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BACKGROUND: Physical fatigue is one of the most disabling symptoms in people with Multiple Sclerosis (PwMS). Several factors might influence the development of fatigue, such as gender, education, body mass index (BMI), Expanded Disability Status Scale (EDSS), disease duration, working status (Ws), physiotherapy (Ph), and disease-modifying therapies (DMTs). Fatigue Symptoms and Impacts Questionnaire-Relapsing Multiple Sclerosis (FSIQ-RMS) is a patient-reported outcome (PRO) that allows one to define the impact of fatigue in PwMS clearly. This study aimed to assess fatigue impact on PwMS by using FSIQ-RMS. METHODS: The participants were enrolled from May to July 2021 in MS Centers of Sant'Andrea Hospital and Policlinico Umberto I Hospital in Rome. Fatigue was evaluated using the FSIQ-RMS, validated, and culturally adapted in Italian. Clinical and demographic data were collected at the same time. RESULTS: We enrolled 178 PwMS [Female 74.16%; RMS 82.58%, SPMS 17.52%]. FSIQ-RMS scores were significantly correlated with EDSS (p-value < 0.01). Analysis of variance between means showed a statistically significant difference between the BMI groups at the 24hours_FSIQ-RMS score and the 7days_FSIQ-RMS score (p < 0.01), with the lower BMI group having the highest scores. Furthermore, perceived fatigue significantly improved both in subjects performing Ph (p < 0.05) and in those who actively work (p < 0.01). CONCLUSIONS: The use of FSIQ-RMS in a real-world setting confirmed that underweight and high levels of disability are closely related to fatigue. In addition, Ph and active Ws are strongly correlated with fatigue in PwMS.

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BACKGROUND: We assessed the relationship between the cognitive development of children and adolescents with phenylketonuria (PKU) and fluctuations in peripheral phenylalanine (Phe) levels. METHODS: We examined the neurocognitive performance of 33 children and adolescents with early treated PKU, of whom 18 were treated with sapropterin dihydrochloride, and 15 were on a classic diet. For 26 weeks, patients were assessed weekly for their blood phenylalanine (Phe) levels. Phe levels were analyzed for fluctuations indicated by the individual standard deviation. Fluctuations were compared to the standard deviation of 26 Phe level measurements before the study interval. We also assessed the concurrent IQ of the patients. This was repeated at one-, two-, and seven-year intervals. RESULTS: Full-scale IQ in patients treated with a classic diet did not change within the follow-up. In patients treated with Sapropterin dihydrochloride, however, there was a considerable gain in full-scale IQ. This was particularly true if blood Phe fluctuations increased in patients of this treatment group. CONCLUSIONS: Sapropterin dihydrochloride enhances Phe tolerance in patients with PKU. Increasing blood Phe fluctuations following enhanced Phe tolerance may indicate that the treatment not only allows patients to relax their Phe-restricted diet but also may support cognitive development in patients.

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The relationship between the gut microbiota and neurocognitive outcomes is becoming increasingly recognized; however, findings in humans are inconsistent. In addition, few studies have investigated the gut microbial metabolites that may mediate this relationship. The objective of this study was to investigate associations between full-scale intelligence (FSIQ) and the composition of the gut microbiota and metabolome in preschool children. Stool samples were collected from a community sample of 245 typically developing children (3-5 years) from the Alberta Pregnancy Outcomes and Nutrition (APrON) cohort. The faecal microbiome was assessed using 16S rRNA sequencing and the metabolome using LC-MS/MS. FSIQ and scores on the Verbal Comprehension, Visual Spatial, Working Memory indices of the Wechsler Preschool and Primary Scale of Intelligence-IV were used to assess neurocognition. Associations between the gut microbiota and FSIQ were determined using Pearson and Spearman correlations, which were corrected for multiple testing and relevant covariates. Verbal Comprehension correlated negatively with both Shannon alpha diversity (r = -0.14, p = 0.032) and the caffeine-derived metabolite paraxanthine (r = -0.22, p < 0.001). No other significant correlations were observed. Overall, the weak to modest correlations between Verbal Comprehension with alpha diversity and paraxanthine provide limited evidence of an association between the gut microbiota and neurocognitive outcomes in typically developing preschool children.

