Type III collagen deficiency and type IV Ehlers-Danlos syndrome.
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Collagen↗
Biomedical subjects
K Hegedüs
Publications and source records attributed to K Hegedüs.
The effects of hypertension on the wall of the large intracranial arteries with special reference to the changes of some connective tissue elements.
The effect of hypertension on the wall of the major cerebral arteries of 62 patients was investigated at the light microscopical level. This series does not include patients with atherosclerosis of the large intracranial arteries. In hypertensive individuals the intracranial arteries showed concentric intimal thickening. Furthermore, the collagenous fibers occurred in the media as early as in the third decade of life in place of reticular fibers. On the basis of literary data and our findings one may suppose that under permanent mechanical influence the arterial smooth muscle cells synthetize predominantly collagenous fibers instead of reticular fibers.
Adult↗
Anatomical patterns of hypoplastic posterior communicating arteries and their implications for cerebrovascular diseases.
A total of 114 undersized posterior communicating arteries (PCoA-s, external diameter less than 1 mm) and 126 larger ones were investigated histologically. Microscopic examination of hypoplastic PCoA-s revealed five different structural patterns. Two of them showed microscopic appearances characteristic of intracranial arteries seen in adults or newborns without vascular disease. Two other types resembled the histologic picture of Moya Moya disease. The last type was thin-walled and dilated. All PCoA-s larger than 1 mm displayed the adult pattern. The PCoA-s with the Moya Moya-like structure and the thin-walled, dilated pattern contained unusually few reticular fibers. It is supposed that Moya Moya disease may be restricted to the PCoA-s. Further, the types of hypoplastic PCoA-s with reticular fiber deficiency are most probably the source of the so-called unexplained subarachnoid hemorrhage regardless of the presence of infundibular widening. The latter statement stems from the observation that the structure of junctional dilatation does not differ from that of other segments of the artery that exhibit this alteration.
Adolescent↗
On the pattern of reticular fibers in the intracranial arteries of mature newborn with and without intracranial hemorrhage.
A detailed histological study was carried out on the major intracranial arteries of 20 mature newborn. A substantial reduction of the reticular fiber content within the wall of the cerebral arteries was observed in all samples originating from infants with intracranial hemorrhage. No other differences occurred when comparisons were made using the conventional staining methods on the vessels from the ten newborn with hemorrhage and the ten others without bleeding. These data indicate that the structural deficiency of the brain vasculature provides the morphological basis for the development of intracranial hemorrhages in the term newborn.
Arteries↗
Pattern of reticular fibres of the major cerebral arteries in cases of unexplained subarachnoid haemorrhage.
The pattern of reticular fibres was studied in the intracranial arteries of six patients who did of subarachnoid haemorrhage without demonstrable cause. In each patient, the media of all of the major intracranial arteries contained much fewer reticular fibres than those of individuals without vascular disease. These fibres were mainly sparser in the outer part of the media, resembling the pattern seen in patients with ruptured berry aneurysms. It is concluded that the deficiency in reticular fibres may predispose to rupture even at the early stage of aneurysm formation.
Adult↗
Experimental focal cerebral ischaemia in rabbits.
A highly reproducible form of experimental embolization of the intracranial arteries is presented in rabbits. The injection of a silver or gold ball into the internal carotid artery caused occlusion predominantly of the middle cerebral artery and/or its branches. At the moment when embolization took place, the characteristic signs of acute cerebral ischaemia occurred in the electroencephalogram, local cerebral blood flow and steady (DC) potentials. Several hours after the ball had been injected the extent of the focal lesions became recognizable on sections stained for myelin. The procedure is simple, rapid, inexpensive and practically always successful. The extent of the lesion may be influenced by the change of both the ball size and the posture of animals. Moreover, the site of occlusion is easily discernible both on radiographs and to the naked eye.
Animals↗
Hypoplasia of the basilar artery. Three case reports.
Three patients, aged 65, 70, and 80 years, respectively, with hypoplastic basilar artery are presented. To our knowledge no similar case has been reported in the literature till now. In one of the cases, the hypoplasia of the basilar artery was an incidental finding. The other two patients had transient neurological symptoms considered characteristic of circulatory insufficiency in the vertebrobasilar system. One of the latter two patients died of acute myocardial infarction and the other patient died after developing occlusion in the cervical part of the right internal carotid artery. The exact nature of hypoplastic basilar artery is uncertain. The possible mechanisms of its occurrence are discussed.
