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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

At least 181 records · Page 10Linked to original sources
PubMed1988

The pedunculopontine nucleus in Parkinson's disease, progressive supranuclear palsy and Alzheimer's disease.

Significant loss of neurons in the pedunculopontine nucleus pars compacta (PPNc), a putative cholinergic nucleus involved in modulating somatic motor activities, has been demonstrated in progressive supranuclear palsy (PSP) and Parkinson's disease but not in Alzheimer's disease. A morphometric study of this nucleus was performed in two cases of PSP and in a cohort of cases of Parkinson's disease, Alzheimer's disease, senile dementia of Alzheimer type (SDAT), and age-matched controls. In PSP a significant 60% neuronal loss in PPNc was associated with neurofibrillary tangles in 40 to 64% of the remaining neurons. In Parkinson's disease there was a significant decrease in cell numbers and density by 53 and 51%, respectively, with Lewy bodies involving 6 to 39% of all neurons. In Alzheimer's disease and SDAT, large neurons were reduced by 29 and 33.8%, respectively, with tangles in 9 to 38% of the remaining cells. The selective affection of this putative cholinergic nucleus in PSP and Parkinson's disease appears to be related to motor dysfunctions in these disorders.

Aged↗
PubMed1987

[Lisuride in the combination treatment of Parkinson disease].

Lisuride, a semisynthetic dopaminergic ergot derivative, was administered in 34 patients with Parkinson's disease (mean age 67.5 years, average duration of illness 7.1 years, mostly stage IV) in an average oral dosage of 1.2 to 1.5 mg (range 0.4 to 3.0 mg) in addition to basic treatment with levodopa and decarboxylase inhibitor. Duration of combined treatment was 3 to 44 months. Daily dose of levodopa could be reduced by 13 to 34%. In the short-term group (3 months) the total disability score improved by 50%, and in the long-term group (up to 44 months) by 46%, with improvement of rigidity, tremor, speech and repeated movements, less of bradykinesia. Similar to treatment with dopamine agonists, after 18 to 24 months, despite increased dosage of levodopa, a slight increase of some clinical features of parkinsonism, particularly bradykinesia and gait disorders, were observed. Daily fluctuation and on-off symptoms often improved in intensity, but were eliminated only in a small number of patients. Discontinuation of lisuride due to adverse effects was necessary in 17.6%. Often, however, preexisting adverse effects of levodopa therapy, particularly psychiatric complications, responded favorably. Lisuride is a new effective agent in the combination treatment of advanced stages of parkinsonism.

Adult↗
PubMed1987

Human brain dopamine receptors in children and aging adults.

Since spontaneous oral dyskinesias are more prevalent in the elderly, and since these movements may be controlled by the balance of brain dopamine D1 and D2 dopamine receptors, we measured the densities of these receptors in 247 postmortem brain striata. In childhood, the densities of D1 and D2 dopamine receptors in the brain striatum rise and fall together. After age 20 years, D1 receptors disappear at 3.2% per decade while D2 receptors disappear at about 2.2% per decade. Overall, therefore, the D1/D2 ratio falls with age. Since perioral motion in rats is dominated by a high D1/D2 ratio, the observed decline in the human D1/D2 ratio with age suggests that the perioral control mechanisms for humans and rats may be different.

Aged↗
PubMed1987

Adjuvant treatment of Parksinson's disease with budipine: a double-blind trial versus placebo.

Budipine, a new 4,4-diphenylpiperidine derivative, and placebo were administered three times daily to 31 patients with Parkinson's disease over a period of 12 weeks. All patients in the two treatment groups received levodopa (plus benserazide) at an optimum and constant dose for at least 2 months before the start of the study and throughout the trial. The additional administration of budipine (daily dose 60 mg) was excellently tolerated by 14 patients, while 2 patients left the study because of mental confusion at an early stage of the trial. The budipine group showed a 22% improvement on the Columbia Rating Scale (median score). Compared with the placebo group (4% improvement), there was a highly significant difference (P less than 0.01, one-tailed test). Of the three main symptoms of Parkinson's disease, the best effect was seen on tremor, and less pronounced effects on bradykinesia and rigidity. Owing to its long half-life (31 h) with little plasma level fluctuations, budipine appears to be an effective agent in the treatment of Parkinson's disease.

