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353 Specific developmental language disorders and electroencephalographic abnormalities
Al 42
Abstracts
139
353 Specific developmental language disorders electroencephalographic abnormalities A S QUINTAS, A LEVY GOMES, R GOUVEIA, M BAPTISTA
Diagnostic rate for the aetiology of mental retardation in a group of 90 children S MASTROYIANNI, E KONTOPOULOS, A PAPARIZOU, D ZAFEIRIOU, I TSIKOULAS Hippocration Hospital of Thessaloniki, First Department Paediatrics, Aristotle University, Thessaloniki, Greece
of
A logical first step in designing public health prevention strategies for mental retardation is to identify its causes. Modem diagnostic techniques and specialized clinical skills are necessary for identifying the various symptoms and diseases causing mental retardation. According to the recent literature, the biomedical causes of mental retardation remain unknown in a high percentage, ranging from 25-65%. This variation is partly related to the inconsistencies in the definition of what determines an aetiology. Since April 1998, 90 children with mental retardation aged 2-16 years (6.56k4.65) were prospectively recorded and investigated, according to a precise diagnostic protocol. All children included in our study had an IQ score below 70 (Wechsler Intelligence Scale for Children-Revised) or severe and persistent global developmental delay. According to the results of our diagnostic work-up, we classified the causes of mental retardation in ‘known‘, ‘possible’, and ‘unknown‘. As known‘ causes of mental retardation, we considered metabolic and other inherited diseases, chromosomal abnormalities, congenital infections, central nervous system birth defects, well documented postasphyxial encephalopathy, cerebral haemorrhage, head trauma, central nervous system infections and brain tumours. Conditions which are frequently associated with mental retardation, such as epilepsy, visual and hearing impairment, low birth weight, dysmorphic features, and suboptimal factors during pregnancy and perinatal period, were considered as ‘possible’ causes. ‘Known’ aetiology for mental retardation was found in 30 patients (33.33%). Prenatal conditions were seen in 18 children; chromosomal abnormalities and other clinical syndromes in ten, metabolic diseases in five, central nervous system birth defects in two, and neglected congenital hypothyroidism in one child. Perinatal and postnatal conditions were found in nine and three children respectively. ‘Possible’ causes of mental retardation were seen in 43 children (47.77%). Severe epilepsy was reported in 16 children, visual and/or hearing defects in four, severe autism in six and dysmorphic features in nine. Suboptimal perinatal factors and intrauterine growth retardation were reported in seven and three children respectively. Five children had clinical characteristics of progressive encephalopathy which remained unclassified despite the extended diagnostic work-up. Most children with mental retardation in our study were categorized in the group of ‘possible’ aetiology. This is consistent with the findings of other studies using similar classification for the causes of mental retardation. We believe that our diagnostic efforts should be focused in this specific group of patients. Experienced clinical judgment in conjunction with better use of modem diagnostic techniques can possibly improve the internationally reported low diagnostic rate for the aetiology of mental retardation.
