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Thomas A.,Dartmouth Hitchcock Medical Center | Rauschkolb P.,Neurociences Institute | Gresa-Arribas N.,IDIBAPS | Schned A.,Dartmouth Hitchcock Medical Center | And 2 more authors.
JAMA Neurology | Year: 2013

IMPORTANCE: N-methyl-D-aspartate receptor (NMDAR) antibody encephalitis is an autoimmune encephalitis that can be paraneoplastic and usually responds to treatment. It is quickly becoming the most common paraneoplastic encephalitis. OBSERVATIONS: We present a case of a woman in her late 30s who developed psychiatric symptoms that progressed to encephalopathy, seizures, autonomic instability, and hyperkinetic movements. The patient was found to have an ovarian teratoma and serum and cerebrospinal fluid NMDAR antibodies. Despite resection of the teratoma and treatment with immunosuppressive therapy, the patient progressed to a minimally conscious state. She was supported medically in our institution for 25 months. During her hospitalization, she was treated with multiple immunosuppressive agents. With each treatment, we analyzed the serum and cerebrospinal fluid for NMDAR antibodies. While there was some initial reduction in the serum antibodies, the spinal fluid antibodies remained persistently elevated. The patient did not have any clinical improvement and eventually died after the family decided to withdraw care. CONCLUSIONS AND RELEVANCE: As far as we know, this case represents the longest active treatment without improvement of a patient with anti-NMDAR encephalitis. The patient had persistently high cerebrospinal fluid and serum antibody titers, which may be of prognostic significance. Copyright 2013 American Medical Association. All rights reserved. Source


Esposito K.,The Second University of Naples | Maiorino M.I.,The Second University of Naples | Ceriello A.,IDIBAPS | Giugliano D.,The Second University of Naples
Diabetes Research and Clinical Practice | Year: 2010

We conducted a systematic review of the available studies that assessed the effect of a Mediterranean diet in type 2 diabetes. We searched publications up to 30 November 2009. Seventeen studies were included. Two large prospective studies report a substantially lower risk (83% and 35%, respectively) of type 2 diabetes in healthy people or in post-infarct patients with the highest adherence to a Mediterranean diet. Five randomized controlled trials have evaluated the effects of a Mediterranean diet, as compared with other commonly used diets, on indices of glycaemic control in subjects with type 2 diabetes. Improvement of fasting glucose and HbA1c levels was greater with a Mediterranean diet and ranged from 7 to 40. mg/dl for fasting glucose, and from 0.1 to 0.6% for HbA1c. No trial reported worsening of glycaemic control with a Mediterranean diet. Two controlled trials in a secondary prevention setting demonstrated that post-infarct patients, including diabetic patients, had cardiovascular benefits from a Mediterranean diet. The evidence so far accumulated suggests that adopting a Mediterranean diet may help prevent type 2 diabetes, and also improve glycaemic control and cardiovascular risk in persons with established diabetes. © 2010 Elsevier Ireland Ltd. Source


Escorsell A.,Liver Unit | Mas A.,Liver Unit | Fernandez J.,Liver Unit | Garcia-Valdecasas J.C.,IDIBAPS
Transplantation Proceedings | Year: 2012

