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1.
Arch Sex Behav ; 51(8): 3765-3789, 2022 11.
Article in English | MEDLINE | ID: mdl-35916987

ABSTRACT

While most studies on sexuality in later life report that sexual desire declines with age, little is known about the exact nature of age effects on sexual desire. Using self-reported dyadic sexual desire relating to a partner, dyadic sexual desire relating to an attractive person, and solitary sexual desire from a large (N > 8000) and age diverse (14.6-80.2 years) online sample, the current study had three goals: First, we investigated relationships between men and women's sexual desire and age. Second, we examined whether individual differences such as gender/sex, sexual orientation, self-rated masculinity, relationship status, self-rated attractiveness, and self-rated health predict sexual desire. Third, we examined how these associations differed across sexual desire facets. On average, the associations between age and both men and women's sexual desire followed nonlinear trends and differed between genders/sexes and types of sexual desire. Average levels of all types of sexual desire were generally higher in men. Dyadic sexual desire related positively to self-rated masculinity and having a romantic partner and solitary desire was higher in people with same-sex attraction. We discuss the results in the context of the evolutionary hypothesis that predict an increase of sexual desire and female reproductive effort prior to declining fertility. Our findings both support and challenge beliefs about gender/sex specificity of age effects on sexual desire and highlight the importance of differentiating between desire types.


Subject(s)
Libido , Sexual Behavior , Female , Humans , Male , Adolescent , Young Adult , Adult , Middle Aged , Aged , Aged, 80 and over , Men , Masculinity , Sexuality , Sexual Partners
2.
J Pediatr ; 247: 160-162, 2022 08.
Article in English | MEDLINE | ID: mdl-35447125

ABSTRACT

A 5-week-old infant born at term was diagnosed with acute necrotizing encephalopathy associated with severe acute respiratory syndrome coronavirus 2 as evidenced by clinical presentation, neuroimaging, and cerebrospinal fluid studies. Our patient was treated with high-dose intravenous methylprednisolone, tocilizumab, and intravenous immunoglobulin with significant short-term clinical improvement but long-term sequelae.


Subject(s)
Brain Diseases , COVID-19 , Brain Diseases/diagnosis , Brain Diseases/etiology , COVID-19/complications , Disease Progression , Humans , Infant , Methylprednisolone/therapeutic use , Neuroimaging
3.
Clin Med Res ; 19(4): 203-207, 2021 12.
Article in English | MEDLINE | ID: mdl-34933953

ABSTRACT

Spondyloarthritis presents in various and occasionally unusual ways that imitates other diseases. Without forthcoming risk factors, such atypical presentation may elude diagnosis for months. The case presented here of a child, aged 4 years, who is negative for human leukocyte antigen B27 (HLA-B27) and with no family history of HLA-B27 related disease, who developed torticollis with neck pain and lymphadenopathy, highlights the necessity of continually evaluating a diagnosis, especially when treatment fails to produce expected results. Painful torticollis in a child with adenopathy often is infectious in nature or potentially due to Griesel syndrome when persistent. Chronic arthritis of the cervical spine may enter the differential diagnosis when torticollis is persistent, and early recognition and aggressive treatment is necessary to prevent permanent functional impairment.


Subject(s)
Spondylarthritis , Torticollis , Child, Preschool , Diagnosis, Differential , HLA-B27 Antigen , Humans , Syndrome , Torticollis/diagnosis , Torticollis/etiology
4.
J Soc Psychol ; 160(2): 164-169, 2020.
Article in English | MEDLINE | ID: mdl-31131721

ABSTRACT

We replicated and extended (N = 207) work on the social values (i.e., obedience, tradition, security, benevolence, universalism, self-direction, stimulation, hedonism, achievement, and power) linked to the Dark Triad traits (i.e., narcissism, psychopathy, and Machiavellianism). Each of the traits was positively associated with values of achievement and power. Psychopathy and narcissism were both negatively correlated with benevolence, and psychopathy and Machiavellianism were negatively correlated with obedience. Psychopathy was also negatively correlated with tradition. Sex differences in the values of tradition, benevolence, and power were mediated by psychopathy. We suggest that high rates of the Dark Triad traits facilitate, for men, holding social values that emphasize standing out whereas low rates facilitate, for women, fitting in.


