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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

At least 289 records · Page 16Linked to original sources

[Neuropathology of renal transplantation (author's transl)].

The neuropathological features are presented of an autopsy series of 43 renal transplant recipients. Inflammatory CNS lesions were found in 18 cases and were attributable to secondary CNS involvement in bacterial or fungal septicaemia (8 cases) or in localized extraneural infections (5 cases). Mixed bacterial-fungal infection and bacterial-fungal-viral triple infection were seen in one case each. Isolated toxoplasmic granulomas were observed in two cases. No tissue changes typical of CNS viral infections, including cytomegaly, were found except for small glial nodules in the brain stem in 7 cases. Massive intracerebral haemorrhages were seen in 2 cases after haemodialysis; subdural haematoma occurred once. Non-specific changes including brain oedema and diffuse glial poliodystrophy were found in many cases; vascular lesions and purpura occurred only seldom. A meningocerebellar malignant lymphoma, probably of metastatic origin was found in one case. Clinico-pathological correlation was poor in 5 cases; this is considered to be due to immunosuppressive therapy, which may mask either clinical expression of severe CNS lesions or morphological expression of viral encephalitis.

Adult↗

Scintigraphy in lymphomas and leukemia of the brain.

Scintigraphy with 99mTc-pertechnetate made it possible to detect focal brain lesions in 4 patients with primary malignant lymphomas of the brain, one patient with CNS involvement with lymphatic immunoblastoma, one patient with Sézary syndrome (mycosis fungoides and T cell leukemia) and one child with a space occupying infiltration of myeloblastic leukemia. The scintigraphic results were verified by morphological data from biopsy or autopsy.

Aged↗

Extracranial metastases of anaplastic cerebral gliomas.

Seven cases are reported of anaplastic cerebral gliomas with metastases outside the neuraxis, seen among about 1500 gliomas. There were two children with anaplastic ependymomas, one adult with oligodendroglioma, and four young to middle-aged adults with astrocytomas grade III and IV. All patients had one or more craniotomies, and four had radiotherapy prior to the appearance of distant tumour deposits. The survival times ranged from 7 to 31 months in cases with gliomas grade II, and from 8 to 18 months with high grade astrocytomas. All seven tumours showed invasion of the meninges, ventricular walls, or both, and in four cases they transgressed the dura and surrounding bone or soft tissues. In six autopsy cases there was widespread dissemination of gliomas through the CSF pathways. Distant metastases involved regional or distant lymph nodes in six patients, the lungs in two, and the vertebrae, pleura, liver, or mediastinum in one patient each. The possible pathways for distant spread or intracranial gliomas and the factors which are considered responsible are briefly discussed.

Adolescent↗

[Intramedullary oligodendroglioma with meningocerebral dissemination (author's transl)].

A 28 year old woman suffered from lumbar radicular signs and sensory deficit for three years before death. A progressive illness with increased intracranial pressure, cerebellar signs, and multiple cranial nerve palsies appeared one year after the onset of symptoms, but craniotomy of the posterior fossa was negative. After development of spastic paraparesis, an intramedullary oligodendroglioma of the lower thoracic cord was partly removed. Death occurred two months later from raised intracranial pressure. At autopsy a diffuse oligodendroglioma was found involving the periventricular regions of the brain, the entire subarachnoid space, the cerebral parenchyma, and the whole length of the thoracic cord. There was a small lumbar syrinx. It is suggested in this case that the dorsal intramedullary oligodendroglioma was the source from which tumour spread diffusely through the cerebrospinal fluid pathways and secondarily invaded the cerebrum. The diagnostic difficulties of diffuse meningeal gliomatosis are discussed.

Adult↗

Intermittent meningitic reaction with severe basophilia and eosinophilia in CNS leukaemia.

Light- and electron-microscope studies were performed on CSF cells from a patient with CNS leukaemia presenting with an intermittent meningitic reaction. Numerous fully matured basophilic and eosinophilic granulocytes with excess glycogen content accompained undifferentiated leukaemic blast cells in the CSF. Such a CSF cell reaction has not been previously reported. It is suggested that this isolated CSF reaction represents a special type of immediate hypersensitivity reaction triggered by an abnormal leukaemic giant cell clone, and mitigated by the accompanying eosinophilic granulocytes.

Acute Disease↗

Agyria-pachygyria (lissencephaly syndrome).

