Intravitreal Aflibercept vs Laser Therapy for Retinopathy of Prematurity: Two-Year Efficacy and Safety Outcomes in the Nonrandomized Controlled Trial FIREFLEYE next.

Importance: Prospective long-term data after retinopathy of prematurity (ROP) treatment with anti-vascular endothelial growth factor injections vs laser therapy are scarce. The FIREFLEYE (Aflibercept for ROP IVT Injection vs Laser Therapy) next trial is prospectively evaluating the long-term efficacy and safety outcomes following ROP treatment with intravitreal aflibercept vs laser therapy. Objective: To evaluate 2-year ophthalmic and safety outcomes after 0.4-mg aflibercept injection or laser therapy in the 24-week randomized (2:1) FIREFLEYE trial (FIREFLEYE outcomes previously reported). Design, Setting, and Participants: This prospective nonrandomized controlled trial performed in 24 countries in Asia, Europe, and South America (2020-2025) follows up participants treated in the FIREFLEYE randomized clinical trial (2019-2021) through 5 years of age. Participants included children born very or extremely preterm (gestational age ≤32 weeks) or with very or extremely low birth weight (≤1500 g) who were previously treated with a 0.4-mg injection of aflibercept compared with laser therapy for severe acute-phase ROP. Data for the present interim analysis were acquired from March 18, 2020, to July 25, 2022. Interventions: Complications of ROP treated at investigator discretion (no study treatment). Main Outcomes and Measures: Efficacy end points included ROP status, unfavorable structural outcomes, ROP recurrence, treatment for ROP complications, completion of vascularization, and visual function. Safety end points included adverse events and growth and neurodevelopmental outcomes. Results: Overall, 100 children were enrolled (median gestational age, 26 [range, 23-31] weeks; 53 boys and 47 girls). Of these, 21 were Asian, 2 were Black, 75 were White, and 2 were of more than 1 race. At 2 years of age, 61 of 63 children (96.8%) in the aflibercept group vs 30 of 32 (93.8%) in the laser group had no ROP. Through 2 years of age, 62 of 66 (93.9%) in the aflibercept group and 32 of 34 (94.1%) in the laser group had no unfavorable structural outcomes. No new retinal detachment occurred during the study. Four children in the aflibercept group (6.1%) were treated for ROP complications before 1 year of age (2 had preexisting end-stage disease and total retinal detachment; 1 had reactivated plus disease; and 1 had recurrent retinal neovascularization not further specified). Most children were able to fix and follow a 5-cm toy (aflibercept group, 118 of 122 eyes [96.7%] among 63 children; laser group, 62 of 63 eyes [98.4%] among 33 children). High myopia was present in 9 of 115 eyes (7.8%) among 5 children in the aflibercept group and 13 of 60 eyes (21.7%) among 9 children in the laser group. No relevant differences in growth and neurodevelopmental outcomes by Bayley Scales of Infant and Toddler Development, Third Edition and Vineland Adaptive Behavior Scales, Second Edition were identified. Conclusions and Relevance: In this nonrandomized follow-up of a randomized clinical trial comparing treatment of severe acute-phase ROP with 0.4-mg injection of aflibercept and laser, disease control was stable and visual function was appropriate in children through 2 years of age. No adverse effects on safety, including growth and neurodevelopment, were identified. These findings provide clinically relevant long-term information on intravitreal aflibercept injection therapy for ROP. Trial Registration: ClinicalTrials.gov Identifier: NCT04015180.

Study of the Interaction between Executive Function and Adaptive Behavior at School in Girls with Fragile X Syndrome.

The aim of this research is to analyze the relationship between executive functions and adaptive behavior in girls with Fragile X syndrome (FXS) in the school setting. This study is part of a larger investigation conducted at the Hospital Parc Tauli in Sabadell. The sample consists of a total of 40 girls (26 with FXS and 14 control) aged 7-16 years, who were administered different neuropsychological tests (WISC-V, NEPSY-II, WCST, TOL) and questionnaires answered by teachers (ABAS-II, BRIEF 2, ADHD Rating Scale). The results show that there is a greater interaction between some areas of executive function (cognitive flexibility, auditory attention, and visual abstraction capacity) and certain areas of adaptive behavior (conceptual, practical, social, and total domains) in the FXS group than in the control group. These results suggest that an alteration in the executive functions was affecting the daily functioning of the girls with FXS to a greater extent.