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Eating disorders have been shown to be associated with epilepsy, typically associated with the temporal lobe and usually of non-dominant hemisphere origin. We report the case of a 56-year-old woman with drug resistant epilepsy, localized to the dominant left hippocampus. She experienced an increasing frequency of seizures over a two-year period associated with loss of appetite and substantial weight loss independent of antiseizure medication changes. Extensive workup eliminated gastrointestinal and paraneoplastic etiologies. There was no history of psychiatric illness, including anorexia nervosa. Pre-surgical workup showed mesial temporal sclerosis on MRI and video-EEG was consistent with ipsilateral medial temporal seizure onset. The patient underwent laser interstitial ablation of the left amygdala and hippocampus, which resulted in a cessation of seizures. Within 24 h of the laser ablation, her appetite returned to normal and, within 8 months she regained 26 lbs. To our knowledge, this is the first case report of a patient with dominant temporal lobe epilepsy with anorexia that was temporally associated with escalating seizure frequency and stopped with treatment and cessation of seizures, suggesting a causal and pathogenic relationship.

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Background: The fragile X premutation carrier state (PM) (55-200 CGG repeats in the fragile X messenger ribonucleoprotein 1, FMR1 gene) is associated with several conditions, including fragile X-associated primary ovarian insufficiency (FXPOI) and fragile X-associated tremor ataxia (FXTAS), with current literature largely primarily investigating older PM individuals. The aim of this study was to identify the prevalence of fragile X-associated neurodevelopmental disorders (FXAND) in a sample of young PM individuals. Methods: This was a retrospective study conducted through a medical record review of PM individuals who were seen either for clinical concerns (probands, 45.9%) or identified through the cascade testing (non-probands, 54.1%) of an affected sibling with fragile X syndrome. Information on the presence of autism spectrum disorder, attention deficit hyperactivity disorder, anxiety, depression, long-term psychiatric medication intake, and cognitive function, based on standardized assessments, was obtained. Molecular data, including CGG repeat number and FMR1 mRNA levels, were also available for a subset of participants. Analysis included descriptive statistics and a test of comparison to describe the clinical profile of PM individuals pertinent to FXAND. Results: Participants included 61 individuals (52 males and 9 females) aged 7.8 to 20.0 years (mean 12.6 ± 3.4) with a mean full-scale IQ of 90.9 ± 22.7. The majority (N = 52; 85.2%) had at least one mental health disorder, with anxiety being the most common (82.0% of subjects), followed by ADHD (66.5%), and ASD (32.8%). Twenty-seven (87.1%) of non-probands also had at least one mental health condition, with probands having lower cognitive and adaptive skills than non-probands. ASD was present in 20 participants (17/52 males and 3/9 females; 15 probands) with significantly lower FSIQ in those with ASD (mean 73.5 vs. 98.0, p < 0.001). Participants with ASD had a higher number of long-term medications compared to those without (2.32 vs. 1.3, p = 0.002). Conclusions: Our findings indicate a high rate of FXAND diagnoses within a cohort of young PM individuals, including those identified via cascade testing, although this was not a population sample. An awareness of the entity of FXAND and the early recognition of the symptoms of associated conditions may facilitate timely and appropriate care for PM individuals.

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Learning and intellectual disabilities are hallmark features of 22q11.2 deletion syndrome. Data are limited, however, regarding influences on full-scale IQ (FSIQ). Here, we investigated possible 22q11.2 deletion parent-of-origin effects. In 535 individuals, we compared FSIQ (≥50), 481 with de novo and 54 with inherited 22q11.2 deletions. In the subsets with data available, we examined parent-of-origin effects on FSIQ. We used linear regression models to account for covariates. Median FSIQ was significantly higher in de novo vs. inherited deletions (77; range 50−116 vs. 67; range 50−96, p < 0.0001). Results remained significant using a regression model accounting for age at IQ testing, sex and cohort site. No significant parent-of-origin differences in FSIQ were observed for de novo deletions (n = 81, 63.0% maternal; p = 0.6882). However, median FSIQ was significantly lower in maternally than in paternally inherited familial deletions (65, range 50−86 vs. 71.5, range 58−96, respectively, p = 0.0350), with the regression model indicating an ~8 point decrement in FSIQ for this variable (p = 0.0061). FSIQ is higher on average in de novo than in inherited 22q11.2 deletions, regardless of parental origin. However, parent-of-origin appears relevant in inherited deletions. The results have potential clinical implications with further research needed to delineate possible actionable mechanisms.

