Epilepsy in patients with pervasive developmental disorder not otherwise specified

Data on epilepsy in pervasive developmental disorder not otherwise specified are few and scanty. Seventy-seven patients with pervasive developmental disorder not otherwise specified were compared with 77 with autistic disorder, matched for age and sex. The 2 groups were divided into 3 subgroups each: A, without electroencephalography (EEG) paroxysmal abnormalities or epilepsy; B, with EEG paroxysmal abnormalities without epilepsy; and C, with epilepsy. Mild mental retardation (P < .01), pathological neurological examination (P < .05), cerebral lesions (P < .01), abnormal EEG background activity (P < .001), and associated genetic pathologies (P < .01) were more common in pervasive developmental disorder not otherwise specified. Familial antecedents for epilepsy prevailed in subgroup C (P < .01). Epilepsy occurred in 35.1% of patients with pervasive developmental disorder not otherwise specified, with no statistically significant difference compared with autistic disorder. The mean age of seizure onset was earlier (2 years 8 months) in pervasive developmental disorder not otherwise specified (P < .000). Seizure outcome was better in autistic disorder. Genetic diseases and cerebral lesions should be investigated in pervasive developmental disorder not otherwise specified to clarify the etiological and clinical features.     

Epilepsy in pervasive developmental disorder without brain MRI abnormalities

Background: Epilepsy has been reported in patients with pervasive developmental disorder (PDD), with an incidence ranging from 5% to 40%; however most of the studies included patients with brain magnetic resonance imaging (MRI) abnormalities (e.g., tuberous sclerosis) and patients with epilepsy whose seizure onset was in the first year of life. Methods: We retrospectively examined 67 patients (45 males, 22 females) with PDD and epilepsy, who did not have brain MRI abnormalities. Patients who had seizures in the first year of life were excluded. We divided the patients into two groups: group A included 34 patients with an IQ < 50, and group B included 33 patients with an IQ ? 50. Results: The median age of epilepsy onset was higher in group A than group B (8.8 vs. 5.2 years, P < 0.01). Only one patient (3%) in group A and nine patients (27%) in group B were classified with generalized epilepsy (P < 0.05). At the last observation, 16 patients (47%) in group A and 25 patients (76%) in group B were seizure-free for ?1 year (not statistically significant). Conclusion: The relationship between PDD and epilepsy may be different between patients with lower (group A) and higher (group B) IQs in patients who do not have brain MRI abnormalities.     

Epilepsy surgery in children with developmental tumours

We report our experience regarding evaluation, surgical treatment and outcomes in a population of 21 children with histopathologically confirmed developmental tumours [nine dysembryoplastic neuroepithelial tumours (DNET), ten gangliogliomas (GG) and two gangliocytomas (GC)] and related epilepsy, analyzing video-EEG, MRI and neuropsychological data, before and after surgery.Most children had focal epilepsy correlating well with lesion location. One patient had epileptic spasms and generalized discharges. Tumours were located in the temporal lobe in 13 patients. Mean age at surgery was 11.16 years. Postsurgical MRI showed residual tumour growth in one DNET. One child had a recurrent ganglioglioma with anaplastic transformation. At latest follow-up (mean 4.68 years) 95.2% of patients were seizure-free and no significant neuropsychological declines were observed. Evidence from our study suggests that, in this setting, surgery should be performed before criteria for refractory epilepsy are met, particularly in cases with early seizure onset, in order to optimize cognitive outcome.     

