Myocardial function reclassification: Echocardiographic strain patterns in patients with chronic Chagas cardiomyopathy and intraventricular dyssynchrony.

BACKGROUND: We aimed to identify, among Chronic Chagas Cardiomyopathy (CCC) patients with left ventricular dysfunction (LVD) and non-left bundle branch block (non-LBBB), subgroups with different functional and mechanical patterns of global longitudinal strain (GLS) and intraventricular dyssynchrony (IVD) at rest and after exercise stress test, and reclassify them using a new echocardiographic approach. METHODOLOGY: In this single-center cross-sectional study, 40 patients with CCC, left ventricular ejection fraction (LVEF) ≤ 35% and non-LBBB underwent rest echocardiography and then treadmill exercise stress echocardiography with GLS and IVD analysis. The sample was divided into four groups, based on GLS and IVD significant variation between rest and exercise: GLS + IVD+ (9 patients); GLS + IVD- (9 patients); GLS-IVD+ (10 patients); GLS-IVD- (10 patients). RESULTS: At rest, median LVEF was 28% (21.3%-33%) and GLS (-7% (-5%/-9.3%), were not different among groups. The average response of GLS was an increase of 0.74% over rest values, and the average response of IVD was a decrease of 6.9 ms. Group GLS-IVD+ presented more dyssynchrony at rest (p = 0.01). Left atrial (LA) volume (higher in GLS-IVD-) (p = 0.022) and TAPSE (higher in GLS + IVD+) (p = 0.015) were also different among groups at baseline. Of the 40 patients evaluated, 27 (67.5%) had very severe LVD (GLS < -8%). In addition, among these patients, 11 patients had contractile reserve after undergoing stress echocardiography. CONCLUSIONS: In patients with CCC, severe LVD and non-LBBB, the evaluation of GLS and IVD between rest and exercise was able to reclassify myocardial function and to identify subgroups with contractile reserve and significant dyssynchronopathy.

Cerebral vasoreactivity is influenced by the prandial state among migraineurs.

OBJECTIVE: To test if the cerebral vasoreactivity among migraineurs is influenced by the prandial state. DESIGN AND METHODS: Eight patients with migraine without aura were studied (mean age = 23.75 years; range = 19 to 32 years; 6 women). We also studied 8 healthy controls (mean age = 20.63 years; range = 18 to 22 years; 3 women), with no history of migraine. Cerebral vasoreactivity was measured on the right side by the breath holding index (BHI) using an EZ-Dop transcranial Doppler instrument, with a 2-MHz transducer fitted on a headband. Serum glucose levels were measured after a 10-hour fasting period and then 10 minutes after a standardized breakfast. RESULTS: In both groups, we found a postprandial (PP) glycemia enhancement (P < .02). Migraineurs showed enhanced PP cerebral vasoreactivity when compared to the fasting state (FS) (BHI-PP = 1.46; BHI-FS = 1.16; P= .02). Among controls, we found a trade to enhancement, but without statistic significance (BHI-PP = 1.28; BHI-FS = 1.11; P= .30). Glucose levels were not significantly correlated to cerebral vasoreactivity in any of the groups (P > .05). CONCLUSION: Our findings suggest that migraineurs have a higher reactivity to hypercapnia during the PP period.

Prevenção secundária da morte súbita: importância do marcapasso definitivo prévio ao implante de cardioversor-desfibrilador implantável (CDI) na sobrevida de pacientes com miocardiopatia chagásica / The relevance of previous permanent pacemaker implant in the survival actuarial curve of chagaapóstrofos ICD patients

Objetivo: Avaliar a importância clínica da presença de marcapasso definitivo (MPD) previamente ao implante de CDI de nossa instituição, foram selecionados 275 submetidos a implante de CDI para prevenção secundária de morte súbita cardíaca (MSC), agrupados de acordo com a cardiomiopatia de base e a presença de MPD prévio ao implante de CDI. As variáveis analisadas foram: sexo, idade, CF-NYHAA, medicações, ritmo cardíaco, FEVE e TVNS. Para análise estatística, utilizou-se o método de Kaplan Meier e o teste de log-rank. Resultados: A amostra reduzida de pacientes com consistentes...

Lesion lateralization in patients with epilepsy and precocious destructive insults.