Subject(s)
Achievement , Antisocial Personality Disorder/psychology , Machiavellianism , Narcissism , Power, Psychological , Social Behavior , Social Values , Female , Humans , Male , Sex Factors , Social Conformity
5.
J Int Neuropsychol Soc ; 24(7): 653-661, 2018 08.
Article in English | MEDLINE | ID: mdl-29745359

ABSTRACT

OBJECTIVES: Rates of cognitive, academic and behavioral comorbidities are elevated in children with epilepsy. The contribution of environmental and genetic influences to comorbidity risk is not fully understood. This study investigated children with epilepsy, their unaffected siblings, and controls to determine the presence and extent of risk associated with family relatedness across a range of epilepsy comorbidities. METHODS: Participants were 346 children (8-18 years), n=180 with recent-onset epilepsy, their unaffected siblings (n=67), and healthy first-degree cousin controls (n=99). Assessments included: (1) Child Behavior Checklist/6-18 (CBCL), (2) Behavior Rating Inventory of Executive Function (BRIEF), (3) history of education and academic services, and (4) lifetime attention deficit hyperactivity disorder (ADHD) diagnosis. Analyses consisted of linear mixed effect models for continuous variables, and logistic mixed models for binary variables. RESULTS: Differences were detected between the three groups of children across all measures (p<.001). For ADHD, academic problems, and executive dysfunction, children with epilepsy exhibited significantly more problems than unaffected siblings and controls; siblings and controls did not differ statistically significantly from each other. For social competence, children with epilepsy and their unaffected siblings displayed more abnormality compared with controls, with no statistically significant difference between children with epilepsy and unaffected siblings. For behavioral problems, children with epilepsy had more abnormality than siblings and controls, but unaffected siblings also exhibited more abnormalities than controls. CONCLUSIONS: The contribution of epilepsy and family relatedness varies across specific neurobehavioral comorbidities. Family relatedness was not significantly associated with rates of ADHD, academic problems and executive dysfunction, but was associated with competence and behavioral problems. (JINS, 2018, 24, 653-661).


Subject(s)
Academic Performance , Attention Deficit Disorder with Hyperactivity/physiopathology , Child Behavior Disorders/physiopathology , Epilepsy/physiopathology , Executive Function/physiology , Family , Social Skills , Adolescent , Attention Deficit Disorder with Hyperactivity/epidemiology , Child , Child Behavior Disorders/epidemiology , Comorbidity , Epilepsy/epidemiology , Female , Humans , Male , Siblings
6.
Adv Methods Pract Psychol Sci ; 1(4): 501-515, 2018 Dec.
Article in English | MEDLINE | ID: mdl-31886452

ABSTRACT

Concerns have been growing about the veracity of psychological research. Many findings in psychological science are based on studies with insufficient statistical power and nonrepresentative samples, or may otherwise be limited to specific, ungeneralizable settings or populations. Crowdsourced research, a type of large-scale collaboration in which one or more research projects are conducted across multiple lab sites, offers a pragmatic solution to these and other current methodological challenges. The Psychological Science Accelerator (PSA) is a distributed network of laboratories designed to enable and support crowdsourced research projects. These projects can focus on novel research questions, or attempt to replicate prior research, in large, diverse samples. The PSA's mission is to accelerate the accumulation of reliable and generalizable evidence in psychological science. Here, we describe the background, structure, principles, procedures, benefits, and challenges of the PSA. In contrast to other crowdsourced research networks, the PSA is ongoing (as opposed to time-limited), efficient (in terms of re-using structures and principles for different projects), decentralized, diverse (in terms of participants and researchers), and inclusive (of proposals, contributions, and other relevant input from anyone inside or outside of the network). The PSA and other approaches to crowdsourced psychological science will advance our understanding of mental processes and behaviors by enabling rigorous research and systematically examining its generalizability.

