Contribution of ambulatory EEG recording (Medilog 9000) in a population of epileptics

Thirty-four epileptic patients, aged 9 to 36, were submitted to A/EEG between May 1987 and July 1988. All patients had a thorough clinical and EEG work-up including long-term conventional EEG, afternoon polygraphic sleep recording and, in some cases, full-night EEG and video monitoring. Patients were divided into 2 groups: group I included 19 patients (18 with symptomatic partial epilepsy (SPE) and 1 with idiopathic generalized epilepsy (IGE) in whom no seizure had ever been recorded in spite of EEG recordings averaging a total of 16 hrs 10 min, awake and asleep); group II included 15 subjects (6 with SPE, 5 with IGE, 3 with symptomatic GE and 1 with undetermined epilepsy) in whom one or several seizures had been recorded. A/EEG was performed in order to: 1) obtain better clinical and EEG characterization of seizures, 2) study the circadian distribution of seizures, 3) verify the efficacy of drug treatment and, 4) establish the epileptic or non-epileptic nature of some ictal events. The results of A/EEG were considered positive in 52.63% of group I patients and in 93.33% of group II patients. The authors discuss the specific advantages of A/EEG vs conventional EEG: recording of seizures with random occurrence, of seizures accompanied by falls, checking the remission of seizures.     

Usefulness of PCR Strategies For Early Diagnosis of Chagas'' Disease Reactivation and Treatment Follow-Up in Heart Transplantation

Heart transplantation (HTx) is a useful therapy for end‐stage Chaga? cardiomyopathy; however, Chagas reactivation remains a mayor complication. Parasitological methods offer poor diagnostic sensitivity, and use of more sensitive tools such as the Polymerase chain reaction (PCR) is usually necessary. In the present study, reactivation incidence and PCR usefulness for early reactivation diagnosis, as well as for treatment response evaluation during follow‐up, were analyzed using Strout parasite detection test, in 10 of 222 consecutive HTx patients suffering Chagas cardiomyopathy. PCR strategies targeted to minicircle sequences (kDNA, detection limit 1 parasite/ 10 mL blood) and miniexon genes (SL‐DNA, 200 parasite/10 mL) were performed to compare parasite burdens between samples. No patients received prophylactic antiprotozoal therapy (benznidazole). Five patients (50%) exhibited clinical reactivation within a mean period of 71.6 days; positive Strout results were observed in most cases presenting clinical manifestations. kDNA‐PCR was positive 38–85 days before reactivation, whereas SLDNA‐PCR became positive only 7–21 days later, revealing post‐HTx parasitic load enhancement present prior to clinical reactivation development. Reactivations were successfully treated with benznidazole and generated negative PCR results. Results observed in this study indicate the value of PCR testing for an early diagnosis of Chagas reactivation as well as for monitoring treatment efficacy.     

Cognitive evolution of a girl submitted to right hemispherotomy when five years old

Since the age of three years the patient suffered from early drug-resistant partial epilepsy with electric status during slow sleep, owing to a micropolygyric malformation of the right fronto-temporo-parietal lobes. The hemispherotomy (when five years of age) was followed by immediate and persistent disappearance of the seizures and withdrawal of the treatment. The transfer of right hemispheric functions to the left hemisphere occurred very early; the child’s development was examined in relation to the restoration of these functions and the age at surgery. The early surgical intervention and the plasticity of the brain – along with an intensive cognitive rehabilitation – seem to be important in determining the favorable global cognitive outcome. Visuo-spatial abilities and multi-modal integration of these functions with memory, attention and language have been the most critical domains and are recently in progress. The rapidity of processing complex tasks is particularly lacking. This seems to be the expression of the defective development of the Central Executive System.     

Myocarditis as a form of relapse in two patients with adult Still’s disease

Still’s disease is a subset of juvenile idiopathic arthritis (JIA) that usually presents with intermittent fever, rash, and arthritis. Extra-articular flares can occur several years after disease onset. We report two cases of adult Still’s disease with myocarditis after several years of being in remission. A 34-year-old Caucasian man with history of systemic juvenile arthritis in remission since age 13 was admitted in hospital with 10 days history of fever, odynophagia, and arthralgias. Chest X-ray and cardiac ultrasound showed cardiac enlargement. An endomyocardial biopsy revealed acute myocarditis. He was treated with methylprednisolone and intravenous gammaglobulin, with improvement of his general condition and cardiac parameters. A 16-year-old Caucasian male patient with history of systemic JIA in remission for the last 7 years was admitted with 7 days history of fever, odynophagia, arthralgias, and myalgias. Two days after admission, he developed chest pain and pericardial rubbing was found on examination. Cardiac ultrasound showed left ventricular dilatation with impaired systolic function, and posterior, inferior and apical-septal wall hypokinesia. Blood test showed elevated creatine phosphokinase levels. He was treated with IV methylprednisolone with normal follow-up cardiac ultrasound. Cardiac involvement in patients with systemic JIA can be the first symptom of disease reactivation, even after many years of disease remission.     

