Myoclonus epilepsy in children and adolescents]

Twelve cases of myoclonus epilepsy initiating in childhood and adolescence were studied clinically, electrophysiologically and biochemically. Myoclonic and epileptic syndromes being most pronounced, the clinical pattern presented certain polymorphism: asymmetry, hyperkinesis, myodystonia, epileptic episodes, mental disorders. The electroencephalographic picture of the disease was variable. Hyperexcretion of glycosaminoglycans and changes in their fraction composition with predominant release of heparan sulphate evidenced for deranged metabolism of mucopolysaccharides.     

Face recognition without awareness

PH has been completely unable to recognise faces since sustaining a closed head injury some four years ago, but can recognise familiar people from their names. His performance on face processing tasks is, however, comparable to that of normal subjects if explicit recognition is not required. Thus he can make same/different identity judgements more quickly for familiar than unfamiliar face photographs, and faster matching of familiar faces is only found for identity matches involving the face's internal features. When making semantic categorisation decisions to printed names he shows interference from distractor faces belonging to an incorrect category, even when the faces in each category are matched on physical appearance. When learning to associate the occupation or the name with photographs of faces, his performance is better with true (face and person's actual name or occupation) than untrue (face and another person's name or occupation) pairings. Covert recognition can also be demonstrated for faces of people PH has only met since his accident. These findings show that in prosopagnosia, much of the processing of familiar faces can remain intact despite absence of awareness that recognition has occurred.     

Road-crossing safety in virtual reality: a comparison of adolescents with and without ADHD.

This study investigated the potential accident-proneness of adolescents with attention deficit hyperactivity disorder (ADHD) in a hazardous road-crossing environment. An immersive virtual reality traffic gap-choice task was used to determine whether ADHD adolescents show more unsafe road-crossing behavior than controls. Participants (ages 13 to 17) were identified with (n = 24) or without (n = 24) ADHD according to a standardized protocol (Schedule for Affective Disorders and Schizophrenia for School-Age Children-Present and Lifetime Version and Conners' Scales), with equal number of boys (n = 12) and girls (n = 12) in each group. ADHD adolescents did not take stimulant medication on the day of testing. Participants with ADHD had a lower margin of safety, walked slower, underutilized the available gap in incoming traffic, showed greater variability in road-crossing behavior, and evidenced twice as many collisions as compared to controls. No sex differences were found. Virtual reality may help identify and educate those at higher risk of being involved in dangerous traffic situations.     

Psychosocial functioning of adolescents with and without paediatric bipolar disorder

BACKGROUND: While there has been a growing interest in the presentation of Bipolar Disorder (BD) in children and adolescents, few studies have investigated the psychosocial functioning of these individuals and its relationship to trauma and suicidal ideation. METHODS: 63 adolescents aged 13-17 participated: 39 controls and 24 with Bipolar Disorder (BD). Group allocation and histories of trauma and suicidal ideation were obtained using the K-SADS-PL and WASH-U-KSADS. Adolescents completed questionnaires covering negative life events, self-esteem, hopelessness, regulation of anger, locus of control and coping. RESULTS: More traumatic events and negative life experiences were reported by the BD group with over 50% of the BD sample indicating a history of trauma compared with 10% of the controls. The BD group reported lower self-esteem, more hopelessness, more negative life events, a more external locus of control and greater difficulties regulating emotion in anger-provoking situations. They were also found to have poorer coping strategies than the controls. Histories of trauma did not differentiate those with and without psychosocial problems. Further, hopelessness was found to be the best predictor of those BD adolescents reporting suicidal ideation. Comorbidity could not account for the differences found. LIMITATIONS: The sample was small and therefore disallowed comparisons among subtypes of BD. Cross-sectional design limited the ability to investigate causal relationships. CONCLUSIONS: This is the first study to document the widespread psychosocial difficulties facing youth with BD, highlighting these issues as important ones to explore during assessment and treatment, particularly in management of affective storms and suicidal risk.     