Destructive brain lesions that occur early in development are often unilateral or asymmetric. We analyzed the lateralization of lesions among a previously reported series of 51 patients with three kinds of destructive lesions based on their topography: specific arterial territory (AT), arterial borderzone territory (Bdz), hemispheric (H). Five patients (all from group Bdz) had bilateral nonlateralizing lesions. The distributions of left- and right-sided lesions were distinct among the groups (P=0.014): in group H, all patients except one presented with right-sided lesions (89%); in group Bdz, left-sided lesions (53%) were more frequent than right-sided lesions (17.7%); in group AT, left- and right-sided lesions were more equally distributed (56 and 44%). Our study suggests that there is a trend toward lesion lateralization among patients with different patterns of precocious destructive lesions. Differences in cerebral maturation and vulnerability between the hemispheres is a possible factor explaining lesion lateralization in early life insults.

Epilepsy and destructive brain insults in early life: a topographical classification on the basis of MRI findings.

Destructive insults of early development can lead to a wide variety of lesional patterns and are a well known cause of epilepsy. The aim of this study is to present a topographic magnetic resonance imaging (MRI) classification of these lesions in adult patients with epilepsy. Thirty-three consecutive patients were divided in three groups according to the topographic distribution of their lesion on MRI: hemispheric (H, n = 7); main arterial territory (AT, n = 18); arterial borderzone (Bdz, n = 8). We analyzed clinical, MRI and magnetic resonance angiography (MRA) data. Status epilepticus (SE) during childhood was more common in group H (7/7) than in the groups AT (1/18) and Bdz (0/8) (P < 0.001). MRA pattern of impaired flow signal in the distal segments of all three major arteries in the affected hemisphere was present in 85.7% of group H patients, and was exclusive to this group. 88.8% (16/18) of patients from group AT presented congenital motor deficit, in contrast to 37.5% (3/8) of group Bdz, and in none of group H (P < 0.001). All patients with Bdz lesions had antecedent of fetal distress, in contrast to 1/7 from group H and 5/18 of group AT (P = 0.001). The MRAs of patients with Bdz lesions were often normal except in those with larger lesions. Our data suggest that in adult patients with epilepsy due to precocious destructive brain insults, a MRI topographical classification distributes them in relatively homogenous clinical groups.

Bases genéticas das cardiomiopatias associadas ou não ao distúrbio da condução intracardíaca / Genetic bases of cardiomyopathies associated or not at the intracardiac conduction disturbances

Os distúrbios do sistema de condução intracardíaco incluem as bradiarritmias e os bloqueios intraventriculares. As causas desse distúrbio são, na maioria dos casos, degenerativas e/ou inflamatórias por cardiopatia adquirida. Raramente, esses distúrbios são congênitos. O conhecimento de múltiplos defeitos genéticos tem permitido o reconhecimento da correlação desses defeitos com cardiomiopatia associada ou não aos distúrbios do sistema de condução intracardíaco. As alterações iônicas e celulares que interagem com o miocárdio heterogêneo também se manifestam ao eletrocardiograma de superfície. Mutações nos genes dos canais de sódio que causam um desvio na curva de ascensão do potencial de ação promovem lentificação da condução do impulso cardíaco, como no bloqueio cardíaco familiar progressivo. Embora a maioria dos casos de cardiomiopatia dilatada seja adquirida, pelo menos 30 por cento são de transmissão hereditária, autossômica dominante, recessiva, ligada ao X ou mitocondrial, podendo afetar recém-natos, crianças, adultos e idosos. Nessas condições, morte súbita pode ser causada por arritmia ventricular e também por bradiarritmia. Síndromes miotônicas hereditárias podem evoluir com desordem cardíaca e algumas delas com especial acometimento do ritmo, como nas doenças de Steiner, Becker, Emery-Dreifuss e Kearns Sayre. O reconhecimento de tais condições hereditárias é de fundamental importância, pois a principal abordagem terapêutica, no momento adequado da evolução, é a estimulação cardíaca artificial permanente, evitando-se dessa maneira, uma morte súbita. Neste capítulo são revisadas as condições genéticas relacionadas aos distúrbios da condução e do ritmo cardíaco e discutidas as mutações genéticas específicas

Lateralization of epileptiform discharges in patients with epilepsy and precocious destructive brain insults.

Unilateral destructive brain lesions of early development can result in compensatory thickening of the ipsilateral cranial vault. The aim of this study was to determine the frequency of these bone changes among patients with epilepsy and precocious destructive lesions, and whether a relationship exists between these changes and epileptiform discharges lateralization. Fifty-one patients had their ictal / interictal scalp EEG and skull thickness symmetry on MRI analyzed. Patients were divided into three main groups according to the topographic distribution of the lesion on the MRI: hemispheric (H) (n=9); main arterial territory (AT) (n=25); arterial borderzone (Bdz) (n=17). The EEG background activity was abnormal in 26 patients and were more frequent among patients of group H (p= 0.044). Thickening of the skull was more frequent among patients of group H (p= 0.004). Five patients (9.8%) showed discordant lateralization between epileptiform discharges and structural lesion (four of them with an abnormal background, and only two of them with skull changes). In one of these patients, ictal SPECT provided strong evidence for scalp EEG false lateralization. The findings suggest that compensatory skull thickening in patients with precocious destructive brain insults are more frequent among patients with unilateral and large lesions. However, EEG lateralization discordance among these patients seems to be more related to EEG background abnormalities and extent of cerebral damage than to skull changes.

