Bickerstaff Brainstem Encephalitis and overlapping Guillain-Barré syndrome in children: Report of two cases and review of the literature.

Bickerstaff Brainstem Encephalitis (BBE) is a rare autoimmune encephalitis, characterized by acute ophthalmoplegia, ataxia and altered state of consciousness. Together with Guillan-Barrè Syndrome (GBS) and Miller-Fisher Syndrome, it forms a spectrum of post-infectious demyelinating diseases. Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on pediatric population. We reviewed all cases of pediatric BBE in the literature, to determine if any patient showed features suggestive for BBE/GBS. Data analysis focused on the diagnostic tests performed (e.g. anti-GQ1b antibodies), neuroimaging and nerve conduction studies (NCS). Further attention was given to the therapeutic management and to patients' outcome. We additionally present two previously unreported pediatric cases. Our review retrieved 19 cases of BBE/GBS, only 2 of which were originally and correctly diagnosed by the authors. The prevalence was higher in male subjects (ratio 3:1) and median age at diagnosis was 8 years. Anti-GQ1b were positive in 46% of the patients, while NCS were altered in 64%. Only 25% of the patients that underwent brain MRI showed abnormal findings. The incidence of BBE/GBS has been underrated in the past, mostly due to an underestimation of the PNS involvement. We therefore suggest to investigate all patients with a clinical picture suggestive of BBE/GBS through electroencephalogram, NCS, brain and spine MRI in order to promptly achieve the correct diagnosis.

Cardiac troponin elevations in chronic renal failure: prevalence and clinical significance.

OBJECTIVES: The aim of the study was investigate the prevalence of abnormal values of cardiac troponin T (cTnT) and cardiac troponin I (cTnI) in patients with chronic renal failure (CRF) and their clinical significance. DESIGN AND METHODS: We investigated the concentrations of cTnT and cTnI in 49 CRF patients without heart disease or diabetes. Cardiac TnT values were measured with a second generation immunoassay and cTnI with two immunoassays with different analytical sensitivity. All CRF patients underwent regular clinical follow-up over a 18-month period. RESULTS: No patients with CRF had elevated values of cTnI when measured with one assay and only 2 patients displayed minimally elevated values with the second assay. In contrast, 23 CRF patients (47%) displayed cTnT concentrations elevated above the upper reference limit. The elevated cTnT values observed were below the values detected in acute myocardial infarction and were not associated with adverse cardiac events during follow-up. CONCLUSIONS: Mildly elevated cTnT concentrations are common in patients with CRF and do not appear to be associated with adverse coronary events.

Alcoholization of the interventricular septum (Sigwart procedure) for treatment of hypertrophic cardiomyopathy.

A patient with NYHA Class III dyspnea under medical treatment for hypertrophic cardiomyopathy underwent alcoholization of the first septal perforator. The procedure induced complete disappearance of the intraventricular gradient, with development of a Q-wave anterior myocardial infarction (peak CK rise = 1645 IU) and a complete right bundle branch block. After 5 months the patient maintains a marked improvement in functional capacity (NYHA Class I).

[Cardiac troponin I and T in unstable angina: incidence, correlation, kinetics of release and prognostic value]. / Troponina i cardiaca e troponina T cardiaca nell'angina instabile: incidenza, correlazione, cinetica di rilasciamento e valore prognostico.

BACKGROUND: The aim of this study was to ascertain the incidence of altered serum cardiac Troponin-T (cTnT) and cardiac Troponin I (cTnI) in patients with unstable angina, the concordance between findings for the two proteins, their release kinetics and their utility in predicting coronary events. METHODS: We studied 32 consecutive patients (pts) admitted to the Coronary Unit with a diagnosis of unstable angina; following Braunwald classification criteria, 5 pts were in class I, 4 class II, 23 class III. A blood sample was taken on admission to hospital and subsequently every 8 hours for two days, a total of 7 samples being obtained per pt. Cardiac-TnT values ranging from 0-0.17 mugr/L (Boehringer Mannheim) were considered normal, as were cTnI values ranging from 0 to 0.7 mugr/L (Stratus-Dade). RESULTS: Among 218 samples, altered cTnT values (0.18-0.68 mugr/L) were found in 19 (3 pts), and 13 of these samples were positive for cTnI (0.8-5.5 mugr/L), while the remaining 6 showed borderline values for cTnI (0.5-0.7 mugr/L). No cTnT negative samples were found to be positive for cTnI. The release kinetics of cTnT and cTnI were comparable in all three cases, with a "plateau" pattern, unlike the kinetics in the course of acute myocardial infarction (AMI). The mean follow-up was 13 months on average (range 1-19). In two pts with altered cTnT and cTnI values, symptoms were controlled with medical therapy, while the remaining patient failed to respond to medical therapy and therefore underwent PTCA. Fifteen months later, they are alive and have not had myocardial infarction. Of the 29 pts with normal cTnT and cTnI values, three developed AMI, which in two cases was fatal. Seven pts were submitted PTCA, seven to aorto-coronary bypass surgery, two were subsequently rehospitalized for a recurrent angina symptoms. In 13 pts complete control of symptoms was achieved with medical therapy. CONCLUSIONS: Our findings demonstrate that the incidence of altered cTnT and cTnI values in pts with unstable angina is low; there is close agreement between findings for the two proteins; in cases of angina, the cTnT and cTnI release kinetics are different from those in AMI. The finding of altered cTnT and cTnI values in the serum of our pts with unstable angina does not appear to be of prognostic value for future coronary events.