Aged↗
PubMed1987

Antineuronal antibodies in small cell lung carcinoma--a significance for paraneoplastic syndromes?

In recent years a cause-effect relationship between the existence of circulating antineuronal antibodies (CANA) and neurological paraneoplastic syndromes has been described by several authors suggesting specifity of such antibodies for paraneoplastic syndromes. The present study is a systematic approach to elucidate the significance of CANA in tumor patients. Forty patients with biopsy-proven small cell cancer of the lung (SCLC) were compared to 70 non-SCLC patients and 20 controls in respect to clinical and neurophysiological findings. CANA were found in 17 patients with SCLC. However, only one of these patients with SCLC and positive CANA displayed a sensory neuropathy of the Denny-Brown type, which appeared to be unrelated to CANA titers and oncological course. Contrary to recent reports, we were not able to confirm an association between the existence of CANA and an increased incidence of paraneoplastic neurological syndromes. These data suggest that the antineuronal antibodies appear to be specific for SCLC, but are not necessarily related to paraneoplastic neurological syndromes.

Aged↗
PubMed1987

Histochemistry of MAO-A and MAO-B in the locus coeruleus of the Mongolian gerbil.

A coupled peroxidation technique for localization of monoamine oxidase (MAO-A and MAO-B), applied to post-mortem fixed tissue of the locus coeruleus of the Mongolian gerbil is demonstrated. Tyramine hydrochloride, beta-phenylethylamine and 5-hydroxytryptamine creatinine sulphate were used as substrates, 1-deprenyl and clorgyline served as specific inhibitors. All three substrates stained the neurons of locus coeruleus in the absence of inhibitor. In the presence of 1-deprenyl, tyramine hydrochloride and 5-hydroxytryptamine creatinine sulphate were metabolized, whereas in the presence of clorgyline no reaction with either substrate could be observed. Immunocytochemical staining of tyrosine hydroxylase (TH) was employed as comparison.

Animals↗
PubMed1987

Human brain D1 and D2 dopamine receptors in schizophrenia, Alzheimer's, Parkinson's, and Huntington's diseases.

Because dopamine D2 receptors are known to be elevated in schizophrenic brain striata, this study examined whether a similar dopamine receptor elevation occurred in other diseases including neuroleptic-treated Alzheimer's and Huntington's diseases. The average D1 density in postmortem striata from Alzheimer's patients was 17.6 +/- 0.1 pmol/g, similar to an age-matched control density of 16.6 +/- 0.4 pmol/g. The average D1 density in schizophrenia patients was 19.0 +/- 0.6 pmol/g, similar to the age-matched control density of 17.9 +/- 0.6 pmol/g. In Parkinson's disease patients, however, the D1 receptor density was elevated, with values of 22.8 +/- 1.2 pmol/g (in patients not receiving L-DOPA) and 19.6 +/- 1.5 pmol/g (in patients receiving L-DOPA) compared to the age-matched control density of 16.0 +/- 0.4 pmol/g. The D2 receptors in Alzheimer's striata averaged 13.4 +/- 0.6 pmol/g (in patients who had not received neuroleptics), almost identical to the control density of 12.7 +/- 0.3 pmol/g. The average D2 density in neuroleptic-treated Alzheimer's striata was 16.7 +/- 0.7 pmol/g, an elevation of 31%, the individual values of which had a normal distribution. In Parkinson's disease patients, the D2 densities were elevated in tissues from patients not receiving L-DOPA (19.9 +/- 1.5 pmol/g in putamen and 14.8 +/- 1.2 pmol/g in striatum) compared to the age-matched control values of 13.0 +/- 0.4 pmol/g and 12.6 +/- 0.3 pmol/g, respectively. In Huntington's disease patients, the D2 density averaged 7.5 +/- 0.4 pmol/g in patients who had not received neuroleptics, but was 10.3 +/- 0.6 pmol/g in those who had. Although all of the D1 and D2 densities in each of the above diseases and subgroups revealed a normal distribution pattern, the D2 densities in schizophrenia displayed a bimodal distribution pattern, with 48 striata having a mode at 14 pmol/g, and the other 44 striata having a mode at 26 pmol/g. Thus, compared to the neuroleptic-induced and unimodal elevations in D2 of 31% in Alzheimer's disease and 37% in Huntington's disease, the schizophrenic striata with a mode of 26 pmol/g (105% above control) appear to contain more D2 receptors than can be accounted for by the neuroleptic administration alone.