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Long-term neurological and neurodevelopmental sequelae are a concerning issue for people with Enterovirus A71 (EV-A71) central nervous system (CNS) infection. Unfortunately, no longitudinal prospective clinical study has systematically investigated the consequences of EV-A71 CNS infection during early life on the later development of other psychiatric disorders. In this naturalistic longitudinal follow-up design, we followed forty-three youth, who got EV-A71 CNS involvement 6-18 years ago and were enrolled in other EV-A71 clinical studies then. Their psychiatric presentation, emotional/behavioral problems, and cognitive issues were examined using a psychiatrist-conducted diagnostic interview, parent- and self-rated questionnaires, and neuropsychological tests, respectively. We compared the prevalence of psychiatric disorders in youth with EV-A71 CNS involvement to a nationally representative cohort. Emotion/behavior and cognition in EV-A71-CNS-infected youth were compared to those in a matched community-based sample of healthy controls and youth with attention-deficit/hyperactivity disorder (ADHD). Compared to a national sample (absolute ADHD prevalence 10.1%), youth with EV-A71 CNS involvement had three times the odds of receiving an ADHD diagnosis (standardized prevalence ratio, 95% CI = 1.8, 4.2; absolute ADHD prevalence 34.9%). No other psychiatric diagnoses were more common in EV-A71-CNS-infected youth. Compared to community-based ADHD youth, EV-A71-CNS-infected youth with psychiatric disorders showed comparable core ADHD symptoms, opposition/defiance, autistic features, and suboptimal sustained attention performance (based on the Conners' Continuous Performance Test), all of which were more severe than healthy controls. EV-A71-CNS-infected youth without psychiatric disorders showed comparable autistic features to EV-A71-CNS-infected youth with psychiatric disorders and ADHD youth. EV-A71 CNS involvement may cause long-term, adverse psychiatric outcomes that develop into an ADHD diagnosis alongside social/communication/emotion problems and autistic features. We recommend earlier identification and intervention of these problems among these children.

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BACKGROUND: Fatigue is a disabling symptom of multiple sclerosis (MS) and impacts on daily life. The Fatigue Symptoms and Impacts Questionnaire-Relapsing Multiple Sclerosis (FSIQ-RMS) is a new 20-item tool that investigates the aspects of fatigue in MS. It concerns motor, cognitive, social, and emotional aspects of fatigue. We aim at validating the Italian version of FSIQ-RMS in an Italian population of MS patients and demonstrate its reliability and construct validity. METHODS: We included patients with diagnosis of MS, age between 18 and 70 years with ability to participate in a 90-min interview. Each patient completed the Italian version of FSIQ-RMS and Fatigue Severity Scale (FSS) at the same time. Construct validity was explored by the exploratory factor analysis; reliability was assessed with Cronbach's alpha; and test-retest stability was examined through intraclass correlation coefficient (ICC). Concurrent validity was calculated using Pearson's correlation. RESULTS: We enrolled 171 patients (126 female and 45 male), 83% with relapsing MS (RMS), and 17% with secondary progressive MS (SPMS). Italian FSIQ-RMS showed a Cronbach's alpha of 0.92; ICC was 0.96. Pearson's correlation coefficient between FSIQ-RMS and FSS total score was statistically significant (p < 0.01); FSIQ-RMS inversely correlated also with BMI and positively with EDSS. CONCLUSION: The Italian version of FSIQ-RMS has excellent psychometric properties and can be used in research and clinical setting to evaluate physical, cognitive, and social fatigue in both RMS and SPMS.

