Elena Santamarta

Usefulness of cardiac magnetic resonance imaging in left ventricular non‐compaction cardiomyopathy

We have read with great interest the paper published recently in your journal by Habib et al. about isolated left ventricular non-compaction in adults. As the authors say, the genetic and clinical features, prognosis and characterization of this fascinating entity remain incompletely understood and the subject of interesting debate in the scientific community. As stated by the authors, difficulties commence with the diagnostic criteria, as different timings of measurement, at systole or diastole, have been proposed. In addition even for experts, making a final diagnosis can be difficult; and echocardiography laboratories have contributed to the great increase in noncompaction cardiomyopathy diagnosis or over diagnosis. Certainly, although both 3D and contrast echocardiography can improve the assessment of this cardiomyopathy, we must not forget that cardiac magnetic resonance imaging (MRI) can also play an important role in its diagnosis. The spatial resolution of this technique, its ability for evaluating cardiac function and what is more, the tissue characterization by the late-gadolinium hyperenhancement sequence makes it an excellent tool for the diagnosis of cardiomyopathies, especially in the case of non-compaction in which differential diagnosis from other entities such as hypertrophic cardiomyopathy, can sometimes be difficult. Late gadolinium enhancement is a surrogate of myocardial fibrosis, Nucifora et al. recently published a report in this journal demonstrating correlation of fibrosis with clinical severity and ventricular dysfunction in patients with non-compaction disease. Thus, fibrosis represents a marker of poor prognosis that could be used as a stratification tool in these patients, similar to that proposed for hypertrophic cardiomyopathy. Many questions remain about left ventricular non-compaction disease, but cardiac MRI can provide some of the answers: a more precise diagnosis based on a better endomyocardial definition and tissue characterization that can help to determine patient prognosis.

Headache as a Crucial Symptom in the Etiology of Convexal Subarachnoid Hemorrhage

Convexal subarachnoid hemorrhage has been associated with different diseases, reversible cerebral vasoconstriction syndrome and cerebral amyloid angiopathy being the 2 main causes.

Late Gadolinium Enhancement in Non-Compaction Cardiomyopathy

The European Society Cardiology of currently considers the non-compaction cardiomyopathy as an un-classified cardiomyopathy.1 It is characterised by the presence of numerous and prominent trabeculations together with deep intertrabecular recesses in a portion of the ventricular wall, principally at the lateral, and apical level, as a result of a congenital anomaly of the endomyocardial development.2 Its clinical presentation is variable, from asymptomatic cases to severe heart failure; other manifestations of the disease include ventricular arrhythmias and systemic embolism. Cardiac Magnetic Resonance Imaging (CMR), because of its high spatial resolution, makes it possible to determine a precise diagnosis of this entity. We present a case of a 44-year-old patient that was diagnosed with dilated idiopathic cardiomyopathy with normal coronaries and moderate dysfunction of the left ventricle that was remitted for a CMR with suspicions of NCC. A Holter study showed frequent episodes of nonsustained ventricular tachycardia, with a maximum oxygen consumption of 14 mL/kg/ min. The CMR carried out with a 1.5 Tesla magnet (Figures 1 and 2, images from the gradient echo and after administering the contrast) showed the anatomic characteristics representative of this entity, as well as a late enhancement of subepicardium and intramyocardial gadolinium in the anterior and septum areas, with an ejection fraction of 35%. The relevance of the CMR in the diagnosis of this cardiomyopathy has been shown previously; however, the role of the gadolinium late-enhancement has not been completely clarified.3 Other authors have described it as an index of scar tissue; a possible correlation between the extension and localisation of the enhancement with the clinical evolution of these patients has been considered, although the number of cases described in existing literature is low.4,5 In addition, the prognosis of the NCC is controversial, and there are still no defined criteria for high risk. CMR studies may provide a great amount of information regarding this complex and still partially unknown entity, not only as a diagnostic tool, but also as an indicator of its prognosis. More follow-up and longer series of patients are needed to understand not only the natural history of non-compaction cardiomyopathy, but also the role that the gadolinium late-enhancement may play regarding this entity.