Adult↗
PubMed1987

Neuropathological aspects of infantile spasms.

A review of the neuropathological findings in 50 personal autopsy cases and in the available literature (214 confirmed cases) of infantile spasms demonstrates that this type of early infantile epilepsy occurs in a wide range of cerebral lesions caused by various noxae during different stages of brain development. With regard to morphology and the presumed time of occurrence of the CNS lesions, four groups can be distinguished: (1) embryofetal lesions, including a) cerebral malformations or developmental disorders-agyria-pachygyria (lissencephaly), micrencephaly, micropolygyrias, (hemi)megalencephaly, agenesis of corpus callosum, tuberous sclerosis, heterotopias, cortical microdysplasias, b) metabolic disorders (leukodystrophies, neurolipidoses, spongy dystrophies, Leigh and Alpers diseases, aminoacidopathies); (2) perinatal and postnatal encephalopathies, e.g. polycystic brain, diffuse and lobar sclerosis, ulegyrias, white matter and basal ganglia scars, status marmoratus, hippocampal sclerosis, and cerebellar atrophy; (3) combined embryofetal (developmental) and perinatal or postnatal brain lesions, particularly association of microdysplasias with secondary anoxic or vascular changes; (4) acute vascular and inflammatory brain injuries; (5) cases without definite brain pathology. Evaluation of the available data indicates that embryo-fetal lesions alone or accompanied and/or superimposed by perinatal or postnatal lesions account for about 61% of the cases confirmed by autopsy, in which infantile spasms can be regarded as fetal epilepsies, while a smaller group is featured by perinatal or postnatal lesions occurring in early age, i.e. affecting the immature brain. Similar lesions are observed in cases showing transition of West syndrome to Lennox syndrome. Negative pathology findings in a small number of cases do not necessarily implicate negative pathobiology.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗
PubMed1987

Neuropathological substrates of Alzheimer's disease and Parkinson's disease.

The pathology of Alzheimer's disease (AD) and Parkinson's disease (PD) is characterized by degeneration of certain vulnerable neuronal populations that display several types of cytoskeletal abnormalities (Alzheimer-type lesions and Lewy bodies, respectively). These may serve as diagnostic markers, although they can be found in both types of disorders and, less frequently, in brains of normal aged individuals. AD pathology shows a preponderance for hippocampal, neocortical, and forebrain cholinergic systems; the hallmark of PD is damage to the nigrostriatal dopaminergic system. Both disorders show frequent involvement of subcortical projection systems that can be similar in quality and distribution, with similar multiple neuromediator dysfunction. The results of morphometric studies of some subcortical nuclei in AD and PD are reported and related with neurochemical data. In addition to classical forms of AD and PD, both types of lesions can coexist suggesting an increased risk of PD in patients with AD and vice versa. The basis for an association between the two disorders is unknown. Many AD cases with signs of PD have additional PD pathology, while cortical Alzheimer lesions may be seen in demented PD patients. However, dementia in PD does not imply coexistent cortical AD pathology; prominent subcortical lesions alone, or in combination with cortical AD pathology, may occur. AD and PD may show some differences in the primary locus of degenerative changes in specific cortical and subcortical neuronal systems, but the causative factors, mutual relations, and relative contributions to the clinical syndromes remain to be elucidated.

Aged↗
PubMed1987

Cellular action of MAO inhibitors.

