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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

At least 271 records · Page 15Linked to original sources

[Adult metachromatic leukodystrophy manifested as schizophrenic psychosis (author's transl)].

An autopsy case of adult metachromatic leukodystrophy (MLD) manifested clinically as schizophrenic psychosis is reported. A 50-year-old man developed progressive mental changes 10 years before his death, and later manifested a schizophrenic syndrome without neurologic deficits or EEG changes. After his death from uremia neuropathology disclosed MLD with demyelination accentuated in the frontal lobes and abundant metachromatic deposits in the preserved areas of cerebral white matter. Neurochemical examination of the demyelinated frontal area showed reduced concentration of cerebrosides and sulfatides, decreased amounts of total lipids in the tissue, and an increase of sulfatides, and particularly of their cerebron fractions in lipid extract. The problems of adult forms of MLD with prolonged course are discussed with special reference to cases showing mainly psychiatric syndromes.

Atrophy↗

Cerebrovascular amyloidosis with cerebral hemorrhage.

More than 1400 necropsies performed on patients with either a nontraumatic cerebral hemorrhage (400 cases) or with dementia over the age of 55 (1010 cases), or both, have been reviewed. There were 15 cases in which a cerebral hemorrhage had occurred together with cerebral amyloid angiopathy all of whom had been demented. Eight of the 15 patients were hypertensive. The 7 non-hypertensives showing only the amyloid change included two cases of "atypical" Alzheimer's disease with acute neurological features, and 5 cases of senile dementia (aged 72 to 78 years) coupled with focal neurological disorders. In the hypertensive patients, aged 67 to 86 years, with a progressive dementing syndrome and acute neurological signs, multiple ball-like hemorrhages (7 cases) and/or cerebral hematomas (3 cases) were associated with a combination of amyloid and hyalinar (hypertensive) angiopathy, often affecting segments of the same pial and cortical vessels. From these data and recent reports on lethal cerebral hemorrhage occurring spontaneously or after neurosurgical procedures in demented old people, cerebral amyloid angiopathy, which is not necessarily associated with systemic amyloidosis or severe (pre)senile cerebral degeneration, may be considered a rare but important cause of cerebral hemorrhage in the aged. The "vascular" type of presenile dementia, occasionally complicated by focal cerebrovascular lesions or bleeds, is considered a variant of Alzheimer's disease. The mechanism leading to formation of cerebral amyloid is unknown.

Aged↗

[The value of imprint cytology in neurosurgical diagnosis (author's transl)].

Cytological smear techniques have been found valuable in obtaining a rapid histological diagnosis during neurosurgical biopsies, although their accuracy is questioned. This method is particularly appropriate in the case of small pieces of tissue which may be difficult to cut in the cryostat. A method developed to obtain multiple touch preparations was used in 332 consecutive neurosurgical biopsies; these were examined and compared with subsequent paraffin sections of the same biopsies. The correct diagnosis was obtained from the smears in 93.7% of the cases, while the diagnostic accuracy of paraffin sections was 98.2%. The imprints also allowed correct grading of gliomas. Most of the errors or inconclusive cytological diagnoses stemmed from failure to distinguish between primary and secondary anaplastic CNS tumours, between neurinomas and fibroplastic meningiomas, or between glial scar or normal brain tissue and isomorphic astrocytomas. The differentiation of these lesions is, however, often difficult even in paraffin sections. In accordance with the results of other authors it is suggested that the touch cytology is one of the most appropriate techniques available for rapid neurosurgical diagnosis.

Cytological Techniques↗

Brain monoamines in metabolic (endotoxic) coma. A preliminary biochemical study in human postmortem material.

Spectrofluorometric assays of DA, 5-HT and 5-HIAA were performed in different areas of postmortem brains of humans who died in hepatic and uremic coma. Brain DA showed a mild general decrease, the average reduction being 20 to 30 percent of the controls. By contrast, 5-HT and 5-HIAA were markedly increased in most brain areas, most significantly in the reticular and raphé nuclei of the brainstem and in some parts of the limbic system. Despite individual and regional differences, the monoamine changes had similar patterns in both hepatic and uremic coma. These data in human brain which confirm previous clinical and experimental studies in hepatic encephalopathy suggest some common disorders of central monoamine metabolism in endotoxic coma of different origin. Brain edema, a constant feature in toxic coma, is probably due to increased 5-HT content in the brain, the pathophysiologic effect of 5-HT on the blood-brain barrier being well established. The prominent changes of indoleamine metabolism in the reticular brainstem and limbic systems may be related to disorders of consciousness.

