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Fifty children and adolescents with intractable epilepsy were treated with lamotrigine. Most of the children had other neuro-impairments, such as mental retardation, cerebral palsy and autism, added to their epilepsy. Five stopped lamotrigine treatment within four months because of side effects. In the 45 children who continued treatment for a mean of 14 months, five became seizure-free and in 16 the seizure frequency was reduced more than 30 percent. Absences and complex partial seizures responded best. In 24 of the 45 children, the parents reported an improvement in the mental state of their child, with better contact, longer attention span and improved alertness. In eight of 13 autistic children, the autistic symptoms decreased during lamotrigine treatment. This also occurred in children with an unchanged seizure situation, indicating a specific positive psychotropic effect of lamotrigine in mentally retarded and autistic children.
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PMID:Intractable epilepsy in children. The efficacy of lamotrigine treatment, including non-seizure-related benefits. 777 Jan 24

In this article, the term non-speaking will be used to refer to those individuals who have limited or no functional speech because of severe physical impairment, neuromuscular or cognitive deficit and whose communication impairment is not due primarily to a hearing problem. Augmentative communication has developed in order to compensate for impairments and disabilities of the non-speaking individual. It is all communication that supplements speech. Pediatric dental patients who may benefit from augmentative communication systems include those with cerebral palsy, multihandicaps, severe mental retardation and autism. The communication board is given as an example of an augmentative communication system. It is a visual display which can use symbols, pictures, letters and words. It allows the non-speaking child to communicate either by listener-assisted scanning or pointing directly to the symbol or word with their hand or eye-gaze, or with an aid such as a pointer or light. A communication board for use in the pediatric dental setting is described. The pediatric dentist should consult with the speech-language pathologist, family/caretaker, special educator/teacher, and health care members when deciding on communication systems for the non-speaking child in the dental setting. Augmentative communication systems need to be individualized for each non-speaking child. The systems need constant evaluation and updating also as the child develops. The ultimate aim of augmentative communication is to help the non-speaking child to have a more active and fulfilling role in everyday life.
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PMID:Augmentative communication for the non-speaking child. 821 93

Single-photon emission computed tomography (SPECT) of the brain has been used to define functional abnormalities in two groups of childhood behavior disorders: (1) a "primary" category in which there is exclusive or predominant presentation with cognitive and/or behavioral dysfunction and (2) encephalopathies, often defined etiologically at the biochemical or molecular level, in which clinical expression includes, but is not confined to, neural dysfunction. Radiopharmaceuticals available for such studies are manifold, but those used to date have been predominantly perfusion agents, eg, Xenon-133 (133Xe) and technetium-99m (99mTc) hexamethylpropylene amine oxime, and studies with [99mTc]bicisate are eagerly awaited. Xenon-133 studies require that the patient be in the field of view of the detector while the tracer is administered. This renders it difficult for a subject to perform cognitive and other exercises while being imaged, because the environment is quite foreign. On the other hand, the 99mTc-labeled perfusion agents permit a scintigraphic "snapshot" of regional cerebral blood flow during a behavioral event without having to have the patient under the imaging instrument. Thus, one can separate the administration of the radiotracer, which can be done under more controlled and physiological conditions, from the actual imaging. In addition, greater spatial resolution is achieved with the technetium-based agents. Currently, multidetector or dedicated annular crystal-type cameras are the preferred brain SPECT devices, and they are essential to applications such as cortical "activation mapping" or tomographic detection of receptor systems. Close attention to technical detail and standardization of the child's behavioral environment during the investigation are critical to a successful study. The relative advantages and disadvantages of qualitative versus semiquantitative analysis of imaging date are reviewed. Among primary behavioral disorders, 133Xe SPECT studies in attention deficit disorder-hyperactivity (ADHD) have suggested a pattern of hypoperfusion of striatal and periventricular structures with sensorimotor cortical hyperperfusion. This pattern is consistent with some neurophysiological models of the disorder. In cerebral palsy, perfusional abnormalities have paralleled clinical deficits and may offer information to help predict outcome. The important field of childhood affective disorders (schizophrenia, juvenile autism, depression, etc) remains largely unstudied with SPECT. Finally, representative examples of the use of SPECT to study perfusion in encephalopathies with behavioral expression (phenylketonuria, MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes) syndrome, Wilson's disease, etc) are given.
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PMID:Brain single-photon emission computed tomography for behavior disorders in children. 837 98