End of Life Intervention Program During COVID-19 in Vall d'Hebron University Hospital.

Introduction: The coronavirus disease 19 (COVID-19) and its consequences have placed our societies and healthcare systems under pressure. Also, a major impact on the individual and societal experience of death, dying, and bereavement has been observed. Factors such as social distancing, unexpected death or not being able to say goodbye, which might predict Prolonged Grief Disorder (PGD), are taking place. Moreover, hospitals have become a habitual place for End of Life (EOL) situations but not in the usual conditions because, for example, mitigation measures prevent families from being together with hospitalized relatives. Therefore, we implemented an EOL program with a multidisciplinary team involving health social workers (HSW) and clinical psychologists (CP) in coordination with the medical teams and nursing staff. Objectives: We aim to describe an EOL intervention program implemented during COVID-19 in the Vall d'Hebron University Hospital (HUVH). We present its structure, circuit, and functions. Descriptive analyses of the sample and the interventions that required psychological and social attention are reported. Material and methods: The total sample consists of 359 relatives of 219 EOL patients. Inclusion criteria were families cared for during the COVID-19 pandemic with family patients admitted to the HUVH in an EOL situation regardless of whether or not the patient was diagnosed with COVID-19. Results: Our program is based on family EOL care perceptions and the COVID-19 context features that hinder EOL situations. The program attended 219 families, of which 55.3% were COVID-19 patients and 44.7% had other pathologies. The EOL intervention program was activated in most of the EOL situations, specifically, in 85% of cases, and 78% of relatives were able to come and say goodbye to their loved ones. An emotional impact on the EOL team was reported. It is necessary to dignify the EOL situation in the COVID-19 pandemic, and appropriate psychosocial attention is needed to try to minimize future complications in grief processes and mitigate PGD.

Pilot study of socio-emotional factors and adaptive behavior in young females with fragile X syndrome.

Girls with Fragile-X-Syndrome (FXS) present high levels of social anxiety, social avoidance, extreme shyness, tendency to social isolation, poor eye contact, learning difficulties, and depression. The aims of the present study, which is based on a group of young females with FXS are: 1) to analyze the possible associations between emotion recognition, theory of mind, and social anxiety, and adaptive behavior, and emotional state; 2) to study the relationship between intelligence quotient (IQ) and adaptive behavior; and 3) to assess whether social anxiety is more prevalent in girls with FXS. The study has 40 female participants aged between 7 and 16 years (26 positive full mutation FXS and 14 as a control group). A neuropsychological assessment was conducted using the following tests: WISC-V, NEPSY-II, SENA, ADHD Rating Scale, BAS, and ABAS-II. In comparison with the control group, the group with FXS presented a greater association between IQ and self-direction ability, and between emotion recognition and leadership. The FXS group presented higher levels of social anxiety and shyness. In the group of girls with FXS, IQ may have prognostic value for both self-direction ability and social adaptation level.

Fragile X syndrome in young females: Influence of executive function on the neurocognitive profile and adaptive behavior.

INTRODUCTION: The aim of this study is to describe the relationship between executive function (EF) and performance in different areas of the neurocognitive profile in young girls with Fragile-X-Syndrome (FXS). METHOD: A neuropsychological assessment was carried out to 40 female participants aged 7-16 years (26 FXS, 14 control group). RESULTS: Regarding intellectual ability, in the group of girls with FXS 3.84 % of the participants obtained IQ scores in the range of moderate ID (IQ 35-40 to 49), 46.15 % in the range of mild ID (IQ 50-70), 38.46 % in the borderline range (IQ 70-85), and 11.53 % within the average range (IQ > 85). EF was found to have a greater influence on adaptive behavior, arithmetic ability, theory of mind, leadership, social integration, social competence, and anxiety/shyness in the group with FXS. CONCLUSIONS: In girls with FXS, EF showed a greater influence on adaptive behavior, arithmetic ability, and social domain.

Bullying Victimization in Young Females with Fragile-X-Syndrome.

