[Neuronal hypertrophy of the subventricular part of the infundibular nucleus in hypophyseotrophic tumors (author's transl)].
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Biomedical subjects
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Clinical and neuropathological findings of a 63-year-old male and a 25-year-old female with Down's syndrome are presented. Neuropathological examination of the older patient revealed intense features of Alzheimer's disease or senile dementia, including congophilic angiopathy and extensive mineral deposits in the globus pallidus and in the white matter of the cerebellum. In the hippocampus of the younger patient, plaque-like bodies and a few neurofibrillary tangles were found. From a survey of the cases hitherto reported in the literature it appears that among patients over 50 years of age it is common to encounter pathological features typical of Alzheimer's disease or senile dementia, that plaque-like bodies may occur in the second decade, neurofibrillary tangles in the third decade and a congophilic angiopathy in the fourth decade. The congophilic angiopathy is a frequent finding. Due to their high frequency, calcium or calciumlike deposits are regarded as important histopathological substrates of Down's syndrome. The clinical symptomatology of the long-surviving patients with Down's syndrome is that of a non-characteristic brain aging process and differs from that of the typical Alzheimer's disease. Organic dementia is not regularly found. Altogether, the anatomical findings in adult patients with Down's syndrome indicate a premature aging of the brain, which becomes more significant and widespread with increasing age.
Morphological structure, distribution and frequency of the so-called "nuclear spheroids" have been studied in the human hypothalamus. The following results were obtained: 1. By light microscopy, the nuclear spheroids turned out to be small or large spheroid bodies inside the nucleus. They are almost homogeneous, rarely granular, vacuolar or reticular and occasionally they are multiple. They are best stained with the cresyl-violet technique. They do not exhibit any histochemical pecularities. Aside from typical nuclear spheroids, irregularly shaped nuclei were frequently seen, associated with increased nuclear folds, transitional stages between nuclear folds and nuclear spheroids were also present. Certain microscopic features suggest invagination and displacement of nuclear membrane and cytoplasm into the karyoplasma. 2. By electron microscopy, the nuclear spheroids consist of nuclear membranes and cytoplasmic constituents. They, therefore, actually represent cytoplasm protruding into the cell nucleus. 3. The nuclear spheroids occur almost regularly and most frequently in the ventromedial field of the tuber cinereum, foremost in the infundibular (= arcuate) nucleus. They numerically increase during life. They are rarely found in the ventromedial nucleus but do occur in most of the adults. In the paraventricular nucleus, the supraoptic nucleus, the lateral nuclei tuberis, and in lateral hypothalamic nucleus, they are found by far less often. They scarcely develop in the preoptic area, in the dorsal field, in the tubero-mamillar complex, in the parafornical nuclear groups, in the premamillary nucleus and in the mamillary nuclei. 4. Within the infundibular (= arcuate) nucleus, the nuclear spheroids are particularly numerous in the basal and basolateral regions, whose neurons, under certain functional conditions--such as the Sheedan syndrome, in the menopause and in association with pituitary neoplasms-, appear hypertrophic. Within the hypertrophic neurons of this region, which we designate the subventricular part of the infundibular nucleus, nuclear spheroids are definitely more frequent, and they are of the large type. 5. A separation of the subventricular part based on the distributional pattern of the nuclear spheroids indicates a pathological karyoarchitectonics which supplements the normal karyoarchitectonics (Bachmann, 1948). 6. The nuclear spheroids seem to be associated with increased neuronal activities, for their frequently strongly correlates with the neuronal hypertrophy which expresses enhanced neuroendocrinological activity.
