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Biomedical subjects

K Jellinger

Publications and source records attributed to K Jellinger.

394 records · Page 22Linked to original sources
PubMedDate not supplied

Levodopa in the treatment of (pre) senile dementia.

A progressive decrease of dopamine and related enzyme activities and a decline in the dopaminergic receptor functions in the striatum and limbic system controlling behavior have been observed in senescent brain. These changes provide a neurochemical basis for some behavioral and mental disturbances of the aged and suggest the possibility of a substitution therapy of neurotransmitter deficiency of senile and presenile dementia. Previous reports on the effects of levodopa treatment of dementia in parkinsonism and aging have given conflicting results. A controlled double-blind study of 6 months' administration of levodopa plus decarboxylase inhibitor given at oral doses of 250 mg daily versus placebo in twelve patients aged 54--60 years showing initial signs of organic dementia was performed. Clinical rating of twelve parameters of organic brain syndrome and psychometric studies using three subtests of the HAWIE (digit span, coding B test and block design) indicate improvement in the overall scores of organic brain syndrome and in particular in the visuospatial functions, visual memory, and visual-motor coordination. In general, improvement increased with the duration of levodopa treatment. These preliminary data suggest some beneficial effect of levodopa treatment in early stages of presenile dementia and thus open new therapeutic aspects in neurogerontology.

Benserazide↗
PubMedDate not supplied

Quantitative changes in some subcortical nuclei in aging, Alzheimer's disease and Parkinson's disease.

In normal aging, AD/SDAT and Parkinson's disease, the changes in subcortical neuronal systems are similar in quality and distribution, but highly variable in their intensity. Some correlations between the intensity and pattern of lesions in some subcortical nuclei with changes in cortical target areas in AD/SDAT suggest that age-related subcortical changes may represent a secondary phenomenon (antograde or retrograde degeneration) due to primary cortical damage. However, severe depletion of subcortical nuclei in both AD/SDAT and PD may also occur without considerable cortical pathology which is more compatible with a concomitant degeneration of both subcortical and intracortical neuronal systems.

Acetylcholine↗
PubMedDate not supplied

On the relationship between measles virus and Alzheimer neurofibrillary tangles in subacute sclerosing panencephalitis.

We have studied the relationship between measles virus and the accumulation of abnormally phosphorylated tau (PHF-tau) in nine cases of subacute sclerosing panencephalitis (SSPE). By assessing the presence of viral intranuclear inclusions and neurofibrillary tangles (NFT) in each case, we found no correlation between presence and amount of measles virus and the numbers of neurons containing PHF-tau. Immunohistochemical double labeling in a case with long duration of disease and severe histopathologic change revealed no strict colocalization of measles virus antigen and PHF-tau throughout different brain regions. In areas containing both antigens, most neurons carrying measles virus did not have a tangle and vice versa, eventhough some colocalization beyond that expected by chance was observed in specific cortical areas. These results indicate that, although secondary to viral infection, NFT formation in SSPE is not restricted to cells carrying viral antigen. Conversely, measles virus infected cells do not necessarily accumulate PHF-tau. This lack of colocalization at the cellular level, throughout different brain areas and among different cases suggests that the formation of NFT in SSPE is not directly induced by the infectious agent. The formation of NFT in this disease appears to be elicited through a specific type of tissue damage and, thus, to be an epiphenomenon. This pathogenetic detail may be of interest for our understanding of the role of neurofibrillary degeneration in the pathogenesis of other more frequent neurodegenerative diseases with cytoskeletal pathology.

Adolescent↗
PubMedDate not supplied

New phenotype of adult alpha-L-iduronidase deficiency (mucopolysaccharidosis I) masquerading as Friedreich's ataxia with cardiopathy.

Clinical, ultrastructural and biochemical studies are reported in a 42-year-old woman presenting with congenital pes cavus who, at the age of 23 years, developed slowly progressive distal amyotrophies, hypesthesia, bilateral hearing loss and severe cardiopathy leading to death. There were skeletal anomalies, mild reduction of motor NCVs, but no corneal opacity, retinitis pigmentosa, organomegaly or vacuolated lymphocytes. Autopsy disclosed severe thickening of fibrous tissues (endocardium, cerebrospinal dura) with accumulation of vacuolated cells containing glycosaminoglycans in numerous membrane-bound cytoplasmic vacuoles, and/or compound multilamellar or zebra-body-like structures. The CNS, in addition to enlarged perivascular lacunes in cerebral white matter with lipid-containing macrophages, showed neuronal lipid storage in thalamus, hypothalamus, hippocampus, brain stem nuclei, spinal motor neurons and Purkinje cell dendrites. Ultrastructurally, lamellated inclusions containing gangliosides were seen in mesenchymal cells, oligodendrocytes, pericytes and Schwann cells. Neurons contained abundant ceroid but no lamellated inclusions. Neurochemistry revealed decrease of alpha-L-iduronidase activity in brain tissue to 4% of normal, normal activities of other lysosomal enzymes, and normal lipid and ganglioside patterns. While the morphology and neurochemistry data are characteristic of mucopolysaccharidosis I, the phenotype of adult alpha-L-iduronidase deficiency mimicking Friedreich's disease has not been described so far.