Transmantle dysplasia in tuberous sclerosis: clinical features and surgical outcome in four children

In the literature, several malformations of cortical development have been described as additional lesions in tuberous sclerosis complex. Among these lesions, a very large focal cortical dysplasia has peculiar magnetic resonance imaging features: a signal abnormality that extends radially inward toward the lateral ventricle from the pachygyric cortical surface plus a homogeneous clinical picture. Affected patients have early-onset drug-resistant epilepsy and severe developmental delay. We describe the clinical, genetic, neurophysiologic, and neuroradiologic characteristics of four patients affected by tuberous sclerosis and this type of cortical dysplasia these patients are of special interest because they have been operated on for their dysplastic lesions. Total control of seizures has been achieved in the three children who underwent a complete lesionectomy. This result cannot be permanent, however, because of the presence of other cortical tubers which could become epileptogenic. All things considered, our choice was to give these children at least temporary relief from severe epilepsy and possibly support for developmental progression.     

Orthotopic heart transplantation in elderly patients: a 10-year experience at a single center

INTRODUCTION: A consensus has not yet been reached regarding the indications for orthotopic heart transplantation (OHT) in elderly patients or the age limit contraindicating the procedure. The objective of this study was to assess OHT outcomes to determine whether elderly patients benefit from the procedure. METHODS: From February 1993 to February 2003, 178 OHTs were performed on recipients of mean age 47.4 +/- 15 years (range, 4 to 74) including 80.3% men. The population was divided into two groups: group A included patients >/= 60 years, and group B those younger than that age. Survival was analyzed for the overall population and for both age groups during a 10-year follow-up period. RESULTS: Group A included 36 patients (20.8%) and group B 142 patients (79.2%). Mean age was 63.7 +/- 2.9 years (60 to 74) in A, and 43 +/- 13.9 years (4 to 59) in B. In-hospital mortality was significantly higher among group A (n = 11, 31.4%) compared to B (n = 17, 12.1%, P =.008). Survival at 1, 5, and 10 years was 61.5% +/- 8%, 58.1% +/- 8.3%, and 49.8% +/- 10.5% group A; and 84.2% +/- 3%, 73.7% +/- 4.1%, and 69.9% +/- 4.7 for group B. Elderly patients showed a lower survival rate (49.8%) compared with the younger group (69.9%) at 10-year follow-up (P =.007). Conditional survival at 9 years failed to show significant differences (A 72.2% vs B 79.6%, P =.4). CONCLUSION: In our population, elderly recipients showed a higher in-hospital mortality. However, when the first post-OHT year was excluded, we found similar survival rates for both age groups.     

Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease.

Two cases of primary pulmonary artery sarcoma resembling chronic thromboembolic disease features are presented. Tumour identification was achieved after pulmonarv thromboendarterectomy, which was indicated by documentation of a prothrombotic state in both patients. A doubtful history of pulmonary emboli or deep venous thrombosis should alert medical personnel to the possible presence of a primary pulmonary artery sarcoma. Advanced imaging methods such as gadolinium-enhanced magnetic resonance imaging could be useful in considering pulmonary thromboendarterectomy. If a tumour is detected, its surgical resection should be considered with caution, taking into account the poor survival results. Invasion of the adventitia or the right ventricle, as documented in the present cases, is unusual. As far as the present authors know, this is the first report of this kind of tumour and its coexistence with an activated protein C resistance state and type II heparin-induced thrombocytopenia.     

Clinical predictors of chronic chagasic myocarditis progression

INTRODUCTION AND OBJECTIVES: Previous prognostic studies of Chagas' disease have focused on mortality associated with end-stage cardiopathy (i.e., heart failure). Our aim was to identify indicators of progression in early-stage Chagas' heart disease. MATERIAL AND METHOD: The study included 856 patients with 3 positive anti-Trypanosoma cruzi test results. Those with heart failure were excluded. Patients were divided into 3 clinical groups: those without heart disease (Group I); those with heart disease but without left ventricular enlargement (Group II); and those with left ventricular enlargement but without heart failure (Group III). The endpoint was progression to a more severe clinical stage or death due to cardiovascular disease. A Cox regression model was used to derive a clinical risk score from clinical, electrocardiographic and echocardiographic variables. RESULTS: At study entry, the patients' mean age was 43.7 years. They were followed up for a mean of 8 years. The following were predictors of heart disease progression: age at entry (HR=1.05; 95% CI, 1.02-1.07; P<.001), left ventricular systolic diameter (HR=1.06; 95% CI, 1.04-1.09; P<.001), intraventricular conduction abnormalities (HR=1.85; 95% CI, 1.02-3.36; P=.04), and sustained ventricular tachycardia (HR=3.97; 95% CI, 1.65-9.58; P=.002). Treatment with benznidazole reduced the risk of progression (HR=0.40; 95% CI, 0.23-0.72; P=.002). The devised clinical risk score was effective in stratifying the likelihood of cardiopathy progression. CONCLUSIONS: Specific clinical indicators and a derived clinical risk score can be used to predict the progression of chronic chagasic myocarditis in patients without heart failure.