Few data are available on the risk of seizures in young children with developmental problems. A retrospective evaluation of 1,946 children 0-5 years of age referred to the Tel Aviv Child Development Center (CDC) between 1981 and 1990 was performed. The study was undertaken to determine the cumulative risk of unprovoked seizures in children referred to a CDC and to assess the risk factors associated with seizures in these children. The center serves the Tel Aviv area for a variety of developmental disabilities. Cumulative risk of seizures and risk factors were assessed using Kaplan-Meier methodology. Unprovoked seizures occurred in 58 patients (3%), including 10 with a single seizure and 48 with two or more seizures. Risk factors for seizures included cerebral palsy (CP) (relative risk [RR] = 28.7), neonatal seizures (RR = 15.2), mental retardation (MR) (RR = 7.8), febrile seizures (RR = 7.7), autism (RR = 3.2), and prematurity (RR = 2.7). The cumulative risk of seizures by age 5 years in children with MR, CP, and MR plus CP was 8%, 47%, and 68%, respectively, compared with 1% in those without MR or CP. On multivariate analysis, CP, MR, prior febrile seizures, and prematurity were associated with an increased risk of seizures. The risk of experiencing unprovoked seizures by age 5 in children with developmental disabilities is 3%, which is fourfold greater than that of the general population. Much of this increased risk is limited to selected subgroups with major disabilities. However, if neither MR nor CP is present, the 1% risk of developing unprovoked seizures by age 5 in children with other developmental problems is not substantially different from that expected in the general population.
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PMID:Unprovoked seizures and developmental disabilities: clinical characteristics of children referred to a child development center. 855 61

A population-based study of active epilepsy was conducted in 6-13-year-old mentally retarded children born between 1975 and 1986. The population at risk comprised 48,873 children. Ninety-eight children were identified, 35 mildly and 63 severely retarded. The prevalence was 2.0 per 1000; 0.7 per 1000 for mildly and 1.3 per 1000 for severely retarded children. Sixty-nine children had at least one additional neuroimpairment. Cerebral palsy was found in 42 children with a majority of spastic/dystonic tetraplegias; visual impairment was present in 24 and autism in 24. Thirty-three children had only a mild or no gross motor disability and mild mental retardation, while 23 had IQs < 20 and a very severe gross motor disability. This study underlines the fact that active epilepsy in mentally retarded children is often associated with additional neuroimpairments, especially a combination of severe cerebral palsy and severe visual impairment.
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PMID:Active epilepsy in mentally retarded children. I. Prevalence and additional neuro-impairments. 856 27

The prevalence of autism spectrum disorders was studied in all children with mental retardation and/or motor disability in a defined geographical region over a two-year follow-up period. In the general population, the prevalence of autistic disorder was 0.09% at the end of the follow-up period -a minimum estimate, as children with average intelligence were not screened. Autism spectrum disorders were found in 19.8% of children with mental retardation, including strictly defined autistic disorder (DSM-III-R criteria) in 8.9%; the two-year follow-up yielded a higher prevalence of 11.7% with autistic disorder. Among children with cerebral palsy, 10.5% had an autism spectrum disorder. Clear co-variation was found between mental retardation, epilepsy and autism spectrum disorders in this population of children with neurodevelopmental disorders.
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PMID:Autism spectrum disorders in children with physical or mental disability or both. I: Clinical and epidemiological aspects. 864 35

The ability to study regional cerebral blood flow (rCBF) is available in many institutions, especially with the spread of multi-headed gamma cameras. The use of this technique in paediatrics requires special attention to detail in the manner of data acquisition and handling the child. The interpretation of the rCBF study in a child requires knowledge of normal brain maturation. The major clinical use in paediatrics is epilepsy because of the advances in surgery and the frequency of complex partial seizures. Other indications in paediatric neurology include brain death, acute neurological loss including stroke, language disorders, cerebral palsy, hypertension due to renovascular disease, traumatic brain injury and migraine. There are paediatric psychological conditions in which rCBF assessment has been undertaken, including anorexia nervosa, autism, Gilles de la Tourette Syndrome (GTS) and attention deficit disorder-hyperactivity (ADHD). This article attempts to review all aspects of rCBF studies in paediatrics.
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PMID:Cerebral blood flow imaging in paediatrics: a review. 900 97