7.
Epilepsy Behav ; 61: 269-274, 2016 08.
Article in English | MEDLINE | ID: mdl-27442497

ABSTRACT

OBJECTIVE: The objective of this study was to identify cognitive phenotypes in children with new-onset focal and generalized idiopathic epilepsies and determine their relationship with epilepsy syndrome, brain structure, neurodevelopmental history, and family characteristics. METHODS: One hundred thirty-eight children with new-onset epilepsy and 95 controls (age: 8-18) underwent neuropsychological, clinical, and quantitative MR evaluations. Control participants' neuropsychological data were subjected to confirmatory factor analysis and then resultant factor scores were applied to participants with epilepsy and subjected to latent class analysis. Identified cognitive phenotypes were examined in relation to epilepsy syndrome, quantitative neuroimaging, and familial and neurodevelopmental variables. RESULTS: Confirmatory factor analysis identified five cognitive factors (verbal, perceptual, speed, attention, executive), and latent class analysis identified three clusters of participants with epilepsy: 1) average and similar to controls, 2) mild impairment across multiple cognitive domains, and 3) impairment across all domains with severe attentional impairment, representing 44%, 44%, and 12% of the epilepsy sample, respectively. Cognitive phenotype membership was not associated with epilepsy syndrome but was associated with increasing abnormalities in brain structure, parental IQ, and features of early developmental history. SIGNIFICANCE: Cognitive phenotypes are present in idiopathic childhood epilepsies that are unassociated with traditional epilepsy syndromes but are associated with measures of brain structure, family history, and neurodevelopmental features.


Subject(s)
Cognition/physiology , Epilepsy/psychology , Executive Function/physiology , Phenotype , Adolescent , Attention/physiology , Brain/diagnostic imaging , Child , Epilepsy/diagnostic imaging , Female , Humans , Magnetic Resonance Imaging , Male , Neuropsychological Tests , Organ Size/physiology
8.
Epilepsy Behav ; 43: 117-21, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25601585

ABSTRACT

OBJECTIVE: This study was conducted to determine the lifetime rate and distribution of supportive academic and educational services provided to children with new- or recent-onset epilepsy and typically developing controls, the relationship of this history to objective academic test performance, and the course of performance over serial evaluations (baseline and 2 and 5years later). METHODS: Research participants were 91 children aged 8-18 at study entry, including 50 youth with recent-onset epilepsy (28 focal [FE] and 22 generalized [GE] epilepsy) and healthy first-degree cousin controls (n=41). The sample with epilepsy included children with uncomplicated epilepsy and normal imaging and development. Lifetime history of a diversity of supportive educational services was determined via a structured interview with parents at the baseline study visit. Associations were examined between these support services and participants' academic performance in reading, spelling, and arithmetic (Wide Range Achievement Test-Revision 3 [WRAT3] [12]) during three serial study visits including baseline and 2 and 5years later. RESULTS: Children with epilepsy had a higher lifetime rate of provision of diverse academic supportive services compared to controls at the baseline visit (52% vs. 18%). These services antedated epilepsy diagnosis in the majority (80.8%) of the children with epilepsy. Among children with epilepsy, children who presented with academic services had significantly lower WRAT3 reading, spelling, and arithmetic performance at baseline and at 2- and 5-year follow-ups. CONCLUSION: A brief structured clinical interview conducted with parents identifies children with epilepsy who are at academic risk at the time of diagnosis, with that risk persisting up to 5years later.