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INTRODUCTION: Fatigue is one of the most common and debilitating symptoms of multiple sclerosis (MS) but is challenging to assess and has not been comprehensively characterized in patients with progressive MS. This study aimed to (1) obtain qualitative evidence from patients with progressive MS to characterize MS-related fatigue concepts and their impacts on health-related quality of life (HRQoL), and (2) evaluate the conceptual frameworks of existing MS-specific fatigue patient-reported outcome (PRO) instruments using study data to determine the most suitable PRO instrument in this population. METHODS: Qualitative interviews were conducted with 30 US participants with confirmed progressive MS and fatigue in the last 6 months to assess their MS-related fatigue. Data were compared with concepts in existing PRO instruments to evaluate their relevance in progressive MS. RESULTS: Physical and mental concepts of fatigue were identified and characterized distinctly from patients with progressive MS. Most patients characterized fatigue as occurring daily and lasting several hours, with negative impacts on HRQoL. Concept mapping to existing MS-specific fatigue PRO instruments supported the Fatigue Severity Impact Questionnaire-Relapsing Multiple Sclerosis (FSIQ-RMS) as the most suitable existing option for assessing fatigue in patients with progressive MS, as it separates physical and mental aspects of fatigue and includes every highly endorsed concept reported by the interviewed patients. CONCLUSIONS: This qualitative study identified meaningful physical and mental fatigue concepts in patients with progressive MS and preliminarily supports the use of the FSIQ-RMS for this population. More research is needed to fully validate this instrument for progressive MS.

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BACKGROUND: Fatigue is among the most frequent and disabling symptoms in patients with relapsing multiple sclerosis (RMS). OBJECTIVE: To measure MS fatigue and its impact on daily life in a real-world US population using an MS-specific patient-reported outcome (PRO) instrument, the Fatigue Symptoms and Impacts Questionnaire-RMS (FSIQ-RMS). METHODS: This ongoing prospective study recruited RMS patients from an online patient community (Carenity) across US. Baseline assessment data are reported. Participants completed questionnaires, including the 20-item FSIQ-RMS questionnaire, with the first seven symptom-related items collected daily for seven days, and the other 13 items on the seventh day assessing impacts of fatigue. The FSIQ-RMS scores range from 0 to 100 (higher score=greater severity). The impact of fatigue on several aspects of patients' lives was rated from 0 (no impact) to 10 (very high impact). Data on disease history, disease status, sleep, social and emotional functioning were also captured. Baseline assessment data of 300 RMS patients are reported while follow-up assessments up to 18 months are planned. RESULTS: 300 RMS participants completed the 7-day assessment (mean age 43.0 years, 88% women). Fatigue was rated as severe, with a mean score of 57.3 for the FSIQ-RMS symptom domain; 3 impact sub-domain scores were 42.3, 43.4 and 50.1 (physical, cognitive/emotional, and coping). Participants who were not in relapse (78%) reported less severe fatigue than those in relapse (22%): mean±SD symptom score of 54.6 ± 17.8 vs. 67.0 ± 19.7, p< 0.001. Fatigue had a higher intensity among those with depression than without (49% vs. 51%, with mean ± SD symptom score of 62.8 ± 16.9 vs. 52.1 ± 19.3, p< 0.001), and among those with sleep disorder than without (27% vs. 73%, 61.2 ± 19.2 vs. 55.9 ± 18.6; p< 0.05). The most common factor associated with increased fatigue was heat exposure (82%). Most participants (52%) reported experiencing fatigue before their MS diagnosis. CONCLUSION: Fatigue influences daily functioning for most patients with RMS. The FSIQ-RMS is a novel and MS-specific PRO measure that can advance the understanding and management of fatigue.

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Objective: This study examined premorbid ability estimate concordance using Test of Premorbid Functioning predicted Full Scale Intelligent Quotient (TOPF-IQ) and Wide Range Achievement Test-Fourth Edition Word Reading (WRAT4-WR). Method: The sample (N = 145) was 28% female with average age and education of 40.6 and 13.2 years, respectively. Outpatient neuropsychological evaluations were conducted in a rehabilitation setting. Measures included the TOPF, WRAT4-WR, Wechsler Adult Intelligence Scale-Fourth Edition, and other neuropsychological tests. Non-WAIS measures defined impairment groups. Analyses included t tests, pairwise correlations, concordance correlation coefficients, and root mean square differences. Results: TOPF-IQ, WRAT4-WR, and Full Scale Intelligent Quotient scores were not significantly different but were lower than normative mean. TOPF-IQ and WRAT4-WR showed acceptable agreement (concordance correlation coefficient = .92; root mean square difference = 5.9). Greater premorbid-current ability differences were observed in the impaired group. TOPF-IQ and WRAT4-WR showed lower but similar agreement with Full Scale Intelligence Quotient in the unimpaired group. Conclusions: Findings support the WRAT4-WR in predicting premorbid ability in rehabilitation settings.