A clinico-pathological report is given on 4 cases of agyria (premature neonate to age 13 months), 3 cases of pachygyria (aged 2,5 to 4,3 years) and a boy aged 4,5 years with temporal pachygyria and frontal microgyrias. Clinical features, more pronounced in agyria than in pachygyria, were microcephaly, frequent facial anomalies, neonatal feeding difficulties, hypotonia with subsequent seizures, hypsarrhythmic EEG pattern in 3 children, arrest of psychomotor development and signs of decerebration. One case of agyria occurred with familial faciorenal dysplasia, two were associated with congenital heart disease, and the fourth with chromosomal abnormality. Morphologically, the colpocephalic brain showed a four-layered agyric pallium with radially aligned cell columns and periventricular heterotopias, lacking differentiation of the claustra, olivary heterotopias and cerebellar dysgenesias in the 4 younger infants. In the agyric neonate additional agenesis of corpus callosum was present. Pachygyric brains showed a six-layered cortex, periventricular heterotopias, lacking differentiation of the claustra, but no cerebello-olivary anomalies. Cytoarchitectonic analysis of the agyric cortex suggests a disorder of neuronal migration during stage III of neocortex formation (Rakic and Sidman) between the 11th and 13th fetal week, while the pachygyric cortex showing the later formed layers II and IV presumable is caused by an attenuated and later disorder acting in early stage IV of neocortex formation, i. e. around or after the 13th fetal week. Additional insula-claustrum dysplasia, olivary and cerebellar anomalies are due to concomittent migration disorders between the 11th and 14th week. Along this period there is a gradient from agyric to normal six-layered cortex, whereas microgyria presumably results from an event occurring after migration has terminated (after the 16th fetal week). Etiological factors of agyria-pachygyria may be both hereditary (familial lissencephaly-syndrome) and environmental ones (prenatal drug application or intrauterine perfusion disorders).

Abnormalities, Multiple↗

[Cavitating neutral fat leukodystrophy with recurrent course].

A girl with non-contributory family history, at the age of 7 years developed a recurrent ataxic-spastic syndrome followed by disorders of consciousness and seizures. She died in a decerebrate state at the age of 10 9/12 years. Diffuse EEG abnormalities changed in accordance with the clinical course. CSF findings were always negative. Brain biopsy performed at the age of 9 5/12 years was non-diagnostic. No clinical signs of adrenal insufficiency were present. Neuropathological examination disclosed severe demyelination of the cerebral white matter with formation of large periventricular cavities, relative sparing of the subcortical arcuate fibers, and peripheral myelin breakdown of the orthochromatic (neutral fat) type. There was complete absence of inflammatory lesions, comparative preservation of axons and oligodendroglia with moderate astroglial reaction. The optic system and cerebellum were not involved. Additional postanoxic changes were seen in the thalamus and hippocampus. The sporadic case is suggested to represent a cavitating form of neutral fat (simple orthochromatic) leukodystrophy which is to be separated from Schilder's diffuse sclerosis and adrenoleukodystrophy. The etiology of the disorder is unknown.

Ataxia↗

The significance of perivascular infiltrations in multiple sclerosis.

143 autopsy cases of multiple sclerosis (19 acute and 124 chronic cases) were analysed histologically for the extent of active demyelination and the degree of infiltration within and outside the demyelinating lesions and in the leptomeninges. The results were compared with the duration of the illness. Infiltrations were found in 60% of all cases but more often (74%) in those with active demyelination. Inflammatory lesions outside demyelinating foci were observed in 27% of the total, and in 80% of them active demyelination was present. Inflammatory lesions in the meninges were present in 41% of the total and in 80% of these were accompanied by active demyelination. The duration of illness correlated with decreasing severity of active demyelination and of perivascular infiltration. Patients treated with cortico-steroids and/or immunosuppressive substances showed no or only moderate inflammatory lesions. The duration of illness in both these groups was significantly longer than the average of untreated patients. The significance of these pathological findings for the CSF cytology in multiple sclerosis is discussed.

Adrenal Cortex Hormones↗

[Morphological aspects of organic brain syndromes (author's transl)].

Organic psychoses are caused by a variety of disorders. In general, they are due to diffuse dysfunction of the brain without any specific anatomical basis, but they may be modified by disorders of some distinct neuronal systems as a result of local accentuation of a dissuse morbid process or localized brain damage. Acute organic psychoses are usually caused by disorders of the blood-brain barrier (cerebral oedema), acute neuronal dysfunction of disorders of synaptic transmission. Acute lesions may be reversible or terminate in stationary defective states or progressive neuronal degeneration. In late stages, the anatomical sequelae of the basic process and of secondary lesions are hardly to be separated. The non-specificity and inconsistency of brain lesions is demonstrated in chronic alcoholic psychoses. In senile organic psychoses there are quantitative correlations between psychopathological, neurophysiological (slowing of the basic rhythm) and morphological changes, mainly characterized by loss of neurons and synaptic contacts. Vascular syndromes are often over-diagnosed clinically. Localized mental syndromes are non-specific, but show characteristic locations, as they are commonly associated with dysfunction of neuronal systems engaged in storing and recall of information (frontobasal region and limbic system). Further detailed studies are needed in order to achieve better correlation between specific features of behavioural and intellectual defects and anatomical location and quantitiy of lesions.

Aging↗

Primary malignant lymphomas of the central nervous system in man.