Epilepsy surgery in children with developmental disabilities

Surgery for treatment of medically uncontrolled epilepsy in children is now widely accepted with reported outcomes similar to those in adults. Epilepsy is reported in 8.8% to 32% of children with mental retardation (MR) and in up to half of children with severe retardation. There has been concern that patients with low IQ will experience unsatisfactory outcomes from epilepsy surgery and not achieve good seizure control. It is appropriate to reassess the prior bias against resective epilepsy surgery in children with MR in view of the changing criteria for potential candidacy for epilepsy surgery in infants and young children. There are three prerequisites for epilepsy surgery: (1) the epilepsy must be medically intractable; (2) the surgery must be feasible, that is, the epileptogenic zone can identified and safely resected; and (3) there is high likelihood of a satisfactory outcome as regards both the epilepsy and the patient's functional status. Patients with MR may have diffuse cerebral dysfunction and diffuse or multifocal epileptogenic regions. Appropriate patient selection is made possible through use of current technology that allows identification of lesions or areas of cerebral dysgenesis, aiding in identification of localized areas of epileptogenesis. Results from various series of patients with MR who have undergone resective surgery for epilepsy have shown that with careful presurgical evaluations, outcomes are similar between patients with normal IQ scores and those with low scores. Surgical protocols specifically for patients with MR and intractable epilepsy are required, including careful definition of desired outcomes.     

Face recognition in children with a pervasive developmental disorder not otherwise specified

This study investigates the accuracy and speed of face recognition in children with a Pervasive Developmental Disorder Not Otherwise Specified (PDDNOS; DSM-IV, American – Psychiatric Association [APA], 1994). The study includes a clinical group of 26 nonretarded 7- to 10-year-old children with PDDNOS and a control group of 65 normally developing children of the same age. Two computerized reaction time tasks were administered: a face recognition task and a control task designed to measure the recognition of abstract visuospatial patterns. The latter were either easy or difficult to distinguish from a set of alternative patterns. The normally developing children recognized the faces much faster than the hardly distinguishable abstract patterns. The children in the PDDNOS group needed an amount of time to recognize the faces that almost equalled the time they needed to recognize the abstract patterns that were difficult to distinguish. The results suggest that, when processing faces, children with PDDNOS use a strategy that is more attention-demanding and, hence, less automatic or Gestalt-like than the one used by the control children. The results are discussed in the light of a theory that explains the development of coherent mental representations.     

Efficacy of porcine secretin in children with autism and pervasive developmental disorder

Secretin, a gastrointestinal (GI) hormone, was reported in a preliminary study to improve language and behavior in children with autism/pervasive developmental disorder (PDD) and chronic diarrhea. To determine the efficacy of secretin, we completed a double-blind, placebo-controlled, crossover (3 weeks) study in children with autism/PDD and various GI conditions using a single dose of intravenous porcine secretin. Children with chronic, active diarrhea showed a reduction in aberrant behaviors when treated with the secretin but not when treated with the placebo. Children with no GI problems are unaffected by either secretin or placebo. The improvement seen with secretin in children with autism/PDD and chronic diarrhea suggests that there may be a subtype of children with autism/PDD who respond to secretin.     

Parent-directed, intensive early intervention for children with pervasive developmental disorder

We examined parent-directed, intensive early intervention for children with Pervasive Developmental Disorder. Children's parents recruited paraprofessional therapists and requested consultations on how to implement the UCLA treatment model in their homes (Smith & Lovaas, 1998). Parents and therapists then received six one-day workshops over a five-month period, with additional consultations for the next 2-3 years. Six boys participated (intake age 35-45 months, intake IQ 45-60). The study addressed 1) the children's skill acquisition during the first five months of treatment; 2) outcome 2-3 years later; 3) treatment quality; and 4) parents' impressions. Five of 6 children rapidly acquired skills when treatment began, but only 2 clearly improved on standardized tests at the 2-3 year follow-up. Therapists usually employed correct treatment procedures but were less consistent than therapists employed at a clinic. Parents reported high satisfaction with treatment. These mixed results highlight the need for multimodal assessment of parent-directed treatment.     

