Faculty Bibliography

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by acute severe thunderclap headaches and evidence of multifocal, segmental, reversible vasoconstrictions of the cerebral arteries. Several precipitating factors have been identified and reported, including the use of recreational substances or sympathomimetic drugs and the postpartum state. CASE DESCRIPTION: Here we present the case of a woman who developed RCVS after the administration of adrenaline (epinephrine) in the setting of an anaphylactic reaction during antibiotic allergy testing. DISCUSSION: To our knowledge, this is the first reported case of RCVS following the administration of exogenous adrenaline. This case contributes to the understanding of the physiopathological mechanisms underlying reversible cerebral vasoconstriction.

INTRODUCTION: Obstructive Sleep Apnea (OSA) is a major risk factor for cardiovascular disease. The goal of this study was to demonstrate whether the use of CPAP produces significant changes in the heart rate or in the heart rate variability of patients with OSA in the first night of treatment and whether gender and obesity play a role in these differences. METHODS: Single-center transversal study including patients with severe OSA corrected with CPAP. Only patients with total correction after CPAP were included. Patients underwent two sleep studies on consecutive nights: the first night a basal study, and the second with CPAP. We also analyzed the heart rate changes and their relationship with CPAP treatment, sleep stages, sex and body mass index. Twenty-minute segments of the ECG were selected from the sleep periods of REM, no-REM and awake. Heart rate (HR) and heart rate variability (HRV) were studied by comparing the R-R interval in the different conditions. We also compared samples from the basal study and CPAP nights. RESULTS: 39 patients (15 females, 24 males) were studied. The mean age was 50.67 years old, the mean AHI was 48.54, and mean body mass index was 33.41 kg/m(2) (31.83 males, 35.95 females). Our results showed that HRV (SDNN) decreased after the use of CPAP during the first night of treatment, especially in non-REM sleep. Gender and obesity did not have any influence on our results. CONCLUSIONS: These findings support that cardiac variability improves as an acute effect, independently of gender or weight, in the first night of CPAP use in severe OSA patients, supporting the idea of continuous use and emphasizing that noncompliance of CPAP treatment should be avoided even if it is just once.

The control of food intake and energy metabolism depends on the complex interaction between energy homeostasis, hedonic control of feeding, and environmental cues. Experimental research in animal models and also the clinical observation of patients with feeding disorders have led to an increased knowledge of the mechanisms that regulate appetite. The hypothalamus is the key component of this network. Hypothalamic nuclei send to and receive signals from the insula, orbitofrontal cortex, nucleus accumbens, dopaminergic reward system, and multiple chemical signals, including peptides and gastrointestinal hormones, to regulate feeding behavior. Recent studies have provided insight into the complex interactions between the homeostatic and hedonic control of feeding behavior, sharing some neurobiological mechanisms with addictions. Identification of potential therapeutic targets will allow the development of useful drugs to treat feeding behavior disorders, such as obesity.

STUDY DESIGN: Several causes of Brown-Sequard syndrome have been described. Endovascular embolization can be used to treat symptomatic vertebral hemangiomas. We describe a previously undocumented case of Brown-Sequard syndrome followed by endovascular embolization with microcoils of a vertebral hemangioma. We also provide a clinical-radiological correlation of this finding and review the relevant literature. CASE REPORT: A 39-year-old male was referred to our hospital for endovascular treatment of a right T9 hemivertebral hemangioma with compromise of the spinal canal. Fifteen minutes after the procedure, the patient developed right lower limb weakness and numbness on the left leg. The emergency magnetic resonance imaging (MRI) of the spine showed no abnormalities. Five days later, a new spinal MRI revealed an infarction in the right half of the spinal cord at T6 and T7 level. This stroke was probably caused by a microcoil ended up in the right sulcocommisural artery. One week after surgery, the patient was able to raise the right leg against gravity, but sensory deficit showed no improvement. CONCLUSIONS: To the best of our knowledge this is the first case of a Brown-Sequard syndrome related to vertebral hemangioma embolization, a relatively safe technique with no important complications made known until this report. Clinicians should always weight the benefits with the potential devastating complications of this therapeutic option.