Adult↗
PubMedDate not supplied

Association of primary cerebral lymphoma with meningioma: report of two cases.

Two cases of association of primary malignant cerebral non-Hodgkin's lymphoma with clinically silent meningiomas are reported. In one case, the malignant lymphoma of the left frontal lobe was diagnosed radiographically and the small parasagittal meningioma was detected at autopsy. In the other case, both CNS neoplasms, a parasagittal meningioma and a multifocal periventricular and brainstem lymphoma, were only detected at autopsy. In both cases, the small meningiomas were located at the cerebral convexity. The concurrence of the slowly growing meningiomas and the highly malignant cerebral lymphomas, seen in two among about 140 confirmed cases of primary CNS lymphomas, is probably coincidental. No other neoplasms were found outside of the CNS.

Aged↗
PubMedDate not supplied

Human neurolymphomatosis in a patient with chronic lymphatic leukemia.

A 62-year-old woman with chronic lymphatic leukemia (CLL) (RAI stage IV) with multiple organ involvement and diabetes mellitus, three months prior to death presented with a symmetrical sensory neuropathy of the upper extremities with little motor impairment and, two months later, sensory atactic neuropathy of the lower limbs. No cranial nerve or CNS impairment was noted. Clinical diagnosis was predominantly sensory neuropathy, but nerve conduction velocities were normal on upper limbs and moderately abnormal on lower limbs, the latter attributing to long lasting diabetes mellitus. The women died from acute subarachnoid hemorrhage. Autopsy revealed CLL of B-cell type with generalized organ involvement and acute craniospinal subarachnoid hemorrhage from ruptured cerebral aneurysm. There was selective neoplastic infiltration of the dorsal root ganglia and peripheral nerves, particularly the median nerve. Although selective infiltration of peripheral nerves by B-cell lymphoma cells was not associated with myelo-axonal degeneration, the relationship of this case to human neurolymphomatosis is discussed.

Axons↗
PubMedDate not supplied

[Histopathologic studies in dementia--comparison with clinical findings].

This paper compares the clinical diagnosis of dementia with the result of the neuropathological brain examination in 23 patients. Aim of this investigation was a verification of the clinical diagnosis. The diagnosis was based on the clinical findings, the Mini Mental State Examination, DSM-III-R classification of dementia, the NINCDS-ADRDA-criteria, results of laboratory tests, EEG, CCT and sonography. A total of 70% of the clinical diagnosis corresponded to the neuropathological results. In 25% of these cases which clinically showed the characteristics of Alzheimer's disease histopathological findings delineated dementia of non-Alzheimer-type. These results underline the importance of additional histopathological investigations in the diagnosis of dementia.

Aged↗
PubMedDate not supplied

Neuropathological aspects of dementias resulting from abnormal blood and cerebrospinal fluid dynamics.

In a large autopsy series of elderly individuals, organic dementia was attributed to (pre-) senile atrophy in 52.8%, to cerebrovascular disease in 22.5%, while 13.6% were of mixed senile and vascular origin, and 1.3% showed communicating hydrocephalus with meningopathies or were of undetermined origin. A survey is given of the morphological criteria of dementia resulting from disorders of cerebral blood supply and CSF circulation. The anatomic basis of vascular dementias are: atherosclerotic encephalopathy with lacunar state or multiple infarcts; granular cortical atrophy resulting from local microcirculation disorders; hypertensive cerebrovascular disease with the common "mixed" cortico-subcortical type, and the rare Binswanger's subcortical type. Atypical cerebral hemorrhage in old individuals rather results from congophilic (amyloid) angiopathy than from hypertensive arteriosclerosis. Multiple infarct dementia may also result from thrombotic microangiopathy, thromboembolic disease or cerebral vasculitides. The anatomical features of dementia associated with communicating "normal-pressure" hydrocephalus (NPH) are meningopathy at the basis or on the convexity, and fibrosis of the choroid plexus and/or arachnoid villi of post-inflammatory or undetermined origin, and other non-specific changes (periventricular gliosis). This condition is also associated with hypertensive cerebrovascular disease and Alzheimer's disease. Cerebral biopsies in NPH as well as in other types of hydrocephalus show enlarged extracellular spaces with otherwise normal neuropil probably resulting from increased transcapillary filtration. In some cases of "idiopathic" NPH no causative anatomical changes are found. The relationship between cerebral tissue changes, abnormal blood and CSF dynamics in these conditions remains to be clarified.

Aged↗