Intellectual disability without epilepsy associated with STXBP1 disruption

STXBP1 (Munc18-1) is a component of the machinery involved in the fusion of secretory vesicles to the presynaptic membrane for the release of neurotransmitters. De novo missense mutations in STXBP1 were recently reported in patients with Ohtahara syndrome, a form of encephalopathy with severe early-onset epilepsy. In addition, sequencing of the coding region of STXBP1 in 95 patients with non-syndromic intellectual disability (NSID) revealed de novo truncating mutations in two patients who also showed severe non-specific epilepsy, suggesting that STXBP1 disruption has the potential of causing a wide spectrum of epileptic disorders in association with intellectual disability. Here, we report on the mutational screening of STXBP1 in a different series of 50 patients with NSID and the identification of a novel de novo truncating mutation (c.1206delT/ p.Y402X) in a male with NSID, but surprisingly with no history of epilepsy. This is the first report of a patient with a truncating mutation in STXBP1 that does not show epilepsy, thus, expanding the clinical spectrum associated with STXBP1 disruption.     

MMPI profiles of depressed adolescents with and without conduct disorder

This study examined whether adolescents with depression would produce significantly different MMPI profiles from adolescents with depression and conduct disorder. Twenty-four adolescents who met DSM-III-R criteria for major depression or dysthymia were included in the study. Nine of these 24 subjects also met DSM-III-R criteria for Conduct Disorder. Results indicated that depressed only adolescents had significantly higher elevations on scales 2 and 0 while those with depression and conduct disorder had significantly higher elevations on scales F and 9. These findings suggest that the presence of conduct disorder in depressed adolescents may lead to a veiling of their affective symptoms, thus potentially causing clinicians to underdiagnose depression in such cases. © 1996 John Wiley & Sons, Inc.     

Brief report: friendships of adolescents with and without diabetes

BACKGROUND: Friendships of adolescents with chronic illness have been rarely studied. OBJECTIVE: To compare the friendships of boys and girls with diabetes with those between healthy adolescents. METHODS: One hundred and thirty-eight adolescents were interviewed at summer camps. Participants indicated their number of close friends and rated friendships for support and conflict. Participants described aspects of their same-sex and other-sex friendships that they liked and disliked. RESULTS: Adolescents with diabetes have friendships that are similar to those between healthy adolescents, with a few exceptions. CONCLUSIONS: Emotional support may be especially valued by girls with diabetes, whereas differences among friends may be less valued among adolescents with diabetes.     

Dysfunctional cognitive control and reward processing in adolescents with Internet gaming disorder

Developmental theories posit that immature cognitive control and excessive reward-seeking capacities may be a risk factor for addictive behaviors during adolescence, but the control and reward capacities have rarely been assessed experimentally in adolescents with Internet gaming disorder (IGD) simultaneously. This electrophysiological study examined inhibitory control and reward processing in adolescents with IGD during a go/no-go task and a gambling task. Behaviorally, the adolescents with IGD exhibited lower inhibitory control, as measured by the accuracy of no-go trials, and more risk seeking, as measured by the proportion of risky choices, than did the controls. Compared with the controls, the adolescents with IGD exhibited decreased no-go P3 and blunted feedback-related negativity (FRN) amplitudes following gains (gain FRN) but not losses. Thus, IGD in adolescents is potentially driven by dysfunction of the control system and the approach system rather than the avoidance system, supporting the neurobiological model of adolescent development.     

Perceived stigma and school attendance among children and adolescents with epilepsy in South Western Uganda

BackgroundEpilepsy is a neurological disorder that has a high worldwide prevalence with eighty percent of the global burden being in low and middle-income countries. There is a high level of perceived stigma among children and adolescents with epilepsy, which has severe debilitating effects and affects school attendance.ObjectiveTo assess the effect of perceived stigma on school attendance patterns among children and adolescents with epilepsy.MethodsWe conducted a cross sectional study among 191 children and adolescents aged from 6–18 years with epilepsy at one large semi-urban hospital and a small rural health center in SouthWestern Uganda. Epilepsy-related perceived stigma was measured using the adapted Kilifi Stigma Scale of Epilepsy and school attendance patterns were assessed using a piloted investigator-designed questionnaire.ResultsChildren with high-perceived stigma were more likely to have never attended school (13.8%) or started school late (average age 5.7 years) compared to those with low-perceived stigma (average age 4.9 years). Additionally, those with high epilepsy-related perceived stigma repeated classes 2.5 times more compared to those with low-perceived stigma.ConclusionThese preliminary findings suggest correlation between high-perceived stigma and disrupted school attendance patterns among children and adolescents with epilepsy, hence the need to address this social challenge.     

