Fatal Outcome in Hemiconvulsion-Hemiplegia Syndrome.

Dec 2012

Jayakody H, Joshi C.

Source

1Division of Child Neurology, University of Iowa Children's Hospital, Iowa City, IA, USA.

Abstract

Hemiplegia-hemiconvulsion-epilepsy syndrome is characterized by prolonged unilateral clonic seizures in a child followed by the development of hemiplegia. Focal status epilepticus results in unilateral cerebral edema of the epileptic hemisphere in the acute phase followed by cerebral hemiatrophy. Literature in the last 5 years does not describe malignant cerebral edema or resultant death. We report a case of a 3-year-old girl with hemiplegia-hemiconvulsion-epilepsy syndrome who died due to malignant cerebral edema and temporal lobe herniation. The first indication of worsening of clinical status after being seizure free was voltage suppression on continuous electroencephalography (EEG). We describe neuroimaging, EEG findings, and neuropathologic findings at autopsy and review pertinent literature. We also evaluate the evolving role of continuous EEG monitoring in the pediatric intensive care unit.

PubMed

For further information:

Hemiconvulsion-Hemiplegia Syndrome.

Hemiconvulsion-hemiplegia-epilepsy syndrome

Myocardial Edema Imaging in Acute Coronary Syndromes

Dec 2012

Michael C. Walls, M.D., David Verhaert, M.D., and Subha V. Raman, M.D., M.S.E.E.

^Abstract

Acute coronary syndromes (ACS) continue to be the most common morbid condition of industrialized nations. The advent of and technical improvements in revascularization and medical therapy have led to a steady decline in mortality rates. However, many patients who suffer unstable angina or myocardial infarction require further testing and risk stratification to guide therapeutic selection and prognosis assignment. Myocardial edema imaging with cardiac magnetic resonance (CMR) affords the ability to define the amount of myocardium at risk, refine estimates of prognosis and provide guidance for therapies with excellent sensitivity compared to standard clinical markers. This review will discuss the rationale for edema imaging, how it is performed using CMR and its potential clinical applications.

Complete Text

NIH Public Access

Rationale for treating edema in Duchenne muscular dystrophy with eplerenone

Rationale for treating oedema in Duchenne muscular dystrophy with eplerenone

May 2012

FRANK LEHMANN-HORN, MARC-ANDRÉ WEBER,¹ ARMIN M. NAGEL,² HANS-MICHAEL MEINCK,³ SIMON BREITENBACH, JOHANNES SCHARRER, and KARIN JURKAT-ROTT

Abstract

Key words: Duchenne muscular dystrophy, eplerenone, cytotoxic oedema

Recently we reported a cytoplasmic sodium overload to cause a severe osmotic oedema in Duchenne muscular dystrophy (DMD). Our results suggested that this dual overload of sodium ions and water precedes the dystrophic process and persists until fatty muscle degeneration is complete. The present paper addresses the questions as to whether these overloads are important for the pathogenesis of the disease, and if so, whether they can be treated. As a first step, we investigated the effects of various diuretic drugs on a cell model of DMD, i.e. rat diaphragm strips previously exposed to amphotericin B. We found that both carbonic anhydrase inhibitors and aldosterone antagonists were able to repolarise depolarised muscle fibres. Since carbonic anhydrase inhibitors are known to have acidifying effects and this might be detrimental to the ventilation of DMD patients, we mainly concentrated on the modern spironolactone derivative, eplerenone. This drug had a very high repolarizing power, the parameter considered by us as being most relevant for a beneficial effect. In a pilot study we administered this drug to a 22-yr-old female DMD patient who was bound to an electric wheelchair and has had no corticosteroid therapy before. Eplerenone decreased both cytoplasmic sodium and water overload and increased muscle strength and mobility. We conclude that eplerenone has beneficial effects on DMD muscle. In our opinion the cytoplasmic oedema is cytotoxic and should be treated before fatty degeneration takes place.

Full Text Article

Cardiovascular magnetic resonance of myocardial edema using a short inversion time inversion recovery (STIR) black-blood technique: Diagnostic accuracy of visual and semi-quantitative assessment

March 2012

Darach O h-Ici,¹ John P Ridgway,² Titus Kuehne,¹ Felix Berger,¹ Sven Plein,³ Mohan Sivananthan,⁴ and Daniel R Messroghli¹

Abstract

Background

The short inversion time inversion recovery (STIR) black-blood technique has been used to visualize myocardial edema, and thus to differentiate acute from chronic myocardial lesions. However, some cardiovascular magnetic resonance (CMR) groups have reported variable image quality, and hence the diagnostic value of STIR in routine clinical practice has been put into question. The aim of our study was to analyze image quality and diagnostic performance of STIR using a set of pulse sequence parameters dedicated to edema detection, and to discuss possible factors that influence image quality. We hypothesized that STIR imaging is an accurate and robust way of detecting myocardial edema in non-selected patients with acute myocardial infarction.