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BACKGROUND: To investigate intellectual development after functional hemispherectomy and to evaluate the favorable or unfavorable factors affecting prognosis. METHODS: Twenty-three patients from the patient follow-up database of the Epilepsy Center of Neurosurgery were selected for this retrospective study. The inclusion criteria of the patients were functional hemispherectomy surgery, surgical age older than 6 years, patients able to complete the intelligence assessment, follow-up time not less than 2 years, and availability of complete preoperative evaluation and postoperative follow-up data. Full Scale Intelligence Quotients (FSIQ) were calculated preoperatively and at the follow-up times for each patient. Correlations between the preoperative to postoperative changes in FSIQ and risk factors (age at epilepsy onset, duration of preoperative epilepsy, postoperative changes in the electroencephalogram, seizure status after surgery, and follow-up time) were analyzed. EpiData 3.1 software was used to establish a database and SPSS 22.0 software was used for data collation and analysis, and used the matching sample Wilcoxon symbol rank and test to conduct the analysis. RESULTS: The data of 23 patients were analyzed. The final follow-up results showed that 73.9% of the patients (17/23) had a significant increase in FSIQ scores (P = 0.006) after surgery, indicating that their cognitive function improved significantly. Successful surgery, successful postoperative seizure control, normalization of postoperative electroencephalogram, shorter disease duration before surgery, and longer recovery periods after surgery (follow-up time) were all related with good intellectual prognosis. CONCLUSIONS: Successful functional hemispherectomy significantly improved the cognitive function of patients. Functional hemispherectomy is a safe and effective treatment for patients with intractable epilepsy caused by hemispheric lesions and can effectively promote intellectual recovery and development.

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OBJECTIVE: High survival rates have led to an increased emphasis on the functional outcomes of children diagnosed with low-grade glioma. Most outcomes research has focused on risks associated with radiotherapy, but less is known about neuropsychological risks for patients treated with surgery alone. Here, the authors sought to examine the neuropsychological trajectories of children diagnosed with a low-grade glioma and monitored up to 6 years postsurgery. Secondarily, they explored demographic and clinical predictors of neuropsychological performance. METHODS: The neuropsychological functioning of 32 patients (median age at diagnosis 10.0 years) was prospectively assessed annually for up to 6 years after surgery (median days from surgery at baseline = 72). Tumor location was predominately supratentorial (65.6%). A combination of performance-based and parent-reported measures was used to assess intelligence, memory, executive functioning, and fine motor control in all patients. RESULTS: Binomial tests at the postoperative baseline revealed that the proportion of children falling below the average range (< 16th percentile) was significantly higher than the rate expected among healthy peers on measures of verbal memory, processing speed, executive functioning, and fine motor control (p < 0.05). Even so, linear mixed models indicated that neuropsychological functioning at the postoperative baseline did not significantly change over time for up to 6 years after surgery across all domains. A larger tumor size was associated with a slower reaction time (p < 0.01). A supratentorial tumor location and history of seizures were associated with more parent-reported executive difficulties (p < 0.01). CONCLUSIONS: While radiotherapy is a known risk factor for neuropsychological deficits in pediatric brain tumor patients, findings in this study indicate that children treated for low-grade glioma with surgery alone (without radiotherapy or chemotherapy) remain susceptible to difficulties with memory, executive functioning, and motor functioning that persist over time. Over half of the children in the study sample required school support services to address neuropsychological weaknesses. Although low-grade glioma is often conceptualized as a benign tumor, children treated for this lesion require ongoing monitoring and intervention to address neuropsychological weaknesses resulting from the tumor itself as well as the surgery.

