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K Jellinger

Publications and source records attributed to K Jellinger.

At least 145 records · Page 8Linked to original sources
PubMed1991

Pulmonary meningioma. Immunohistochemical and ultrastructural features.

Two cases of solitary primary pulmonary tumors showing the immunohistochemical and ultrastructural features of meningothelial meningiomas are presented. The benign clinical and radiologic course, the negative computed tomography scan of the brain (case 1), and negative neuropathologic investigation (case 2) support the diagnosis of a primary pulmonary meningioma rather than a metastazing malignant intracranial meningioma. Negative neuroendocrine markers (neuron-specific enolase, chromogranin, bombesin) and the lack of neurosecretory granules by electron microscopy confirm the diagnosis of this rare pulmonary tumor.

Adult↗
PubMed1990

A high ratio of chromogranin A to synaptin/synaptophysin is a common feature of brains in Alzheimer and Pick disease.

Chromogranin A and synaptin/synaptophysin were characterized by immunological methods in human autopsy brain tissue from patients with Alzheimer's and Pick's disease. In immunoblots there was no qualitative difference between the antigens in control and diseased brain, but significant quantitative differences were found. In all Alzheimer cases there was a significantly lower level of synaptin/synaptophysin, whereas chromogranin A was higher in 4 out of 5 cases and in all cases relative to synaptin/synaptophysin. An analogous finding was obtained for Pick's disease. Immunohistologically a consistent staining of neuritic plaques for chromogranin A, but not for secretogranin II was found in Alzheimer cases. In Pick's disease the characteristic Pick bodies showed an analogous specific immunostaining.

Aged↗
PubMed1990

Reflection of changes in membrane constituents in various regions of Alzheimer brains to differential scanning thermograms.

To test whether changes in basic biochemical membrane constituents were reflected in membrane fluidity measurements, protein, total lipids, triglycerides, lecithin, cholesterol and alkaline phosphatase were determined in frontal cortex, hippocampus, putamen and nucleus basalis Meynert (NbM) of DAT brains and controls and compared to differential scanning thermograms. Biochemical changes were most pronounced in the hippocampus, while thermostability was altered in the NbM and the frontal cortex. The results indicate that changes in protein content, but not in lipid composition were reflected in alterations of thermostability of the brain regions examined.

Aged↗
PubMed1990

Urinary dopamine sulfate: regulations and significance in neurological disorders.

Dopamine-3-O-sulfate (DA-3-O-S) and dopamine-4-O-sulfate (DA-4-O-S) are important end products of L-dopa metabolism. Therefore they may give indications of disturbances in the peripheral metabolism of catecholamines, when measured in urine samples of patients with Parkinson's disease (PD). In addition, information about the reliability of DA sulfatation after L-dopa therapy may be of significance for its role in the elimination of DA from the peripheral nervous system. Although DA-3-O-S appears to be the predominant sulfo-conjugate in urine, there are no changes in PD nor in depression syndrome compared to controls with or without other neurological disorders. By contrast, DA-4-O-S is significantly decreased in de novo PD subjects. However, a similar reduction is notable in patients with other neurological disorders. In depressed persons the loss of this compound was less pronounced as compared to de novo PD. Treatment with combined L-dopa therapy caused increased excretion of DA-3-O-S, while changes in DA-4-O-S were only marginal. It is concluded that urinary DA-3-O-S cannot be used as marker for PD, while DA-4-O-S is significantly reduced in a variety of neurological disorders and in particular in de novo PD. Further studies are necessary to elucidate its role as possible peripheral marker to distinguish preclinical PD and depression syndrome.

Adult↗
PubMed1990

Memory deficits in advanced Parkinson's disease.

Apart from global dementia various isolated cognitive deficits have been described in Parkinson's disease (PD). We investigated 31 non-demented Parkinsonian patients in their late stages of disease and 50 control subjects with regard to verbal memory. Eleven patients suffered from an isolated verbal memory deficit as defined by two list learning tasks using the Buschke selective reminding procedure. The isolated memory impairment did not depend on depression but was associated with longer duration of PD. Twelve demented PD patients were comparable to PD patients with isolated memory impairment with regard to age at onset and duration of PD. We speculate that the isolated memory impairment in PD is associated with isolated neuronal loss in the nucleus basalis of Meynert, without cortical or limbic pathology of the Alzheimer's type.

Aged↗
PubMed1990

Brain iron and ferritin in Parkinson's and Alzheimer's diseases.

