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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

At least 217 records · Page 12Linked to original sources
PubMed1982

Morphological and biochemical changes in the aging brain: pathophysiological and possible therapeutic consequences.

Significant reduction in brain weight and in the number of cortical neurons with increase of astroglia in aging brain and SDAT are associated with decreased synthesis and turnover of some neurotransmitters, particularly affecting the DAergic system. Progressive loss of TH activity reaching in SDAT almost the low levels of Parkinson disease is associated with progressive decline in DA concentration in the nigro-striatal system. Increase in MAO-B activity and in the ratio of MAO-B: MAO-A reported by some authors in aging brain and SDAT, however, was not confirmed in human frontal cortex in both Parkinson disease and SDAT. However, the location of both types of MAO in human brain is debatable, since preliminary studies indicate that, unlike in rat brain, MAO-B appears to be the major degradating enzyme of biogenic amines in human brain, while MAO-A might be associated, at least in part, with neuronal structures. Reduction in DAergic parameters in aging brain are also reflected in a decrease of adenylate cyclase activity and of D2 DA receptors. Animal data on decrease of DA-receptor density in the striatum with age were confirmed in human Parkinson disease and Alzheimer disease. These disturbances in neuronal feedback systems may be responsible for pathophysiological and behavioral changes in old age.

Aged↗
PubMed1982

Cerebral atrophy in Parkinson syndrome.

Review of 100 autopsy cases of Parkinson disease (PD), 100 age and sex matched controls, 16 cases of senile Parkinson (sP), 85 cases of senile dementia of Alzheimer type (SDAT) and 55 cases of Alzheimer disease (AD) showed that the average brain weight in PD was not significantly less than in controls but was much higher than in sP and SDAT. PD shows only slight but insignificant increase of cortical neuronal loss and Alzheimer lesions (AL) as compared to controls, while in PD the AL are significantly less severe than in sP, SDAT and AD. There is no increased simultaneous occurrence of PD and AD. Several types of PD are separated: PD with dementia with a) subcortical PD lesions combined with AD or severe AL, b) severe AL with little nigral damage; c) severe AL with cortical Lewy bodies; PD without dementia with no or little AL. Demented PD patients with severe AL are older than non-demented ones without AL. In general, there is good correlation between the degree of dementia and the severity of AL in both PD and controls.

Age Factors↗
PubMed1982

Adjuvant treatment of Parkinson's disease with dopamine agonists: open trial with bromocriptine and CU 32-085.

Bromocriptine (CB-154) and the 8-alpha-ergoline CU 32-085, two dopamine receptor agonists, were administered at different times to two series of 22 patients with Parkinson's disease, most of whom took levodopa (plus benserazide) at optimum dosage. The addition of bromocriptine (mean daily dose 32 mg; after 6 months 40 mg) led to a 38.5% reduction of levodopa, while CU 32-085 (mean daily dose 15.2 mg; after 6 months 17.5 mg) permitted a 33.7% reduction in levodopa. The mean dose in two patients on CU 32-085 monotherapy was 55 mg/day. A total of 15 patients tolerated adequate doses of bromocriptine (5-75 mg/day, mean duration of treatment 7.5 months) and 15 patients long-term treatment up to 14 months with CU 32-085 (dose range 1-60 mg/day; mean duration 8.8 months). Both groups showed a significant improvement of "total disability score' at 6 months by 56% and 67%, respectively, and after 6 months by 69% and 69.4%, respectively, with a significant decrease of all types of disability. All patients with fluctuations and "on-off' effects rapidly improved on both compounds. Bromocriptine and CU 32-085 were discontinued in seven patients each (32%) because of adverse effect including mental changes (for with bromocriptine, two with CU 32-085), nausea and vomiting (one and two, respectively), hypotension (one each) and increased tremor plus vomiting (one with CU 32-085). Although adverse effects were similar to those observed with levodopa, CU 32-085 in general showed less severe dyskinesia and mental changes but more frequent nausea than bromocriptine and levodopa. While the results of treatment with bromocriptine and CU 32-085 were comparable, the antitremor effect of the latter drug developed more rapidly, even at low dosage. Both compounds were useful in the management of patients with advanced Parkinson's disease, CU 32-085 having a stronger effect on tremor, bradykinesia, fluctuations and "on-off' effects than bromocriptine.