Methods

Forty-six consecutive patients with acute myocardial infarction underwent CMR (day 4.5, +/- 1.6) including STIR for the assessment of myocardial edema and late gadolinium enhancement (LGE) for quantification of myocardial necrosis. Thirty of these patients underwent a follow-up CMR at approximately six months (195 +/- 39 days). Both STIR and LGE images were evaluated separately on a segmental basis for image quality as well as for presence and extent of myocardial hyper-intensity, with both visual and semi-quantitative (threshold-based) analysis. LGE was used as a reference standard for localization and extent of myocardial necrosis (acute) or scar (chronic).

Results

Image quality of STIR images was rated as diagnostic in 99.5% of cases. At the acute stage, the sensitivity and specificity of STIR to detect infarcted segments on visual assessment was 95% and 78% respectively, and on semi-quantitative assessment was 99% and 83%, respectively. STIR differentiated acutely from chronically infarcted segments with a sensitivity of 95% by both methods and with a specificity of 99% by visual assessment and 97% by semi-quantitative assessment. The extent of hyper-intense areas on acute STIR images was 85% larger than those on LGE images, with a larger myocardial salvage index in reperfused than in non-reperfused infarcts (p = 0.035).

Conclusions

STIR with appropriate pulse sequence settings is accurate in detecting acute myocardial infarction (MI) and distinguishing acute from chronic MI with both visual and semi-quantitative analysis. Due to its unique technical characteristics, STIR should be regarded as an edema-weighted rather than a purely T2-weighted technique.

Complete Text

NIH-PubMed

Extrafoveal changes following intravitreal bevacizumab injections for macular edema secondary to branch retinal vein occlusion: an mfERG and OCT study.

Dec 2012 

Park S, Cho IH, Park TK, Nam WH, Ohn YH.

Source

Department of Ophthalmology, College of Medicine, Soonchunhyang University, 1174 Jung-dong, Wonmi-gu, Bucheon, 420-767, Korea.

Abstract

PURPOSE:

To evaluate the functional and structural changes of extrafoveal macula after intravitreal bevacizumab (IVB) injection in patients with macular edema due to branch retinal vein occlusion (BRVO) using multifocal electroretinogram (mfERG) and optical coherence tomography (OCT).

METHODS:

A total of 19 eyes of 19 patients with macular edema due to BRVO received three consecutive IVB injections with a 6-week interval. Spectral domain optical coherence tomography (SD-OCT), mfERG, and fluorescein angiography (FA) were performed at baseline. The macular area was divided into four quadrants (Q1-Q4) based on FA. The mean retinal thickness (MRT) and mfERG parameters in each of the four quadrants were measured at baseline and 4 weeks after the third injection.

RESULTS:

The MRT in the four quadrants improved significantly after IVB injections (p < 0.01 for Q1 and Q2, p < 0.05 for Q3 and Q4) compared to baseline. The significant improvements in mfERG responses were seen in Q1 and Q2. In Q1, there were 68 and 56 % improvement in N1 and P1 amplitude, respectively (p < 0.01). N1 and P1 amplitude in Q2 increased significantly by 43 and 46 %, respectively, compared to baseline (p < 0.05). The MRT and P1 amplitude were significantly correlated at baseline in Q1 and Q2, but no significant correlations were found after three IVB injections.

CONCLUSIONS:

The injection of IVB improved functional and structural outcomes in the primarily affected half of the extrafoveal macula effectively. The measurements of structural and functional changes using mfERG and OCT may be appropriate for monitoring the effects of IVB injection in BRVO patients.

Springer

Small bowel angioedema due to acquired C1 inhibitor deficiency: a case report and overview.

Dec 2012

Oostergo T, Prins G, Schrama YC, Leeuwenburgh I.

Source

Departments of aInternal Medicine bNephrology, Sint Franciscus Gasthuis, Rotterdam, The Netherlands.

Abstract 

Acquired angioedema is a rare disorder caused by an acquired deficiency of C1 inhibitor. It is characterized by nonpitting, nonpruritic subcutaneous or submucosal edema of the skin, or of the respiratory or gastrointestinal tract. When localized in the gastrointestinal tract, it can cause severe abdominal pain, mimicking an acute surgical abdomen, or chronic recurrent pain of moderate intensity. We report a case of a 48-year-old man presenting with recurrent episodes of hypotension and abdominal pain. Computed tomography of the abdomen showed edema of the small bowel. The first determinations of C1 inhibitor level and activity, measured in a symptom-free period, were normal. Repetition of the laboratory tests in the acute phase, however, showed a low C1 inhibitor level. Further diagnostic work-up indicated an acquired C1 inhibitor deficiency caused by a monoclonal gammopathy. He was treated with tranexamic acid as prophylaxis for his frequent attacks and to date, he has remained symptom free. Acquired C1 inhibitor deficiency is a rare cause of angioedema and is, among others, related to autoantibodies and abnormal B-cell proliferation, for example monoclonal gammopathy. The diagnosis of acquired C1 inhibitor deficiency is made on the basis of the medical history and on the level and activity of plasma C4, C1q, and C1 inhibitor. In case of high suspicion and a normal C1 inhibitor activity, it is recommended to repeat this test during an angioedema attack. Early diagnosis is important for the treatment of severe, potentially life-threatening attacks and to start prophylactic treatment in patients with frequent or severe angioedema attacks.

