Biomedical subjects
Publications and source records attributed to W Kamphorst.
We present a case of spinal subarachnoid hemorrhage due to an ependymoma of the filum terminale in a 23-year-old male. Clinical signs indicating a spinal origin of the subarachnoid hemorrhage are discussed. Subarachnoid hemorrhages are only rarely caused by an intraspinal tumor, most of which are located in the cauda equina. Our findings in this case proved the value of MRI examination in tumors of the cauda equina.
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The case of a 7-year-old boy with a spinal epidural extraosseous Ewing's sarcoma (EES) is presented. He is in complete remission without neurologic deficit 40 months after diagnosis. Another 15 cases were found in the literature and are discussed together with this patient. Twelve of them were male patients. The mean age of the patients was 17.5 years (range, 4 to 47). Symptoms included back pain and/or radicular pain (100%), paresis of one or both legs (83%), sensory disturbances, and bladder and bowel dysfunction. The mean diagnostic delay was 5.8 months. Each patient underwent laminectomy; complete resection of the tumor was impossible in more than 50% of the cases. Most patients received radiation therapy and/or chemotherapy. Four patients suffered from local recurrence, eight from metastases. Ten (63%) patients died, 1 to 48 months (mean, 16) after diagnosis. The differential diagnosis is discussed, including disk herniation and several benign and malignant tumors.
A literature review of pregnancy-related vertebral haemangiomas, pregnancy-related haemangiomas outside the nervous system and its coverings, and pregnancy-related meningiomas was performed. All three conditions tended to occur during the first pregnancy, the second or third trimester, to ameliorate postpartum, and to recur during a subsequent pregnancy. These results suggested a hormonal influence on the clinical expression of haemangiomas and meningiomas. To test whether a hormonal factor is also operative in the (not pregnancy related) symptomatic and asymptomatic counterparts of these conditions we also reviewed the literature concerning the symptomatic and asymptomatic haemangiomas and meningiomas for the following: frequency, sex and age distribution. No arguments were found to suggest that steroid hormones are strong factors in the development of asymptomatic and symptomatic lesions. The pregnancy-related counterparts have to be considered as subsets with their own behaviour.
Although the reason for development of saccular aneurysms in humans has still not been elucidated, the process might be observed and explained using a reproducible aneurysm model in vivo. In this paper, a sequential pathological study of the experimental saccular aneurysms was made. No additional influential factors were applied. At all stages of the development between the induction and 6 months, the histology of the aneurysm wall was similar to that of human aneurysms. The imbalance of the degeneration and regeneration processes during the development of these experimental aneurysms is discussed according to the pathological findings in different periods. Haemodynamic stress and different regenerative speeds in different arterial tissues must be responsible for the saccular aneurysm formation and growth.
Haemodynamic factors in the formation and development of saccular aneurysms have been widely studied. Saccular aneurysms could appear and grow at the side of the increased blood flow. The effects of contralateral carotid ligation on the formation and growth of our experimental saccular aneurysms were studied. Measurement and pathological examination showed that the haemodynamic changes could facilitate the development of saccular aneurysms, but by itself could not bring about their formation.
In this study we applied the technique described in the previous paper (see pp. 103-106 of previous issue (1991); 13(2)) to rats with experimental saccular aneurysms. We found that the mean sizes and some growth ratios of experimental aneurysms were significantly larger in the intermittent blood pressure elevation groups than in the control groups. The increase of growth ratios was proportional to the number of transient blood pressure elevations. The morphological structure of these saccular aneurysms showed that the regenerative processes in the aneurysm wall had obviously been interfered with in animals with intermittent blood pressure elevation. The effective mechanism of this influential factor is discussed.
The clinical data and computed tomographic findings of 64 patients with solitary supratentorial brain lesions were presented to two panels of six experienced clinicians. The diagnoses predicted by these clinicians were compared with each other (interobserver variation) and with the definite diagnosis, which in almost all cases was based on histologic examination of the involved tissue (validity of predicted diagnosis). The interobserver agreement was only moderate. The predicted diagnoses agreed with the definite diagnoses in only 57% of cases. A high number of errors were made in distinguishing between high-grade and low-grade glioma and between high-grade glioma and cerebral metastasis, and in the detection of primary cerebral lymphoma. Possible implications of these findings for clinical practice are discussed.
A new and reproducible saccular aneurysm model has been developed at the bifurcation of the common carotid artery in rats. The details of the experimental methods and results are described. It is strongly suggested that the internal elastic lamina is a critical layer in saccular aneurysm formation, because an experimental saccular aneurysm can be produced immediately by transluminally damaging the inside of the arterial wall at the bifurcation of the common carotid artery. This saccular aneurysm model has several advantages: (i) it can be induced quickly and the success rate approaches 100% in rats; (ii) this technique can produce satisfactory experimental saccular aneurysms for other aneurysm studies, and in the future it will also be possible to use this technique to produce experimental saccular aneurysms in cerebral arteries of large animals.