Subject(s)
Epilepsy/psychology , Underachievement , Adolescent , Age of Onset , Child , Education , Educational Status , Epilepsies, Partial/psychology , Epilepsy/diagnosis , Epilepsy, Generalized/psychology , Female , Humans , Intelligence Tests , Longitudinal Studies , Male , Mathematics/education , Neuropsychological Tests , Reading
9.
Dev Med Child Neurol ; 55(3): 278-82, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23216381

ABSTRACT

AIM: The aim of this study was to determine the relationship between parent and child Full-scale IQ (FSIQ) in children with epilepsy and in typically developing comparison children and to examine parent-child IQ differences by epilepsy characteristics. METHOD: The study participants were 97 children (50 males, 47 females; age range 8-18y; mean age 12y 3mo, SD 3y1mo) with recent-onset epilepsy including idiopathic generalized (n=43) and idiopathic localization-related epilepsies (n=54); 69 healthy comparison children (38 females, 31 males; age range 8-18y; mean age 12y 8mo, SD 3y 2mo), and one biological parent per child. All participants were administered the Wechsler Abbreviated Scale of Intelligence (WASI). FSIQ was compared in children with epilepsy and typically developing children; FSIQ was compared in the parents of typically developing children and the parents of participants with epilepsy; parent-child FSIQ differences were compared between the groups. RESULTS: FSIQ was lower in children with epilepsy than in comparison children (p<0.001). FSIQ of parents of children with epilepsy did not differ from the FSIQ of the parents of typically developing children. Children with epilepsy had significantly lower FSIQ than their parents (p<0.001), whereas comparison children did not. The parent-child IQ difference was significantly higher in the group with epilepsy than the comparison group (p=0.043). Epilepsy characteristics were not related to parent-child IQ difference. INTERPRETATION: Parent-child IQ difference appears to be a marker of epilepsy impact independent of familial IQ, epilepsy syndrome, and clinical seizure features. This marker is evident early in the course of idiopathic epilepsies and can be tracked over time.


Subject(s)
Epilepsy/physiopathology , Intelligence/physiology , Adolescent , Adult , Biomarkers , Case-Control Studies , Child , Epilepsies, Partial/physiopathology , Female , Humans , Male , Parents/psychology , Wechsler Scales
10.
Epilepsia ; 51(10): 2038-46, 2010 Oct.
Article in English | MEDLINE | ID: mdl-20384719

ABSTRACT

PURPOSE: To characterize prospective neurodevelopmental changes in brain structure in children with new and recent-onset epilepsy compared to healthy controls. METHODS: Thirty-four healthy controls (mean age 12.9 years) and 38 children with new/recent-onset idiopathic epilepsy (mean age 12.9 years) underwent 1.5 T magnetic resonance imaging (MRI) at baseline and 2 years later. Prospective changes in total cerebral and lobar gray and white matter volumes were compared within and between groups. RESULTS: Prospective changes in gray matter volume were comparable for the epilepsy and control groups, with significant (p < 0.0001) reduction in total cerebral gray matter, due primarily to significant (p < 0.001) reductions in frontal and parietal gray matter. Prospective white matter volume changes differed between groups. Controls exhibited a significant (p = 0.0012) increase in total cerebral white matter volume due to significant (p < 0.001) volume increases in the frontal, parietal, and temporal lobes. In contrast, the epilepsy group exhibited nonsignificant white matter volume change in the total cerebrum (p = 0.51) as well as across all lobes (all p's > 0.06). The group by white matter volume change interactions were significant for total cerebrum (p = 0.04) and frontal lobe (p = 0.04). DISCUSSION: Children with new and recent-onset epilepsy exhibit an altered pattern of brain development characterized by delayed age-appropriate increase in white matter volume. These findings may affect cognitive development through reduced brain connectivity and may also be related to the impairments in executive function commonly reported in this population.