The aim of this study is to investigate the risk associated with girls with fragile X syndrome (FXS) suffering bullying in the role of a victim and its effects on their adaptive behavior, socialization style, and emotional state. A neuropsychological assessment was carried out on a sample of 40 participants (26 FXS positive and 14 control group) using the following instruments: WISC-V, SENA, BAS-2, ABAS-II. The results show that the group of girls with FXS presented higher ratios of lack of social support and isolation from classmates. This finding suggests that problems with social interaction and communication in the group of girls with FXS could lead to difficulties in interpreting social signals and identifying situations of bullying correctly, placing them in a very vulnerable situation.

Benzodiazepine Use Disorder and Cognitive Impairment in Older Patients: A Six-Month-Follow-Up Study in an Outpatient Unit in Barcelona.

OBJECTIVE: Adverse health effects including cognitive impairment have been described in older adults with benzodiazepine misuse, although the literature about this issue is scarce. The present study aimed to assess cognitive decline in older adults with benzodiazepine use disorder and changes in cognitive state at the 6-month follow-up, as well as whether patients achieved abstinence. METHOD: A 6-month follow-up longitudinal study was conducted in an outpatient drug center in Barcelona in a sample of older adults (≥65 years old) who had benzodiazepine use disorder. The sample was compared with an equivalent control group. A neuropsychological protocol was performed at baseline and after 6-month follow-up covering the most important cognitive domains. RESULTS: The final sample comprised 33 patients with an average age of 73.5 years. At baseline, patients presented impairment in several domains compared with the control group: visual immediate recall (p < .001), visual delayed recall (p < .001), copy (p < .001), working memory (p < .003), immediate verbal learning (p < .002), total words learned (p < .009), set switching (p < .001), verbal fluency (p < .007), speed processing (p < .002), solving problems (p < .006), nonverbal fluency (p < .004), and sustained attention in all three areas omissions (p < .001), variability (p < .001), and perseverance (p < .005). At 6-month follow-up, patients achieving abstinence showed improvement compared with patients in active consumption in visual delayed recall (p < .006), total words learned (p < .010), and verbal fluency (p < .013). CONCLUSIONS: Benzodiazepine misuse in older adults may produce negative effects on cognitive skills. Recovery of some of these cognitive deficits may be possible with benzodiazepine abstinence.

Sex differences in an old adult sample with substance use disorder: A 6 months follow-up study.

Substance use disorder (SUD) is a worldwide concern that has its own particularities regarding age and sex. This study aims to assess the differences between old SUD women and men regarding socio-demographics, clinical factors and outcomes. A 6-months follow-up longitudinal study was conducted in an outpatient center, on a convenience sample of 115 SUD old adults (≥65 years old, average age of 71.57). Descriptive, bivariate, and multivariate analyses were performed. Data showed statistical significant differences between men and women related to sociodemographic variables (marital status, coexistence, criminal records and stress factors), medical and psychiatric conditions (women suffer higher rates of depression and anxiety, with worse health-related quality of life), family records (women had more presence of family psychiatric records) and SUD related parameters (men tend to use more alcohol, had an early onset, consume higher doses, report more craving and more tobacco life use while women had higher rates of prescription drugs use). At 6-month follow-up, the whole sample showed excellent rates of adherence and abstinence, without sex differences. The study points out sex differences on several sociodemographic and clinical variables, indicating their specific needs. This research could facilitate better approaches by considering a sex perspective in SUD old adults.

Alcohol use disorder and cognitive impairment in old age patients: A 6 months follow-up study in an outpatient unit in Barcelona.

There has been little research about deleterious effects, including cognitive impairment, related to hazardous long-term alcohol use in old adults. This study aims to assess cognitive decline in old patients with alcohol use disorder and changes in cognitive state at 6 months follow-up, achieving or not abstinence. A six-month follow-up study was conducted in an outpatient center in Barcelona on a sample of old adults (≥65 years old) who had hazardous alcohol use. The sample was compared with healthy volunteers adjusted for age, sex and years of education. A neuropsychological protocol was performed at baseline and after 6 months follow-up covering four cognitive domains: attention, visuospatial abilities, memory and executive functions. Several domains were significant impaired at baseline: visual immediate and delayed recall, working memory, immediate verbal learning, total words learned, set switching and sustained attention. At 6 months reassessment, alcohol abstinence was achieved in 93.5% of patients and it was detected a trend towards improvement in direct mean scores of all cognitive areas, although it was not significant. The current study points out a cognitive impairment in many areas secondary to alcohol long-term hazardous use in old adults. A trend towards cognitive improvement after recovery was detected in most patients.