Sites of action of monoamine oxidase (MAO) inhibitors in the human brain were analyzed by studying the distribution of the respective MAO subtype, MAO-A or -B. Blood brain barrier (BBB), glial cells and neurons of several brain areas were examined with biochemical and immunocytochemical methods. Whereas MAO-A was localized in microvessels and neurons of locus ceruleus, MAO-B was present predominantly in neuronal cells of dorsal raphe nucleus. The neurons of substantia nigra revealed no immunohistochemically detectable MAO. Astrocytes expressed both subtypes and were observed in all cortical areas examined and all over the brain stem including medulla reticular formation, pontine reticular formation, reticular formation of mesencephalon, midbrain reticular formation, red nucleus, substantia nigra, caudate nucleus and putamen (sections according to DeArmond et al., 1976). MAO activity in several brain areas seems mostly to be due to astrocytes rather than to neuronal cells. As MAO inhibitors have beneficial effects in the treatment of malfunctions of aminergic transmitter systems (Johnstone and Marsh, 1973; Robinson et al., 1973; Birkmayer et al., 1977; Knoll, 1981; Zarifian, 1984; Riederer and Youdim, 1986) and a large amount of MAO activity seems to be localized in brain astrocytes, the function of glial cells especially with regard to their neuromodulating capacity should be focused in more detailed research in future.

Aged↗
PubMed1986

Neuropathology of Rett syndrome.

Autopsy studies in 8 girls with the Rett syndrome dying between 4 and 15 years showed: Diffuse cerebral atrophy/micrencephaly, with a decrease in brain weight by 13.8 to 33.8% of age-matched controls, apparently related to the duration of the disorder; Mild, but inconsistent diffuse cortical atrophy without developmental disorders apart from occasional microdysgenesis (three cases), but increased amounts of neuronal lipofuscin, and occasional mild astrocytic gliosis; Mild, but inconsistent spongy changes in cerebral and cerebellar white matter, optic nerve (two cases), and myelinated fascicles of the brainstem tegmentum, without signs of dys- or demyelination, and apparently different from the spongy myelinopathy common to aminoacidopathies; Most conspicuous was an underpigmentation of the substantia nigra which contained many fewer well-pigmented neurons for age (53-73%), and fewer pigmented granules per neuron, while the total number of nigral neurons and the triphasic substructure of neuromelanin were normal for age. No pathologic changes were seen in locus coeruleus, nucleus basalis of Meynert, and nucleus dorsalis raphe; Electron microscopy of autopsy material from an 11-year-old girl showed increased amounts of neuronal lipofuscin without signs of a storage disorder. Reactive and degenerating axons in the caudate nucleus were possibly related to the nigral changes, suggesting some dysfunction of the dopaminergic nigro-striatal system, while the synaptic organization of the neostriatum appeared unaffected. Peripheral nerve from a patient dying in advanced stage showed increased numbers of unmyelinated (regenerated?) axons, with almost no demyelination and few remyelinated axons, suggesting axonal degeneration rather than hypomyelination, but exogenous factors (malnutrition) cannot be excluded. The pathogenetic mechanisms of the morphologic brain lesions and their relations to clinical and neurochemical findings in Rett syndrome are unknown and deserve further intensive investigations.

Adolescent↗
PubMed1986

Dopamine D2 receptor density remains constant in treated Parkinson's disease.

D2 dopamine receptor densities were measured in postmortem samples of the caudate nucleus and putamen from 36 parkinsonian patients. The relationship between the age of the patient, duration of the disease, and duration of L-dopa therapy versus density of brain D2 dopamine receptors was examined using [3H]spiperone. Receptor density in parkinsonian tissues was constant over the age range of 56 to 90 years, as was the case for control tissues. Density did not change with duration of disease up to 24 years. Treatment with L-dopa did not cause progressive reduction in receptor density. The diminished clinical response in the final stages of Parkinson's disease is not due to receptor dropout, and must depend on other factors.

Age Factors↗
PubMed1986

Multifocal spinal angiosarcoma after chordotomy.

A male aged 60 is reported who, 5 years after chordotomy at the Th 2/3 level, developed acute paraplegia of the legs and a sensory transverse lesion due to an extradural tumour of the Th 1-4 level. Neuropathology revealed transverse necrosis of the thoracic spinal cord (Th 1-4) due to an intradural tumour at the upper thoracic and lumbar spinal levels, the thoracic malignancy arising at the site of the previous chordotomy, with a suture being observed within the tumour mass. Histology, positive immunostaining of tumour cells with Factor VIII antigen, a specific marker of endothelial cells and the ultrastructural demonstration of Weibel-Palade bodies in endothelial cells of the tumour vessels suggested a malignant mesenchymal tumour of angiosarcoma type presumably arising from the spinal meninges, and broadly invading the spinal cord. The usual relation of this extremely rare intraspinal vasoformative malignancy to previous chordotomy is discussed.