Psychiatric disorders in individuals with pervasive developmental disorder

Pervasive developmental disorders (PDD) refer to the group of heterogeneous conditions that make up a continuum or spectrum of autistic disorders and share a core triad of impairments consisting of qualitative disturbances in social interaction and verbal and nonverbal communication and imagination. It has long been believed that the prevalence of autism was 2-4 per 10,000 children. However, studies using broader definitions of autistic disorder have suggested that the prevalence of autism spectrum disorder may be as high as 90 per 10,000 and that a greater proportion of individuals with PDD have intellectual levels above 70. Clinicians and researchers have commented on the apparent increase in prevalence of the disorder and have offered a number of explanations, including better recognition and diagnosis of the autism spectrum of disorders and a real increase in the disturbance. It is being increasingly recognized that individuals with PDD are at risk for a wide array of psychiatric disturbances, including affective disorder, anxiety disorders, schizophrenia-like psychosis, aggression, antisocial behavior, and Tourette's disorder (TD). Evidence indicates that PDD is significantly related to the comorbid psychiatric disorders and TD. Because PDD is not rare, individuals with autism spectrum disorder may represent significant subgroups of severely emotionally disturbed patients referred for psychiatric treatment. Because of lack of awareness that the clinical manifestations of PDD are heterogeneous and often mild and that comorbid psychiatric disturbances may obscure the symptoms of the developmental disorder, the diagnosis of PDD may be missed. Implications for practicing clinicians are discussed.     

Clinical features of obsessive-compulsive disorder with pervasive developmental disorder

Recently, psychiatrists have been paying attention to the presence of PDD (Pervasive Developmental Disorder) in difficult cases of mental disorder. We have found that patients with treatment-resistant OCD (Obsessive-Compulsive Disorder) are very likely to have PDD. If the presence of PDD can be recognized at an early stage based on the clinical features of patients with OCD, it would lead to effective treatment. However, there has been little research on the epidemiology of OCD patients with PDD, especially in adults. In this study, we investigated the percentage of PDD in adult OCD and the clinical characteristics of OC symptoms, comparing between OCD patients with or without diagnoses of PDD. The results showed that, in 48 patients with OCD, as many as 13 (27%) were diagnosed with PDD (PDD+). We identified several items in the Y-BOCS (Yale-Brown Obsessive Compulsive Disorder) symptom checklist that showed significantly higher rates in PDD+. In the 'Obsession' checklist, the items were obsession with need for symmetry or exactness, needing to know or remember and bothered by certain sounds/noises and the fear of losing things. In the 'Compulsion' checklist, the items were checking, repetition, ordering/ arranging, and hoarding/collecting compulsions. Among these items, needing to know or remember, hoarding/collecting compulsion, and the fear of losing things seemed to be closely related. The need to know or remember was considered to be the core factor. This relationship was found only in PDD+, and so it may prove useful in clinical guides to identify PDD+ among patients with OCD.     

Randomized trial of intensive early intervention for children with pervasive developmental disorder

Young children with pervasive developmental disorder were randomly assigned to intensive treatment or parent training. The intensive treatment group (7 with autism, 8 with pervasive developmental disorder not otherwise specified--NOS) averaged 24.52 hours per week of individual treatment for one year, gradually reducing hours over the next 1 to 2 years. The parent training group (7 with autism, 6 with pervasive developmental disorder NOS) received 3 to 9 months of parent training. The groups appeared similar at intake on all measures; however, at follow-up the intensive treatment group outperformed the parent training group on measures of intelligence, visual-spatial skills, language, and academics, though not adaptive functioning or behavior problems. Children with pervasive developmental disorder NOS may have gained more than those with autism.     

Visual completion and complexity of visual shape in children with pervasive developmental disorder

Much evidence has been gathered for differences in visual perceptual processing in individuals with Autistic Spectrum Disorder. The presence of the fundamental process of visual completion was tested in a group of children with Pervasive Developmental Disorder (PDD), as this requires perceptually integrating visual structure into wholes. In Experiment 1, it was investigated whether visual completion is present for simple partly occluded shapes in a group of children with PDD and a typically developing group. In Experiment 2, the presence of contextual influences in visual completion was investigated for the two groups. A total of 19 children with PDD and 28 controls who were matched for chronological age and IQ took part in two primed-matching tasks. For both groups, visual completion was present and for both groups, contextual influences were found to be dominant in this process. However, only for the group with PDD no priming effects (PEs) were found from less regular primes on congruent test pairs. The group with PDD did integrate visual information into wholes and did this in a contextually dependent way. However, for more complex shapes, visual completion is weaker for this group.     