Are patients with bipolar disorder and comorbid attention-deficit hyperactivity disorder more neurocognitively impaired?

OBJECTIVE: Research on neurocognitive impairment in adult patients with comorbid bipolar disorder (BD) and attention-deficit hyperactivity disorder (ADHD) is very scarce. This study assessed the neurocognitive profile of a comorbid group (BD+ADHD) compared with that of pure BD (pBD) group, pure ADHD (pADHD) group and healthy controls (HCs). METHODS: This was a three-site study comprising 229 subjects: 70 patients with pBD, 23 with BD+ADHD, 50 with pADHD, and 86 HCs. All patients with BD had been euthymic for at least 6 months. Neuropsychological performance was assessed using a comprehensive neurocognitive battery. RESULTS: Our results showed that all the clinical groups had poorer performance than the HCs in all the neurocognitive domains except for executive functions. No significant differences were observed between the pBD and BD+ADHD groups in any of the cognitive domains, with these two groups showing greater impairment than the pADHD group in executive functions and visual memory. CONCLUSIONS: Our results, although preliminary, suggest that the BD+ADHD group showed the same neurocognitive profile as pBD patients, most likely reflecting the same neurobiological basis. On the other hand, the pADHD group showed a more selective moderate impairment in attention.

Long-Term Treatment with Citicoline Prevents Cognitive Decline and Predicts a Better Quality of Life after a First Ischemic Stroke.

Stroke, as the leading cause of physical disability and cognitive impairment, has a very significant impact on patients' quality of life (QoL). The objective of this study is to know the effect of citicoline treatment in Qol and cognitive performance in the long-term in patients with a first ischemic stroke. This is an open-label, randomized, parallel study of citicoline vs. usual treatment. All subjects were selected 6 weeks after suffering a first ischemic stroke and randomized into parallel arms. Neuropsychological evaluation was performed at 1 month, 6 months, 1 year and 2 years after stroke, and QoL was measured using the EuroQoL-5D questionnaire at 2 years. 163 patients were followed during 2 years. The mean age was 67.5 years-old, and 50.9% were women. Age and absence of citicoline treatment were independent predictors of both utility and poor quality of life. Patients with cognitive impairment had a poorer QoL at 2 years (0.55 vs. 0.66 in utility, p = 0.015). Citicoline treatment improved significantly cognitive status during follow-up (p = 0.005). In conclusion, treatment with long-term citicoline is associated with a better QoL and improves cognitive status 2 years after a first ischemic stroke.

Neuropsychological impairments in anorexia nervosa: a spanish sample pilot study.

This work was aimed at obtaining a profile of neuropsychological impairments in young Spanish participants with anorexia nervosa (AN) to demonstrate that right-hemisphere and frontal capacity impairments are present not only in the acute phase but also after weight recovery in a Spanish sample compared with a healthy control group. Twelve patients with AN in the acute phase (body mass index [BMI] < 17) were compared both to 16 healthy control subjects and 12 weight-recovered AN participants (BMI ≥ 17) matched by age, IQ, and educational level by utilizing a wide neuropsychological battery. Differences were found between AN groups only for long-term verbal memory, which worsens as BMI increases. Among participants with AN as a group, results showed differences in speed of information processing, working memory, visual memory, and inhibition, unrelated to attentional capabilities. We cannot support the hypothesis of a specific right cerebral dysfunction in patients with AN. A general cognitive dysfunction, primarily in information processing, working memory, visual and verbal memory, as well as frontal impairments such as impulsivity and poor behavioral control, appeared unrelated to BMI. We support previous works affirming that neuropsychological impairments in AN are not a consequence of the illness but a risk factor for it to develop.

Relationship between poor decision-making process and fatigue perception in Parkinson's disease patients.