Antigens↗
PubMed1986

Vascular malformations of the central nervous system: a morphological overview.

Vascular malformations of the central nervous system (C.N.S.) are classified by size, location, and morphologic type, distinguishing capillary telangiectasias, cavernous malformations, venous angiomas, arteriovenous malformations (AVMs) including varix of the great vein of Galen, and other vascular malformations (e.g. Sturge-Weber syndrome). The morphology and predominant location pattern of the different types of vascular malformations in the brain and spinal cord, and their embryology are reviewed. In the brain and its coverings, all types mainly AVMs and venous angiomas do occur, representing 5-9% of all intracranial space-occupying lesions and 20-40% of the sources of surgically treated intracranial hemorrhages. 50-80% of the angiomas are located in the cerebral hemispheres, 10-18% in central brain areas (basal ganglia, internal capsule, choroid plexus), and 10-30% in the posterior fossa. The major types of cerebral vascular malformations are described with reference to their anatomical features, location, chief arterial and venous supply, and prominent complications. Spinal vascular malformations, accounting for 3 to 12% of spinal space-occupying lesions, include vertebral, extradural, dural, subpial and intramedullary angiomas which occur as isolated or complex vascular anomalies and may involve various covering layers at the same level. The preferential occurrence of angiomas on the dorsal surface of the cord and in the caudal regions is related to the embryologic development of spinal vasculature. Frequent association of spinal angiomas (20-25%) with other vascular anomalies and dysplasias emphasizes their hamartomatous nature and developmental origin. Spinal angiomas include capillary telangiectasias with extra- or intradural and, rarely, intramedullary location, cavernomas, mainly arising in vertebral bodies, venous angiomas, mainly located in vertebral bodies and in the extradural space, and AVMs constituting the commonest type, that may affect both the pial and radicular vessels and can penetrate into the cord. They present as simple AV fistulas, cirsoid angiomas with localized vascular plexuses and large complex convolutions ("juvenile" type). The complications of spinal angiomas include subarachnoid hemorrhage, rare epidural hematoma, hematomyelia, compression lesions of the cord and roots, and ischemic changes causing chronic progressive radiculomyelopathy, previously referred to as Foix-Alajouanine syndrome. Chronic damage to the cord and spinal roots results from pressure effects, thrombosis of the abnormal vessels, disorders of venous drainage, and "steal" phenomena related to the vascular anomalies.

Arteriovenous Malformations↗
PubMed1985

Multifocal neuropathy with vasculitis in hypereosinophilic syndrome. An entity or drug-induced effect?

A 56-year-old male with a 2-year history of bronchial asthma, together with pulmonary infiltration and marked eosinophilia, developed a subacute multifocal sensorimotor neuropathy. Electrodiagnostic studies demonstrated both multifocal and generalized nerve involvement. Sural nerve and muscle biopsies revealed axonal degeneration with almost complete loss of myelinated fibres, lymphomononuclear vasculitis of interstitial vasa nervorum without eosinophils, and neurogenic atrophy of muscle without angiitis. Although eosinophilia decreased drastically with corticosteroid treatment, neuropathy rapidly progressed to total disability. The patient died from pulmonary embolism 4 months after the onset of neurological signs. Autopsy disclosed vasculitis of epineurial vessels of peripheral nerves and severe axonal neuropathy, particularly of the lower limbs, without vasculitis or other inflammatory lesions in any other organ system, including the lungs. Retrospective analysis revealed that the onset of pulmonary infiltration and eosinophilia coincided with the administration of cromolyn sodium (Intal), which is known to produce PIE syndrome (pulmonary infiltration and eosinophilia), vasculitis and allergic granulomatosis, while multifocal neuropathy with vasculitis appears not to have been reported in connection with this substance.

Biopsy↗