When to suspect and thus investigate for inborn errors of purine and pyrimidine metabolism is a dilemma for even the most observant investigator. Often parents of affected children, or a history involving siblings, can provide valuable clues. The recognition of new purine and pyrimidine disorders requires skill and serendipity. But even identifying known disorders can prove difficult, since they cover a broad spectrum of illnesses, can have more than one symptom, or lead to early death. This problem is compounded by the fact that they are relatively recently described and therefore often little known, either in the clinic or laboratory. The considerable heterogeneity in clinical expression within families as well as between families means that asymptomatic homozygotes may not be recognized or can present at any time from early childhood through adolescence up to their eighth decade. Consequently, all siblings should be screened. These disorders should be suspected in any case of unexplained anaemia, failure to thrive, susceptibility to recurrent infection, or neurological deficits with no current diagnosis, including autism, cerebral palsy, delayed development, deafness, epilepsy, self-mutilation, muscle weakness, the inability to walk or talk, and-unusual in children and adolescents-gout, sometimes with renal disease. Some disorders present with radiolucent kidney stones, in acute or chronic renal failure, alone or with any of the above, or as an intolerance/sensitivity to therapy (e.g. 5-fluorouracil in malignancies or azathioprine immunosuppression in organ transplantation), often with life-threatening consequences. Several parameters need to be evaluated to ensure correct diagnosis. Pitfalls which can mask diagnosis using only a single test are renal failure, blood transfusion, diet or drugs.
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PMID:When to investigate for purine and pyrimidine disorders. Introduction and review of clinical and laboratory indications. 921 Nov 94

Developmental delay is frequently used to identify children with delay in meeting developmental milestones in one or more streams of development. There is no consensus on the specific definition. Developmental delay is best viewed generically as a chief complaint rather than a diagnosis. A child suspected to have delays should always be assessed in each of the major streams of development: expressive and receptive language, including social communication; visual problem solving (nonverbal cognition); motor development; neurobehavioral development; and social-emotional development. A model developed by the National Center for Medical Rehabilitation Research is used to compare existing classifications of developmental delays. This model defines the five domains in the disability process: pathophysiology, impairment, functional limitation, disability, and societal limitation. An etiology domain is added. This model is used to illustrate how existing classification systems of cerebral palsy, mental retardation, autism, and language delay draw on information from one or more domains. The model illustrates some of the conflicts between different systems. For example, most classification systems for cerebral palsy emphasize only impairment (spasticity, dyskinesias, and topography). The current definition and classification system for mental retardation focuses on functional limitations (IQ), disability, and societal limitations, ignoring pathophysiology and details of impairment. Given the complexity of neurodevelopmental disabilities, it is unlikely that a single classification system will fit all needs.
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PMID:Classification of developmental delays. 954 35

In the past, studies have been conducted to determine dental and dental hygiene students' attitudes toward the disabled following their clinical experience. The purpose of this study was to identify how dental hygiene students' attitudes toward treating clients with disabilities changed between the start and the end of their didactic and clinical rotation. Earlier research had examined dental hygiene students' attitudes toward individuals with disabilities following either a limited didactic and clinical course addressing disabilities or at the completion of their dental hygiene education. Few investigations had examined students' attitudes prior to and at the completion of a long-term course on clients with disabilities. The aim of this investigation was to determine if a one-year clinical and didactic course addressing various disabilities would result in a significant change in the students' attitudes and comfort level when treating clients with disabilities. The survey researched the attitudes of 18 senior dental hygiene students in the special care clinic at Baylor College of Dentistry, Texas. The students received a pre- and post-modified survey of the "Dental Students' Attitudes Toward the Handicapped Scale" and were asked to rank their responses from strongly agree (5) to strongly disagree (1). The following areas were assessed: Group 1 (Positive Perceptions of Educational Training); Group 2 (Negative Attitude Toward Treating Persons with Disabilities); Group 3 (Providing Dental Services); Group 4 (Negative Perceptions of Educational Training); and Group 5 (Comfort Level when Treating Persons with Disabilities). There was a significant improvement in the students' attitudes in Groups 1, 2 and 4 with p-values for the questions in these groups ranging between p < 0.001 to p < 0.05. In Group 3, only two out of the five questions yielded a significant change in attitude, p < 0.001 to p < 0.01. Following the rotation, when asked about their comfort level in treating clients with disabilities (Group 5), the students reported being comfortable treating persons with autism p < 0.01, cerebral palsy p < 0.05, quadriplegia and paraplegia p < 0.05.
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PMID:Dental hygiene students' attitudes toward treating individuals with disabilities. 961 52


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