BACKGROUND: Fatigue is a common non-motor symptom in Parkinson's disease patients. The reasons for its perception are not completely understood. One suggested possibility might be that perceived fatigue is related with abnormal interpretation of somatic symptoms. It has been described that somatic markers misinterpretation leads to poor decision-making. We hypothesized that fatigued Parkinson's disease patients would show poorer performance than non-fatigued in a decision-making task. METHODS: To test our hypothesis, 89 Parkinson's disease patients were assessed for the presence of fatigue using the Parkinson Fatigue Scale. All patients were also administered scales evaluating psychopathology and neuropsychological tests, including the Iowa Gambling Task. RESULTS: 33 (37.1%) patients fulfilled the established criteria for fatigue. In the univariate analysis, fatigued patients showed higher levels of anxiety (state: p = 0.001, trait: p < 0.001), impulsivity (p = 0.051), and depression (p < 0.001) than non-fatigued patients. No statistically significant differences in other neuropsychological test results (Stroop, Trail Making Test, Tower of London) were found between fatigued and non-fatigued patients except for the Iowa Gambling Task, in which fatigued patients showed poorer performance (p = 0.001) after controlling for confounding factors. CONCLUSIONS: These results suggest that fatigued Parkinson's disease patients may present abnormal decision-making process, which may reflect abnormal processing of somatic markers when faced with an activity that requires effort.

Driving and legal status of Spanish opioid-dependent patients.

BACKGROUND: Opioid dependent patients have legal problems, driving violations and accidents more frequently than the general population. We have hypothesized that those patients currently driving may have better legal outcomes than those who do not possess a valid driving license. With this aim we have analyzed the information gathered in the PROTEUS study regarding the legal and driving statuses and assessed the possible association between them. The PROTEUS study was an observational, cross-sectional, descriptive, multicenter nationwide representative study, conducted in Spanish healthcare centers for opioid dependent patients. FINDINGS: The driving and legal statuses of a population of opioid dependent patients ≥ 18 years and enrolled in Opioid Agonist Therapy treatment centers in Spain, were assessed using a short specific questionnaire and the EuropASI questionnaire to highlight distinct individual clinical needs. 621 patients were evaluable (84% men, 24.5% active workers). 321 patients (52%) drove on a regular basis. Nineteen percent of patients had some problem with the criminal justice system. There was a significant difference (p = 0.0433) in status, according to the criminal justice system, between patients who drove on a regular basis and those who did not, with a higher percentage of patients with non-pending charges among usual drivers. CONCLUSIONS: Regular drivers showed fewer legal problems than non-regular drivers, with the exception of those related to driving (driving violations and drunk driving). Driving is a good prognostic factor for the social integration of the patients and policies should be implemented to enable these patients to drive safely under medical authorization. The legal description will be useful to assess treatment efficacy.

Long-term treatment with citicoline may improve poststroke vascular cognitive impairment.

BACKGROUND: Cognitive decline after stroke is more common than stroke recurrence. Stroke doubles the risk of dementia and is a major contributor to vascular cognitive impairment and vascular dementia. Nonetheless, few pharmacological studies have addressed vascular cognitive impairment after stroke. We assessed the safety of long-term administration and its possible efficacy of citicoline in preventing poststroke cognitive decline in patients with first-ever ischemic stroke. METHODS: Open-label, randomized, parallel study of citicoline vs. usual treatment. All subjects were selected 6 weeks after suffering a qualifying stroke and randomized by age, gender, education and stroke type into parallel arms of citicoline (1 g/day) for 12 months vs. no citicoline (control group). Medical management was similar otherwise. All patients underwent neuropsychological evaluation at 1 month, 6 months and 1 year after stroke. Tests results were combined to give indexes of 6 neurocognitive domains: attention and executive function, memory, language, spatial perception, motor speed and temporal orientation. Using adjusted logistic regression models we determined the association between citicoline treatment and cognitive decline for each neurocognitive domain at 6 and 12 months. RESULTS: We recruited 347 subjects (mean age 67.2 years, 186 male (56.6%), mean education 5.7 years); 172 (49.6%) received citicoline for 12 months (no significant differences from controls n = 175). Demographic data, risk factors, initial stroke severity (NIHSS), clinical and etiological classification were similar in both groups. Only 37 subjects (10.7%) discontinued treatment (10.5% citicoline vs. 10.9% control) at 6 months; 30 (8.6%) due to death (16 (9.3%) citicoline vs. 14 (8.0%) control, p = 0.740), 7 lost to follow-up or incorrect treatment, and 4 (2.3%) had adverse events from citicoline without discontinuation. 199 patients underwent neuropsychological evaluation at 1 year. Cognitive functions improved 6 and 12 months after stroke in the entire group but in comparison with controls, citicoline-treated patients showed better outcome in attention-executive functions (OR 1.721, 95% CI 1.065-2.781, p = 0.027 at 6 months; OR 2.379, 95% CI 1.269-4.462, p = 0.007 at 12 months) and temporal orientation (OR 1.780, 95% CI 1.020-3.104, p = 0.042 at 6 months; OR 2.155, 95% CI 1.017-4.566, p = 0.045 at 12 months) during the follow-up. Moreover, citicoline group showed a better functional outcome (modified Rankin scale ≤2) at 12 months (57.3 vs. 48.7%) without statistically significant differences (p = 0.186). CONCLUSIONS: Citicoline treatment for 12 months in patients with first-ever ischemic stroke is safe and probably effective in improving poststroke cognitive decline. Citicoline appears to be a promising agent to improve recovery after stroke. Large clinical trials are needed to confirm the net benefit of this therapeutic approach.