Semiquantitative histological evaluation of brain iron and ferritin in Parkinson's (PD) and Alzheimer's disease (DAT) have been performed in paraffin sections of brain regions which included frontal cortex, hippocampus, basal ganglia and brain stem. The results indicate a significant selective increase of Fe3+ and ferritin in substantia nigra zona compacta but not in zona reticulata of Parkinsonian brains, confirming the biochemical estimation of iron. No such changes were observed in the same regions of DAT brains. The increase of iron is evident in astrocytes, macrophages, reactive microglia and non-pigmented neurons, and in damaged areas devoid of pigmented neurons. In substantia nigra of PD and PD/DAT, strong ferritin reactivity was also associated with proliferated microglia. A faint iron staining was seen occasionally in peripheral halo of Lewy bodies. By contrast, in DAT and PD/DAT, strong ferritin immunoreactivity was observed in and around senile plaques and neurofibrillary tangles. The interrelationship between selective increase of iron and ferritin in PD requires further investigation, because both changes could participate in the induction of oxidative stress and neuronal death, due to their ability to promote formation of oxygen radicals.

Aged↗
PubMed1990

Clinicopathological analysis of dementia disorders in the elderly.

The relative incidence of the major types of dementia disorders and the agreement rates between clinical and pathological diagnosis were analysed in consecutive autopsy series of 675 demented subjects from 3 hospitals (mean age 79.5 years, SD 9.6). Clinical assessment followed the DSM-III and ICD-9-NA criteria and NINCDS/ADRDA criteria for probable Alzheimer disease (AD) (McKhann et al. 1984), histological criteria for the diagnosis of AD those of the NIH/AARP Work Group (Khachaturian 1985) using a 4-degree rating scale for plaques and tangles in neocortex and hippocampus (Morris et al. 1988), and the criteria by Tierney et al. (1988) for 'pure' AD. Vascular dementia (MID) and other disorders were diagnosed according to current pathologic criteria. Clinical diagnosis of AD/SDAT was made in 59.2%, of MID in 21.7%, of mixed AD + MID in 3.1%, and of Parkinson's disease (PD) and other disorders in 16%. At autopsy, 76.7% fulfilled histological criteria for AD/SDAT, but only 60% were 'pure' forms, while 8.2% had additional features of PD and 7.9% coexisting vascular lesions indicating mixed SDAT + MID. 15.7% were MID with no or very little AD pathology, 7.4% other CNS disorders. 0.3% of the brains showed no abnormality beyond age-related changes. AD/SDAT had its highest incidence in a psychiatric population, MID and PD + SDAT in general and geriatric hospital cohorts. The overall coincidence rates for clinical and pathological diagnosis of AD/SDAT were 85.2%, for MIX and MID 60.5-61.9%, but only 51% for PD-PD/AD. These data and the results of other recent studies emphasize the need for more appropriate clinical and pathological criteria in the diagnosis of the dementias.

Aged↗
PubMed1990

Peripheral nerve involvement in the Rett syndrome.

Clinical and neurophysiological studies were performed on 4 girls aged 9 to 19 years with the Rett syndrome (RS) (stages II and III). In addition to constant foot deformity (pes planus) and brisk tendon reflexes in the lower limbs, they showed muscular atrophy of the calves and mild atrophy of the extensor digitorum brevis muscle. Motor NCVs were within normal values for age, but distal latencies in two girls and compound action potential in one were abnormal, suggesting mild distal, predominantly motor neuropathy. The upper limbs showed nothing abnormal except for mild hand deformities. Neuropathology in 2 autopsy cases (aged 11 and 17 years, stage IV) and sural nerve biopsies in three girls aged 2, 3 and 17 years, all clinical stage II-III without inanition, revealed mild distal axonopathy without demyelination. Selected axons in sural nerve of a girl aged 17 and in the dental pulp of a girl aged 9, both at clinical stage II, showed increased numbers of neurofilaments. Skeletal muscle in two girls aged 11 and 17 years (both stage IV) exhibited mild alterations in the sarcoplasmic reticulum with occasional disordered myofibrils and tubular structures in the Z-filaments, but no neurogenic atrophy or abnormal mitochondria. The reported data do not support spinal denervation (motor neuron degeneration) in RS, but suggest late onset denervation due to mild distal axonopathy of hitherto unknown origin.

Adolescent↗
PubMed1990

Pigmentary type of orthochromatic leukodystrophy with early onset and protracted course.

