1/32. Detection of beta-A4 amyloid and its precursor protein in the muscle of a patient with juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt-Sjogren).Muscle biopsy tissue from a patient affected by the juvenile form of neuronal ceroid lipofuscinosis (NCL) was studied immunohistochemically using antibodies to beta-amyloid peptide and amyloid precursor protein. Positive reaction in muscle was specifically localized to autophagic vacuoles and blood vessel walls. Increased acid phosphatase reaction suggested enhanced lysosomal activity. We hypothesize that beta-amyloid is deposited in NCL muscle by a lysosomal mechanism similar to that proposed in other disorders involving beta-amyloid.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/32. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/32. Cerebral angiofibroma: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibromatous tumors are very rare lesions, with few reported cases. CLINICAL PRESENTATION: We report the case of a 34-year-old male patient who experienced seizures resulting from a cystic lesion in the left occipital region, which remained unchanged for 11 years. After the seizures increased in number, magnetic resonance imaging revealed a large cyst with a tumor nodule. INTERVENTION: A left occipital craniotomy was performed, and the tumor was removed. Pathological studies, including immunohistochemical and ultrastructural analyses, indicated that this neoplasm was composed of fibrous and angiomatous components, and a diagnosis of cerebral angiofibroma was established. CONCLUSION: Cerebral and meningeal fibromas are rare neoplasms that differ from solitary fibrous tumors and fibrous meningiomas. When a number of prominent blood vessels are present in a cerebral or meningeal fibroma, a diagnosis of angiofibroma can be considered. It is possible that some nodular brain tumors that were previously described as meningioangiomatosis could be reclassified as cerebral or meningeal angiofibromas.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/32. stroke in a young man with fibromuscular dysplasia of the cranial vessels with anticardiolipin antibodies: a case report.A case of fibromuscular dysplasia (FMD), presenting with a non-hemorrhagic infarct is reported. Positivity of anticardiolipin antibodies suggested an immune response. A 40-year-old man presented with sudden onset of stroke, preceded by similar ischemic attacks. Computed tomography (CT) of the brain showed a recent non-hemorrhagic infarct in the left middle cerebral artery (MCA) territory and an old right MCA territory infarct. serum was positive for anticardiolipin antibodies. These above findings were confirmed at autopsy. A portion of the internal carotid artery and the middle cerebral arteries on both sides revealed features of FMD, with thrombosis. This case suggests an immune mechanism for FMD, hitherto unobserved in the cerebral circulation.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
5/32. serum tryptase analysis in a woman with amniotic fluid embolism. A case report.BACKGROUND: Recent studies have noted a striking similarity between amniotic fluid embolism (AFE) and anaphylaxis. serum tryptase levels may therefore serve as a marker of mast cell degranulation in AFE cases. CASE: A 40-year-old woman, gravida 6, para 4, experienced the acute onset of facial erythema, eclampsia-type seizures, severe hypoxia, cardiac arrest and disseminated intravascular coagulation while in early active labor. The patient was declared dead 37 minutes after the onset of resuscitative efforts. At autopsy, fetal squames were found within the pulmonary tree, uterine blood vessels and brain. A peripheral venous blood specimen, obtained approximately one and a half hours postmortem, revealed a tryptase level of 4.7 ng/mL (normal, < 1). CONCLUSION: An elevated serum tryptase level, in conjunction with our patient's clinical history, adds further supporting evidence to the concept of AFE as an anaphylactoid syndrome of pregnancy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/32. Meningioangiomatosis: MR imaging and pathological correlation in two cases.Meningioangiomatosis is a rare, benign neoplastic disorder involving the cortex and leptomeninges. The pathological findings are characterised by proliferation of meningothelial cells and leptomeningeal vessels and calcifications within the mass. We experienced two cases of pathologically confirmed meningioangiomatosis, one as a solitary cortical mass with calcification and the other as a cortical lesion manifested as extensive intracranial haemorrhage. On MRI, the first case showed an isointense cortical mass in the left frontal lobe and homogeneous enhancement on the contrast-enhanced study. The second case showed a target-like lesion with a peripheral dark signal rim on T2-weighted images accompanied by extensive haemorrhage in the adjacent frontal lobe and lateral ventricles.