A mathematical model of internal time processing in temporal lobe epilepsy.

Based on known relationships between epileptic and nonepileptic cortical cerebral blood flow, electrocorticographic factors and epileptogenicity, a mathematical model for internal time processing is derived. The model suggests that the human brain has mechanisms for internal processing of real, reverse and imaginary time.     

Discontinuing antiepileptic medication in children with epilepsy after two years without seizures. A prospective study

Antiepileptic medications were discontinued in 88 children with epilepsy of various causes who had been free of seizures for two to four years. The subjects were then followed for six months to five years (mean, 22 months). Sixty-six (75 per cent) remained free of seizures. Electroencephalographic characteristics, type of seizure, and age at onset were important in predicting outcome. Specific electroencephalographic features (such as the presence or absence of slowing or spikes) were more predictive than simple classification of an electroencephalogram as normal or abnormal. A history of complex partial seizures that had been controlled for two years carried a relatively favorable prognosis (P less than 0.05), whereas a history of atypical febrile seizures carried a poor prognosis (P less than 0.05). The variable of a younger age at onset was also associated with a better outcome (P less than 0.05), but only if accompanied by electroencephalographic slowing. Altogether, six variables (the interaction of age at onset with electroencephalographic slowing, electroencephalographic spikes, atypical febrile seizures, complex partial seizures, electroencephalographic slowing, and electroencephalographic change) were statistically significant (P less than 0.05) in predicting outcome. We conclude that in most children with epilepsy who have been free of seizures for two or more years, antiepileptic medications can safely be discontinued, and that it is possible to predict reasonably well which children will remain free of seizures.     

Sleeping-waking rhythm and frequency of seizures in epilepsy without psychoses and with chronic psychoses

Sleeping-waking is a basic biological rhythm [1, 2]. A study of sleep in epilepsy established that changes in the EEG vary with different sleep phases [3–5]. Activation of epileptic electroencephalographic phenomena was observed more frequently during NREM (non-rapid eye movement) phases; the REM, or paradoxical, sleep phase seemed to inhibit such phenomena. On this basis some authors [3–5] are attempting far-reaching conclusions concerning a connection between separate sleep phases and the development of epileptic seizures. To a certain degree, a similar interpretation is focusing on the problem of the relationship of seizures and their frequency to the sleeping-waking rhythm. In this connection, it must be noted that in themselves EEG changes and epilepsy, a disease in which convulsive episodes play a substantial role, are not identical [6]. Horyd and Baransia-Teruszak [7] found no reliable correlation between EEG changes and type of seizure. There was a correlation between frequency of seizures and the electroencephalographic picture in only 30% of the cases. The authors believe that in the remaining 70% the connection between EEG shifts and clinical manifestations of epilepsy is more complex. On the other hand, as N. N. Bemin et al. indicated [1], based on hypotheses developed by N. P. Bekhtereva [8] on flexible and rigid connection, sleep is one of the processes that is regulated by flexible connections and for this reason the sequence and duration of sleep phases is exceptionally variable and depends on most various external and internal factors.Translated from Zhurnal Nevropatologii i Psikhiatrii imeni S. S. Korsakova, Vol. 82, No. 6, pp. 801–806, June, 1982.     