Subject(s)
Brain/pathology , Epilepsy/pathology , Adolescent , Age of Onset , Atrophy/pathology , Brain/growth & development , Cerebral Cortex/growth & development , Cerebral Cortex/pathology , Child , Cohort Studies , Epilepsy/diagnosis , Female , Frontal Lobe/growth & development , Frontal Lobe/pathology , Humans , Longitudinal Studies , Magnetic Resonance Imaging/statistics & numerical data , Male , Parietal Lobe/growth & development , Parietal Lobe/pathology , Prospective Studies
11.
Epilepsia ; 51(2): 257-67, 2010 Feb.
Article in English | MEDLINE | ID: mdl-19780796

ABSTRACT

PURPOSE: Anecdotal reports have described cortical malformations in epileptic patients with Sturge-Weber syndrome (SWS). No data are available regarding the prevalence and significance of this association. METHODS: We reviewed retrospectively the clinical profile, preoperative magnetic resonance imaging (MRI) studies, and pathology reports of all patients with SWS and medically intractable epilepsy evaluated in our epilepsy surgery program between 1979 and 2006. RESULTS: Twelve patients (male/female = 7/5) were identified. Mean age at seizure onset was 11.1 +/- 16.7 months. Seizures occurred daily in seven patients and weekly in five patients. A facial port-wine stain was noted in 10 cases. Eleven patients evidenced developmental delay and eight were hemiparetic. Eight patients underwent excisional surgery for epilepsy (mean age 10.3 +/- 6.5 year), including hemispherectomy (n = 4) and focal cortical resection (n = 4). Tissue was available for neuropathology in six operated cases and revealed polymicrogyria (n = 3) and cortical dysplasia (n = 4). Polymicrogyria was associated with cortical dysplasia in one child. Brain MRIs were reviewed in 10 of 12 patients and were consistent with cortical malformations in all cases. CONCLUSIONS: We conclude that cortical malformations are frequent in patients with medically intractable epilepsy and Sturge-Weber-syndrome and may be the primary cause of epilepsy.


Subject(s)
Cerebral Cortex/abnormalities , Epilepsy/diagnosis , Epilepsy/surgery , Sturge-Weber Syndrome/diagnosis , Sturge-Weber Syndrome/surgery , Brain Mapping , Cerebral Cortex/surgery , Child, Preschool , Electroencephalography , Epilepsy/epidemiology , Female , Functional Laterality/physiology , Hemispherectomy , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging , Male , Malformations of Cortical Development/diagnosis , Malformations of Cortical Development/surgery , Neuropsychological Tests , Preoperative Care , Prognosis , Sturge-Weber Syndrome/epidemiology , Tomography, X-Ray Computed
12.
Epilepsia ; 49(11): 1847-58, 2008 Nov.
Article in English | MEDLINE | ID: mdl-18785880

ABSTRACT

PURPOSE: To characterize patterns and determinants of normal and abnormal cognitive development in children with new onset epilepsy compared to healthy controls. METHODS: Longitudinal (2-year) cognitive growth was examined in 100 children, age 8-18 years, including healthy controls (n = 48) and children with new onset epilepsy (n = 52). Cognitive maturation was examined as a function of the presence/absence of two neurobehavioral comorbitiies (attention deficit hyperactivity disorder and/or academic problems) identified at the time of epilepsy diagnosis. Groups were compared across a comprehensive neuropsychological battery assessing intelligence, academic achievement, language, memory, executive function, and psychomotor speed. RESULTS: Children with new onset epilepsy without neurobehavioral comorbidities were comparable to healthy controls at baseline, rate of cognitive development, and follow-up assessment across all neuropsychological domains. In contrast, the presence of neurobehavioral comorbidities was associated with significantly worse baseline and prospective cognitive trajectories across all cognitive domains, especially executive functions. CONCLUSION: The presence of neurobehavioral comorbidities at the time of epilepsy onset is a major marker of abnormal cognitive development both prior to and after the onset of epilepsy.