AIM: To analyse our experience in the treatment of refractory chronic migraine, episodic frequent refractory migraine (>/=10 days/month), with onabotulinumtoxin A (OnabotA). PATIENTS AND METHODS: Retrospective analysis of patients with refractory migraine who underwent, at least two sessions of OnabotA pericranial injections following the PREEMPT protocol between 2008 and 2012. The efficacy of OnabotA was evaluated comparing the basal situation with 12-16 weeks after the second session. We analysed the subjective improvement of the patients, number of days with headache, preventive and abortive drugs consumption, and adverse effects. RESULTS: Forty-one patients (37 women, 4 male) were identified. 65.8% patients experienced subjective improvement after OnabotA treatment. 36.58% responded (reduction of > 50% in headache days). Differences between days with headache before the first session (24.5 +/- 7.3), and 12-16 weeks after the second session (17.4 +/- 11.6), as well as the differences between the number of abortive drugs taken before the first session (26.8 +/- 23.1) and 12-16 weeks after the second session (16.7 +/- 19.3), were statistically significant (p < 0.001). Subgroups analysis showed that all differences were significant, except for the reduction of the number of days with headache in patients with episodic frequent refractory migraine. CONCLUSION: Our work shows that treatment with OnabotA is safe and useful in patients with episodic and chronic refractory migraine, including those patients with medication overuse headache.

INTRODUCTION: Brain death is generally accepted as a concept to indicate death. It was introduced about 40 years ago, and it was considered the ideal situation for donation of organs. METHODS: During this time, however, there have been problems in the understanding of this concept both in the medical profession and in the general population. University students from medical and non-medical schools were tested for their understanding of this concept. RESULTS: Our results show that less than one third of the non-medical students identified brain death as death. The data from the medical students changed as they progressed through their studies, but only 2/3 of the graduating medical class believed that brain death is death. CONCLUSION: Similar results have been seen in other universities around the world, and a renewed effort on the re-education of the concept of brain death may be worthwhile. Although we cannot extrapolate these results to the general population, the confusion is probably similar; hence an effort should be made to solve this problem.

Familial dysautonomia (FD) is a rare hereditary disorder caused by mutations within the gene that encodes for I-kappa-B kinase complex associated protein (IKAP). A deficiency of IKAP affects the development of primary sensory neurons including those carrying baroreflex afferent volleys, a feature that explains their characteristic sensory loss and labile blood pressure. This review describes the history, the genotype of FD and the unusual cardiovascular autonomic phenotype of these patients. We outline the main consequences of a failure to receive information from arterial baroreceptors, including the characteristic "autonomic storms" and severe end-organ target damage.

Orthostatic hypotension (OH) defined as a reduction of systolic blood pressure of at least 20 mmHg or diastolic blood pressure of at least 10 mmHg within 3 min of active standing or head-up-tilt (HUT) is common in Parkinson disease (PD). We compared the frequency of OH when assessed by head-up-tilt test (HUT) versus active standing in 233 patients with PD. 116 patients (73 men and 43 women) underwent a 60degree HUT and 117 patients (62 men and 53 women) underwent an active standing procedure. Blood pressure and heart rate were measured before and after 3 min in the upright position. The average dose of levodopa and direct dopaminergic agonists and the frequency of other medications was similar in both cohorts. The prevalence of OH was 70 % in those undergoing HUT and 41 % in those undergoing active standing (p<0.001). However, patients undergoing HUT were significantly older (72.1 vs. 61.2 years, p<0.001) and had higher systolic blood pressure while supine (151 vs. 134 mmHg, p<0.001). Prevalence of OH by age showed that the 40-50 years old group (n:15) had 20 % prevalence of OH with HUT versus 40 % with active standing (NS); in the 50-60 years old group (n:38), 33 % had OH with HUT versus 47 % with active standing (NS), in the 60-70 years old group (n:67), 78 % had OH with HUT versus 43 % with active standing (p<0.004), and in the 70-80 years old group (n:85), 60 % had OH with HUT and 36 % with active standing (p<0.04). Thus, in younger patients with PD active standing and HUT showed similar prevalence of OH. However, among PD patients 60 years and older the prevalence of OH was significantly higher with HUT than active standing. These findings have practical implication for diagnosis and clinical management