Realce tardío del gadolinio en la miocardiopatía no compactada

La Sociedad Europea de Cardiología actualmente considera la miocardiopatía no compactada (MNC) como una miocardiopatía no clasificada. Se caracteriza por la presencia de numerosas y prominentes trabeculaciones junto con recesos intertrabeculares profundos en un segmento de la pared ventricular, principalmente a nivel lateral y apical, resultado de una anomalía congénita del desarrollo endomiocárdico. Su presentación clínica es variable, desde casos asintomáticos hasta insuficiencia cardiaca severa; otras manifestaciones propias de la enfermedad son las arritmias ventriculares y la embolia sistémica. La cardiorresonancia magnética (CRM), por su alta resolución espacial, permite un diagnóstico preciso de esta entidad. Presentamos el caso de una paciente de 44 años diagnosticada de miocardiopatía dilatada idiopática con coronarias normales y disfunción moderada del ventrículo izquierdo, que fue remitida para CRM por sospecha de MNC. Un estudio Holter había puesto de manifiesto episodios frecuentes de taquicardia ventricular no sostenida, con un consumo máximo de oxígeno de 14 ml/kg/min. La CRM realizada con un equipo de 1,5 Teslas (figs. 1 y 2, imágenes de eco de gradiente y tras administración de contraste) puso de manifiesto las características anatómicas propias de esta entidad, así como un realce tardío de gadolinio subepicárdico e intramiocárdico de localización anterior y septal, con una fracción de eyección del 35%. La relevancia de la CRM en el diagnóstico de esta miocardiopatía ya ha sido puesta de manifiesto previamente; sin embargo, el papel del realce tardío de gadolino no ha sido totalmente aclarado. Otros autores lo han descrito como indicio de tejido fibroso y cicatrizal; también han planteado una posible correlación de la extensión y la localización del realce con la evolución clínica de estos pacientes, si bien el número de casos descritos en la literatura es escaso. Por otro lado, el pronóstico de la MNC es controvertido, sin que hasta el momento se hayan definido los criterios de alto riesgo. Los estudios de CRM pueden aportar mucho en esta entidad compleja y todavía no completamente conocida, no sólo como herramienta diagnóstica, sino también en cuanto a su pronóstico. Son necesarios mayores seguimientos y series de pacientes más largas para conocer tanto la historia natural de la miocardiopatía no compactada como el papel que el realce tardío de gadolino pueda desempeñar en esta entidad.

Chronic migraine does not increase posterior circulation territory (PCT) infarct-like lesions

UNLABELLED Two population-based studies have found an increased prevalence of posterior circulation territory (PCT) infarct-like lesions in migraine, which seemed to increase with attack frequency. OBJECTIVE To determine whether chronic migraine (CM) patients are at increased risk of PCT infarct-like lesions. METHODS We prospectively obtained brain MRIs from adult women fulfilling CM criteria. To keep radiologists blinded we also obtained brain MRIs in 15 episodic migraine (EM) patients. MRIs were acquired on a 1.5 T unit. Protocol included whole brain weighted images in sagittal T1 (5 mm slices), axial FLAIR T2 (3 mm) and combined proton density and T2 fast spin echo (3 mm). Two independent neuroradiologists carefully analyzed all the images. RESULTS One hundred women with CM participated. Their ages ranged from 18 to 68 years (mean 43.7) and the length of CM ranged from 0.5 to 38 years (mean 9.8). Sixty-three patients (63%) had at least one vascular risk factor. Thirty-three met analgesic overuse criteria. Fifty-one had a history of migraine with aura attacks, though aura frequency was below one per month in all patients except one. Eleven were not on preventatives. We found PCT infarct-like lesions in only 6 CM patients aged 42-64 years (mean age 54 years) who had at least two vascular risk factors. CONCLUSIONS As frequency of PCT infarct-like lesions in our CM patients was in the low range than that found for EM in general population studies, we conclude that frequency of migraine attacks itself is not a factor increasing PCT infarct-like lesion risk.

Fluctuating neurological symptoms in demyelinating disease mimicking an acute ischaemic stroke

Fluctuating neurological symptoms in an older patient most often point towards a cerebral ischaemic pathology. The authors present a 66-year-old male patient suffering from a fluctuating right hemiparesis, with an initial diagnosis of ischaemic stroke. The brain and cervical MRI showed demyelinating lesions with abnormal cerebrospinal fluid (CSF) and visual evoked potentials and the patient was successfully treated with intravenous corticosteroids. Demyelinating disease in older patients could be more frequent than expected. It should be considered even in older patients with fluctuating neurological symptoms. MRI and CSF analysis are critical to provide an accurate diagnosis.