Subject(s)
Attention Deficit Disorder with Hyperactivity/epidemiology , Attention Deficit Disorder with Hyperactivity/physiopathology , Brain/physiopathology , Cognition Disorders/epidemiology , Epilepsy/epidemiology , Achievement , Adolescent , Age of Onset , Attention Deficit Disorder with Hyperactivity/diagnosis , Child , Cognition Disorders/diagnosis , Comorbidity , Diagnostic and Statistical Manual of Mental Disorders , Educational Measurement , Female , Follow-Up Studies , Humans , Male , Neuropsychological Tests , Prospective Studies , Psychomotor Disorders/diagnosis , Psychomotor Disorders/epidemiology , Severity of Illness Index , Surveys and Questionnaires
13.
Dev Med Child Neurol ; 49(7): 493-7, 2007 Jul.
Article in English | MEDLINE | ID: mdl-17593119

ABSTRACT

The aim of this study was to characterize the distribution, timing, and risk factors for psychiatric comorbidity in children with recent onset epilepsy. Children aged 8 to 18 years with recent onset epilepsy (<1 year in duration) of idiopathic etiology (n=53) and a healthy comparison group (n=50) underwent a structured psychiatric diagnostic interview to characterize the spectrum of lifetime-to-date history of comorbid psychiatric disorder. There was no significant difference between the children with recent onset epilepsy and healthy comparison children in sex (31 males, 22 females vs 23 males, 27 females) or mean age 12.7y [SD 3.3] vs 12.7y [SD 3.2]). Children with recent onset epilepsy exhibited an elevated rate of lifetime-to-date Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV) Axis I disorders compared with the comparison group. They showed significantly higher rates of depressive disorders (22.6 vs. 4%, p=0.01), anxiety disorders (35.8 vs 22%, p<0.05), and attention-deficit-hyperactivity disorder (26.4 vs 10%, p=0.01) with elevated but less prevalent rates of oppositional defiant and tic disorders. A subset of children with epilepsy (45%) exhibited DSM-IV Axis I disorders before the first recognized seizure, suggesting the potential influence of antecedent neurobiological factors that remain to be identified. The increased prevalence of psychiatric comorbidity antedating epilepsy onset may be consistent with the presence of underlying neurobiological influences independent of seizures, epilepsy syndrome, and medication treatment.


Subject(s)
Epilepsy/epidemiology , Mental Disorders/epidemiology , Adolescent , Chi-Square Distribution , Child , Comorbidity , Diagnostic and Statistical Manual of Mental Disorders , Female , Humans , Male , Psychiatric Status Rating Scales
14.
Brain ; 129(Pt 10): 2609-19, 2006 Oct.
Article in English | MEDLINE | ID: mdl-16928696

ABSTRACT

Abnormalities in cognition, academic performance and brain volumetrics have been reported in children with chronic epilepsy. The nature and degree to which these problems may be present at epilepsy onset or may instead become more evident over time remains to be determined. This study characterizes neuropsychological status, brain structure and their interrelationship in children with recent-onset epilepsy compared with healthy controls. Children (age: 8-18 years) with recent-onset idiopathic epilepsy (n = 53) and healthy controls (n = 50) underwent comprehensive neuropsychological assessment and quantitative volumetric measurement of segmented (grey and white matter) volumes of total cerebrum and lobar regions. Compared with controls, children with recent-onset epilepsy exhibit a pattern of mild diffuse cognitive impairment, regardless of epilepsy syndrome, as well as academic underachievement that in a subset of children antedates the first recognized seizure. There are no overall differences in MR morphometric analyses of total cerebral or lobar tissue volumes. Controls show a strong association between cognitive development and increasing cerebral tissue volume (especially white matter volume), an association that is absent in children with epilepsy. Children with a history of academic achievement problems exhibit the most abnormal cognitive function and have significant volumetric reductions in left occipital and parietal lobe grey matter. Patients with idiopathic epilepsy exhibit cognitive dysfunction and academic underachievement at the onset of the disorder, irrespective of epilepsy syndrome, and indications of antecedent neurocognitive impairment are present in a subset of children. Volumetric abnormalities are not yet apparent in the epilepsy group as a whole, but there are indications of an altered structure-function relationship in epilepsy, and the subset of children with prior history of academic problems have abnormal volume of posterior left hemisphere grey matter. These early abnormalities need to be integrated into lifespan models of the neuropsychology of epilepsy.


