Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid.

Bullous pemphigoid (BP) is an autoimmune bullous disease, associated with autoantibodies directed against the hemidesmosomal components BP180 and BP230. In this study for the first time different laboratories have analyzed the autoantibody profile in the same group of 49 prospectively recruited BP patients. The results show that: 1) disease severity and activity correlated with levels of IgG against the BP180-NC16A domain, but also against a COOH-terminal epitope of BP180, 2) distinct epitopes of the BP180 ectodomain other than BP180-NC16A were recognized by 96% of the BP sera; and 3) the combined use of BP180 and BP230 ELISA led to the detection of IgG autoantibodies in all the BP sera. These results demonstrate the usefulness of the combined ELISAs based on various BP180 and BP230 fragments in establishing the diagnosis of BP and support the concept that BP180 is the major autoantigen of BP.

Exercise release of cardiac natriuretic peptides is markedly enhanced when patients with coronary artery disease are treated medically by beta-blockers.

OBJECTIVES: This study sought to identify determinants of the exercise rise in plasma levels of cardiac natriuretic peptides (NPs) in patients with coronary artery disease (CAD). BACKGROUND: During stress, there is a variable rise in the plasma level of NPs, but this rise frequently reaches levels that are known to lower the cardiac load and that thus might be beneficial to CAD patients. METHODS: Plasma venous concentrations of atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) were determined at rest and peak exercise in 104 patients with chronic CAD who were referred to exercise thallium-201 ((201)Tl) single-photon emission computed tomography (SPECT) and radionuclide angiography. RESULTS: The extent of scarred myocardium by (201)Tl-SPECT and patient age were the best independent predictors of NP concentrations at rest, but also of increases in NP concentration during exercise (all p < 0.001). Moreover, beta-blocking treatment was an additional and strong independent predictor of the increase in NP concentrations at exercise (p < 0.001 for ANP; p = 0.001 for BNP). On average, exercise increases in NP concentrations were more than twice as high in patients with (n = 55) than in those without (n = 49) beta-blocker treatment (ANP: +49 +/- 63 vs. +22 +/- 25 ng/l, p = 0.01; BNP: +24 +/- 5 vs. +11 +/- 15 ng/l, p = 0.04), whereas NP concentrations at rest were equivalent in the two groups (ANP: 34 +/- 34 vs. 30 +/- 33 ng/l, p = NS; BNP: 85 +/- 152 vs. 57 +/- 101 ng/l, p = NS). CONCLUSIONS: Patients with chronic CAD exhibit much higher exercise releases of ANP and BNP when they are treated with beta-blockers. This enhanced secretion of potent vasodilating and natriuretic agents constitutes an original therapeutic mechanism for further protecting diseased hearts against stress.

Ictal SPECT in a case of pure musicogenic epilepsy.

A 39-year-old, right-handed woman had seizures for two years which were always triggered by exposure to various types of music: the first occurred while she listened to a tune she particularly liked, Con Te Partiro, by Andrea Boccelli. Other triggering factors were various types of music such as supermarket background music and polyphonic singing or instrumental music played by family members. The seizures had a stereotyped course: she felt anxious, tearful, then occurred slight obtundation, during which she smacked her lips and moved restlessly. There was no complete loss of consciousness, but some degree of amnesia. She never experienced a generalized tonic-clonic seizure, but reported rare spontaneous feelings of déjà-vu that had begun at the same time as the induced seizures. There were no other spontaneous attacks; only one seizure was apparently provoked, not by music but by a loud background noise in her office. She was a music lover and a singer. Interictal EEG showed independent slow waves over the temporal regions. Several seizures with EEG localisation over the right temporal region were elicited after several minutes of exposure to music. Monoauricular stimulation with the same music produced a seizure when applied to the left ear but was ineffective when applied to the right ear. Ictal SPECT demonstrated right temporal hyperperfusion. MRI was normal. On high dose of carbamazepine, seizure frequency decreased. The addition of topiramate resulted in full seizure control. Musicogenic epilepsy is a rare form of reflex epilepsy. Pure cases, when patients do not experience unprovoked seizures, are exceptional. Our report confirms the implication of the right temporal lobe in this epilepsy.