A quantitative structural analysis of the ventromedial area of the human hypothalamus was performed using an automatic image analysing computer. We divided the ventromedial area into two nuclei, nucleus ventromedialis (N. vm.) and nucleus infundibularis (N. inf.). N. vm. was studied in 6 cases, N. inf. in 7 cases. The following findings were obtained: 1. No significant differences of cell population became apparent between the left and right sides of both nuclei. Aside from a female case above 80 years of age, so far, no correlation with cellular distribution of age and sex, respectively, could be established. 2. In N. vm. the distribution of large and small nerve cells depends on the region within this nucleus. Small nerve cells appear more frequent in the medial region than in the central and in the lateral regions. On the other hand, significant distributional differences of nerve cells in the dorsal, central, and basal regions as well as in oro-caudal direction are absent. Data of individual cases and their mean value are conforming. Cell density (cell number/volume) does not show any regional dependence both in the frontal plane and in the oro-caudal direction; it amounts to approximately 170/mum3 in the entire nucleus. 3. In the frontal plane plane N. inf. does not disclose marked regional differences of cell population. Large neurons are slightly more frequent in the basal-medial region as compared to the dorsal region. An 81-year-old woman displayed a conspicuous increase of large nerve cells in the basal and centrolateral regions, obviously hypertrophy of the potential "subventricular nucleus" as described by SHEEHAN and KOVACS, which is, in our opinion, a part of the N. inf. In young subjects (below the age of 40 years) the frequency of large nerve cells decreases in oro-caudal direction. Identical cell populations suggest that the cuadal end of N. inf. extends to the floor of the mammillary recessus. Cell density in the frontal plane appears higher in the centrolateral region (230/mum3) compared to the dorsal and basal regions (190-200/mum3). Cell density increases in oro-caudal extent; it decreases, however, in the most caudal part.
The long-term results of 12 stereotaxic operations on 11 multiple sclerosis patients with incapacitating intention tremor were evaluated and compared with the experiences of other authors. The selection of the patients, the criteria applied for the success and the length of the follow-up period influenced the reported results. Considering not only the relief of the intention tremor but the overall performance after the operation only a certain group of patients seemed to profit by neurosurgical treatment. Applying this criterion of overall performance and evaluation only 3 out of 11 patients in our series had real benefit from the operation. The reasons for this small number of good or moderate results are given with brief discussion of some of the cases. History, course and fatal outcome of one patient are presented in detail together with the neuropathological findings. According to the literature and the limited number of our own cases the following indications for stereotaxic operations on MS patients can be established: 1. Tremor and hyperkinetic movements should be the dominant features of the symptomatology. 2. The overall performance should be essentially improved by the operation. 3. Patients in the terminal stage of the disease gain little from the procedure, whereas patients with longstanding more benign course are the best candidates.
The clinical findings and course in the first 2 cases of multiple sclerosis are described, in whom it was possible to isolate a virus from brain tissue by early sterile autopsy and fusion technique. It is noteworthy that in one of these cases multiple sclerosis probably occurred in female members of the family through three consecutive generations. A report is made on an additional case, in which no virus could be isolated, but in which electron microscopic studies showed two types of virus particles: nucleocapsid-like structures and particles identical in form and size with papova virus. Electron microscopic findings and attempts to cultivate a virus in multiple sclerosis published by other authors are discussed. It is pointed out, that the results described provide as yet no proof for the viral etiology of multiple sclerosis. They support the hypothesis, however, that a virus may play an essential role in the etiology and/or pathogenesis of this disease. Problems in relation to brain autopsy in multiple sclerosis are discussed. Early sterile autopsy is considered the most practicable possibility for obtaining tissue material for culturing and for ultrastructural studies in multiple sclerosis.
1) Coronal sections measuring exactly 4 mm in thickness of 106 human brains (212 cerebral hemispheres) were cut with the Göttinger Hirnmakrotom. Planimetric volumetry of various macroscopically delineated structures was performed on photographs of the sections. 2) The volumes ovtained from 58 "normal cases" were used for determining preliminary standards as well as mean values and standard deviations for age and sex. The female-male ratio of the structures measured varies between 86 and 92%. Comparing right and left a predominance of the left pallidum for both sexes is apparent showing an error probability of less than 5%. In "normal" men a significant predominance of the rightsided frontal structures, located anterior to the anterior commissure, have been found (error probability of less than 1%). 3) Regarding the 48 "abnormal cases", striatum and pallidum show a uniform picture in Huntington's disease, namely an extreme shrinkage. The pallidum shrinks to a similar extent as the striatum, although its neurones are not substantially affected by this system atrophy. Other structures do not display similarly uniform changes in this disease. 4) In Parkinson's syndrome a tendency of the pallidum to enlarge -- though statistically not significant -- is seen. This raises the question whether a constitutional hyperplasia of this structure is sometimes involved in the pathogenesis. 5) In Pick's disease, it is not only the histologically impressive centers of shrinkage of the cerebral cortex that are atrophic, but, to a somewhat lesser degree, also the whole telencephalon. 6) In an 18-year-old girl with malignant obsessional neurosis (schizophrenia?) the volume of the striatum was highly above average values enlarged. 7) Bibliographical data of macroscopic-quantitative brain research reveal many problems which can be solved today due to improved methods.