1/100. Adrenocortical insufficiency.Primary adrenocortical insufficiency is a rare disease which may present with protean clinical symptoms and signs. At one end of the spectrum is the patient with a long clinical history with marked pigmentation and documented episodes of crises which may follow relatively minor viral illnesses. The other extreme is illustrated by patients with a rapid deterioration in health culminating in unexpected or unexplained death. In the latter instances, the forensic pathologist may well be involved, and the case will present a significant challenge to diagnosis. We report 5 cases of primary and secondary adrenocortical insufficiency seen at the Victorian Institute of forensic medicine in 1 year. One adult man presented as a case of sudden unexplained death. In 2 cases, the diagnosis of adrenocortical insufficiency was raised following postmortem examination and confirmed by the treating physician following further consultation. In the remaining 2 cases, the diagnosis was known prior to postmortem examination and the diagnosis confirmed.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
2/100. andersen syndrome autosomal dominant in three generations.andersen syndrome is a rare entity and comprises potassium sensitive periodic paralysis, ventricular arrhythmia, and an unusual facial appearance; syncope and sudden death have also been reported. The recognition of the characteristic face permits an early diagnosis in order to detect the severe systemic manifestations that are associated with this syndrome. The genetic defect is not linked to any other form of potassium sensitive periodic paralysis nor is it related to that of the long qt syndrome; nevertheless, a prolonged QT interval can be detected in a significant proportion of the cases. Sixteen cases of this syndrome have been described. We report on a three-generation family with 10 affected members. To our knowledge, this is the largest number of cases reported in one family. We noted some additional minor anomalies such as broad forehead and malar hypoplasia. Our patients had variable expression in the classical triad and of the severity of the systemic manifestations. Five of 8 affected studied members did not have a long QTc, which has been suggested as a constant finding in this syndrome.- - - - - - - - - - ranking = 3keywords = long (Clic here for more details about this article) |
3/100. oxygen desaturations triggered by partial seizures: implications for cardiopulmonary instability in epilepsy.PURPOSE: The occurrence of hypoxemia in adults with partial seizures has not been systematically explored. Our aim was to study in detail the temporal dynamics of this specific type of ictal-associated hypoxemia. methods: During long-term video/EEG monitoring (LTM), patients underwent monitoring of oxygen saturation using a digital Spo2 (pulse oximeter) transducer. Six patients (nine seizures) were identified with oxygen desaturations after the onset of partial seizure activity. RESULTS: Complex partial seizures originated from both left and right temporal lobes. Mean seizure duration ( /-SD) was 73 /- 18 s. Mean Spo2 desaturation duration was 76 /- 19 s. The onset of oxygen desaturation followed seizure onset with a mean delay of 43 /- 16 s. Mean ( /-SD) Spo2 nadir was 83 /- 5% (range, 77-91%), occurring an average of 35 /- 12 s after the onset of the desaturation. One seizure was associated with prolonged and recurrent Spo2 desaturations. CONCLUSIONS: Partial seizures may be associated with prominent oxygen desaturations. The comparable duration of each seizure and its subsequent desaturation suggests a close mechanistic (possibly causal) relation. Spo2 monitoring provides an added means for seizure detection that may increase LTM yield. These observations also raise the possibility that ictal ventilatory dysfunction could play a role in certain cases of sudden unexpected death in epilepsy in adults with partial seizures.- - - - - - - - - - ranking = 2keywords = long (Clic here for more details about this article) |
4/100. Control of idiopathic ventricular fibrillation by implantable cardioverter-defibrillator in a child who survived sudden death.Idiopathic ventricular fibrillation (VF) is extremely rare in children who have not previously undergone cardiac surgery. patients resuscitated from idiopathic VF remain at risk for recurrence. The use of an implantable cardioverter-defibrillator (ICD) effectively prevents such recurrences. We report the case of a 12-year-old girl who had a history of recurrent syncope and had survived an episode of VF. Serial studies after prolonged but successful resuscitation, including echocardiography, an electrocardiogram (ECG), and coronary angiography failed to reveal abnormal cardiac structures responsible for VF. No abnormal conduction pathways or abnormal early or late after depolarization were found on electrophysiologic study. The ST segments of the 12-lead ECG remained normal after procainamide challenge. The patient underwent ICD implantation 2 weeks after admission and syncope did not recur during a follow-up of 14 months. This report emphasizes that idiopathic VF may be responsible for syncope in children. ICD therapy prevents the recurrence of idiopathic VF and the associated risk of sudden death.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