Face processing in schizophrenia: defining the deficit

BACKGROUND: Abnormalities of face affect naming and face recognition occur in schizophrenia but it is not clear whether the deficits reflect wider underlying impairments of perception, memory, language or executive function. METHOD: Twenty-six patients with schizophrenia were compared with 23 healthy volunteers on neuropsychological tests and tests of face and affect processing. Face and non-face tests were compared at four levels of processing: visuo-spatial perception, recognition memory, language and naming, and executive function. We examined relationships with drug dose, duration of illness and pre-morbid and current IQ. RESULTS: Patients and controls did not differ in estimated pre-morbid IQ but current IQ was 12 points lower in patients. At each level of processing there were correlated deficits of face and non-face processing in the patients that were mostly independent of IQ decline. Impaired face and non-face visuo-spatial function and recognition performance were generally correlated with drug dose. Impairments in naming face emotions were correlated with other non-face naming tasks independently of drug dose. Patients performed less well than controls in classifying faces by emotion while ignoring identity and this was associated with poorer performance in Wisconsin Card Sorting. CONCLUSIONS: The pattern of results suggests that deficits in face processing reflect three wider neuropsychological impairments: a drug-related impairment of visual imagery, and disease-related impairments of semantic retrieval and executive function.     

以健康测量量表评价癫癎及哮喘患儿生活质量的对照研究

目的以健康测量量表（SF-36）评估癫癎及哮喘患儿的生活质量。方法采用SF-36量表对2007年6至12月在复旦大学附属儿科医院神经专科、呼吸专科及儿童保健门诊就诊的癫癎患儿、哮喘患儿和健康儿童进行生活质量评定。结果研究期间85例癫癎患儿（癫癎组）、81例哮喘患儿（哮喘组）和87名健康儿童（正常对照组）进入分析。3组儿童的年龄、性别和受教育程度相匹配。①疾病及治疗情况：癫癎组和哮喘组服用1种药物者分别为70／85例和59／81例,癫癎组63／85例和哮喘组56／81例患病后规则服药,两组在服药种类和依从性上差异无统计学意义（P均〉0．05）;癫癎组36／85例至本次调查前至少有1年癫癎未发作;哮喘组29／81例最大呼气峰流速监测持续保持个人最佳值的80％以上。②生活质量评价：癫癎组的生活质量总分和8个分项的终得分与正常对照组差异均有统计学意义（P〈0．01）;哮喘组的生活质量总分及生理功能、身体疼痛、总体健康和活力4个分项的终得分与正常对照组差异均有统计学意义（P〈0．01）;无论疾病控制与否,癫癎组生活质量均较哮喘组差;癫癎组情感功能及精神健康状况明显差于哮喘组,其心理障碍不随疾病控制而明显改善。结论①癫癎和哮喘患儿生活质量较健康儿童明显下降;②癫癎患儿较哮喘患儿生活质量损害更为明显;③SF-36量表可作为评价疾病控制程度的手段,辅助评价疾病治疗的疗效;④癫癎患儿的心理障碍应引起临床高度关注。     

Feedback processing in adolescents with prenatal cocaine exposure: an electrophysiological investigation

Impaired cognitive control is a consequence of cocaine exposure. Difficulty with feedback processing may underlie this impairment. We examined neural correlates of feedback processing using event-related potentials (ERPs) in 49 prenatally cocaine-exposed (PCE) and 34 nondrug exposed (NDE) adolescents. Adolescents performed a reward-feedback task with win/no-win feedback in a chance-based task. We investigated amplitude and latency of the feedback-related negativity (FRN) and P300 ERP components and source-based estimates elicited during feedback processing. PCE adolescents had smaller P300 amplitudes for no-win feedback, and source analysis in the P300 time window revealed differences between groups localized to the dorsal anterior cingulate cortex.     

Comparison between children and adolescents with and without chronic benign pain: consultation rate and pain characteristics.

The aim of the study was to determine whether children with chronic benign pain are in contact with their general practitioner (GP) more frequently than those without chronic benign pain. A random sample of children and adolescents aged between 0 and 18 years of age was drawn from the records of ten general practices. According to their responses to a pain questionnaire, subjects were assigned to the chronic benign pain group (n = 95) if they had pain of more than three months' duration, or to the control group (n = 105) if they had pain of less than three months' duration or no pain at all. All the subjects had an average GP consultation rate of 2.6 contacts per year. No significant age and sex differences were found. Chronic benign pain in childhood and adolescence is not related to increased use of healthcare services, suggesting that somatisation does not play a major role in children with chronic benign pain.