Heart involvement in systemic sclerosis: present but not evident

A 42 year old woman, with a previous diagnosis of systemic sclerosis (SSc) with Raynaud‘s phenomenon and skin involvement, came into the Emergency Department complaining of dyspnea and palpitations. Previous echocardiogram practised several months ago was normal. Pulmonary pressure and pulmonary function tests were also normal. ANAs were positive while RNP and anti Scl 70 antibodies were negative. Physical exam revealed severe skin sclerosis with sclerodactilia. Tachycardia without murmurs and a normal jugular venous pressure. No crackles or rales over lungs. No peripheral oedema. ECG showed an atrial flutter, previously unknown while thorax radiography and contrast enhanced spiral CT ruled out pulmonary disease or thromboembolism. Cardioversion of atrial flutter was practised after transoesophageal echocardiogram which exluded thrombus in left atrium, with good clinical evolution. Cardiac-MRI was carried out with a 1.5 T MRI scanner. It revealed a diffuse, anular and non-coronary distribution myocardial late gadolinium hyperenhancement, without hypertrophy or contraction abnormalities. (Figure 1: Short axis and two chamber view. SSFP sequence and late gadolinium hyperenhancement sequence, note the anular enhancement). Myocardial fibrosis in SSc has been reported in 50–80% of necropsy series. It can manifest as biventricular cardiac failure, both atrial and ventricular arrhytmias, like in our case, and even with sudden death. The diagnosis of myocardial fibrosis is difficult by non-invasive methods. Several studies have analyzed the late myocardial fibrosis by MRI. Usually it has a linear pattern limited to the midwall layer with invariable sparing of the subendocardium and epicardium [1, 2]. Patients with abnormal Holter studies and those with long duration of Raynaud‘s phenomenon

Parkinson’s Disease Database Analysis of Stereotactic Coordinates Related to Clinical Outcomes

Parkinson’s Disease is one of the leading movement disorder diseases. It is the fourth most common neurological disease, after migraine, stroke and epilepsy. The motor symptoms of the disease significantly impair daily living and quality of life and exact a high burden on both patients and their caregivers. Deep Brain Stimulation is a proven therapy for this disease, getting positive outcomes while reducing medication. In this paper, stereotactic system used for Deep Brain Stimulation (DBS) procedures will be described. Different planning methods will be observed and compared to the gold standard normally used, neurophysiological coordinates recorded intra-operatively. MRI, CT scan and direct calculation of stereotactic coordinates will be compared and group in three different groups, according to DBS therapy outcomes: “very good DBS therapy”, “good DBS therapy” and “not major improvement”.

Long-Term Results of Deep Brain Stimulation of the Mamillotegmental Fasciculus in Chronic Cluster Headache

Background: Deep brain stimulation (DBS) and the proper target for chronic cluster headache (CCH) are still subjects of controversy. Objectives: We present our long-term results of analysis of the target and its structural connectivity. Methods: Fifteen patients with drug-resistant CCH underwent DBS in coordinates 4 mm lateral to the III ventricular wall and 2 mm behind and 5 mm below the intercommissural point. The clinical parameters recorded were the number of weekly attacks, pain intensity, and duration of the headache. Structural connectivity was studied using 3-T MR diffusion tensor imaging (DTI). Results: All of our patients improved from a mean of 39 attacks/week to 2; pain intensity decreased from 9 to 3 out of 10, and the mean cephalalgia duration decreased from 53 to 8 min. The mean stereotactic coordinates of the effective contact location were 6.1 mm lateral to the midcommissural point and 1.2 mm behind and 4.0 mm below the intercommissural point. DTI analysis showed that this target was connected to tracts and nuclei of the posterior mesencephalic tegmentum, specifically the dorsal longitudinal and mamillotegmental fasciculi. Conclusions: Our data showed DBS to be a safe and useful procedure for the treatment of drug-resistant CCH; the rate of improvement was higher than those found in other series. Although these are promising results, larger series targeting those fasciculi with a longer follow-up are needed.

Neuropsychiatric Systemic Lupus Erythematosus and Cognitive Impairment: A Complex Issue with a Dark Prognosis

Aims: Neuropsychiatric systemic lupus erythematosus (NPSLE), may present as a wide range of symptomatology where cognitive impairment could play a key role in the prognosis of SLE patients. Presentation of Case: A 24 year-old female was admitted in 1999 in unconsciousness. Cranial MRI showed two thalamo-mesencephalic lesions and the patient was discharged home with a diagnosis of metabolic and/or toxic encephalopathy. One year later systemic lupus erythematosus was diagnosed. Following up showed memory disturbance with several strokes and atrophy in neuroimaging, and positivity of antiphospholipid antibodies. Anticoagulation was indicated with an irregular compliance. In March 2010 the patient was admitted due to generalized articular pain and hemoptoic sputum due to bad control of the anticoagulation. One month later she was admitted to Intensive Care Unit due to acute pulmonary edema and acute renal failure. In June 2010, the