Aged↗

Morphological changes in anaplastic gliomas treated with radiation and chemotherapy.

The effects of antineoplastic treatment on gliomas are related to tumour cell cycle and proliferation kinetics, glioma tissue architecture, and the surrounding environment. Morphological changes induced by radiation and chemotherapy are characterized by cell necrosis and severe alterations in cell and nuclear morphology caused by changes in the cell kinetic parameters which, however, may also occur spontaneously in untreated anaplastic gliomas. Comparative studies of cytological imprints and routine histological preparations of biopsy and autopsy specimens were performed in four groups of anaplastic astrocytomas and glioblastomas (78 cases) with postsurgical irradiation, combination chemotherapy, and CCNU treatment, and without specific postsurgical treatment (control group). Following radiation and chemotherapy, in addition to increased necrosis and vascular response, a variety of characteristic but nonspecific changes were observed in cell and nuclear morphology with prominent formation of multinucleated giant and monstrous cells, irregular and hyperchromatic nuclei, and severe cytoplasmic degeneration indicating both inhibition of cell division and cell damage. Statistically significant findings were a posttreatment increase in the number of multinucleated giant and monstrous cells and a decrease in the number of mitoses. These changes were more pronounced after chemotherapy than after radiation, while no significant dissimilarities were found between combination chemotherapy and CCNU. The implications of these changes on the mechanisms of antitumour treatment in anaplastic gliomas are discussed.

Brain Neoplasms↗

[Abnormal scintigrams in demyelinating diseases of the brain (author's transl)].

In 6 patients with acute or exacerbating demyelinating disease and in 2 cases suffering from adrenoleucodystrophy (Schilder's disease) brain scintigraphy revealed areas with increased isotope uptake. The pathological foci were verified by autopsy in 4 cases and by inspection in 1. These foci are difficult to distinguish from other localized brain diseases causing increased isotope uptake; in addition to the clinical course and to spinal fluid abnormalities reversibility of the scintigraphic lesions indicate demyelinating diseases. The typical pattern of symmetrical lesions within the white matter and the progression of abnormalities from the occipital to the frontal lobe speak in favor of Schilder's disease.

Adult↗

Pathology of intracerebral hemorrhage.

Spontaneous intracerebral haemorrhages are dealt with on the basis of a great number of clinical and neuropathological cases. They amounted to 4.9 per cent of the patients admitted to the hospital and 5.2 per cent of the autopsies. In two thirds hypertension was found about 20 per cent were ruptured aneurysms. In 3 per cent of the respective autopsies no cause was found. The clinical courses are dealt with in greater detail. Eighty per cent of the hypertensive haemorrhages are located in the cerebral hemisphere; among these, 60 to 80 per cent show ventricular perforations. This percentage is still higher in thalamus--brain stem haemorrhages. Two theories on the aetiology are discussed. Traumatic haemorrhages are especially dealt with in connection with late apoplexy. Deformations of the cerebral vessels, too, are described in detail. Among the aneurysms, 82 per cent belong to the circulus willisi. Angiomas were found in 15.2 per cent of the autopsies in case of cerebral haemorrhages, but in 58 per cent of the surgically treated intracerebral haemorrhages. These figures are higher than those given in the relevant literature. Among 1600 cerebral tumours, there were 2.2 per cent massive cerebral haemorrhages. The cases are compiled in a table.

Aneurysm, Infected↗

[Extraneural metastases of anaplastic gliomas (author's transl)].

8 cases are reported of anaplastic cerebral gliomas with metastases outside the neuraxis, which were seen among about 1600 gliomas. The series included 4 males and 4 females ranging in age from 5 to 58 years at the time of death. There were 2 children with anaplastic ependymomas, one adult with oligodendroglioma, and 5 young or middle-aged adults with astrocytomas grade III or IV. All patients had one or more craniotomies, and 5 had radiotherapy before the appearance of remote tumor deposits. The survival times ranged from 7 to 31 months in cases with gliomas grade II, and from 8 to 18 months in high-grade astrocytomas. All tumors showed invasion of the meninges and/or ventricle walls, and in 4 cases they transgressed the dura and surrounding bone or soft tissues. In 6 autopsy cases there was widespread dissemination of gliomas through the CSF pathways. Remote metastases involved regional or distant lymph nodes in 7, the lungs in 2, the vertebrae, lungs, pleura, parotis and mediastinum in one patient each. The possible pathways for distant spread of intracranial gliomas and the factors which are considered responsible for their rare occurrence are discussed.