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/32. Anticholinergics induce eclamptic seizures.Severe preeclampsia is a pathophysiological disorder specific to pregnancy and characterized by vasoconstriction and hypercoagulability. eclampsia (convulsion associated with preeclampsia) and hemolysis, elevated liver enzymes, and low platelet count associated with preeclampsia (hellp syndrome) are serious complications in patients with severe preeclampsia. They are thought to be characterized by generalized vasoconstriction and reduction in blood flow to various organs that may be explained by increased sensitivity of the vascular smooth muscles and increased vasopressors. liver involvement in eclampsia and preeclampsia is referred to as hellp syndrome, and epigastric and right upper quadrant pain is often a symptom of severe preeclampsia and may be indicative of imminent convulsions. In addition, marked dilatation of the stomach and the colon is often demonstrated in these patients. These phenomena result from the hyperactivity of the sympathetic nervous system but are not caused by the hyperactivity of the parasympathetic nervous system. The authors experienced two cases of eclamptic seizures after the administration of an anticholinergic (scopolamine butylbromide) in patients with severe preeclampsia complicated by hellp syndrome. Anticholinergics, blocking agents of the parasympathetic nervous system, can enhance the hyperactivity of the sympathetic nervous system; therefore, vasospasms of the vessels may be easily aggravated, and eclamptic seizures may be induced in patients with severe preeclampsia, especially in those complicated by hellp syndrome. The administration of anticholinergics should be avoided in patients with severe preeclampsia, especially when there is epigastralgia.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/32. September 2002: 24-year-old female with a 6-month history of seizures.The September 2002 COM. A 24-year-old female presented with a history of 3 generalized seizures, the first of which had occurred 6 months before admission. Her neurological examination was normal, but upon admission her MRI showed a small cystic lesion in the left parieto-occipital region. The lesion was hyper-intense on T-2 weighted images and did not show contrast enhancment. At surgery, the tumor was found to be deep to the cortex and was a cyst with amber fluid surrounded by gliotic brain. Microscopically, the tumor was well-demarcated from the surrounding tissues, which showed reactive changes, including Rosenthal fibers. The tumor was composed of GFAP-positive glial cells, which were arranged in a pseudopapillary fashion around blood vessels. In between, the tumor cells were positive for neuronal markers. The diagnosis was papillary glioneuronal tumor (PGNT), a relatively recently described lesion that may be a variant of ganglioglioma. The current literature on PGNTs is reviewed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/32. Mini-infarct encephalopathy associated with uncommon microvessel convolute formation presenting with presenile dementia.A female patient started to suffer from transient ischemic attacks when she was 47 years of age, followed by increasing predominantly left-side spastic tetraparesis, generalized seizures and progressive dementia over a period of 11 years. She died when she was 58 years of age. On gross examination the brain showed enlarged ventricles and arteriosclerotic changes of large extracerebral vessels of the circulus arteriosus. Microscopic examination of the atrophic brain showed innumerable incomplete microinfarcts in the white and gray matter throughout all parts of the brain. In the white matter these lesions were characterized by small foci of demyelination and loss of oligodendrocytes while occasionally some scavenger cells were seen. axons seemed to be unaffected or displayed irregular axonal regeneratory growth. Any inflammatory reaction failed. In the cerebral cortex and subcortical nuclei the lesions showed loss of neurons and decrease in synaptophysin expression. Intracerebral arteries showed fibrosis or fibrohyalinosis of the entire intracerebral small-vessel network. In addition, numerous uncommon clusters of angioma-like telangiectatic vessels were observed. Medium-sized ischemic infarcts were found in the right putamen and adjacent internal capsule region, left-side dorsolateral brain stem and cerebellar hemisphere as well as a left-side pyramidal tract degeneration. Contralateral pseudohypertrophy of the inferior olivary nucleus was seen. The clinical and the neuropathologic observations made in this patient are compatible with small vessel disease characterized by a multicentric special and not yet described type of incomplete mini-infarcts in cerebral cortex and white matter accompanied by some larger ischemic infarcts of the common type in brain stem and cerebellum.- - - - - - - - - - ranking = 8keywords = vessel (Clic here for more details about this article) |
10/32. MELAS: clinical phenotype and morphological brain abnormalities.We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial dna (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. In all three cases there was a reduced immunocytochemical staining for mtDNA-encoded subunits of the respiratory chain, confirming the presence of a defective mitochondrial protein synthesis in this disease. Mitochondrial abnormalities were mostly confined to multiple areas of different size and shape, in agreement with the focal character of the brain pathology in MELAS, and were most prominent in the cerebral cortex, providing a morphological contribution to the explanation of the cognitive regression of the patients. Immunoreactivity for mtDNA-encoded subunits was reduced in the walls of many pial and intracerebral arterioles of different brain regions but there was no clear correlation between territories of affected vessels and distribution of the histological and immunohistochemical lesions. Cerebral focal lesions in MELAS might have a metabolic nature and several pathogenetic mechanisms might be involved in the genesis of stroke-like episodes when there is a local increased ATP demand.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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