Child Development and Neurology de Santa Maria, Lisbon, Portugal
Departments,
and
Hospital
Introduction: Children with specific developmental language disorders (SDLD) are reported to have a high incidence of epilepsy and/or abnormal EEG findings (respectively S-28% and 20-93%). The EEG abnormalities are found more frequently in subjects who have forms of SDLD involving impairments in comprehension, occur or increase during sleep and are usually paroxysmal. There is, however, some discussion regarding their meaning and the existence of a causal relation between EEG abnormalities and SDLD has not yet been confirmed. The authors carried out a retrospective analysis of children diagnosed as having SDLD and with available EEG data, in order to study the incidence of EEG abnormalities and the relation between them and the language problems in that population. Materials and methods: The diagnostic criteria applied for inclusion in the study were: an impairment in language development as demonstrated by scores on an appropriate standardized test (Reynell’s developmental scale) substantially below those obtained from a measure of non-verbal intellectual capacity (Griffiths scale), which was within normality. Significant deafness and social deprivation were excluded. SDLD subtypes were defined according to the DSM-IV classification. We reviewed the records of the patients with the diagnosis of SDLD evaluated from 1990 to 1999 in the Child Development Department, Hospital Santa Maria, Lisbon, and selected the cases having available EEG data. Results: From a total of 126 children with identified SDLD, 49 had available EEG data, including a sleep EEG in 28; 9.3% (12/129) had a family history of epilepsy; 4.7% (6/129) children had epilepsy; 4.5% (3/67) of the cases with expressive language disorder (ELD) and 6.8% (3/44) of those with mixed receptive-expressive language disorder (MLD). The following data concern the group with available EEG data (n =49): the mean age was 4.3 years. 27 ELD, 19 MLD and three phonological disorders (I’D) were found: 18 of the 49 children (36.7%) had an abnormal EEG, including six with epilepsy (three with ELD and three with MLD). In these six cases, the EEG showed spikes (four focal - two left/two right, one bilateral and one diffuse), spike-waves (one diffuse) and in two of them an abnormal organization of the electrical activity during sleep. The abnormalities found were aggravated during sleep in three cases. In the subgroup with EEG data who did not have epilepsy, 27.9% (12/43) of the children had an abnormal EEG, including eight with spikes or spike-waves (four focal - two right/two left, one bilateral and three diffuse), two with slow waves (focal left) and three with an abnormal organization of the sleep EEG. In this subgroup, EEG abnormalities were present in 16.7% (4/24) of the children with ELD, 43.8% (7/16) of those with MLD and 33.3% (l/3) of those with I’D and occurred during sleep in 10 of the 12 cases (83.3%). In the last evaluation performed, six children (33.3%) from the group having EEG abnormalities
A143
Abstracts
(including one with epilepsy) had significant problems ZIS two (6.5%) in the other group.
residual
Discussion: In this population of children with SDLD, epilepsy was more frequent than one would expect in the general population (4.7% ~1s0.4%), the highest rate being found in the patients with mixed language disorder. Concerning the EEG abnormalities reported, the difference was even more significant. In fact, 27.9% of the children without epilepsy had abnormal (most paroxysmal type) findings in their EEGs (~1s 1.64.7% in the general population). These tended to occur more frequently in patients in whom language comprehension was impaired and were significantly more common during sleep. The outcome was more favourable in the group without EEG abnormalities. Most of these data are similar to others previously published and, in our opinion, reinforce the need to carry out prospective and wider studies, in order to better understand the correlation between EEG findings and clinical aspects in SDLD, as well as its implications.
276 Treatment
outcome
in shunted hydrocephalus
J J ROTTEVEEL,’ I HEINSBERGEN,’ A GROTENHUIS3 Departments of ‘Paediatric 3Neurosurgery, University Netherlands
N ROELEVELD,2
Neurology, ‘Epidemiology, Hospital, Nijmegen, The
and
Purpose: To identify the main risk factors for a poor outcome in children with a shunted hydrocephalus. Material and methods: The medical records of patients born between 1984 and 1992 who had a shunt operation for hydrocephalus before 1994, were analysed retrospectively. The following data were collected: general information, aetiology of hydrocephalus; information about shunt operations (timing of drain implantation, number and indication of revisions); several outcome parameters (school type, seizures and morbidity parameters according to the WHO classification).