Gastronoenterology & Hepatology

Flash Pulmonary Oedema after Relief of Haemodialysis Graft Stenosis.

Dec 2012 

Vélez-Martínez M, Weinberg BD, Mishkin JD.

Source

Cardiology Division, UT Southwestern Medical Center at Dallas, USA; Department of Internal Medicine, UT Southwestern Medical Center at Dallas, USA. Electronic address: mariella.velez-martinez@phhs.org.

Abstract

Heart failure (HF) and chronic kidney disease (CKD) are undoubtedly very much interrelated, especially in patients with end-stage renal disease (ESRD) who are dependent on renal replacement therapy. Haemodialysis (HD) is of particular interest in cardiovascular patients due to the creation of a haemodialysis vascular access and the haemodynamic changes associated with it. Adequate HD though is very dependent on a properly functioning vascular access. Unfortunately, these surgical vascular accesses are vulnerable to stenoses and occlusions. Percutaneous endovascular treatment of these stenoses is often performed and has been found to be safe and effective. Despite its frequent use, acute medical complications of this percutaneous procedure have not been well-documented. In this report, we describe a patient who developed flash pulmonary oedema after balloon angioplasty treatment of an arteriovenous graft (AVG) stenosis.

Elsevier SciVerse

Glioma-related edema: new insight into molecular mechanisms and their clinical implications.

Dec 2012

Lin ZX.

Source

Department of Neurosurgery, The First Affiliated Hospital, Fujian Medical University, Fuzhou, Fujian 350005, P. R. China lzx1967@sina.com.

Abstract

Glioma-related edema (GRE) is a significant contributor to morbidity and mortality from glioma. GRE is a complicated process involving not only peritumoral edema, but also the water content of the tumor body. In terms of etiology, this condition derives from both GRE in the untreated state and GRE secondary to clinical intervention, and different cell types contribute to distinct components of GRE. Peritumoral edema was previously believed to loosen glioma tissue, facilitating tumor-cell invasion; however, the nutrition hypothesis of the tumor microecosystem suggests that tumor cells invade for the sake of nutrition. Edema is the pathologic consequence of the reconstructed trophic linkage within the tumor microecosystem. Glioma cells induce peritumoral brain edema via an active process that supplies a suitable niche for peritumoral invasive cells, suggesting that glioma-related peritumoral brain edema is determined by the invasive property of tumor cells. There are differences between pivotal molecular events and reactive molecular events in the development of GRE. Molecular therapy should target the former, as targeting reactive molecular events will produce undesired or even adverse results. At present, brain glioma angiogenesis models have not been translated into a new understanding of the features of brain images. The effect of these models on peritumoral brain edema is unclear. Clinical approaches should be transformed on the basis of new knowledge of the molecular mechanism of GRE. Exploring clinical assessment methods, optimizing the existing control strategy of GRE, and simultaneously developing new treatments are essential.

PubMed

Stellate ganglion block may prevent the development of neurogenic pulmonary edema and improve the outcome.

Nov 2012

Zhang L, Yao J, Zhang T, Jin J, Zeng X, Yue Z.

Source

Department of Anesthesiology, The Second Affiliated Hospital of Harbin Medical University, Harbin 150086, Heilongjiang Province, China.

Abstract

Neurogenic pulmonary edema (NPE) is an acute and serious complication after a central nervous system insult with high mortality. The pronounced activation of sympathetic nervous system and the release of vasoactive substances are necessary prerequisites for the development of NPE. We introduce a hypothesis that stellate ganglion block (SGB) may prevent NPE development on the basis of the inhibition of sympathetic overactivation, reduction of the concentration of norepinephrine and attenuation of baroreflex sensitivity, and improve the outcome by improving cerebral blood flow and pulmonary circulation and maintaining cardiovascular stability. In clinical practice, the guidance technique and close monitoring might guarantee the safety of SGB. If our hypothesis is supported by further experiments, this may open a new doorway for the treatment of NPE.

Elsevier

Cardiogenic Acute Pulmonary Edema

Image from the article:

Cardiogenic Acute Pulmonary Edema – Causes, Symptoms, Diagnosis And Treatment





From:  Heart Failure and Your Lungs



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