Subject(s)
Brain/pathology , Epilepsy/pathology , Epilepsy/psychology , Adolescent , Case-Control Studies , Child , Cognition , Educational Status , Female , Humans , Magnetic Resonance Imaging , Male , Neuropsychological Tests
15.
Epilepsy Behav ; 8(1): 272-7, 2006 Feb.
Article in English | MEDLINE | ID: mdl-16352471

ABSTRACT

OBJECTIVE: Excessive daytime sleepiness (EDS) and sleep complaints are common among adults with epilepsy. We hypothesized that children with epilepsy have worse daytime sleepiness compared with controls. METHODS: Children with and without epilepsy between ages 8 and 18 were recruited for the study. Parents and children were asked to fill out the Pediatric Sleep Questionnaire (PSQ) and Pediatric Daytime Sleepiness Scale (PDSS), respectively. The Mann-Whitney U test was used for group comparisons, with the Fischer exact or chi2 test for categorical variables. Regression analysis was used to identify predictors of EDS. RESULTS: Twenty-six patients and matched controls were recruited for the study. Parents of children with epilepsy more often reported EDS (P < 0.001), symptoms of sleep-disordered breathing (P < 0.001), and parasomnias (P < 0.001) compared with controls. On the PDSS, children with epilepsy reported worse daytime sleepiness scores compared with controls (15.48 +/- 6.4 vs 11.88 +/- 5.25, P = 0.037). Based on conditional logistic regression modeling, symptoms of excessive daytime sleepiness [corrected] (OR = 15.3, 95% CI = 1.4-166.6) and parasomnias (OR = 12.4, 95% CI = 1.01-151.6) were significantly associated with having epilepsy when adjusted for duration of nightime sleep. Further, 10 children (38.5%) with epilepsy reported positive sleep-disordered breathing, whereas no one in the control group reported SDB (P < 0.001) [corrected] Epilepsy syndrome, anticonvulsants used, and presence or absence of seizure freedom, however, were not significant predictors of EDS among patients. CONCLUSIONS: Daytime sleepiness appears to be common in children with epilepsy, and may be due to underlying sleep disorders. Further confirmatory studies are needed using screening questionnaires and formal sleep studies to systematically study the prevalence of sleep complaints and role of sleep disorders in these patients.


Subject(s)
Disorders of Excessive Somnolence/epidemiology , Epilepsy/complications , Parasomnias/epidemiology , Sleep Apnea Syndromes/epidemiology , Adolescent , Child , Disorders of Excessive Somnolence/etiology , Female , Humans , Logistic Models , Male , Parasomnias/etiology , Parents , Pilot Projects , Sleep Apnea Syndromes/etiology , Surveys and Questionnaires
16.
Am J Med Genet A ; 124A(2): 202-8, 2004 Jan 15.
Article in English | MEDLINE | ID: mdl-14699622