301 Evidence-based practice and the care of handicapped children P SZATMARI McMaster
University,
Hamilton,
Ontario,
Canada
Evidence-based medicine refers to the use of the best available empirical evidence to address important clinical problems. It is based on the notion that different levels of evidence exist to answer clinical questions and that certain skills are needed in asking the right clinical questions, retrieving the information, critically appraising the available evidence and then systematically applying that evidence to the actual clinical situation. The concept of evidence-based practice is gaining wide currency in North America and elsewhere. There is certainly a great need to apply evidence-based principles to the care of handicapped children. For example, there are enormous variations in the diagnosis and treatment of several handicapping conditions of childhood including the hyperkinetic syndrome, autism and other chronic neurological disorders of childhood. Many interventions are employed for these children for which very little evidence of their efficacy exists. Moreover, treatments that have been demonstrated to be effective on the basis of high quality evidence are not being applied. Although there remain many obstacles to the implementation of evidence-based practice among health care professionals working with handicapped children, steady progress is being made in North America. There are also special problems in implementing evidence-based practice with parents of these children. With the explosion of information on the Internet and the interest of the media in health issues, parents are now aware of a bewildering array of diagnostic and treatment options that are available for their handicapped children. Parents often feel that they cannot wait for the best available evidence and are requesting services and intervention for their handicapped children that may or may not be effective and may or may not have potential to do harm. Several recent examples in autism will be presented to illustrate these issues. The lecture will conclude with concrete suggestions to improve communication between health care professionals and parents so that the important goals of evidence-based practice and shared decision-making can be achieved without conflict and rancour.
Results: We found that children with a post-haemorrhagic hydrocephalus faired poorly. Children with hydrocephalus due to spina bifida or a congenital malformation had considerably better outcome scores. Regarding the timing of drain implantation, we found a poorer mental and linguistic development in children operated more than 1 month after diagnosis. Furthermore, we found that patients with more than two revisions have a higher chance of developing mental impairment, especially if associated with drain infections.
F TORGAL-GARCIA, R GOUVEIA, A FERNANDES, A TEIXEIRA, A MARTINS, N LACERDA
Conclusion: Haemorrhage and more than two revisions are risk factors for a poor outcome. We also found that delay in surgical treatment might be an important risk factor for a poor outcome in hydrocephalic children.
Purpose: To assess the neurodevelopmental outcome of a group of children at the ages of 3 and/or 5 years followed at the Child Development Unit of the Department of Paediatrics of Hospital de Santa Maria,
384 Neurodevelopment factors
of children
with perinatal
risk
Child Development Unit, Department of Paediatrics, Hospital de Santa Maria, University of Lisbon, Lisbon, Portugal
Abstracts
139
353 Specific developmental language disorders electroencephalographic abnormalities A S QUINTAS, A LEVY GOMES, R GOUVEIA, M BAPTISTA
Diagnostic rate for the aetiology of mental retardation in a group of 90 children S MASTROYIANNI, E KONTOPOULOS, A PAPARIZOU, D ZAFEIRIOU, I TSIKOULAS Hippocration Hospital of Thessaloniki, First Department Paediatrics, Aristotle University, Thessaloniki, Greece
of
A logical first step in designing public health prevention strategies for mental retardation is to identify its causes. Modem diagnostic techniques and specialized clinical skills are necessary for identifying the various symptoms and diseases causing mental retardation. According to the recent literature, the biomedical causes of mental retardation remain unknown in a high percentage, ranging from 25-65%. This variation is partly related to the inconsistencies in the definition of what determines an aetiology. Since April 1998, 90 children with mental retardation aged 2-16 years (6.56k4.65) were prospectively recorded and investigated, according to a precise diagnostic protocol. All children included in our study had an IQ score below 70 (Wechsler Intelligence Scale for Children-Revised) or severe and persistent global developmental delay. According to the results of our diagnostic work-up, we classified the causes of mental retardation in ‘known‘, ‘possible’, and ‘unknown‘. As known‘ causes of mental retardation, we considered metabolic and other inherited diseases, chromosomal abnormalities, congenital infections, central nervous system birth defects, well documented postasphyxial encephalopathy, cerebral haemorrhage, head trauma, central nervous system infections and brain tumours. Conditions which are frequently associated with mental retardation, such as epilepsy, visual and hearing impairment, low birth weight, dysmorphic features, and suboptimal factors during pregnancy and perinatal period, were considered as ‘possible’ causes. ‘Known’ aetiology for mental retardation was found in 30 patients (33.33%). Prenatal conditions were seen in 18 children; chromosomal abnormalities and other clinical syndromes in ten, metabolic diseases in five, central nervous system birth defects in two, and neglected congenital hypothyroidism in one child. Perinatal and postnatal conditions were found in nine and three children respectively. ‘Possible’ causes of mental retardation were seen in 43 children (47.77%). Severe epilepsy was reported in 16 children, visual and/or hearing defects in four, severe autism in six and dysmorphic features in nine. Suboptimal perinatal factors and intrauterine growth retardation were reported in seven and three children respectively. Five children had clinical characteristics of progressive encephalopathy which remained unclassified despite the extended diagnostic work-up. Most children with mental retardation in our study were categorized in the group of ‘possible’ aetiology. This is consistent with the findings of other studies using similar classification for the causes of mental retardation. We believe that our diagnostic efforts should be focused in this specific group of patients. Experienced clinical judgment in conjunction with better use of modem diagnostic techniques can possibly improve the internationally reported low diagnostic rate for the aetiology of mental retardation.