Length of illness does not predict cognitive dysfunction in chronic fatigue syndrome.

Neuropsychological studies have shown cognitive impairment in chronic fatigue syndrome (CFS), particularly in information-processing speed. The aim of this study was to examine the evolution of cognitive impairment in CFS. The evolution is one of the most disabling aspects of the CFS, and it has received little attention in the literature. Fifty-six women with CFS were assessed with neuropsychological tests. Patients were divided into three groups based on the duration of the disease. There were no differences between groups in terms of cognitive function. The cognitive impairment in CFS was not found to be more severe with longer disease duration. These data suggest that there is no progressive cognitive impairment in patients with CFS. Therefore, the cognitive deficits in CFS should be treated with cognitive rehabilitation programs focused on improving emotional distress associated to the illness and on promoting functional abilities.

Effects of branched-chain amino acids supplementation in patients with cirrhosis and a previous episode of hepatic encephalopathy: a randomized study.

OBJECTIVES: Protein intake impacts on nutritional status and may determine the recurrence of hepatic encephalopathy (HE). A low-protein diet has been considered the standard treatment after an episode of HE, while branched-chain amino acids (BCAA) have been shown to improve minimal HE. We performed a study to investigate the long-term effects of supplementing a protein-controlled diet with BCAA. METHODS: A randomized, double-blind, multicenter study that included 116 patients with cirrhosis and a previous episode of HE was conducted in four tertiary care hospitals. All patients received a standard diet of 35 kcal/kg per day and 0.7 g of proteins/kg per day and a supplement of 30 g of BCAA (BCAA group) or maltodextrin (MDX group) during 56 weeks. RESULTS: The actuarial risk of remaining free of HE did not differ between groups (BCAA=47%, MDX=34%, P=0.274), but patients in the BCAA group exhibited a better outcome on two neuropsychological tests and an increase in the mid-arm muscle circumference. Recurrence was associated with low plasma albumin at baseline and a decrease in sodium and an increase in creatinine during follow-up. Patients with recurrence of HE exhibited a lack of improvement in global cognitive function. CONCLUSIONS: Diet supplementation with BCAA after an episode of HE does not decrease recurrence of HE. However, supplementation with BCAA improves minimal HE and muscle mass. Identification of risk factors for recurrence of HE may allow the development of new preventive therapies that could decrease the neuropsychological sequelae of repeated episodes of HE.

Hepatic encephalopathy is associated with posttransplant cognitive function and brain volume.