Clinical features of epilepsy with pervasive developmental disorder

Purpose

To clarify the clinical features of patients with epilepsy and pervasive developmental disorder (PDD). Methods: We examined 12 outpatients with epilepsy as well as PDD at Seiai Rehabilitation Hospital. Results: The patients comprised 7 males and 5 females. The initial neurological symptoms appeared between 5 months and 4 years of age. The interval between the initial neurological symptoms/developmental delay and seizure onset ranged from several months to twenty years. The seizures started at 10–19 years of age in 8 out of the 12 cases. The types of seizures were astatic-drop in 2 cases, tonic-to-astatic in one, atypical absence (decreased consciousness) and generalized tonic clonic seizures (GTCS) in 3 cases, GTCS in 4 cases, or myoclonic and psychomotor in 2 cases. The mental development distributed from normal to extremely severe retardation. Paroxysmal abnormalities on eegs were focal at the frontal area in 7 cases (58%) and other findings in 5 cases. Presumptive risk factors were prenatal in 6 cases (family history for PDD in 1 case, for epilepsy in 1, twin pregnancy in 2 cases, and other in 2 cases), perinatal in 2 patients, postnatal in 1, and unknown in 3 cases. Conclusions: The seizures occurred most frequently after the onset of neurological symptoms or developmental delay. The frontal lobe dysfunction was associated with seizure onset in 58% of the cases based on the EEG findings. The risk factors were prenatal in 50% of the cases.     

Abnormal spatial frequency processing in high-functioning children with pervasive developmental disorder (PDD).

OBJECTIVE: Basic abnormalities in visual information processing could be associated with the local visual bias often found in subjects with PDD. Therefore, the present study investigated the existence of deficits in spatial frequency processing at an early sensory level in children with PDD. METHODS: Visual evoked potentials (VEPs) and VEP dipole sources elicited by high and low spatial frequency gratings were analyzed in high-functioning children with PDD and matched controls. RESULTS: Around 80 ms (N80-latency) children with PDD did not show the same robust differences between high and low spatial frequencies in VEP amplitude and VEP brain sources as controls, because of atypical processing of high frequencies. Analyses at the P1-latency (130 ms) revealed that, although similar inferior-medial brain sources were activated for the processing of both spatial frequencies in the PDD and control group, source strength in response to both frequencies was weaker in the PDD compared to control group. Moreover, additional superior-lateral brain sources were activated during the processing of both frequencies in the PDD group. CONCLUSIONS: Decreased specialized processing of high and low spatial frequencies might be a robust characteristic of PDD. Early in processing abnormalities in high spatial frequency processing seem to occur in PDD. At a later phase in processing there seems to be both atypical high and low spatial frequency processing. Considering that the processing of specific spatial frequencies plays an important role in the processing of global and local aspects of hierarchical stimuli and faces and of emotions, present data suggest that peculiarities in PDD subjects with respect to these stimuli might be related to an abnormality in more fundamental visual processes. SIGNIFICANCE: A basic abnormality in visual frequency processing is established in children with PDD.     

Clinical significance of tics and attention-deficit hyperactivity disorder (ADHD) in children with pervasive developmental disorder

The goal of this study was to examine the clinical significance of co-occurring tics and attention-deficit hyperactivity disorder (ADHD) as indicators of a more complex symptomatology in children with and without pervasive developmental disorder. Parents and teachers completed a Diagnostic and Statistical Manual of Mental Disorders-IV-referenced rating scale for 3- to 5- (n = 182/135) and 6- to 12- (n = 301/191) year-old children with pervasive developmental disorder and clinic controls, respectively. The percentage of children with tic behaviors varied with age: preschoolers (25%, 44%) versus elementary schoolchildren (60%, 66%) (parent and teacher ratings, respectively). For many psychiatric symptoms, screening prevalence rates were highest for the ADHD + tics group and lowest for the group with symptoms of neither, but the pattern of group differences varied by age group and informant. In general, there were few differences between the ADHD only and tics only groups. The pattern of ADHD/tic group differences was similar for both children with and without pervasive developmental disorder. We concluded that these findings support the notion that the co-occurrence of ADHD and tics is an indicator of a more complex psychiatric symptomatology in children with pervasive developmental disorder.     

