Evaluation of perampanel in patients with intellectual disability and epilepsy

Snoeijen‐Schouwenaars, Francesca M.; Ool, Jans S. van; Tan, In Y.; Schelhaas, Helenius J.; Majoie, Marian

doi:10.1016/j.yebeh.2016.10.013

Cited by 34 publications

(31 citation statements)

References 14 publications

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“…38 Seizure freedom rates vary widely in observational studies with perampanel and will be influenced heavily by patient populations and by the exact definition of seizure freedom and the calculation method. Our figure of 9% (seizure-free at 1 year and for ≥6 months) is lower than some reports that use similar definitions (14% 39 ), higher than others (0%, 34,37,40,41 4%, 33 6% 35,42 ), and higher than in the phase III clinical trials (~4% seizure-free over a 13-week maintenance period). 5 We also calculated the pragmatic seizure freedom rate (3.1%)-the number seizure-free expressed as a proportion of the FAS (n = 2396).…”

Section: Discussioncontrasting

confidence: 79%

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

et al. 2018

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Across a large, treatment-resistant population, add-on perampanel was retained for ≥1 year by 48% of individuals, and 9% were seizure-free for ≥6 months. TEAEs were in line with previous reports in routine clinical use, and less frequent than in the clinical trial setting. No new or unexpected TEAEs were seen. Despite the limitations of observational studies, our data indicate that some individuals may derive a marked benefit from the use of perampanel.

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Section: Discussioncontrasting

confidence: 79%

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

et al. 2018

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“…Unlike what suggested in literature, mean of VIQ (91.74) was higher than mean of PIQ (86.87). This could be due to the use of alternative strategies for problem solving that require the use of verbal reasoning ability, learned through experience to compensate for their present difficulties, or may even be linked to the effects of hormonal therapies [24]. On the other hand, in our other SCAs subjects, mean IQ was 68.71, VIQ was 69.36 and PIQ was 74.72, in line with previous studies.…”

Section: Discussionsupporting

confidence: 89%

“…The typical cognitive profile is mainly characterized by the presence of the discrepancy between scores on performance tasks and those achieved in the verbal subtests, in favor of the former. Some studies have shown that verbal IQ scores are 10 points below the average compared with those of performance IQ [24]. This discrepancy may change during the life stages: differences in verbal conceptual and nonverbal reasoning skills may diminish over time, with the former no longer being such an area of deficit.…”

Section: Discussionmentioning

confidence: 99%

Variability in Cognitive Behavioral Phenotypes in Klinefelter Syndrome (KS) and Other Sex Chromosomal Aneuploidies (SCAs)

Verri¹,

D’Angelo²,

Cremante³

et al. 2017

Andrology

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Sex Chromosomal Aneuploidies (SCAs) are the most frequently occurring chromosomal abnormalities with an incidence of 1 in 450 births. Males with SCAs are known to have variability in their developmental profile. Aim of this paper is to illustrate clinical variability in the different SCAs. The sample was composed by 53 subjects (mean age=21.16 years, range: 13-54) with karyotype 47, XXY (73%), 49, XXXXY (7%), 48, XXYY (9%), mosaicism 47, XXY/48, XXXY (2%), 47, XYY (5%), 48, XXXY (2%), 49, XXXYY (2%). Only 5 subjects have been diagnosed prenatally (4 KS and 1 XXYY). Primary caregivers completed a comprehensive questionnaire detailing birth, medical, developmental and psychological history. Cognitive and behavioral assessment was performed with clinical interviews with DSM IV criteria and psychometric questionnaires (WISC-R, WAIS-R, CPM, Token Test, VABS, SCL90, and SCQ). Twenty-one sex and age matched subjects karyotypically normal were also evaluated from the behavioural point of view. Mean IQ in typical KS was 87.45 ± 2 ds (sd=20.12) range [21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] while in CPM the other SCAs subjects scored 22.27 (range 10-35) and 22.50 (range 9-31) in the Token Test (p<0.05). VABS scores documented more marked impairment on adaptive behavior in atypical SCAs subjects. SCL90 documented an elevation of paranoid scale in the 70% of KS subjects and 50% of other SCAs. Autistic traits were present in 67% of the other SCAs subjects and in the 18% of KS at the SCQ. A precise identification of the cognitive and behavioral phenotype in different SCAs may enhance the clinical treatment, anticipatory guidance, and care throughout the lifespan.

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“…More than one third of the patients in our series affected by ID developed PER-related aggressive behavior, which required discontinuation of the treatment in all of them. This finding is in agreement with the findings of recent studies by Snoeijen-Schouwenaars et al (2017) and Huber and Schmid (2017) in patients with epilepsy and IDs who reported behavioral AEs, including, aggression, irritability mood changes, in about 40 and 50% of subjects, respectively. These observations should alert us to the possibility that PER may be not tolerated in a non-negligible proportion of epilepsy patients with IDs.…”

Section: Discussionsupporting

confidence: 93%

Foreground and background: an interview with Peter Singer and three arguments against naturalism

Pacherie¹

2017

Functional Neurology

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Evaluation of perampanel in patients with intellectual disability and epilepsy

Cited by 34 publications

References 14 publications

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

Perampanel in routine clinical use across Europe: Pooled, multicenter, observational data

Variability in Cognitive Behavioral Phenotypes in Klinefelter Syndrome (KS) and Other Sex Chromosomal Aneuploidies (SCAs)

Foreground and background: an interview with Peter Singer and three arguments against naturalism

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