1/157. noonan syndrome and cavernous hemangioma of the brain.We present two patients with multiple characteristics that occur in Noonan phenotype and cavernous hemangioma of the brain. The first patient, who had been diagnosed radiographically as having a cavernous hemangioma in the left basal ganglia at age 15 years, developed massive intracerebral hemorrhage, resulting in sudden death at home at 19 years. The second patient, who was diagnosed radiographically as having a cavernous hemangioma in the left parietal lobe at age 17 years, is being followed carefully (the patient is currently 18 years old). A review disclosed four cases of structural cerebrovascular abnormalities with or without subsequent hemorrhage. Neither these four patients nor our two patients had any severe anomalies in the heart or large vessels, which are frequently seen in patients with noonan syndrome. Cerebrovascular abnormalities might have a significant influence on the prognosis of patients with noonan syndrome, especially those having no severe abnormalities in the heart or large vessels.- - - - - - - - - - ranking = 1keywords = brain, cerebral (Clic here for more details about this article) |
2/157. Unexpected death in persons with symptomatic epilepsy due to glial brain tumors: a report of two cases and review of the literature.Two cases of unexpected death in persons with epileptic seizures due to a brain tumor are presented which encompassed an astrocytoma WHO grade II and an anaplastic astrocytoma WHO grade III. A 35-year-old man was found somnolent and disoriented at home. A computed tomography (CT) scan revealed a tumor of the right frontal lobe suggestive for an oligodendroglioma. During an angiographic examination the patient experienced an epileptic seizure. Some weeks later, the man was found dead in front of his house with a fresh bite mark of the tongue. Neuropathological examination revealed an astrocytoma WHO grade II of the right frontal lobe. A 47-year-old man plunged into a swimming-pool and was found submerged some minutes later. After resuscitation he survived comatose for 8 days but finally died due to severe hypoxic brain damage. He had been operated on a brain tumor of the temporal lobe 1 year before the accident. Neuropathological examination revealed residual tumor tissue at the operation site corresponding to an anaplastic astrocytoma WHO grade III. Although rare, death in persons with epileptic seizures due to brain tumors is an important mechanism of death encountered by the forensic pathologist.- - - - - - - - - - ranking = 1.4448714485945keywords = brain (Clic here for more details about this article) |
3/157. Sudden death due to a central neurocytoma.The central neurocytoma is a common, usually intraventricular tumor with bland histologic features. We report a case of a 51-year-old man who died suddenly. At autopsy, a neurocytoma with acute hemorrhage filled the anterior left lateral ventricle. The tumor matrix and surrounding brain tissue contained accumulations of hemosiderin. Previously, 2 cases of central neurocytoma with associated hemorrhage have been reported. Hemorrhage appears to be a serious complication associated with these neoplasms.- - - - - - - - - - ranking = 2.4064443525133keywords = ventricle, intraventricular, neoplasm, brain (Clic here for more details about this article) |
4/157. Sudden death in an infant caused by rupture of a basilar artery aneurysm.Ruptured aneurysms of the cerebrovasculature in infancy and early childhood, except for "giant" aneurysms and arteriovenous malformations, are rare. seizures, loss of consciousness, and apnea are the usual presenting signs in infancy; symptoms such as headache or visual disturbances and signs such as cranial nerve compression or meningeal irritation commonly found in older children or adults are absent in infants. However, the morphologic findings (i.e., subarachnoid and retinal hemorrhage, and occasionally subdural hemorrhage) may be mistaken for inflicted trauma, especially if the aneurysm is not identified. Sudden death caused by rupture of a cerebral aneurysm has not been previously described in an infant. This report outlines the investigation and autopsy findings in a 7-month-old infant who died unexpectedly as a result of rupture of a complex basilar artery aneurysm.- - - - - - - - - - ranking = 0.096955344628452keywords = cerebral (Clic here for more details about this article) |
5/157. Sudden unexpected death associated with HHV-6 in an adolescent with tuberous sclerosis.A 14-year-old female with tuberous sclerosis and history of seizures was found dead in bed at home 3 days after she had been assessed as doing well at a routine neurology clinic appointment. She had been treated with an antiepileptic drug, felbamate, for 36 months and had been seizure-free except for one seizure episode 5 months before death. Postmortem examination revealed cerebral edema, with uncal and tonsillar herniation, and pulmonary edema, consistent with seizure-induced apnea. Multiple microglial nodules with mature perivascular lymphocytic cuffing and diffuse infiltrates were identified around subependymal tuberous sclerosis giant cell nodules. Immunostaining and electron microscopy revealed human herpesvirus-6-infected macrophages, astrocytes, lymphocytes, and endothelial cells in the subependymal tuberous sclerosis lesions and choroid plexus. Subacute human herpesvirus-6 encephalitis is postulated to have precipitated a seizure and thus sudden unexpected death in epilepsy in this otherwise stable adolescent patient.- - - - - - - - - - ranking = 0.096955344628452keywords = cerebral (Clic here for more details about this article) |