5/100. Morphometric study of nucleus ambiguus in multiple system atrophy presenting with vocal cord abductor paralysis.AIM: To identify lesions responsible for vocal cord abductor paralysis (VCAP) in multiple system atrophy (MSA), we performed a morphometric study of the nucleus ambiguus which innervates the intrinsic laryngeal muscles. methods: Two autopsied cases of MSA presenting with VCAP and one control were examined. Both cases of MSA showed selective neurogenic atrophy of the posterior cricoarytenoid muscles among the intrinsic laryngeal muscles, while no abnormalities were seen in the control. From a block of the medulla oblongata, sections 10 microm thickness were cut serially without spacing and stained with cresyl violet. The ambiguus neurons were counted in all the sections to make a histogram. RESULTS: In the control case, ambiguus neurons showed densely populated areas and sparsely populated areas alternately with significant difference in the mean neuronal density between two areas. In MSA, ambiguus neurons were significantly decreased in number at all levels. It indicates that the neurogenic atrophy of the posterior cricoarytenoid muscle is derived from the neuronal loss of the nucleus ambiguus. CONCLUSION: Though it has still been controversial whether or not the ambiguus neurons are decreased in number in MSA with VCAP, we speculated possible reasons for the disagreement on the involvement of the nucleus ambiguus as follows: different mechanism of VCAP are playing role, and histometric data have been disturbed by factors such as split-cell counting error and marked variation in the distribution of the ambiguus neurons.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
6/100. Sudden death from pelvic hemorrhage after bilateral central fracture dislocations of the hip due to an epileptic seizure.Fracture and dislocation of major joints may be caused by the forceful tonic muscular contractions of seizure activity. A 77-year-old man who was found dead in bed with no sign of external trauma had bilateral central fracture dislocations of the femoral head through the acetabular floor with fatal pelvic hemorrhage and extensive pulmonary fat and bone marrow embolism. He had epilepsy, but the last seizure was 6 years earlier, and he had long discontinued medication. The fractures were attributed to a new unwitnessed seizure. This is the twentieth case of central fracture dislocation of the hip since 1970, when better anesthesia eliminated convulsive therapy-induced fractures. The authors review these 20 cases. seizures followed inflammation, infarction or neoplasia of the brain, eclampsia, metabolic or iatrogenic causes, or epilepsy (6 cases, 2 of which had no prior seizures for 5 years). There were 11 men (mean age, 64 years) and 9 women (mean age, 47 years). Fractures were unilateral in 13 and bilateral in 7. Additional fractures (in vertebrae, shoulders, or femur) were present in eight. Only eight had prior bone disease. Local symptoms led to diagnosis in most, but two were identified incidentally on imaging. The current patient was the only one to die suddenly, but six other patients presented with shock and three died (one of whom had injuries that led to a suspicion of manslaughter). Central fracture-dislocation of the hip is a rare and little known consequence of seizures, with strong potential for misdiagnosis and lethal complications.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
7/100. death of a psychiatric patient during physical restraint. Excited delirium--a case report.We report the case of a young man with a diagnosis of paranoid schizophrenia and multiple drug abuse who died in hospital following a period of prolonged physical restraint. The literature is reviewed, possible factors contributing to death discussed and measures which may reduce the incidence of such deaths in the future highlighted.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
8/100. Hepatic abscess formation and unexpected death: a delayed complication of occult intraabdominal foreign body.A case of unexpected death in a 65-year-old woman, caused by a migrating foreign body that resulted in multifocal hepatic abscesses, is reported. The foreign body was subsequently identified as a portion of chicken fibula. The prolonged time course of the illness, with relatively nonspecific symptoms and signs, resulted in establishment of the diagnosis only at autopsy.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
9/100. Sudden death due to rupture of an omental metastatic tumor arising from cardiac angiosarcoma. A case report.A 32-year-old man underwent surgical excision of a malignant hemangioendothelioma of the heart and received multidisciplinary treatment. Thirty-three months later, he underwent a second surgical treatment for a recurrent tumor of the posterior chest wall of the right thorax. Five months after this surgery (thirty-eight months after the initial surgery), a second recurrent tumor in the right thorax developed. Although radiotherapy and recombinant interleukin-2 were administered, anemia of unknown origin (hemoglobin 6.7 g/dl) developed. The patient died of sudden shock due to a rupture of an omental metastatic tumor. The patient survived for 41 months after the first surgical resection. We present this case because it is the first reported case of sudden death due to bleeding from an omental metastatic tumor, and because our patient was the second-longest survivor after surgical treatment for cardiac angiosarcoma.- - - - - - - - - - ranking = 1keywords = long (Clic here for more details about this article) |
10/100. Familial qt prolongation and risk of sudden death.Two sisters with the syndrome of familial QT prolongation in the ECG and syncope are presented. A recently suggested mechanism of the syndrome is presented and preventive measures to reduce the risk of sudden death associated with it are proposed.- - - - - - - - - - ranking = 333.26415882319keywords = qt, long (Clic here for more details about this article) |
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