Nonvisualization of axillary sentinel node during lymphoscintigraphy: is there a pathologic significance in breast cancer?

UNLABELLED: The aim of this study was to define the factors associated with nonvisualization of a sentinel node (SN) in the axilla area during preoperative lymphoscintigraphy. METHODS: We retrospectively studied 332 women with T0, T1, or T2 <3-cm, N0 invasive breast cancer who underwent a sentinel lymph node biopsy procedure. All patients had intradermal and intraparenchymal injection of 37 MBq (99m)Tc-sulfur colloid in a total volume of 4 x 0.1 mL, above and around the tumor. Anterior and lateral static views were obtained a few minutes and 2-4 h after injection. Surgery was performed the next day. The SNs were localized intraoperatively with the aid of patent blue dye and using a hand-held gamma-probe. SNs were analyzed by serial sections stained with hematoxylin-eosin, with the adjacent section stained with anticytokeratin antibodies. Different parameters, such as the number of positive lymph nodes, presence of lymphovascular invasion, tumor size, tumor grade, histology (invasive vs. in situ), prior excisional biopsy, and patient age were analyzed to determine whether they had any significant correlation with nonvisualization of SNs in the axillary area. RESULTS: An axillary SN was successfully visualized on the preoperative lymphoscintigraphy in 302 of 332 patients (90.7%). No axillary drainage was found in 30 patients on the delayed images, even after a second injection of radiocolloid, and 5 of 30 patients showed uptake outside the axillary area. Positive nodes were identified in 86 of 302 patients (28.5%) with successful axillary drainage and in 19 of 30 patients (63.3%) with unsuccessful axillary drainage. More than 4 invaded axillary nodes (P < 0.0001) and the presence of lymphovascular invasion in the breast tumor (P = 0.004) were the only significant variables on univariate analysis, although multivariate analysis showed that only the increased number of invaded nodes was statistically significant. CONCLUSION: Patients with unsuccessful axillary mapping have an increased risk for axillary involvement.

Value of (99m)Tc-ECD SPET for the diagnosis of dementia with Lewy bodies.

Despite improved diagnostic accuracy, differentiation of dementia with Lewy bodies (DLB) and Alzheimer's disease (AD) on the basis of clinical findings remains problematic. The purpose of this retrospective study was to evaluate the utility of technetium-99m ethyl cysteinate dimer (ECD) single-photon emission tomography (SPET) as a potential tool for the diagnosis of DLB and discrimination from AD. Cerebral perfusion patterns detected by (99m)Tc-ECD SPET were compared in patients presenting with a probable diagnosis of DLB ( n=34) or AD ( n=28). Tracer distribution was quantified using the region of interest technique in eight symmetrical paired zones and expressed as a perfusion index (ratio of mean uptake in a brain region to that in the cerebellum). Comparison of findings in the DLB and AD groups demonstrated significant differences in mean perfusion indexes in the right occipital region ( P=0.004), left occipital region ( P=0.005) and left medial temporal region ( P=0.013). Mean perfusion indexes in the right and left occipital regions were lower in DLB than in AD patients. Conversely, the mean perfusion index in the left medial temporal region was lower in AD than in DLB patients. DLB was correctly identified in 22 patients (sensitivity, 65%) while AD was correctly identified in 20 patients (specificity, 71%). In the DLB group, right and left occipital perfusion indexes were 0.95 or more in all eight non-hallucinating patients, and bilateral occipital hypoperfusion was observed in 15 of the 26 patients with visual hallucinations (57.7%). To our knowledge, this is the first study in which (99m)Tc-ECD SPET has been used exclusively for the diagnosis of DLB. The results suggest that brain perfusion scintigraphy could be helpful in distinguishing DLB from AD if diagnosis based on clinical criteria alone is difficult. The findings also support a link between visual hallucinations and structural/functional changes in the occipital region in DLB patients.