The pigmentary type of orthochromatic leukodystrophy (OLD) is a rare disorder in adults; only one questionable childhood case has been observed. We report the sporadic case of a male aged 26 years with early onset and protracted course. He presented retarded motor development from birth with ataxic gait and, at age 13 years, developed progressive mental and neurologic deterioration with tetraparesis, ataxia and seizures and died in a disabled, mute state. Repeated CT scans showed progressive diffuse cerebral atrophy and low density of the hemispheric white matter. Autopsy revealed OLD with pigmented macrophages and glial cells, ultrastructurally showing storage of lipofuscin and ceroid with multilamellar bodies or finger-print profiles. Abnormal cytoplasmic inclusions in reduced oligodendroglial cells suggest demyelination due to a primary defect of oligodendroglia in this rare disorder.

Adult↗
PubMed1990

Normal mitochondrial genome in brain from patients with Parkinson's disease and complex I defect.

The mitochondrial genome codes for 13 proteins which are located in the respiratory chain. In postmortem brain of patients with Parkinson's disease, decreased activity of complex I of the respiratory chain could be demonstrated. Because seven subunits of complex I are coded by the mitochondrial genome, we analyzed the mitochondrial DNA of human postmortem substantia nigra, putamen, and frontal cortex by the Southern blot technique. No deletions of the mitochondrial genome could be demonstrated, thus indicating that either subunits which are encoded by the nuclear genome are decreased or enzyme activity is diminished by metabolites, toxins, or increase of Fe3+.

Aged↗
PubMed1990

Dopamine uptake sites and dopamine receptors in Parkinson's disease and schizophrenia.

Dopamine uptake sites, labelled by 3 nM [3H]GBR 12935, were reduced by 28-34% in post-mortem Parkinson's diseased striata (n = 22) compared to control striata (n = 28). There was no significant difference between post-mortem striata from schizophrenics (n = 16) and controls. Both dopamine D1 and D2 receptors are consistently elevated in Parkinson's diseased striata from patients who have not been medicated with L-dopa pre-mortem. Both these receptors are down-regulated by L-dopa pre-mortem. In schizophrenia, however, while the density of D1 receptors is similar to control, the D2 receptors are consistently elevated.

Adult↗
PubMed1990

New developments in the pathology of Parkinson's disease.

Recent data on neuronal cytoskeletal pathology of Lewy bodies, a major anatomical hallmark of PD, suggest pathological phosphorylation of neurofilament proteins with ubiquitination, but their relation to neuromelanin and cell degeneration is unknown. Quantitative morphological studies in PD indicate that in addition to nigrostriatal dopaminergic pathways, other dopaminergic, noradrenergic, serotonergic, and cholinergic systems are involved, causing multiple neuromediator dysfunctions. Dementia in PD is not only related to cortical AD pathology, but also to a variety of central nervous system lesions.

Alzheimer Disease↗
PubMed1989

Accumulation of abnormally phosphorylated tau precedes the formation of neurofibrillary tangles in Alzheimer's disease.

The intraneuronal accumulation of paired helical filaments in the form of neurofibrillary tangles is one hallmark of the brain pathology in Alzheimer's disease. At certain predilection sites, a small number of similar lesions are also present in the brains of the majority of aged non-demented individuals. As suggested by several studies before, these abnormal cytoskeletal structures contain determinants of microtubule-associated protein tau and ubiquitin. The present study uses a morphological classification of neurofibrillary tangles into different stages of maturation, as suggested by Alzheimer in 1911, and shows by quantitative immunocytochemistry that early stages of neurofibrillary degeneration contain abnormally phosphorylated tau. Immunoreactivity for the altered tau is seen not only in tangles but also in the cytoplasm of some nerve cells lacking neurofibrillary tangles. Similar numbers of such immunoreactive neurons without tangles are present in age-matched non-demented individuals as in Alzheimer cases, but are absent in young controls. In contrast, incorporation of an epitope, recognized by a monoclonal antibody (3-39) raised to paired helical filaments, which is directed against a determinant residing in the 50-65 amino acid residue region of ubiquitin occurs late in the process of tangle maturation and is most pronounced in extracellular 'ghost tangles'. It is suggested that the accumulation of abnormally phosphorylated tau is one of the earliest cytoskeletal changes in the process of tangle formation. Exposure of certain ubiquitin epitopes in the pathological fibers may reflect an unsuccessful attempt of proteolytic degradation.

Adult↗