Laterization of epileptiform discharges in patients with epilepsy and precocious destructive brain insults

Lesões cerebrais destrutivas unilaterais ocorridas em fase precoce do desenvolvimento podem resultar em espessamento compensatório da calota craniana ipsilateral. O objetivo deste estudo foi determinar a freqüência destas alterações ósseas em pacientes com epilepsia e lesões destrutivas precoces e avaliar se há associação entre estas alterações e lateralização de descargas epileptiformes. Foram analisados EEGs interictais / ictais e espessura do crânio pela RM de 51 pacientes. Os pacientes foram divididos em três grupos de acordo com a distribuição topográfica da lesão à RM: hemisférico (H) (n=9); território arterial (AT) (n=25); fronteira arterial (Bdz) (n=17). A atividade de base no EEG foi anormal em 26 pacientes e foi mais freqüente entre os pacientes do grupo H (p=0,044). Espessamento unilateral da calota craniana foi mais freqüente entre os pacientes do grupo H (p=0,004). Cinco pacientes (9,8 por cento) apresentaram discordância lateralizatória entre as descargas epileptiformes e lesão estrutural (quatro deles com atividade de base anormal, e apenas dois deles com espessamento da calota craniana). Em um destes pacientes, o SPECT ictal revelou forte evidência de falsa lateralização pelo EEG. Os achados sugerem que o espessamento compensatório da calota craniana é mais freqüente entre pacientes com lesões unilaterais e extensas. No entanto, a lateralização de descargas epileptiformes parece estar mais relacionada ao grau de alteração da atividade de base e extensão da lesão cerebral do que às alterações ósseas.

Early development destructive brain lesions and their relationship to epilepsy and hippocampal damage.

Fifty-one consecutive adult patients with epilepsy and early development destructive brain lesions were divided into three main groups according to the topographic distribution of the lesion on magnetic resonance imaging: hemispheric (H) (n=9); main arterial territory (AT) (n=25) and arterial borderzone (Bdz) (n=17). Eight (89%) patients from group H presented status epilepticus in the first 5 years of life, five of them associated with fever. Seventeen of the 25 patients from group AT (76%) had an obvious hemiparesis observed early in life. In addition, major prenatal events were significantly more common in the group AT compared with the other two groups. Among patients from group Bdz, prenatal or postnatal events were not identified, except for one patient. Conversely, nine patients from group Bdz (60%) showed a history of perinatal complications. Hippocampal atrophy (HA) was determined by visual analysis in 74.5% of all patients and by volumetry in 92%. The frequency of HA was comparable among groups, but patients from group H presented the most severe atrophy and more frequent hyperintense T2 hippocampal signal. In conclusion, these three groups of patients with early destructive lesions and epilepsy (H, AT and Bdz), appear to have distinct pathogenic mechanisms. Our data show that there is a striking association of HA with different patterns of neocortical destructive lesions of early development. This association seems to be related to a common and synchronic pathogenic mechanism. The recognition of the pattern and degree of HA among these patients with intractable seizures may influence the surgical rationale.

[Idiopathic generalized epilepsies misdiagnosed as partial epilepsies]. / Epilepsias generalizadas idiopáticas diagnosticadas incorretamente como epilepsias parciais.

Idiopathic generalized epilepsy (IGE) is often not recognized with serious consequences on the sufferers. We examined factors contributing to the missed diagnosis of IGE in 41 adults attending our epilepsy clinic with diagnosis of partial epilepsy who had semiology or EEG findings suggesting a possible differential diagnosis. After careful re-evaluation, the diagnosis of IGE was established in 25 patients: 22 (88%) with JME, one with juvenile absence, one with perioral myoclonia with absences, one with eyelid myoclonia with typical absences. Myoclonic jerks, the hallmark of the JME and other IGE, were not usually reported by patients or misdiagnosed as focal motor seizures. Brief and infrequent absence seizures and focal EEG abnormalities were other factors contributing to not recognizing JME. All 25 patients did not achieve seizure control before re-evaluation of diagnosis. After appropriate diagnosis of IGE and change of AED to valproate or valproic acid, 19 (76%) became seizure free and six (24%) had a significant improvement on seizure control. Association with lamotrigine provided further improvement in three of these patients. An appropriate questioning to identify myoclonic and absence seizures and a proper interpretation in the context of whole clinical constellation are essential for a correct seizure classification and diagnosis of IGE in adults.