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OBJECTIVE: This study investigated the auditory sensory-perceptual level of specific learning disorder (SLD) and explored relationships among neuropsychological assessments for SLD, auditory processing, and short and long latencies of auditory event-related potentials (ERPs). METHODS: Fifteen children (7-14 years old) comprised the control group; 34 children comprised the SLD group. Audiologic assessments included tone audiometry, acoustic immittance measurements, acoustic reflex, central auditory processing, brainstem evoked response audiometry, and long latency potentials (P3 and N2). Children's intelligence levels were assessed with 2 intelligence batteries, 1 verbal and 1 non-verbal, as well as with visuomotor skills. RESULTS: Multiple regression showed a significant interaction effect of APE tests and P3/N2 over Wechsler Scale performance in freedom of distractibility indexes and multiple subtests. Errors in the Bender Visual Motor Gestalt Test were predicted by lower parental education, lower performance in APE tests: dichotic digits and pediatric/synthetic sentence identification-ipsilateral, and longer P3/N2 latencies, particularly regarding integration and rotation distortions. CONCLUSIONS: Children with altered auditory processing exhibit a specific cognitive profile, including lower verbal and spatial reasoning performance, that is sensitive to parental education level. SIGNIFICANCE: Children with SLD should undergo a complete multimodal examination to identify their specific difficulties and needs.

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The associations between growth during early life and subsequent cognitive development and physical outcomes are not widely known in low-resource settings. We examined postnatal weight and height gain through early life and related these measurements to the nutritional status and intellectual development of the same children when they were between 7 and 9 years old. Mothers had enrolled in an randomised controlled trial to evaluate the effect of prenatal micronutrient supplementation on birth weight. Their children were born in 2004, their height and weight were measured at 6, 12, 18 and 24 months of age and were followed up between October 2012 and September 2013 (at ages 7-9 years, n 650). Height-for-age, weight-for-age and BMI-for-age were used to describe the nutritional status, and the Wechsler Intelligence Scale for Children fourth edition was used to measure the intellectual function. Multilevel linear and logistic modelling was used to estimate the association between early growth and subsequent growth and intellectual function. After adjustment, weight gain from 6 to 12 months of age was associated with Full-scale Intelligence Quotient, Verbal Comprehension Index, Working Memory Index and Perceptual Reasoning Index. Weight gain during early life was associated with subsequent nutritional status. For every 1 kg increase in weight during the 0- to 6-month period, the OR for underweight, thinness and stunting at 7-9 years of age were 0·19 (95 % CI 0·09, 0·37), 0·34 (95 % CI 0·19, 0·59) and 0·40 (95 % CI 0·19, 0·83), respectively. Weight gain during the periods of 6-12 months of age and 18-24 months of age was also associated with a lower risk of being underweight. Weight gain during early life was associated with better growth outcomes and improved intellectual development in young school-aged children.

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OBJECTIVE: The aim of this study was to ascertain whether age at surgery has an impact on later neurodevelopmental outcomes for children with sagittal synostosis (SS). METHODS: The developmental outcome data from patients who had surgery for SS and who attended their routine preoperative, 6-7 months postoperative, and 5-year-old developmental assessments (yielding general quotients [GQs]) (n = 50), 10-year-old IQ assessment (n = 54), and 15-year-old IQ assessment (n = 23) were examined, comparing whether they had surgery at < 7 months, 7 to < 12 months, or ≥ 12 months). RESULTS: There was no significant effect for age at surgery for GQ at 5 years of age, but there was a significant effect (p = 0.0001) for those undergoing surgery at < 7 months in terms of preoperative gross locomotor deficit that resolved by 6-7 months postoperatively (increase of 22.1 points), and had further improved by 5 years of age (total increase of 29.4 points). This effect was lessened when surgery was performed later (total increase of 7.3 points when surgery was performed at ≥ 12 months). At 10 years of age, 1-way ANOVA showed a significant difference in Full Scale IQ (FSIQ) score (p = 0.013), with the highest mean FSIQ being obtained when surgery was performed at < 7 months of age (score 107.0), followed by surgery at 7 to < 12 months (score 94.4), and the lowest when surgery was performed at ≥ 12 months (score 93.6). One-way ANOVA for the Performance IQ (PIQ) was very similar (p = 0.012), with PIQ scores of 101.4, 91.4, and 87.3, respectively. One-way ANOVA for Verbal IQ (VIQ) was again significant (p = 0.05), with VIQ scores of 111.3, 98.9, and 100.4, respectively. At 15 years, 1-way ANOVA showed a significant difference in PIQ (p = 0.006), with the highest mean PIQ being obtained when surgery was performed at < 7 months (score 104.8), followed by surgery at 7 to < 12 months (score 90.0), and the lowest when surgery was at performed at ≥ 12 months of age (score 85.3). There were no significant results for FSIQ and VIQ, although there was a similar trend for better outcomes with early surgery. CONCLUSIONS: The findings of this study add to the literature that suggests that early surgery for SS may result in improved neurodevelopmental outcomes, with surgery optimally undertaken when patients are < 7 months of age, and with those undergoing surgery at ≥ 12 months performing the least well. These results also have potential implications for ensuring early diagnosis and referral and for the type of surgery offered. Further research is needed to control for confounding factors and to identify the mechanism by which late surgery may be associated with poorer neurodevelopmental outcomes.