After 2 and 3 months we re-exposed 20 experimental saccular aneurysms. Under the operating microscope, we measured their sizes and compared them with the sizes immediately after induction. All aneurysms had grown significantly and none was thrombosed. Pathological examinations showed that the sac of the aneurysm was similar to that of the human saccular aneurysms. Based on the operating microscopy and pathological findings we postulate that abnormal histological structure and haemodynamic stress are the major causes of aneurysm enlargement. We also noted that there were some regenerative processes in these experimental aneurysm walls. The results prove that this model is reliable, because these aneurysms have the main characteristics of human saccular aneurysms, not only grossly but also microscopically.
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We reviewed the literature concerning primary brain and spinal tumors with first manifestation or acceleration of symptoms during pregnancy or within the first postpartum week and encountered four new cases in our center. The incidence of brain tumors that become symptomatic during pregnancy appears to be decreased compared with that in age-matched women. The relative frequency of the different primary brain tumor types is not changed by pregnancy. The number of meningiomas gradually tends to increase during pregnancy, with gliomas and spinal vascular tumors accumulating in the first and third trimesters, respectively. Postpartum amelioration of symptoms has especially been described for meningiomas and spinal vascular tumors. We conclude that different types of tumors are influenced at different stages of pregnancy. Although progesterone receptors predominate compared with estrogen receptors, no definite causal relationship with progesterone has been established.
A patient with a chronic lymphocytic hypophysitis (LYHY) following an idiopathic lymphocytic meningitis is described. Computed tomography scan of the sella turcica suggested a pituitary adenoma with suprasellar extension, whereas hormonal investigations showed a functional lesion at the suprapituitary level. A transsphenoidal biopsy revealed diffuse lymphocytic infiltration of the adenohypophysis without characteristics of a sarcoid lesion. The possible relationship between idiopathic (viral?) lymphocytic meningitis and LYHY is discussed.
A case of concordant oligodendroglioma in monozygotic twins is reported. The twins were also concordant for uterine leiomyoma and one twin partner had fibroadenoma and lipoma of the breast and myelofibrosis. As very few cases of concordant glioma in monozygotic twins have been published and no such cases in dizygotic twins, a genetic influence in the aetiology of glioma can only be suggested.
Cytogenetic studies on neuroblastomas arising in children have revealed consistent abnormalities of the short arm of chromosome number 1. Partly because of the rare occurrence of neuroblastomas in adults, extensive cytogenetic studies in this group of patients have not been performed. We report a case of a neuroepithelioma (neuroblastoma) arising in a 50-year-old male patient. On chromosome analysis of a metastasis, a stemline with karyotype 47,XY, +der1 (1 qter---1 cen::1q21---1 qter) was identified. The possible consequences of this result and those of results previously reported in the literature are discussed.
A very rare hemangioblastoma of the filum terminale with sciatic pain and partial cauda syndrome in a 36-year-old man is presented. The difference between solitary hemangioblastoma, hemangioblastomatosis and von Hippel-Lindau disease is stressed. The literature is reviewed and the diagnosis of cauda equina neoplasms is discussed. Even nowadays often the final diagnosis is made late and only after operation and microscopical tissue examination.
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With immunohistoperoxidase techniques the presence of plasma (serum) proteins was investigated in senile plaques, congophilic angiopathy, neurons and glial cells in brains of patients with Alzheimer's dementia. Other investigators have found plasma proteins in brain parenchyma and suggested that blood-brain barrier dysfunction might be a primary factor in the pathogenesis of Alzheimer's dementia. These studies were performed on formol-fixed brains of patients with Alzheimer's dementia. In the present study we investigated both frozen and formol-fixed brain tissues. The influence of post-mortem delay, prolonged formol fixation and differences in clinical course on detection of plasma proteins by immunocytochemical techniques was also studied. Findings in cases with Alzheimer's dementia were compared with findings in nondemented controls with or without neurological disorders. Plasma proteins could not be demonstrated in the neuropil of a number of patients with Alzheimer's dementia. Moreover, plasma proteins were also found in neuronal cells and astrocytes in brains of nondemented controls. We discussed whether or not cytochemical detection of plasma proteins in the neuropil of post-mortem obtained brains is a reliable technique to investigate blood-brain barrier dysfunction. In our opinion there are, at the moment, no convincing arguments for blood-brain barrier dysfunction in Alzheimer's dementia.