Aim: To assess the prognostic value of noninvasive indocyanine green (ICG) clearance (ICG-pulse-densitometric method [PDR]) for the outcome of liver grafts after transplantation. Methods: ICG-PDR, hepatic artery resistance index, cardiac output, transaminases, prothrombin time, bilirubin, albumin, hematocrit at 48 to 72 hours after transplantation were analyzed with reference to outcome among 59 liver graft recipients. Results: Two grafts were lost at 10 and 88 days during the initial hospitalization. These two patients only differed from the other recipients in the need for packing (1/2 versus 3/57) and degree of hypoproteinemia (46 ± 0 versus 51 ± 7.8 g/L), whereas they had similar ICG-PDR values (16.7%/min and 21.8%/min versus 17.3%/min ± 7.2%/min). Seven patients showed an ICG-PDR ≤ 8.8%/min, a previously identified cutoff for early postoperative complications. These patients versus the other 52 significantly differed in prothrombin index (47.9% ± 15.9% versus 64.3% ± 11.7%, P =.001) and bilirubin (8.3 ± 3.2 versus 3.3 ± 2.9 mg/dL, P =.0001). Early postoperative complications - primary graft nonfunction, hepatic artery thrombosis, or septic shock - responsible for an ICG-PDR ≤ 8.8%/min were observed in 2/7 patients. Interestingly, six cases developed an early (range: 3-15 days) rejection episode. In all the cases rejection suspected by analytical abnormalities was confirmed by liver biopsy. Among the overall series of patients, ICG-PDR significantly correlated with serum albumin (r = 0.345; P =.007), bilirubin (r = -0.514; P =.0001), and hematocrit (r = 0.462; P =.0001) but not with transaminases, prothrombin index, cardiac output, or hepatic artery resistance index. Actuarial 72-month probability of graft survival was 75%. Overall, 14 grafts were lost over a median follow-up of 78 months (range 1-99 m). There were no significant differences among early ICG-PDR values among grafts lost vs retained upon follow-up. Conclusion: ICG-PDR measured once early after liver transplantation did not offer relevant information to predict individual patient outcomes in the immediate postoperative phase. This lack of prognostic value may have been due to the multiple confounding factors involved in ICG metabolism after liver transplantation. © 2012 Elsevier Inc. All rights reserved. Source


Gutierrez F.,Institute of Neuroscience | Garriz M.,Institute of Neuroscience | Peri J.M.,Institute of Neuroscience | Ferraz L.,Biomedical Research Institute Sant Pau | And 6 more authors.
Evolution and Human Behavior | Year: 2013

Extreme personality traits in humans often have detrimental life consequences, so they have long been supposed to be diseases. However, many other species display personality variants that are maintained due to their fitness advantages; in this case, they are construed as strategies. To examine the fitness costs and benefits of pathological personality traits in humans, we measured features of the A (socially odd, distrustful), B (incentive-seeking, selfish) and C (fearful, inhibited) clusters with the Personality Diagnostic Questionnaire-4. + (PDQ-4. +) in a sample of 738 outpatients. Fitness relevant parameters like mating success, reproductive output, self preservation, and access to status were assessed with the Life Outcome Questionnaire. No fitness advantages were found for high-A subjects. In contrast, high-B subjects tripled low-B subjects with regard to mating success and had 39% more offspring. Further, high-C subjects outperformed low-C subjects in attaining status and avoiding risks. These findings help explain the commonness of some extreme personality traits in humans, and suggest that they should be seen as evolutionary strategies rather than as diseases. © 2013 Elsevier Inc. Source


Pratcorona M.,IDIBAPS | Pratcorona M.,Erasmus University Rotterdam | Abbas S.,Erasmus University Rotterdam | Sanders M.A.,Erasmus University Rotterdam | And 6 more authors.
Haematologica | Year: 2012

Somatic mutations in the additional sex comb-like 1 (ASXL1) gene have been described in various types of myeloid malignancies, including acute myeloid leukemia. Analysis of novel markers, such as ASXL1 mutations, in independent clinical trials is indispensable before considering them for clinical decision- making. We analyzed 882 well-characterized acute myeloid leukemia cases to determine the prevalence and prognostic impact of ASXL1 exon12 mutations. Truncating ASXL1 mutations were present in 46 cases (5.3%). ASXL1 mutations were inversely associated with FLT3 internal tandem duplications and mutually exclusive with NPM1 mutations. ASXL1 mutations were an unfavorable prognostic factor as regards survival (median overall survival 15.9 months vs. 22.3 months; P=0.019), with a significantly lower complete response rate (61% vs. 79.6%; P=0.004). In multivariate analyses, ASXL1 mutations were independently associated with inferior poor overall survival (HR 1.52, P=0.032). In conclusion, ASXL1 mutations are common mutations in acute myeloid leukemia and indicate a poor therapy outcome. © 2012 Ferrata Storti Foundation. Source

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