Aged↗
PubMed1982

Adult GM2 gangliosidosis masquerading as slowly progressive muscular atrophy: motor neuron disease phenotype.

Ultrastructural and biochemical studies were performed on postmortem material of a 67-year-old woman presenting with proximal muscle weakness in the legs, slurred speech, and mental subnormality. The symptoms began at age 19 and showed extremely slow progression, mimicking progressive muscular dystrophy. A brother suffered from a similar chronic neuromuscular disease, and two sisters died at an early age from unknown "nervous" diseases. Autopsy disclosed abundant lipid accumulation in CNS neurons and severe cerebellar cortical atrophy of the granule cell type. Skeletal muscle showed a terminal stage of denervation atrophy with severe lipomatosis; intrafusal fibers of muscle spindles contained lipid deposits. Complex lamellar cytoplasmic inclusions often resembling membranous cytoplasmic bodies or stacked membranes were seen in cells of the brain. In addition, there were various lipopigment bodies, fingerprint profiles, rare polyglucosan bodies, rodlike structures, and filamentous sheaves, particularly in substantia nigra. Accumulation of gangliosides GM2 and GA2 in the cerebral cortex was demonstrated by thin-layer chromatography. Determination of hexosaminidase activity was not possible (formalin-fixed material). This observation, in addition to the cases reported by Navon et al. [1981] and Johnson [1982], is suggested to represent a new phenotype of adult-onset GM2 gangliosidosis referred to as motor neuron disease phenotype, which can be differentiated from other adult-onset lipidoses and motor neuron disorders. Our paper emphasizes the importance of ultrastructural demonstration of lamellar inclusions for the differential diagnosis of ceroid lipofuscinosis, and the value of biochemical studies in the diagnostic clarification of atypical neuromuscular disorders.

Adult↗
PubMed1982

[Antagonism of the ammonia effect on serotonin receptors by L-valine].

Ammonia and valine influence the activity of serotonin receptors in membranes of frontal cortex taken from brain removed at postmortem: Ammonia reduces maximal binding capacity of serotonin receptors, and this effect can be antagonized by valine. This modulatory effect of valine seems to be efficient at a lower level in tissues of patients with hepatic coma. Branched chain amino acids may have efficient therapeutic effects in hepatic coma due firstly to effects on the blood brain barrier by a well known mechanism of action and secondly to molecular interaction at the synaptic membrane.

Aged↗
PubMed1981

Detection of glycosaminoglycans in human gliomas by histochemical methods.

Sulphatized mucopolysaccharides or glycosaminoglycans (GAG) were examined by histochemical methods in biopsy specimens of 69 human cerebral gliomas. The intensity of cell-surface associated GAGs, particularly of the -SO3-groups, was highest in isomorphic oligodendrogliomas and astrocytomas except for pilocytic forms. It showed progressive decrease with increasing degree of anaplasia of these tumors. Anaplastic gliomas only rarely show a very weak reaction for -SO3-groups which was completely absent in glioblastomas, where GAGs are only found in blood vessel walls and mesenchymal stroma. In accordance with observations in experimental gliomas, the rapid loss of membrane-associated GAGs in anaplastic gliomas is considered to indicate a progressive cellular dedifferentiation of glial cells due to hitherto unknown metabolic changes.

Astrocytoma↗
PubMed1981

Chronic encephalitis in X-linked agammaglobulinaemia.

Clinicopathological and immunological findings are reported in a boy with X linked agammaglobulinaemia (XLA), who after multiple episodes of purulent infections in various organs developed encephalitis associated with excemata and non suppurative arthritis. Inspite of gammaglobulin administration he developed progressive spastic tetraparesis, convulsions, mental deterioration and blindness and died in a decerebrate state at the age of 8 years. Necropsy revealed a general diminuition of the lymphoid tissue. The atrophic brain showed multiple cystic destructions and chronic, but still active polioencephalitis with prominent inflammatory involvement of basal ganglia and brain stem. Symmetric Wernicke-like lesions were seen in inferior corpora quadrigemina. The aetiology of this particular type of chronic progressive encephalitis in XLA is unknown but some kind of viral infection is to be considered.