ABSTRACT

Regional skin hypoplasia has been described in several genetic syndromes, including focal dermal hypoplasia (FDH), microphthalmia with linear skin defects (MLS), oculocerebrocutaneous syndrome (OCCS), and terminal osseous dysplasia and pigmentary defects (TODP). All but OCCS have been reported to follow an X-linked inheritance pattern. We describe a 14-year-old girl with clinical features overlapping with these disorders. She had mild mental retardation, macrocephaly, microphthalmia, right-sided morning glory optic disc anomaly, palmar and lip pits, and polysyndactyly. A swirling pattern of skin hypopigmentation, papular hypopigmented and herniated skin lesions reminiscent of FDH most prominent over her face, head, hands, and feet was evident. Brain magnetic resonance imaging (MRI) showed polymicrogyria (most severely in the perisylvian and mesial frontal regions), enlarged left lateral ventricle, partial agenesis of the corpus callosum, and optic nerve tumor on the right. Dermatopathologic examination of the skin lesions was consistent with basaloid follicular hamartomas. The skin and digit anomalies observed overlap with FDH, but polymicrogyria, basaloid follicular hamartomas, optic nerve tumor, and morning glory anomaly have not previously been described in FDH. Skin defects in MLS are linear and the eyes typically have sclerocornea. Polymicrogyria has been described in OCCS, but not in any of the other three syndromes. The limb anomalies in TODP are reductions rather than polysyndactyly. Skin defects are localized to the face, and digital fibromas usually occur. While significant overlap exists between all four of the syndromes discussed, we believe that the constellation of anomalies observed in this girl most likely comprises a newly recognized syndrome.


Subject(s)
Abnormalities, Multiple/pathology , Brain/abnormalities , Focal Dermal Hypoplasia/pathology , Optic Disk/abnormalities , Abnormalities, Multiple/genetics , Adolescent , Female , Humans , Karyotyping , Polydactyly/pathology , Syndactyly/pathology , Syndrome , Toes/abnormalities
17.
J Clin Anesth ; 14(3): 201-5, 2002 May.
Article in English | MEDLINE | ID: mdl-12031753

ABSTRACT

STUDY OBJECTIVE: To assess complications of regional as well as general anesthesia in parturients with Chiari I malformation. DESIGN: Retrospective chart review. SETTING: Academic medical center. PATIENTS: All parturients in our institution who had the diagnosis of Chiari I malformation and delivered in our hospitals over a 50-year period. MAIN RESULTS: 12 parturients delivered 30 babies. Three deliveries were facilitated with general anesthesia. Nine deliveries were facilitated with central axis anesthesia, six with epidural anesthesia, two with a single injection of a spinal anesthetic, and one with a continuous spinal catheter. The patient who received a continuous spinal catheter developed a postdural puncture headache that resolved with an epidural blood patch. None of the patients who received general, spinal, or epidural anesthesia for their deliveries developed symptoms or had exacerbation of preexisting symptoms of Chiari I malformation. CONCLUSIONS: General anesthesia, as well as spinal and epidural anesthesia, appeared to be safe and effective in our series of vaginal or cesarean delivery patients. The small number of patients in our series does not negate the cautious recommendations of others, but suggests that general anesthesia, as well as spinal or epidural anesthesia, can be used safely and effectively in these patients.


Subject(s)
Anesthesia/adverse effects , Arnold-Chiari Malformation/complications , Parturition/physiology , Academic Medical Centers , Adolescent , Adult , Female , Humans , Middle Aged , Pregnancy , Retrospective Studies
18.
Neurosurg Clin N Am ; 13(1): 35-9, viii, 2002 Jan.
Article in English | MEDLINE | ID: mdl-11754315

ABSTRACT

The preoperative evaluation of patients with suspected cortical dysplasia involves detailed neurologic and neuropsychologic evaluations, electroencephalography, and comprehensive neuroimaging and functional studies. The goal is to identify a focal region of seizure onset and to assess for motor impairments, speech and language difficulties, or developmental delay. Although the electroencephalogram abnormalities in cortical dysplasia are nonspecific, they typically include unusual high-amplitude (slowing and focal) activity. The association of cortical dysplasia with cardiovascular, dermatologic, and other systemic disorders is discussed.


Subject(s)
Cerebral Cortex/abnormalities , Electroencephalography , Epilepsy/congenital , Brain Mapping , Cerebral Cortex/physiopathology , Cerebral Cortex/surgery , Child , Epilepsy/physiopathology , Evoked Potentials/physiology , Humans , Neuroglia/physiology , Neurons/physiology , Neuropsychological Tests , Prognosis , Synaptic Transmission/physiology
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