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OBJECTIVEThe goal of this study was to clarify the long-term outcome of endoscopic third ventriculostomy (ETV) in pediatric hydrocephalus in light of the ETV Success Score (ETVSS), shunt dependency, and intellectual development.METHODSThe authors retrospectively analyzed pediatric patients with hydrocephalus who underwent ETV between 2002 and 2012 and who were followed for longer than 5 years as a single-center cohort. The data of the patients' pre- and postoperative status were collected. The relationships between ETVSS and the full-scale IQ as well as shunt dependency were analyzed. The usefulness of ETVSS for repeat ETV and the change of radiological parameters of ventricle size before and after ETV were also analyzed. The success of ETV was defined as no requirement for further CSF diversion procedures.RESULTSFifty ETVs were performed in 40 patients. The average ETVSS was 61 and the success rate at 6 months was 64%. The mean follow-up was 9.9 years (5.2-15.3 years), and the long-term success rate of ETV was 50%. The Kaplan-Meier survival curve continued to show a statistically significant difference among patients with a low, moderate, and high ETVSS, even after 6 months (p = 0.002). After 15 months from the initial ETV, no patients required additional CSF diversion surgery. There was no statistical significance between ETVSS and the long-term full-scale IQ or shunt dependency (p = 0.34 and 0.12, respectively). The radiological improvement in ventricle size was not associated with better future educational outcome.CONCLUSIONSThe ETVSS was correlated with the long-term success rate. After 15 months from the initial ETV, no patients required an additional CSF diversion procedure. The ETVSS was not considered to be correlated with long-term intellectual status.

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OBJECTIVEPituitary adenomas and the treatment required for the underlying neuropathology have frequently been associated with cognitive dysfunction. However, the mechanisms for these impairments remain the subject of much debate. The authors evaluated cognitive outcomes in patients treated with or without Gamma Knife radiosurgery (GKRS) for an underlying pituitary adenoma.METHODSThis was a retrospective, institutional review board-approved, single-institution study. A total of 51 patients (23 male, 28 female) treated for pituitary adenoma were included in this neurocognitive study. Twenty-one patients underwent GKRS following transsphenoidal surgery, 22 patients were treated with transsphenoidal surgery alone, and eight patients were conservatively managed or were treated with medical management alone. Comparisons using psychometric tests of general intellectual abilities, memory, and executive functions were made between the treatment groups, between male and female patients, and between patients with Cushing's disease and those with nonfunctioning adenoma (NFA).RESULTSThe entire patient sample, the NFA group, and the GKRS group scored significantly below expected on measures of both immediate and delayed memory, particularly for visually presented information (p ≤ 0.05); however, there were no significant differences between the patients with Cushing's disease and those with NFA (t ≤ 0.56, p ≥ 0.52). In those who underwent GKRS, memory scores were not significantly different from those in the patients who did not undergo GKRS (t ≤ 1.32, p ≥ 0.19). Male patients across the sample were more likely to demonstrate impairments in both immediate memory (t = -3.41, p = 0.003) and delayed memory (t = -3.80, p = 0.001) than were female patients (t ≤ 1.09, p ≥ 0.29). There were no impairments on measures of general intellectual functioning or executive functions in any patient group. The potential contributions of tumor size and hormone levels are discussed.CONCLUSIONSOverall, pituitary adenoma patients demonstrated relative impairment in anterograde memory. However, GKRS did not lead to adverse effects for immediate or delayed memory in pituitary adenoma patients. Cognitive assessment of pituitary adenoma patients is important in their longitudinal care.