Agammaglobulinemia↗
PubMed1981

Giant-cell glioblastoma of the thoracic cord.

A case of giant-cell glioblastoma occurring in the upper thoracic cord of a male aged 76 without evidence of intracranial lesion is reported. Partial tumour resection with decompression and radiotherapy did not improve the patient's neurological deficit.

Aged↗
PubMed1981

Transitional states of central serotonin receptors in Parkinson's disease.

Crude membrane preparations from the frontal cortex of controls and parkinsonian patients were used to demonstrate affinity changes of the specific 3H-5-hydroxytryptamine (5-HT) binding sites. Two such sites were notable in controls, a finding consistent with earlier observations. In Parkinson's disease, both high- and low-affinity sites are significantly decreased. Additional experiments either with prolonged incubation times or pre-incubation with N-ethylmaleimide change the two affinities to a single high-affinity or low-affinity constant. The concept of transitional states of 5-HT receptors is discussed and seems to have important implications in the treatment of parkinsonism.

Aged↗
PubMed1981

Cerebral space-occupying cysts following radiation and chemotherapy of malignant gliomas.

Eight cases of cerebral cyst formation among 50 patients (= 16%) with malignant supratentorial gliomas treated by surgery, megavoltage radiation, and multiple-agent chemotherapy are reported. Five of them developed signs of intracranial hypertension or progressive neurological deficit, while in three patients cerebral cysts were detected by CT without clinical deterioration. At operation or autopsy, or both, the large fluid-filled, smooth-walled cysts were lined by glio-mesenchymal scar tissue with no or little tumour recurrence in five, while three patients showed large recurrent tumour masses associated with necrosis and cyst formation. Clinical signs or CT evidence, or both, of cerebral cysts developed 4 to 12 months (average 10 months) after the first craniotomy, and 3 to 9 months after termination of radiotherapy, usually after the second to fourth course of polychemotherapy. The cystic cavities which are attributed to increased necrosis and other effects of radiation and cytostatic treatment, may mimic tumour progression or recurrence, and cerebral abscess, but are usually recognized by CT. Surgical treatment produced transient clinical improvement in 5 patients, but usually did not prevent the fatal outcome of the disease, which in these paticrosis and other effects of radiation and cytostatic treatment, may mimic tumour progression or recurrence, and cerebral abscess, but are usually recognized by CT. Surgical treatment produced transient clinical improvement in 5 patients, but usually did not prevent the fatal outcome of the disease, which in these paticrosis and other effects of radiation and cytostatic treatment, may mimic tumour progression or recurrence, and cerebral abscess, but are usually recognized by CT. Surgical treatment produced transient clinical improvement in 5 patients, but usually did not prevent the fatal outcome of the disease, which in these patients occurred 3 weeks to 6 months after surgical treatment of cyst formation, their life span ranging from 9 to 22 months. The pathogenesis and clinical problems related to cerebral cysts arising following multimodality treatment of malignant brain tumours are discussed.

Adult↗
PubMed1981

[Glioblastoma multiforme developing after a gunshot injury of the brain (author's transl)].

We report the case of a 28-year-old male who suffered a frontal penetrating gunshot injury with subsequent bifrontal brain abscess and subdural empyema, and five years later developed a large bifrontal tumour at the precise site of the meningo-cerebral scar and posttraumatic defect. Histological examination showed a glioblastoma multiforme adjacent to the dural scar and, in addition, old suture material was found within the glioma tissue. In spite of combined radiation and polychemotherapy the patient died eleven months after partial tumour resection. The temporal and local association of missile injury with subsequent recurred abscess and scar formation and the malignant glioma is highly suggestive of a causal relationship between trauma and the development of a brain tumour.

Adult↗