Biomedical subjects

W Jänisch

Publications and source records attributed to W Jänisch.

At least 19 recordsLinked to original sources

PubMed2000

[Chronic encapsulated intracerebral hematoma]].

The purpose of this case report is to describe chronic encapsulated intracerebral hematoma (CEIH), a rare and not fully understood brain lesion. The differentiation from chronic cerebral hematoma, cerebral abscess, and intra-axial tumor is based on clinical and imaging criteria. The diagnosis is confirmed by histological analysis. In the English medical literature, we found 39 patients reported with this lesion. The key feature is the formation of a capsule between a hematoma and cerebral parenchyma. The reasons for this formation are not known. In our patient, an arteriovenous malformation was located outside but adjacent to the CEIH.

Adult↗

PubMed2000

[Diagnostic imaging in viral encephalitis].

The diagnostic procedure in viral encephalitis is based on the synopsis of clinical signs and symptoms, serological data, CSF analysis and diagnostic imaging findings. This article summarizes the findings of those viral encephalitides most frequently encountered in Western Europe. MRI is more sensitive than CT for the detection of inflammatory brain lesions due to the higher contrast resolution. The pattern of parenchymal damage is highly specific in only some viral encephalitides (e.g., the frequently hemorrhagic lesions of structures of the limbic system in herpes simplex virus type I encephalitis; the symmetric and confluent lesions of the frontal white matter of progressive diffuse leukoencephalopathy in AIDS). In the majority of viral encephalitides MRI demonstrates the location and extension of parenchymal damage. The specific diagnosis in terms of the causative agent is based on serological studies.

Brain↗

PubMed2000

[Septic-embolic and septic metastatic brain abscess].

The hematogenous spread of bacteria, fungi and protozoa may also reach the brain vessels, which happens mostly through septic emboli. From such an embolus a metastatic focal encephalitis and later a septic-embolic brain abscess may arise. The most frequently underlying infections that may cause septic emboli are bacterial endocarditis as well as bacterial infections of artificial heart valve prostheses. Congenital heart malformations with a right-to-left shunt also play here a certain role. Basically, however, all septic conditions and bacteriemias may cause septic-embolic brain abscesses. They occur frequently as multiple lesions. MRI is superior to CT in depicting the different stages of evolution from focal encephalitis, through the hardly encapsulated early abscess, to the formation of a membrane and later a dense fibrous capsule. The medical treatment of a brain abscess requires properly performed CT or MRI follow-up examinations in order to realize early enough a possible growing of such a lesion.

Brain↗

PubMed2000

[Clinical aspects and diagnostic imaging in sarcoidosis of the nervous system].

The central nervous system is frequently affected during the course of sarcoidosis. Many of these lesions remain without clinical correlates. Unenhanced and contrast enhanced CT and MRI play an important role during the diagnostic work-up of patients with sarcoidosis and suspected or proved CNS involvement. This article summarizes the most frequent manifestations of neurosarcoidosis and provides illustrative examples of MRI findings.

Brain↗

PubMed2000

Cystic lesions of the pineal region--MRI and pathology.

Pineal lesions are rare. Tumours in this location comprise 0.4-1% of intracranial tumours. They grow mainly as solid-mass lesions, and cystic tumours are not common. On MRI, a cystic configuration is associated usually with non-neoplastic pineal lesions rather than with a tumour, but analysis does not allow cystic pineal tumours to be distinguished from glial cysts with certainty. We compared neuroradiological and pathological data from 13 cystic pineal lesions, analysing preoperative MRI. Formalin-fixed, paraffin-embedded surgical specimens were stained routinely and immunocytochemically, using the streptavidin-biotin-complex method. Histology revealed six pineocytomas, four glial cysts, an arachnoid cyst, a low-grade astrocytoma and a teratoma. Signal characteristics of pineocytomas were similar in many respects to those of glial pineal cysts. Histomorphological analysis allowed unambiguous discrimination between pineocytomas and glial pineal cysts.

Adolescent↗

PubMed1998

[Incidence of endomycoses in the autopsy material of the Berlin Charité Hospital].