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OBJECTIVE: How the brain supports intermediate-term preservation of memory in patients who have undergone unilateral medial temporal lobe resection (MTLR) has not yet been demonstrated. To understand the neural basis of episodic memory in the intermediate term after surgery for temporal lobe epilepsy (TLE), the authors investigated the relationship between the activation of the hippocampus (HIP) during successful memory encoding and individual memory capacity in patients who had undergone MTLR. They also compared hippocampal activation with other parameters, including structural volumes of the HIP, duration of illness, and age at seizure onset. METHODS: Thirty-five adult patients who had undergone unilateral MTLR at least 1 year before recruiting and who had a favorable seizure outcome were enrolled (17 left MTLR, 18 right MTLR; mean follow-up 6.31 ± 2.72 years). All patients underwent a standardized neuropsychological examination of memory function and functional MRI scanning with a memory-encoding paradigm of words and figures. Activations of the HIP during successful memory encoding were calculated and compared with standard neuropsychological memory scores, hippocampal volumes, and other clinical variables. RESULTS: Greater activation in the HIP contralateral to the side of the resection was related to higher postoperative memory scores and greater postoperative memory improvement than the preoperative baseline in both patient groups. Specifically, postoperative verbal memory performance was positively correlated with contralateral right hippocampal activation during word encoding in the left-sided surgery group. In contrast, postoperative visual memory performance was positively correlated with contralateral left hippocampal activation during figure encoding in the right-sided surgery group. Activation of the ipsilateral remnant HIP was not correlated with any memory scores or volumes of the HIP; however, it had a negative correlation with the seizure-onset age and positive correlation with the duration of illness in both patient groups. CONCLUSIONS: For the first time, a neural basis that supports effective intermediate-term episodic memory after unilateral MTLR has been characterized. The results provide evidence that engagement of the HIP contralateral rather than ipsilateral to the side of resection is responsible for effective memory function in the intermediate term (> 1 year) after surgery in patients who have undergone left MTLR and right MTLR. Engagement of the material-specific contralesional HIP, verbal memory in the left-sided surgery group, and visual memory in the right-sided surgery group were observed.

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OBJECTIVEShunt surgery is the most common treatment for hydrocephalus, but it is associated with several long-term complications. Endoscopic choroid plexus coagulation (CPC) and endoscopic third ventriculostomy (ETV) are alternative surgeries that may avoid the need for shunt surgery. Although the short-term efficacy and safety of CPC have been reported in previous studies, long-term outcome, including not only avoiding shunt placement but also intellectual development, remains to be elucidated. The purpose of the present study was to investigate the long-term outcome of CPC.METHODSThe study population comprised patients who had infantile hydrocephalus treated by endoscopic CPC before the age of 24 months and who were followed until at least 5 years of age. Retrospective review was performed using the medical charts. The authors assessed educational status and the full-scale intelligence quotient (FSIQ) using the Wechsler Intelligence Scale for Children (WISC) IV as the means to evaluate the intellectual development.RESULTSFourteen patients with infantile hydrocephalus underwent CPC with or without ETV as a primary surgery. There were no intraoperative complications. In 7 patients (50%), hydrocephalus was successfully controlled without shunt placement. Six patients (43%) eventually required shunt placement. In one patient hydrocephalus was controlled by additional ETV. In the shunt-independent group, 4 patients went to age-appropriate school or achieved age-appropriate development according to intelligence quotient (IQ), 1 patient went to specialized school, and 2 patients had disabilities. In the shunt-dependent group, 4 patients went to an age-appropriate school or achieved age-appropriate development by IQ, 1 patient went to specialized school, and 1 patient had disabilities. The mean FSIQ score in 3 patients without shunts was 90 (range 89-91) and the mean FSIQ score in 4 patients with shunts was 80 (range 48-107). There was no significant difference in the rate of normal development between the shunt-independent group and the shunt-dependent group (p = 0.72).CONCLUSIONSThe CPC with or without ETV can be a safe and effective treatment in children with infantile hydrocephalus. Long-term control of hydrocephalus and normal intellectual development can be achieved in successful cases. Further prospective studies should be required to elucidate appropriate indications.

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