Sixty-eight primary malignant lymphomas of the CNS exclusively confined to the brain and its leptomeninges from a series of about 8000 intracranial neoplasms (incidence 0.85%) were examined and classified according to current histopathologic criteria. Average age at onset of symptoms was 55 years, mean duration of illness to time of diagnosis was 3 months. Survival averaged 1,8 months with supportive care, but 17,2 months with surgery, radiation and/or chemotherapy. CSF cytology was useful and reliable tool for clinical diagnosis. The cerebral hemispheres were affected in about 50%, the basal ganglia in 18%, posterior fossa in 10%, while multifocal lesions amounted to 22%. All CNS tumors were of the diffuse type of non-Hodgkin's lymphomas; no follicular (germinal center) lymphomas were observed. Three histological patterns comparable to extraneural lymphomas were distinguished: Immunoblastoma (reticulosarcoma) occurred most frequently (58.8%), lympho-plasmacytoid immunocytoma constituted 28 percent, while lymphoblastic lymphoma occurred least frequently (13.2%). There were no significant differences with regard to onset, location, growth pattern or clinical course except for a much poorer prognosis of lymphoblastic lymphoma. Although there are no definite cytological differences between malignant lymphomas arising in extraneural sites or as primary lesions in the CNS, the latter showed a much greater proportion of phagocyting histiocytes (and microglia) and a frequent occurrence of plasmacytes and their precursors which apparently exceded pure host reaction. The prognostic value of modern classification schemes for CNS lymphomas needs further critical evaluation.

Adolescent↗

SSPE-like inclusion body disorder in treated childhood leukemia.

Clinico-pathological report on a boy with cytostatically treated leukemia, dying with cerebral symptoms after passing clinical measles 10 weeks before death. At autopsy, numerous nuclear inclusion bodies in glial and nerve cells were found. By electron microscopy, nuclear inclusions appeared as loosely arranged smooth tubules, corresponding to paramyxovirus nucleocapsids. Frequently, cytoplasmic changes appeared too, consisting of incomplete tubular structures and an abundant dense "fuzzy" material. No regular tubuli of the coated granular type were present, as in common measles virus infection, nor any mature viral structures or differentiation of the surface membrane. The lack of maturation in cytoplasm together with a predominance of nuclear changes suggested a slow type of measles virus infection, while the particular cytoplasmic changes suggested a defect in synthesis of granular nucleocapsids, possibly a basic factor for the slow type of the viral infection. Possible pathogenetic factors are discussed.

Brain↗

Histological subtypes and prognostic problems in meningiomas.

The incidence of the various histological subtypes of meningiomas was examined in 1238 patients with surgically treated meningiomas, about 80% arising within the cranial cavity. The histological classification used was that of Courville (1950) and Rubinstein (1972), but "angioblastic" meningiomas were segregated into 3 groups: highly vascularized meningiomas, hemangioblastomas, and hemangiopericytomas. Endotheliomatous and transitional forms constituted 85% of the total (71.5% of intracranial tumors), fibroblastic forms 6.6 and 7.5%, respectively, and highly vascularized (endotheliomatous or transitional) meningiomas 5.2% of the intracranial tumors, while true "angioblastic" meningiomas (hemangioblastomas and hemangiopericytomas) amounted to 2.8% of the total (3.1% of the intracranial tumors). 1.2% were "atypical" (so-called malignant) meningiomas; true meningeal sarcomas were excluded. The incidence of recurrence in patients surviving at least 5 years after apparently complete removal of the tumor was 13% for all sites, and 14.2% for intracranial tumors, but almost twice as high after partial removal. There were no significant differences in the recurrence rate and intervals between first and second operation according to the various histological subtypes of meningiomas, except for hemangiopericytomas which recurred with significantly higher frequency and, together with atypical meningiomas, at much shorter intervals than the others. The prognostic significance of some histological criteria in "non-angiomatous" meningiomas was examined in 211 patients surviving at least 5 years after apparently complete removal of the tumor. Among the recurrences, there was a significantly higher degree of cellularity and increased mitotic rate and, probably, of cortical invasion, while nuclear pleomorphism, increased vascularity, and focal necroses showed no definite differences. The presence of mitotic figures alone appeared to be of no prognostic value. While most recurrent meningiomas did not change their basic morphological type significantly, about 12.5% of the recurrences appeared to have a different rate of growth as suggested by increased cellularity and mitotic rates. In 2 cases an isomorphic (benign) meningioma became a true spindle cell sarcoma.

Adult↗

[Oculocerebrovertebral syndrome].

After description of the oculovertebral syndrome Weyers-Thier a personal observation is reported. It was characterized by severe cerebral malformations (agenesis of the olfactory lobes, incomplete agenesis of corpus callosum with large sagittal arachnoidal cyst) associated with polycystic brain damage. The relationship of this "oculovertebral syndrome" to the "oculoauriculovertebral syndrome" is discussed. The combination of various malformations in our observation indicates an embryonic lesion between the 4th and 11th week. Additional polycystic brain damage was due to perinatal anoxy. The causal factor of the malformative syndrome remains unknown, but similar lesions were experimentally induced by fetal hypoxia.

Brain↗