Child Development and Neurology de Santa Maria, Lisbon, Portugal
Departments,
and
Hospital
Introduction: Children with specific developmental language disorders (SDLD) are reported to have a high incidence of epilepsy and/or abnormal EEG findings (respectively S-28% and 20-93%). The EEG abnormalities are found more frequently in subjects who have forms of SDLD involving impairments in comprehension, occur or increase during sleep and are usually paroxysmal. There is, however, some discussion regarding their meaning and the existence of a causal relation between EEG abnormalities and SDLD has not yet been confirmed. The authors carried out a retrospective analysis of children diagnosed as having SDLD and with available EEG data, in order to study the incidence of EEG abnormalities and the relation between them and the language problems in that population. Materials and methods: The diagnostic criteria applied for inclusion in the study were: an impairment in language development as demonstrated by scores on an appropriate standardized test (Reynell’s developmental scale) substantially below those obtained from a measure of non-verbal intellectual capacity (Griffiths scale), which was within normality. Significant deafness and social deprivation were excluded. SDLD subtypes were defined according to the DSM-IV classification. We reviewed the records of the patients with the diagnosis of SDLD evaluated from 1990 to 1999 in the Child Development Department, Hospital Santa Maria, Lisbon, and selected the cases having available EEG data. Results: From a total of 126 children with identified SDLD, 49 had available EEG data, including a sleep EEG in 28; 9.3% (12/129) had a family history of epilepsy; 4.7% (6/129) children had epilepsy; 4.5% (3/67) of the cases with expressive language disorder (ELD) and 6.8% (3/44) of those with mixed receptive-expressive language disorder (MLD). The following data concern the group with available EEG data (n =49): the mean age was 4.3 years. 27 ELD, 19 MLD and three phonological disorders (I’D) were found: 18 of the 49 children (36.7%) had an abnormal EEG, including six with epilepsy (three with ELD and three with MLD). In these six cases, the EEG showed spikes (four focal - two left/two right, one bilateral and one diffuse), spike-waves (one diffuse) and in two of them an abnormal organization of the electrical activity during sleep. The abnormalities found were aggravated during sleep in three cases. In the subgroup with EEG data who did not have epilepsy, 27.9% (12/43) of the children had an abnormal EEG, including eight with spikes or spike-waves (four focal - two right/two left, one bilateral and three diffuse), two with slow waves (focal left) and three with an abnormal organization of the sleep EEG. In this subgroup, EEG abnormalities were present in 16.7% (4/24) of the children with ELD, 43.8% (7/16) of those with MLD and 33.3% (l/3) of those with I’D and occurred during sleep in 10 of the 12 cases (83.3%). In the last evaluation performed, six children (33.3%) from the group having EEG abnormalities
A143
Abstracts
(including one with epilepsy) had significant problems ZIS two (6.5%) in the other group.
residual
Discussion: In this population of children with SDLD, epilepsy was more frequent than one would expect in the general population (4.7% ~1s0.4%), the highest rate being found in the patients with mixed language disorder. Concerning the EEG abnormalities reported, the difference was even more significant. In fact, 27.9% of the children without epilepsy had abnormal (most paroxysmal type) findings in their EEGs (~1s 1.64.7% in the general population). These tended to occur more frequently in patients in whom language comprehension was impaired and were significantly more common during sleep. The outcome was more favourable in the group without EEG abnormalities. Most of these data are similar to others previously published and, in our opinion, reinforce the need to carry out prospective and wider studies, in order to better understand the correlation between EEG findings and clinical aspects in SDLD, as well as its implications.