Aged↗
Reticular fiber deficiency in the intracranial arteries of patients with dissecting aneurysm and review of the possible pathogenesis of previously reported cases.
The pattern of reticular fibers in the tunica media of the major intracranial arteries was investigated in two patients with dissecting aneurysm. In numerous circumscribed areas, the reticular fibers were absent close to the internal elastic lamina in all major arteries of each patient. It is suggested that the subintimal deficiency in reticular fibers results in insufficient fixing of the tunica intima to the media contributing to their separation when the internal elastic lamina becomes defective. The presumed etiologies of the previously reported cases are reviewed and the possible origin of the deficiency in reticular fibers is discussed.
Adolescent↗
Ectasia of the basilar artery with special reference to possible pathogenesis.
The pattern of reticular fibers and some other microscopic features of all the major intracranial arteries were examined in two patients with ectatic basilar artery. In order to make a comparison possible the major cerebral arteries of 105 individuals with advanced atherosclerosis were also investigated. This series includes 40 patients with berry aneurysms. Defects in the elastic lamina and reticular fiber deficiency in the muscular layer were common pathologic features in patients with ectatic basilar artery and in those with berry aneurysm. In the ectatic basilar arteries these alterations were much more conspicuous. In the other atherosclerotic arteries sampled from patients having no malformations, the density of reticular fibers in the media was preserved. It is concluded that atherosclerosis may not have a basic role in the pathogenesis of arterial ectasias, but the severe reticular fiber deficiency in the media associated with extensive defects in the elastic lamina forms the morphologic basis of ectasias. Furthermore, the arterial ectasias and berry aneurysms are most probably different manifestations of the same underlying disorder.
Aged↗
Histopathological study of major intracranial arteries in premature infants related to intracranial hemorrhage.
The incidence of various types of intracranial hemorrhage (ICH) and the microscopic appearance of the major intracranial arteries were investigated in 112 premature infants, and related to birth weight. In none of the premature newborns, regardless of the presence of ICH, did the intracranial arteries contain true elastic elements. The wall of the basilar artery was significantly thinner in all premature infants with any kind of ICH. Within each body-weight group those with ICH had intracranial arteries with fewer reticular fibers than did those without ICH. The density of the reticular fibers in the cerebral arteries of premature neonates without ICH did not seem to depend on maturity. The authors believe that the hypoplasia of the major intracranial arteries and the reticular fiber deficiency most probably involve the whole vascular system of the brain and may predispose to hemorrhage.
Cerebral Arteries↗
Fibromuscular dysplasia of the basilar artery. Case report with autopsy verification.
Seven histologically confirmed cases of intracranial fibromuscular dysplasia (FMD) have been published. A new case of intracranial FMD confined to the basilar artery occurred in a 32-year-old man. The proliferation of fibroblasts and smooth-muscle cells could be observed only in the intima detaching from the media. The neurologic symptoms corresponding to akinetic mutism occurred nine days after pulmonary lobectomy for aspergilloma. We believe that this pure intimal type of FMD consisting of cells without elastic and collagen fibers represents a more undifferentiated and therefore more progressive form of the disease. The FMD can be considered as an overwhelming reaction of so-called pluri-potential smooth-muscle cells of the arterial wall to various stimuli.
Adult↗
Case report of infarction in the region of the posterior spinal arteries.
Spinal cord infarction in the region of the posterior spinal arteries is reported in a 62-year-old woman. The softening was restricted to the lumbosacral region involving two segments. Sectioning the affected and adjacent segments serially no occlusion was found in the posterior spinal arteries. Besides the circumscribed infarction the microscopic picture of the spinal cord was characteristic of vascular myelopathy. The underlying disorders of the previously reported cases and the predisposing factors contributing to the development of infarction are discussed. It is concluded that an insufficient anastomotic network plays the essential role in the pathogenesis of the spinal cord infarction.
Arteries↗
Direct involvement of intracerebral arteries in Takayasu's arteritis.