Hepatic encephalopathy (HE) is a common complication of cirrhosis that is associated with brain atrophy and may participate in impaired cognitive function after liver transplantation. This study analyzes the relationship of HE with cognitive function and brain volume after transplantation. A total of 52 consecutive patients with cirrhosis (24 alcohol abuse, 24 prior HE, 14 diabetes mellitus) completed a neuropsychological assessment before liver transplantation and again, 6 to 12 months after transplantation. In 24 patients who underwent the posttransplant assessment, magnetic resonance imaging was performed in addition, with measurement of brain volume and relative concentration of N-acetylaspartate (NAA) and creatine/phosphocreatine (Cr), a neuronal marker, by magnetic resonance spectroscopy. Neuropsychological assessment prior to transplantation identified minimal HE in 28 patients. All cognitive indexes improved after liver transplantation, but 7 patients (13%) showed persistent mild cognitive impairment. Global cognitive function after transplantation was poorer in patients with the following variables before liver transplantation: alcohol etiology, diabetes mellitus, and HE. Brain volume after transplantation was smaller in patients with prior HE. Brain volume correlated to NAA/Cr values (r = 0.498, P = 0.013) and poor motor function (r = 0.41, P = 0.049). In conclusion, the association of HE with cognitive function and brain volume suggests that having experienced HE before liver transplantation impairs the posttransplantation neurological outcome.

SPECT imaging for brain improvement quantification in a patient with cerebrotendinous xanthomatosis.

Cerebrotendinous xanthomatosis is a rare recessive autosomal disease caused by mutations of the sterol 27-hydroxylase gene (CYP27), which leads to reduced synthesis of bile acids, particularly chenodeoxycholic acid (Cali et al, J Biol Chem. 1991;266:7779-7783; Gallus et al, Neurol Sci. 2006;27:143-149). The disease is characterized by progressive neurologic dysfunction due to accumulation of cholestanol in neurologic tissues (Moghadasian et al, Arch Neurol. 2002;59:527-529; Selva-O'Callaghan et al, Rheumatology. 2007;46:1212-1213). Long-term treatment with chenodeoxycholic acid can arrest or even reverse progression of the disease (Pierre et al, J Inherit Metab Dis. In press).Brain SPECT with 740 MBq of Tc-99m ethyl cysteinate dimmer, using a double-head gamma camera (Siemens E.cam) with high-resolution, low-energy parallel collimators was performed in our patient at onset and 2 years after starting chenodeoxycholic acid treatment. SPECT acquisitions were performed using a 360-degree orbit, 1 image/30 seg/3 degree, and 128 × 128 matrix. Reconstruction was by means of filtered back-projection, Butterworth 5/0.25, without attenuation correction. Pre- and post-SPECT dicom images were reoriented into Talairach space using NeuroGam (Segami Corporation). To visually identify abnormal perfusion regions, volume render brain image was computed, where abnormal perfusion regions were found by comparing with age-matched normal database, and Brodmann areas (BA) were quantified. Pre- versus post-treatment changes were computed by means of relative percentage between counts. Post-treatment SPECT showed better perfusion than pretreatment SPECT with an increase between 5% and 10% in frontal cortex (BA 9, BA 24, BA 32, BA 46, BA 47), parietal cortex (BA 5, BA 31), and temporal cortex (BA 20, BA 22, BA 28, BA 36, BA 37, BA 38), and with an increase of more than 10% in frontal cortex (BA 45) and parietal cortex (BA 23). This case illustrates the benefit of bile acid therapy for halting and even reversing neurologic retardation in this condition.

[The role of depression in cognitive impairment in patients with chronic fatigue syndrome]. / El rol de la depresión en el déficit cognitivo del paciente con síndrome de fatiga crónica.

BACKGROUND AND OBJECTIVE: To analyze the role of depression in cognitive deficits of patients with chronic fatigue syndrome (CFS). PATIENTS AND METHODS: 57 women with CFS were assessed by neuropsychological tests that included measures of attention: CalCap, Mental control of the WMS-III, PASAT, forward and backward digits (WAIS-III), symbol digit modalities test (SDMT); executive functions: Stroop Test, Trail Making Test (TMT A y B), FAS, Tower of London; memory: Auditory-Verbal Learning Test (AVL), Rey Complex Figure (RCF), and psychomotor skills: Grooved Pegboard. The raw scores on the tests were adjusted according to normative data and transformed to T scores. The sample was divided into two groups based on the presence or absence of depression, assessed by clinical interview and administration of the Hospital Anxiety and Depression Scale (HADS). This study compared neuropsychological test scores between the two groups. RESULTS: CFS patients showed cognitive deficit in attention and executive functions, regardless of the presence of depression. There were no significant differences between the two CFS groups. CONCLUSIONS: The cognitive impairments in patients with CFS are not secondary to the presence of depression. These results should be taken into account in the implementation of therapeutic programs in these patients.