Epilepsias generalizadas idiopáticas diagnosticadas incorretamente como epilepsias parciais / Idiopathic generalized epilepsies misdiagnosed as partial epilepsies

A epilepsia generalizada idiopática (EGI) frequentemente näo é diagnosticada corretamente em adultos, com sérias consequências para os pacientes. O objetivo deste estudo foi avaliar os fatores mais frequentemente associados a dificuldades no diagnóstico diferencial entre epilepsias parciais e generalizadas em adultos. Avaliamos 41 pacientes com diagnostico de crises parciais complexas com elementos de anamnese e EEG indicando um possível diagnóstico diferencial. Foi possível a mudança do diagnóstico de epilepsia parcial para EGI em 25 pacientes: 22 (88 por cento) com EMJ; um com ausência juvenil, um com síndrome de ausências com mioclonias periorais e um com ausência com mioclonias palpebrais. Mioclonias, uma das características da EMJ e outras formas de EGI, geralmente näo eram espontaneamente relatadas pelos pacientes. Abalos mioclônicos unilaterais eram confundidos com crises parciais motoras. Ausências breves e pouco frequentes e anormalidades focais no EEG contribuíram para o näo reconhecimento de EGI. Todos os 25 pacientes apresentavam crises sem controle adequado antes da revisäo diagnóstica. Após o diagnóstico correto e mudança para monoterapia com acido valpróico ou valproato de sódio, 19 (76 por cento) ficaram livre de crises e seis (24 por cento) dos 25 pacientes apresentaram melhora significativa. A associaçäo de lamotrigina em três destes pacientes propiciou reduçäo significativa da frequência de crises. Em conclusäo, anamnese detalhada e questionamento direcionado para determinar a presença de mioclonias e crises tipo ausência e a sua interpretaçäo no contexto clínico säo fundamentais para o diagnóstico correto das EGI em adultos

Crossed cerebellar atrophy in patients with precocious destructive brain insults.

OBJECTIVE: To analyze the frequency and pathogenetic factors of crossed cerebellar atrophy (CCA) in adult patients with epilepsy secondary to destructive brain insults of early development. METHODS: We studied 51 adult patients with epilepsy and precocious destructive lesions. Patients were divided into 3 groups according to the topographic distribution of their lesions on magnetic resonance imaging: group A, hemispheric (n = 9); group B, main arterial territory (n = 25); and group C, arterial border zone (n = 17). We evaluated the presence of CCA visually and with cerebellar volumetric measurement, correlating it with the clinical data. Other features shown on magnetic resonance imaging, such as the thalamus, brainstem, and middle cerebellar peduncle, were also carefully analyzed. RESULTS: Seven patients (13%) had CCA that was associated with the extent of the supratentorial lesion (6 from group A, 1 from group B, and none from group C; P<.001). Status epilepticus was present in 6 patients from group A and in none from the other groups. There was an association between the antecedent of status epilepticus and CCA (P<.001). All patients had atrophy of the cerebral peduncle ipsilateral to the supratentorial lesion and 4 had contralateral atrophy of the middle cerebellar peduncle. The duration of epilepsy was not associated with the presence of CCA (P =.20). CONCLUSIONS: Our data suggest that in patients with epilepsy and destructive insults early in life, the extent of the supratentorial lesion as well as the antecedent of status epilepticus play a major role in the pathogenesis of CCA. Recurrent seizures do not seem to be relevant to the development of CCA.

Congenital destructive hemispheric lesions and epilepsy: clinical features and relevance of associated hippocampal atrophy

We studied the clinical, EEG and MRI findings in 19 patients with epilepsy secondary to congenital destructive hemispheric insults. Patients were divided in two groups: 10 with cystic lesions (group 1), and 9 with atrophic lesions (group 2). Seizure and EEG features, as well as developmental sequelae were similar between the two groups, except for the finding that patients of group 2 more commonly presented seizures with more than one semiological type. MRI showed hyperintense T2 signal extending beyond the lesion in almost all patients of both groups, and it was more diffuse in group 2. Associated hippocampal atrophy (HA) was observed in 70 percent of group 1 patients and 77.7 percent of group 2, and it was not correlated with duration of epilepsy or seizure frequency. There was a good concordance between HA and electroclinical localization. The high prevalence of associated HA in both groups suggests a common pathogenesis with the more obvious lesion. Our findings indicate that in some of these patients with extensive destructive lesions, there may be a more circumscribed epileptogenic area, particularly in those with cystic lesions and HA, leading to a potential rationale for effective surgical treatment