276 Treatment
outcome
in shunted hydrocephalus
J J ROTTEVEEL,’ I HEINSBERGEN,’ A GROTENHUIS3 Departments of ‘Paediatric 3Neurosurgery, University Netherlands
N ROELEVELD,2
Neurology, ‘Epidemiology, Hospital, Nijmegen, The
and
Purpose: To identify the main risk factors for a poor outcome in children with a shunted hydrocephalus. Material and methods: The medical records of patients born between 1984 and 1992 who had a shunt operation for hydrocephalus before 1994, were analysed retrospectively. The following data were collected: general information, aetiology of hydrocephalus; information about shunt operations (timing of drain implantation, number and indication of revisions); several outcome parameters (school type, seizures and morbidity parameters according to the WHO classification).
301 Evidence-based practice and the care of handicapped children P SZATMARI McMaster
University,
Hamilton,
Ontario,
Canada
Evidence-based medicine refers to the use of the best available empirical evidence to address important clinical problems. It is based on the notion that different levels of evidence exist to answer clinical questions and that certain skills are needed in asking the right clinical questions, retrieving the information, critically appraising the available evidence and then systematically applying that evidence to the actual clinical situation. The concept of evidence-based practice is gaining wide currency in North America and elsewhere. There is certainly a great need to apply evidence-based principles to the care of handicapped children. For example, there are enormous variations in the diagnosis and treatment of several handicapping conditions of childhood including the hyperkinetic syndrome, autism and other chronic neurological disorders of childhood. Many interventions are employed for these children for which very little evidence of their efficacy exists. Moreover, treatments that have been demonstrated to be effective on the basis of high quality evidence are not being applied. Although there remain many obstacles to the implementation of evidence-based practice among health care professionals working with handicapped children, steady progress is being made in North America. There are also special problems in implementing evidence-based practice with parents of these children. With the explosion of information on the Internet and the interest of the media in health issues, parents are now aware of a bewildering array of diagnostic and treatment options that are available for their handicapped children. Parents often feel that they cannot wait for the best available evidence and are requesting services and intervention for their handicapped children that may or may not be effective and may or may not have potential to do harm. Several recent examples in autism will be presented to illustrate these issues. The lecture will conclude with concrete suggestions to improve communication between health care professionals and parents so that the important goals of evidence-based practice and shared decision-making can be achieved without conflict and rancour.
Results: We found that children with a post-haemorrhagic hydrocephalus faired poorly. Children with hydrocephalus due to spina bifida or a congenital malformation had considerably better outcome scores. Regarding the timing of drain implantation, we found a poorer mental and linguistic development in children operated more than 1 month after diagnosis. Furthermore, we found that patients with more than two revisions have a higher chance of developing mental impairment, especially if associated with drain infections.
F TORGAL-GARCIA, R GOUVEIA, A FERNANDES, A TEIXEIRA, A MARTINS, N LACERDA
Conclusion: Haemorrhage and more than two revisions are risk factors for a poor outcome. We also found that delay in surgical treatment might be an important risk factor for a poor outcome in hydrocephalic children.
Purpose: To assess the neurodevelopmental outcome of a group of children at the ages of 3 and/or 5 years followed at the Child Development Unit of the Department of Paediatrics of Hospital de Santa Maria,
384 Neurodevelopment factors
of children
with perinatal
risk
Child Development Unit, Department of Paediatrics, Hospital de Santa Maria, University of Lisbon, Lisbon, Portugal