Psychotherapy of children with pervasive developmental disorders

In recent years, the role of psychotherapy in the treatment of children with pervasive developmental disorders, such as autism and Asperger's Syndrome, has been questioned. Advances in neuropsychiatry, neuropsychology, and genetics, as well as the refinement of behavioral and educational techniques, have relegated discussions about psychotherapy mostly to reviews about the history of treatment for autism. Even when psychotherapeutic work is suggested, it is typically only very structured supportive counseling for older and high functioning children and adolescents that is considered. This paper argues that there is a central role for psychotherapy for children with pervasive developmental disorders, and that psychotherapeutic interventions should begin at a younger, rather than older age.     

Affect comprehension in children with pervasive developmental disorders

Affect comprehension was studied in children with pervasive developmental disorders (PDD) and normal children matched for mental age. Three matching tasks were used: matching objects (a nonsocial control task), matching faces, and matching affects. The three tasks were developed to be of equal difficulty for normal children. Children were also tested for comprehension and expression of affect terms. The PDD children were impaired on affect matching relative to the normal controls. The PDD children were impaired on face and affect matching relative to their own performance on object matching, whereas the normal children were not. Within the PDD sample, object matching was correlated with mental age measures but not with measures of social behavior and play, but face and affect matching were significantly correlated with mental age as well as social behavior and play. Individual PDD children who showed relative deficits on face or affect matching tended to be more socially impaired than PDD children whose face and affect matching was consonant with their mental age. Results are discussed in terms of possible etiologies of the social deficit in PDD children, and the importance of subtypes within this population.This work was supported in part by National Institute of Mental Health grants (MH28605 to authors Waterhouse and Fein, and MH40162 to authors Fein and Waterhouse), and from NINCDS (NS20489 to the CNS-INS Task Force on Nosology of Higher Cerebral Function Disorders in Children). We gratefully acknowledge the help and support of staff and students of the League School, Newton, the Phillips Elementary School, Watertown, Kids are People, Brookline, the Russell Cooperative Preschool, Watertown, Just for Kids Preschool, Watertown, Massachusetts; Chepachet Elementary School, Chepachet, Rhode Island; Search Day School, Ocean, Eden Institute, Princeton, Princeton Child Development Institute, Princeton, Egg Harbor Township Regional Day School, Egg Harbor, and St. John of God School, West Mill Grove, New Jersey.     

Integrity of lateral and feedbackward connections in visual processing in children with pervasive developmental disorder

Enhanced visual detail processing in subjects with pervasive developmental disorder (PDD) has been related to impairments in feature integration. The functional integrity of two types of neuronal connections involved in visual feature integration, namely horizontal and feedbackward connections, were tested. Sixteen children with PDD and 17 age- and IQ-matched control children (mean age 13.3 years) were included. In a texture segregation task the difference in ERP response to homogeneous and checkered visual stimuli was determined. Additionally, in a contour integration task subjects had to point out a contour consisting of colinearly aligned Gabor signals in backgrounds increasing in noise. Children with PDD showed a normal performance on the contour integration task, suggesting that neurons in the primary visual cortex of children with PDD can effectively integrate the activity of local detectors that process different aspects of the same object information by making use of long-range lateral connections. The amplitude of ERP activity related to texture segregation was also not different between the PDD and control groups, indicating functional visual feedback mechanisms between V1 and higher order areas in subjects with PDD. However, a difference in latency of texture-segmentation related activity between the groups was noted. This effect did not reach significance, which could be due to the small N of the study. Therefore, the data need replication in a study with larger samples before more definitive conclusions can be drawn.