6/157. Clinical and pathologic study of two siblings with arrhythmogenic right ventricular cardiomyopathy.ARVC is a cardiomyopathy in which the right ventricular myocardium is replaced by fibroadipose tissue. Males are affected slightly more often than females and, in those cases which are familial, the pattern of inheritance is usually autosomal dominant with incomplete penetrance. We examined the hearts of two sisters, ages 17 and 14, with no family history of heart disease. The older sibling, who was previously considered healthy, died suddenly, while the younger sibling developed congestive heart failure and received a cardiac transplant. An autopsy of the older sibling and examination of the younger sibling's excised heart revealed severe examples of ARVC with minor differences. A thick cap of fibroadipose tissue covered most, if not all, of each right ventricle and was transmural in some areas. Microscopically, lelt ventricles contained extensive myocyte disarray and multifocal fibrosis. The coronary arteries displayed intimal hyperplasia with disruption of the internal elastic lamina, similar to fibromuscular dysplasia. These two cases comprise a unique familial grouping in a polymorphic disease. Despite the male predominance and autosomal dominant inheritance in ARVC, the only members affected in this family were female, and an autosomal dominant pattern of inheritance, even with incomplete penetrance, would be unusual. In addition, we identified changes in the coronary arteries similar to fibromuscular dysplasia and corroborated recently reported changes in the left ventricle of patients with ARVC, providing evidence that this disease, in its most severe form, involves the entire heart.- - - - - - - - - - ranking = 3.0577563799915keywords = ventricle (Clic here for more details about this article) |
7/157. A case of sudden death caused by fibromuscular dysplasia.After sudden unexpected death in a previously healthy 42 year old woman, necropsy examination showed myocardial infarction caused by occlusion of a vital coronary artery by fibromuscular dysplasia. This is a rare arterial disease with a clinical onset usually in the third or fourth decades of life. The aetiology is not fully understood but since it affects vital (cerebral and coronary) blood vessels it often has fatal consequences.- - - - - - - - - - ranking = 0.096955344628452keywords = cerebral (Clic here for more details about this article) |
8/157. Sudden death in a child due to an intrathoracic paraganglioma.A 12-year-old boy under treatment for asthma was found dead in his home. The autopsy revealed a large posterior mediastinal mass that completely compressed the upper lobe of the right lung and the associated airways. This mass extended from the right costovertebral sulcus into the thoracic spinal canal through the spinal foramen and compressed the spinal cord. It was located in the epidural space and was adherent to a nerve root. The histologic and immunocytochemical features were that of a paraganglioma. Although neurogenic tumors are the most common posterior mediastinal masses in the pediatric population, paragangliomas are rare, and spinal involvement has not been described in children. In addition, sudden death has not been reported in association with any of the 13 cases of posterior mediastinal paraganglioma described in the literature as involving the spine. This case illustrates an unusual cause of sudden death in a pediatric patient due to a benign neoplasm.- - - - - - - - - - ranking = 0.30559236273744keywords = neoplasm (Clic here for more details about this article) |
9/157. Sudden death and Wegener's granulomatosis of the pituitary.Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.- - - - - - - - - - ranking = 0.18060893107431keywords = brain (Clic here for more details about this article) |
10/157. Postmortem diagnosis of cerebral malaria.Human cerebral malaria is a frequent encephalopathy that occurs in the endemic tropical-subtropical zones. There are a smaller number of imported cases in continental zones where the diagnosis sometimes remains difficult to establish. Fifteen days after the death of a 36-year-old male French citizen in africa, an investigation to determine the cause of death was conducted. Histologic examination of the brain permitted the diagnosis of cerebral malaria. Because of the popularity of overseas tourism and because this disorder may appear as "sudden death," these victims may be referred to a forensic pathologist. This case demonstrates the role a forensic pathologist may play in determining the cause of death in cerebral malaria.- - - - - - - - - - ranking = 0.85929634347347keywords = brain, cerebral (Clic here for more details about this article) |
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