From 1970 to 1993, a total of 93 endomf1p4es confirmed by post-mortem examination was diagnosed in the autopsy material of the Berlin Charité, a large hospital with an average of 1,500 hospital beds and maximum medical care. These comprised 51 candidoses (54.8%), 24 aspergilloses (36.6%), five cryptococcoses (5.4%), one zygomycosis, 1 trichosporosis and one coccidioidomycoses. This corresponded to 0.7% of the 13,375 decreased persons autopsied during this period. The frequency of autopsy was 85.3%. In 3,770 cases (2,418 adults and 1,352 children), brain dissection was performed. An adequate clinical putative diagnosis was made in only six out of 28 patients (18 adults, 10 children) with histologically confirmed cerebral mycosis [11 candidoses (39.3%), 10 aspergilloses (35.7%), five cryptococcoses (17.9%), one trichosporosis and one coccidioidomycosis]. About 80% of the mycoses of the CNS thus remained undetected while the patients were alive. Against the background of the continuing reduction in the frequency of autopsy in the Federal Republic of Germany, the observations made in the present paper underscore the demand for improved efficiency of mycological in-vivo diagnoses in the hospital and laboratory.

Adult↗

PubMed1996

p53 gene mutations in human astrocytic brain tumors including pilocytic astrocytomas.

Recent molecular biological studies have shown evidence for a distinct pathogenesis of pilocytic astrocytomas based on alterations other than mutations of the tumor suppressor gene p53. To prove these data, the authors screened a series of 42 astrocytic human brain tumors with a relatively high proportion (16.6%) of the pilocytic variant for the presence of p53 mutations, using the polymerase chain reaction (PCR) and single-strand conformation polymorphism (SSCP) analysis, followed by DNA sequencing. Mutations were found in one of seven (14.3%) pilocytic astrocytomas, in one of 18 (5.6%) low grade astrocytomas, and in one of four (25%) anaplastic astrocytomas, but in none of 13 glioblastomas. Sites of missense mutations were in exon 8 (codons 281 and 282), and exon 5 (codon 151). Silent mutation was found in exon 9 (codon 324), which was related to pilocytic astrocytoma. This is, to the authors' knowledge, the first report that shows a p53 mutation in pilocytic astrocytomas. However, the p53 mutation was only found in one of seven tumors of this entity and was a silent mutation, which does not lead to change of amino acids. Thus, the significance of this alteration for the development of this special tumor type seems to be low. Nevertheless, it may be a sign of genetic instability and is thus suggested to be of certain pathogenetic relevance. The p53 findings concerning the other tumors are in accordance with the view of p53 gene mutations to be early events in astrocytoma formation.

Adolescent↗

PubMed1995

Involvement of chromosome 22 in ependymomas.

We have karyotyped a total of twelve ependymomas using GTG-banding including seven for which preliminary results have already been published. One case showing hyperdiploid main line with two marker chromosomes was further analyzed by nonisotopic chromosome in situ suppression hybridization. It was shown that the marker chromosomes consisted of 1q, 14q and 1q, and 22q. The possible role of chromosome 22 in ependymomas and the usefulness of fluorescence in situ hybridization for cytogenetic analysis in tumor investigation are discussed.

Adolescent↗

PubMed1994

[Epidemiology of primary tumors of the central nervous system in children and adolescents. A population-based study].

The incidence of primary CNS tumours in children and adolescents (0-20 years of age) was investigated for the time period 1970-1989 using the records of the East German National Cancer Registry. 2906 such tumors were registered. 93.2% of them were histopathologically confirmed. The incidence was 31.0/1,000,000 for the male and 25.9 for the female population. 2719 (93.6% of all tumours) were intracranially and only 187 (6.4%) intraspinally situated. The incidence of the CNS tumours increased during the time period of the investigation. This increase was more pronounced in the male and less in the female population. The most common tumour types were gliomas (38.7% of all registered CNS tumours), medulloblastomas (14.0%) and ependymomas (10.9%). 130 primary CNS tumours occurred in infants (less than 1 year of age). Among these tumours were 21 ependymomas and 21 choroid plexus tumours.

Adolescent↗

PubMed1994

Nucleolar organizer regions (AgNORs) in primary and recurrent gliomas. A retrospective study.