A man aged 20 with sudden onset of headaches and rapidly evolving spastic quadriparesis died within 3 weeks. Pathologic examination revealed intensive inflammation confined mainly to the adventitia of the aorta, the internal carotids, and all the major intracranial arteries. The gross changes and the lymphocytic and plasma cell nature of the inflammatory infiltrates were typical of Takayasu's arteritis. Thrombosis of the right internal carotid artery resulted in ischemic necrosis of the ipsilateral hemisphere. Quite recent thrombus occluded the left internal carotid artery. This seems to be the first case report on histologically proven extensive direct involvement of intracranial vessels in Takayasu's disease. The differential diagnostic aspects of the case are also briefly discussed.
Adult↗
Some observations on reticular fibers in the media of the major cerebral arteries. A comparative study of patients without vascular diseases and those with ruptured berry aneurysms.
In spite of the large number of papers about the changes in the cerebral arterial wall occurring under various conditions, no systematic study can be found in the literature on the reticular fibers surrounding the smooth muscle cells of the media. The histologic examination of the major cerebral arteries of 70 individuals without vascular diseases revealed a dense network of fine reticular fibers in the media showing a rather uniform distribution. This pattern did not change significantly from birth up to the age of 50 years and was independent of intimal proliferation with aging. Contrary to this, all the major cerebral arteries of 35 patients who died from rupture of berry aneurysms under the age of 50 contained a considerably smaller amount of reticular fibers, predominantly in the outer part of the media. It is concluded that people having few reticular fibers in their arterial wall are susceptible to develop aneurysms, and the partial lack of these fibers may play an important role in the development of cerebral aneurysms.
Adolescent↗
The adult form of subacute necrotizing encephalopathy.
The rare adult form of subacute necrotizing encephalopathy in a 49-year-old man is reported. Six months before admission the previously healthy and nonalcoholic patient developed progressive hemiparesis of the left side and instability of gait. Ventriculography revealed marked narrowing of the aqueduct, and after ventriculocisternotomy the patient became akinetic and mute. The post-mortem histologic examination revealed pathologic changes characteristic of subacute necrotizing encephalopathy. This case demonstrates that this rare disease in an adult may cause both akinetic mutism and aqueductal stenosis.
Brain Diseases↗
Adjacent astrocytoma and ependymoma: dependent mixed neoplasia or unusual collision tumors?
Concurrent neoplastic transformation of different cellular elements resulting in an intraventricular and intraparenchymal tumor is described. An infiltrating astrocytoma of the left frontal lobe had been partially removed from a 43-yr-old woman. Four months later she died, and the autopsy revealed a recurrent astrocytoma and a closely apposed large intraventricular ependymoma. Both light microscopy and ultrastructural findings established the different nature of the two tumors. Theoretically they could represent collision or composite tumors. Another possibility would be that a relatively longstanding ependymoma induced the astrocytic neoplasm. The clinical history and the sequential morphological analysis, i.e., biopsy and autopsy sampling suggest that the ependymoma developed due to the inductive influence of the astrocytic growth.
Adult↗
Burkitt-type lymphoma and reticulum-cell sarcoma. An unusual mixed form of two intracranial primary malignant lymphomas.
An unusual mixed type of two primary malignant lymphomas in a 50-year-old woman is reported. The patient had been in good health until 4 months before her death, when she developed epileptic seizures and neurological symptoms attributed to a lesion of the brainstem. Pathologic examination revealed a circumscribed tumor mass in the right parietal lobe, which was shown microscopically to contain two different forms of malignant lymphoma. The histologic features of the peripheral part of the tumor were typical of a Burkitt-type lymphoma, while the central part showed the features of a reticulum-cell sarcoma. The cells of the latter type diffusely infiltrated both temporal lobes, the cerebellum, and the brainstem. It is concluded that in the absence of systemic involvement, the Burkitt-type lymphoma also could be derived from the primitive reticulum cells of perivascular sheaths.
Brain Neoplasms↗
Defects of elastic lamina in middle cerebral artery. A possible cause of a primary intracerebral hemorrhage in a young woman.
Circumscribed defects in the elastic lamina as a possible cause of intracerebral hemorrhage are reported. The abnormality was present in the right middle cerebral artery of a 24-year-old women. The patient died of large intracerebral hemorrhage which extended into the lateral ventricle on the same side and to the subarachnoid space. In the absence of vascular malformation and secondary degenerative changes attributed to hypertension these defects of the internal elastic lamina associated with the rise of the blood pressure appear to lead ultimately to bleeding.
Adult↗