Adolescent↗

Human central nervous system lesions following radiation therapy.

With the increasing number of high-energy irradiation sources, the cases of radiolesions of the CNS are also increasing. The irradiation effects on nerve and tumour tissue and the various kinds of irradiation damage are discussed. They become manifest as (a) acute radionecrosis (rare) and (b) late reactions which are subdivided into delayed lesions and the more frequently occurring late necrosis proper. In early and early-delayed lesions, morphologically local spongy demyelination with loss of the oligodendroglia and astrocytic reaction are seen while vascular damage is characteristic of late irradiation damage. The author's observations include clinic, pathology, and time/dose dependence of the irradiation damage of brain and spinal marrow. The tolerance values of the spinal cord and the relative biological effects of the irradiation are dealt with. In spite of a good knowledge of clinic and pathology of the radiolesions and the improvement of irradiation techniques, the widely differing individual reactions of the CNS to ionising rays remain partly unclear.

Acute Disease↗

Involvement of the central nervous system in malignant lymphomas.

A retrospective histologic study of 145 consecutive autopsy cases of systemic malignant lymphomas (including lymphatic leukemias) was performed. The classification followed the Kiel Classification (Gérard-Marchant et al., 1974). There was an overall secondary CNS involvement in 26.2% of the total or in 30.4% of the non-Hodgkin's lymphomas including ALL, with intracranial lesions in 21.4 and 26.1%, respectively, and spinal epidural spread in 5.5 (5.1%). Peripheral nerve involvement was seen in almost 40% of the examined cases. Ten further cases were isolated ("primary") intracranial lymphomas without evidence of extraneural deposits or systemic lymphatic disease. The CNS complications in non-Hodgkin's lymphomas were diffuse meningeal and/or perivascular infiltration with or without invasion of the nervous parenchyma, and did not differ from those in CNS leukemia. Isolated solid mass lesions in the brain were only present in 7% of the secondary CNS lymphomas, but were seen in all instances of "primary" cerebral lymphomas. The incidence of CNS complications was highest in lymphoblastic lymphomas including ALL (39%), CLL (31%), immunocytic lymphoma (29%), less frequent in immunoblastic (18.7%), and centrocytic lymphomas (16.6%). No intracranial lesion was observed in centroblastic-centrocytic and centroblastic lymphomas which only produced epidural spread. Bone marrow involvement was present in 92.8% of the cases with secondary CNS lesions, and in 83.2% of the epidural lymphomas. Leukemic conversion, present in 44% of the total (52% with ALL), was demonstrated in 83.3% of the cases with secondary brain lesions, but was hardly combined with epidural spread. The histologic pattern of CNS lesions in non-Hodgkin's lymphomas and their frequent association with leukemic conversion suggest the importance of hematogenous dissemination rather than of direct spread from bone marrow or local manifestation in multisystem disease. Isolated ("primary") lymphomas of the CNS which are morphologically identical with the extraneural lymphomas may represent a primary, often lethal manifestation of a multisystem disease with or without secondary generalization.

Brain Diseases↗

[Cerebral involvement in the Sézary syndrome (author's transl)].

Two foci of histologically verified mycosis fungoides were found in the left parietal region of a 45-year-old man with involvement of the brain during an exacerbation of leukemia. There was perivascular and diffuse infiltration with lymphoma-like cells and occasional giant (Lutzner) cells but no involvement of the meninges. Both before and after operation the CSF contained a few atypical lymphocytes and mononuclear cells, one of which showed ultrastructurally the deeply clefted nucleus of Sézary cells. Atypical cells were confirmed morphologically in the peripheral blood, as well as by cytochemical (beta-glucuronidase) and immunological (rosettes with SRBC) markers, after the postoperative leucocytosis had subsided. It is suggested that this CNS involvement during a leukemic conversion phase of mycosis fungoides is consistent with the Sézary syndrome.

Brain Neoplasms↗