To assess the diagnostic and prognostic relevance of nucleolar organizer regions (AgNORs) the silver-stained AgNOR structures of 46 human gliomas (22 primary gliomas, 21 first and 3 second recurrences) from 28 patients were counted and measured. Surgical specimens of two neuropathological centres were included in this retrospective study. The tumours were classified into 8 groups according to WHO criteria of diagnosis and grading and their state of recurrence: 1: primary glioma grade II; 2: primary glioma grade III; 3: glioblastoma grade IV; 4: first recurrence of glioma grade II; 5: first recurrence of glioma grade III; 6: first recurrence of glioblastoma grade IV; 7: second recurrence of glioma grade II and 8: second recurrence of glioma grade III. AgNOR numbers demonstrated a positive correlation with increasing histological grade. Similarly, higher levels of AgNORs were in general found in recurrent tumours compared to primary tumours with few exceptions in groups, in which few numbers only could be analyzed. The present study demonstrates the utility of evaluating AgNOR in assessing malignant gliomas and indicates that recurrences have a higher proliferative potential than original tumours. The problems of standardization of the AgNOR technique are especially crucial for the retrospective evaluation of fixed and embedded pathological material.

Adult↗

PubMed1994

[Tuberculosis of the CNS--once again a current neurosurgical disease].

Tuberculosis is relative uncommon in developed countries. The incidence is still considerable in India, in the Middle East and South America. Neurosurgically important manifestations of tuberculosis are intracranial tuberculomas, tuberculous meningitis with hydrocephalus and tuberculous spondylitis. Therefore experiences in the management of these three kinds of neurotuberculosis are described and an overview of the literature is given. The diagnosis of intracranial tuberculomas is difficult because the symptoms are similar to those of many other types of intracranial tumor and the CT-findings are unspecific. Clinical recognition of neurotuberculosis rests mainly on the evidence of the general disease. A positive skin test supports the diagnosis. The management of tuberculomas enclosed stereotactic biopsy to confirm the diagnosis, medical treatment and surgery. But surgery is only indicated for larger symptomatic mass lesions producing midline shift and severe intracranial hypertension. With antituberculous drugs treated tuberculomas begin to decrease in size within the first two months of therapy. Hydrocephalus is a common complication of tuberculous meningitis. Due to the low incidence the first diagnosis in industrialized countries is a hydrocephalus of unknown origin. The diagnosis of tuberculous meningitis is suspected in any case of subacute meningitis with a CSF profile demonstrating a predominance of lymphocytes, elevated protein value, hypoglycaemia and negative stains for bacterial or fungal organisms, because positive smears for acid-fast bacilli are seldom. Without treatment, tuberculous meningitis is invariably fatal within 4 to 6 weeks. Therefore, when ever tuberculous meningitis is suspected, antituberculous therapy must be started.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1994

[Primary diffuse leptomeningeal leiomyomatosis].

A female patient aged 28 fell ill with manifestations of sensory aphasia, headache and signs of cerebral compression. Except for a bilateral papilledema there were no further pathological clinical and paraclinical findings. Later on, amaurosis, deafness, anosmia and generalized muscular hypotonia developed. The nuclear magnetic resonance image revealed a major accumulation of contrast medium in the leptomeninx. Biopsy demonstrated a mesenchymal neoplasm in the leptomeninx. After a strong rise in intracranial pressure, the patient died from a bulbar brain syndrome. Microscopy revealed a diffuse neoplasm limited to the leptomeninx of brain and spinal cord as well as the immediate neighbourhood of small cortical vessels which, by morphological criteria, was classified as low-grade malignant. With the aid of electron microscopy, the tumour cells could be identified as descendants of smooth muscle cells.

Adult↗

PubMed1994

Comparative genomic hybridization of human malignant gliomas reveals multiple amplification sites and nonrandom chromosomal gains and losses.

Nine human malignant gliomas (2 astrocytomas grade III and 7 glioblastomas) were analyzed using comparative genomic hybridization (CGH). In addition to the amplification of the EGFR gene at 7p12 in 4 of 9 cases, six new amplification sites were mapped to 1q32, 4q12, 7q21.1, 7q21.2-3, 12p, and 22q12. Nonrandom chromosomal gains and losses were identified with overrepresentation of chromosome 7 and underrepresentation of chromosome 10 as the most frequent events (1 of 2 astrocytomas, 7 of 7 glioblastomas). Gain of a part or the whole chromosome 19 and losses of chromosome bands 9pter-23 and 22q13 were detected each in five cases. Loss of chromosome band 17p13 and gain of chromosome 20 were revealed each in three cases. The validity of the CGH data was confirmed using interphase cytogenetics with YAC clones, chromosome painting in tumor metaphase spreads, and DNA fingerprinting. A comparison of CGH data with the results of chromosome banding analyses indicates that metaphase spreads accessible in primary tumor cell cultures may not represent the clones predominant in the tumor tissue.