[Cardiac troponin I in unstable angina]. / La troponina I cardiaca nell'angina instabile.

BACKGROUND: Unstable angina implies high risk of myocardial infarction and sudden death. Increased levels of cytoplasmatic enzymes and proteins (creatine phosphokinase, MB creatine phosphokinase troponin T, etc.) were described in unstable angina, providing information about incoming major coronary events. Cardiac troponin I (cTn-I) is a structural protein inhibiting the actinomyosine ATPase; it is only found in myocardial cells. Serum titration of cTn-I has been recently introduced into clinical practice as a sensitive and specific marker of myocardial cellular necrosis. OBJECTIVES: The aim of our prospective study was to assess the presence of cTn-I in blood samples of patients with unstable angina and no signs of myocardial necrosis. Furthermore we intended to test the possible use of cTn-I in unstable angina as a prognostic marker of major coronary events on short and middle term. METHODS: We studied 33 consecutive patients admitted to our CCU for unstable angina. According to unstable angina Braunwald's classification, 6 patients were included in the first class, 4 patients in the second class and 23 patients in the third class. We excluded patients with acute or recent myocardial infarction. Blood samples of all patients were obtained at the time of CCU admission and every eight hours in the first and second day. Serum cTn-I titration was performed with the sandwich immunoenzymometric method, recently introduced by Diagnostic Pasteur. Two months follow-up was carried out in order to survey major coronary events and revascularization procedures, either angioplasty or coronary artery bypass surgery. RESULTS: No patients with unstable angina exhibited cTn-I in their blood samples; accordingly, cTn-I was not found in the first blood sample of a patient who underwent myocardial infarction during hospitalization. During the follow-up 2 patients died of myocardial infarction, 9 patients had surgical revascularization and 5 patients angioplasty. CONCLUSIONS: CTn-I is a sensitive and specific marker of myocardial necrosis. It is not found in patients with unstable angina; therefore it has no role as a prognostic marker of major coronary events.

[The effect of gallopamil on myocardial perfusion in angina of effort]. / Influenza del gallopamil sulla perfusione miocardica nell'angina da sforzo.

To value stress tolerance and stress myocardial perfusion before and after a week of oral therapy with gallopamil 150 mg daily, we studied 10 patients suffering from stable effort angina. We performed bicycle exercise stress testing and thallium scintigraphy (Tl) with planar technique in 3 projections (anterior-posterior and oblique left anterior at 45 and 70 degrees) according to the current standards. We valued systolic and diastolic blood pressure (SBP-DBP), heart rate (HR) and HR-SBP product at rest, at symptoms stress-induced and at the end of the procedure. Moreover we valued work threshold of chest discomfort and ischemia, the maximal work capacity and the perfusion defects according to a Tl score obtained dividing the 3 projections in 5 segments and fixing a value according to the observed perfusion from 0 = normal perfusion to 3 absent perfusion. We observed a significant reduction of basal HR (77 vs 71, p = 0.05), SBP (147 +/- 15 vs 131 +/- 15 mmHg, p = 0.001), DBP (91 +/- 6 vs 83 +/- 6 mmHg, p = 0.002). Work threshold of chest discomfort and ischemia significantly arose (8 +/- 3 vs 11 +/- 4 min., p = 0.002; 6 +/- 3 vs 10 +/- 4 min., p = 0.001). The HR-SBP product at the maximal work capacity and the Tl score significant decreased (31650 +/- 6239 vs 29406 +/- 5418, p = 0.003; 8 +/- 2 vs 5 +/- 1, p = 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

[Use of myocardial thallium-201 scintigraphy in the evaluation of infarct area]. / Utilizzo della scintigrafia miocardica con Tallio-201 nella valutazione dell'area infartuale.

In twenty-four patients admitted of the Coronary Care Unit for myocardial infarction, without signs of previous necrosis and uncomplicated course, amplitude of infarct size was estimated by 201-Thallium perfusion scintigraphy. The obtained results were compared with residual ventricular function (i.e. angiographic ejection fraction) and with other methods of fibrotic area evaluation such as electrocardiographic score and CPK and CK-MB dismission curve. Concordance was observed among: scintigraphy and ejection fraction, scintigraphy and electrocardiographic score, scintigraphy and CPK peak.