycosis. The etiopathogenesis of the changes and the clinical-morphological correlations of this very rare infectious disease of the central nervous system are discussed.

Actinomycosis↗
PubMed1989

[Aspergillosis of the brain in a patient with pancytopenia].

The here presented case concerns a 74-year-old woman treated for many years with steroids and immunosuppressive drugs during pancytopenia. Seven days before her death she developed severe headaches and visual disorders, rapidly increasing disturbances of consciousness and a temperature of 40 degrees C. The course of the disease was violent with meningeal syndrome and cerebral coma. The cerebrospinal fluid was purulent, pleocytosis 1235/ml, with prevalence of polymorphonuclear leucocytes (88%) and an protein level increased to 210 mg%. In autopsy no mycotic changes were found in the internal organs whereas in microscopic examination of the brain the dominant finding was the presence of numerous filamentous bodies with septa characteristic of aspergillus which were visible both in microthrombi in the lumen of meningeal and interstitial vessels and in the areas of extensive necroses in both cerebral hemispheres and the brain stem as well as within granulomas occurring on the edges of necrotic foci and in the inflammatory infiltration of the brain base meninges. The presence of aspergillus hyphae was usually associated with a severe inflammatory reaction of polymorphonuclear leucocytes, acidophilic and plasmatic cells in microabscesses and in the inflammatory infiltration of meninges or in the form of granulomas composed mainly of multinucleated giant cells of Langhans or of foreign body type and mononuclear cells. The etiopathogenesis of these changes is discussed. The role of the facilitating factor could have been played by protracted therapy with steroids and immunosuppressive drugs and/or by pancytopenia itself which is probably associated with abnormal immunological response. Noteworthy is the fact that the seldom described mycotic changes caused by aspergillus concerned in the present case the central nervous system exclusively.

Aged↗
PubMed1989

[Clinico-morphologic correlations in cerebral infarction after recurrent hemorrhage].

Clinical-morphological analysis of cases in which, in the course of vasogenic necrotic brain lesion, hemorrhagic foci appeared within the infarcted area, was performed. The material comprised 355 cases with encephalomalacia (223 women and 123 men). The patients died at age between 39 and 96 years. In the clinical picture a sudden onset of the disease prevailed, with hemiparesis or hemiplegia associated in 216 cases with disturbances of consciousness. The course of the disease was in general progressive. Only in 30 patients improvement of clinical state was observed. The time of survival varied from several hours to several weeks. Most patients (140) died in the first week in deep cerebral coma or state of decerebration. In nine cases clinical symptomatology of herniation and secondary hemorrhages into the brain stem was found. A sudden onset and violent course of the disease resulted in a diagnosis of primary hemorrhage with penetration to the subarachnoid space or ventricular system in 32 patients. Only in six cases was a secondary hemorrhage into the primary infarction diagnosed clinically. Hemorrhagic malacia was most frequently (in 255 cases) located in region vascularized by the middle cerebral artery and very seldom, the basilar artery or cerebellar arteries. Malacia was accompanied by considerable brain edema. This was particularly intense in extensive infarctions and persisted even in cases with considerable long survival. In 45 cases secondary hemorrhagic foci were found within brain stem. The morphology of the infarction foci was variable in most cases. Independently to fresh unreactive diffuse necrosis, sometimes areas with various advancement of resorption and organization processes were observed in the same case. Both in areas of fresh reactionless malacia and of advanced macrophage-glial disintegration, or even in areas of advanced gliomesodermal organization, stand-like or spherical hemorrhages could be seen merging sometimes into extensive pools. The morphology of hemorrhagic foci was largely dependent on their size and localization. The hemorrhagic foci located close to the depth of the cortical sulci very often destroyed the cortex and white matter, and penetrated to the subarachnoid space (141 cases). In was usually almost impossible to detect the source of bleeding within the necrotically changed area. The resorption reaction at the edge of extensive hemorrhagic foci was in general minimal. A lesion of the vessel wall, caused by stasis of long duration, resulting from disorders of the vascular function in the course of defficient blood supply and ischemic brain edema are considered the main causes of bleeding into the pale infarction.

Adult↗
PubMed1989

[Clinical diagnosis of Jakob-Creutzfeldt syndrome--analysis of 6 cases].

The authors present an analysis of the clinical course of 6 cases of Creutzfeldt-Jakob disease (patients were aged from 27 to 59 years). The diagnosis was established during the life of the patients. In the neurological status dementia and syndromes of pyramidal and extrapyramidal lesions predominated. Neuropathological examinations in 5 cases demonstrated also considerable cerebellar damage, however, clinical signs of this damage were noted in two patients only. EEG findings were of greatest importance among the laboratory investigations.

Adult↗
PubMed1978

[Circulatory disorders in the area of supply of the vertebrobasilar arteries in clinical and morphological picture].

The material comprised 40 autopsied cases in which vasogenic lesions in the brain stem were the only area of central nervous system damage or preceded the involvement of the cerebral hemispheres. Only 3 of them had been diagnosed correctly during the life of the patients. The material was divided into three groups: I. 22 cases with very dramatic onset suggesting in neurological appearance injury to the hemispheres, leading in most cases (15 out of 22) to death within 1 week. II. 18 cases in which the survival was from 2 months to 6 years. The vasogenic damage to the brain stem preceded considerably the development of brain softening or supratentorial haemorrhage 12 cases, in 7 cases the brain stem lesion was clinically silent in neurological examination. Fresh infarctions (group I) in the brain stem were located usually in the region of vascularization of the short circumferential and paramedian arteries, mainly in the basal part of the pons and midbrain. In group II single or multiple cavities were situated unilaterally or bilaterally mainly in the pons, at the watershed of the vascularization areas of paramedial and short or long circumflex arteries. The foci in the brain stem were associated in most cases (32 out of 40) with vascular lesions in the remaining areas of the same vascularization region. In the discussion on the pathogenesis the significance of degenerative changes in the walls of the vessels in this region and presence of thrombi in them for the development of necrotic changes has been stressed.

Adult↗