Adolescent↗

PubMed1993

Age-related nonrandom chromosomal abnormalities in human low-grade astrocytomas.

We report a cytogenetic investigation of 55 low-grade astrocytomas in 52 patients, 15 children and 37 adults. In addition to numerical aberrations such as trisomy 7 and gonosomal losses, we found structural and/or numerical aberrations of chromosome 1 in eight astrocytomas. There was a striking difference between the rearranged chromosomes in pediatric and adult patients. Whereas the pediatric tumors revealed monosomies 1p with accompanying trisomy 1q, the astrocytomas in adults showed partial or complete monosomies 1q.

Adolescent↗

PubMed1993

[Pathomorpholigical findings in ketothiolase deficiency].

The post-mortem findings in two brothers who had suffered from clinically and biochemically confirmed ketothiolase deficiency are reported. They had died as a consequence of metabolic-acidotic crisis at the age of 6 years and 9 months and 4 years and 1 month, respectively. Autopsy revealed cardiac hypertrophy and brain pathology in both children. The latter consisted of loss of neurons, spongiosis and slight reactive astrocytosis affecting parasagittal areas of the parietal and occipital cortex, visual cortex, putamen, caput nuclei caudati and claustrum. Furthermore demyelination of the visual pathways, including chiasma opticum, was seen. Changes in both hemispheres were almost symmetric. In the younger child, changes were less severe than in the older one in whom the course of the disease had been longer. To the best of our knowledge this is the first report of autopsy findings in siblings with ketothiolase deficiency.

Acetyl-CoA C-Acyltransferase↗

PubMed1993

How to run a "brain bank"? Clinical and institutional requirements for "brain banking".

"Brain Banking" or prospective sampling of tissues relevant to the study of neurological disease is a complex task which needs organization at various levels of operation such as the establishment of a donor system, recruitment of clinical assessment centres, establishment of standardized assessment protocols, the inauguration of logistic structures for brain removal and transport to the bank, proper storage of patient data and tissues, histological verification of the disease and availability of tissue and clinical data to researchers. This effort certainly promises to bear fruit since there is a striking lack of precise prospective studies into etiology and pathogenesis in most neurological diseases especially in the field of the neurodegenerative diseases.

Brain↗

PubMed1992

[Expression of immunohistochemical differentiation markers in normal and transformed neoplastic neuroectodermal stem cells].

The cells of primitive neuroectodermal tumours may undergo differentiation and, eventually, may be transformed to neurons, glial cells, and ependymal cells. Early stages of neuroectodermal differentiation may primarily be determined by means of immunohistochemical methods. Immunohistochemical investigations were performed on brains of human foetuses obtained from the 18th to 36th weeks of pregnancy, with a view to elucidating the process of maturation during foetal development and to determining the antigens identifiable in cells in the course of differentiation, following fixation in formalin and embedding in paraffin. Gliafibrillar acid protein (GFAP) and vimentin proved to be of particularly high stability and, consequently, were easily detectable from paraffin material. The same antigens were focally recordable also from eight of 17 primitive neuroectodermal tumours. Clues were rare in these tumours as to neuronal differentiation. This was attributed to instability of neurofilament proteins under conditions of formalin fixation and paraffin embedding.

Adult↗

PubMed1992

[DNA synthesis activity and growth fractions of brain tumors].

Histoautoradiographic and immunohistochemical studies of biopsies from 27 brain tumors yielded the following preliminary conclusions: 3H-thymidine labeling indices and Ki-67 growth fraction indicate the proliferative activity more reliable than counting of mitoses, the fraction of DNA synthesizing tumor cells and the Ki-67 growth fraction show an equal behaviour, as expected, the immunohistochemical values are higher than the labeling indices because all tumor cells within the proliferation cycles express the nuclear antigen. By the increase of the number of tumors and the use of the different cell kinetic methods on the same tumor tissue the prerequisite to grading of brain tumors as a basis of therapeutic strategy can be improved.

Astrocytoma↗