Showing papers in "Developmental Medicine & Child Neurology in 1998"

Behaviours caregivers use to determine pain in non-verbal, cognitively impaired individuals

Abstract: To create a checklist of behaviours that caregivers could use to determine pain in non-verbal individuals with mental retardation, primary caregivers were recruited by the Division of Neurology and interviewed using a semistructured interview. Caregivers of 20 individuals were asked to recall two instances of short, sharp pain and two of longer-lasting pain and describe the individual's behaviour. Transcribed interviews were reviewed by two of the authors and sets of non-overlapping items were developed. Average age of the 20 individuals was 14.5 years (range 6 to 29 years) and language level averaged 10 months as scored by the MacArthur Communicative Development Inventory. All had mental retardation and 18 had epilepsy and spastic quadriplegia or hemiparesis. Thirty-one behaviours were extracted from the interviews. The specific behaviours were often different from one child to another but the classes of behaviours (Vocal, Eating/Sleeping, Social/Personality, Facial expression of pain, Activity, Body and limbs, and Physiological) were common to almost all children. Reliability of using the checklist on interviews was very good (kappa=0.77). The checklist has excellent content validity and will be useful for caregivers of cognitively-impaired, non-verbal individuals to report on pain behaviours. Further research is needed to additionally assess its validity and sensitivity.

Effectiveness of speech intervention for phonological disorders: a randomized controlled trial

Abstract: Thirty children of preschool age with severe phonological disorders were randomly assigned to two treatment groups. Group 1 received treatment for 4 months followed by 4 months without treatment while group 2 underwent 4 months without treatment followed by 4 months of treatment. The outcome measures used were the Assessment of Phonological Processes-Revised (APP-R), the Goldman-Fristoe Test of Articulation (GFTA), the Percentage Consonants Correct (PCC), and Mean Length of Utterance (MLU). Group 1 showed significant differences on scores of phonological measures (APP-R, GFTA, and PCC) after the first 4 months of the study. At the 8-month assessment point the measures for conversational speech intelligibility continued to be significantly different, with group 1 scores higher than those of group 2. The expressive language measure did not detect a difference between groups at any time; however group 1 scores were consistently higher than group 2 scores.

Relation between blindness due to retinopathy of prematurity and autistic spectrum disorders: a population-based study.

Abstract: Children with blindness due to retinopathy of prematurity (ROP)--who are at greatly increased risk of cerebral damage--have been noted to have a high rate of autistic symptoms, but systematic controlled studies have been lacking. A controlled population-based study was performed; one group was blind due to ROP (N=27) and the other was congenitally blind due to hereditary retinal disease (N=14). Fifteen of the 27 children with ROP had autistic disorder. All these children were mentally retarded and about one-third of them had cerebral palsy. In the comparison group, two of the 14 children had autistic disorder. It is concluded that there is a strong association between ROP and autistic disorder. The association is most probably mediated by brain damage and is largely independent of the blindness per se.

MRI brain changes in subjects with Down syndrome with and without dementia

Abstract: Individuals with Down syndrome (DS), a disorder of known genetic etiology (trisomy of chromosome 21), exhibit several types of structural brain abnormalities that are detectable pathologically and by MRI. In addition, in middle age, individuals with DS develop histological and, in some cases, clinical features of Alzheimer's disease (AD). Abnormalities in MRI scans of 50 adults with DS, 11 of whom had clinical dementia, are described and compared with those of 23 cognitively normal, healthy subjects who were matched for age, sex, and race. Qualitative visual analogue scale (VAS) ratings on MRI hard copies for all subjects and computer-aided volume measures for a subsample of subjects were carried out. On VAS, subjects with DS had larger lateral ventricles, a higher frequency of posterior fossa arachnoid cysts/megacisterna magna and fewer scans rated as normal compared with controls. Quantitatively, total brain and gray-matter volumes were reduced in DS, as were the volumes of the left hippocampus and amygdala; ventricle volumes were larger. Post hoc comparisons of subjects with DS with and without dementia revealed that on VAS the former had more generalized atrophy for age, mesial temporal shrinkage, and third ventricular enlargement. Similarly, total brain, left hippocampus, and left amygdala volumes were reduced quantitatively in subjects with DS with dementia, while ventricular volumes were increased.

Are skinfold measurements suitable to compare body fat between children with spastic cerebral palsy and healthy controls

Abstract: The aim of this study was to assess whether percentage of body fat (BF) can be predicted adequately from skinfold measurements in comparative studies of children with spastic cerebral palsy (CP) and healthy control children. The deuterium dilution technique (D2O) was used as a reference method. In contrast with what was expected, %BF predicted from skinfold measurements was considerably lower than that determined by the D2O method in children with CP, whereas in the controls this was not seen. A proportionally large internal fat deposit and a different distribution of subcutaneous fat in children with CP may be responsible for this. It was concluded that skinfold measurements are not suitable for predicting %BF in comparative studies of children with CP and control children. For evaluation of diet- or exercise-related interventions in children with CP, the use of skinfold measurements seems to be justified. However, it may be more appropriate to use skinfold thicknesses without converting them to BF.

Development of perioral muscle activity during suckling in infants : a cross-sectional and follow-up study

Abstract: The activity of the perioral muscles during breastfeeding in infants was investigated using EMGs. Fifty-six infants aged from 1 to 5 months were classified into five groups according to month of age in the cross-sectional study. Follow-up was carried out on 18 infants whose mean age was 2.5 months at the initial examination, and 4.8 months at the second. During suckling, EMGs were recorded unilaterally from the temporalis (TM), the masseter (MM), the orbicularis oris (OM), and the suprahyoid (SM) muscle groups. The activity of the SM increased significantly with age, while there was no appreciable increase in the activity of the TM, MM, and OM in either the cross-sectional study or the follow-up. However, total muscle activity was shown to increase significantly in both parts of the study. These findings suggest that the active tongue- and jaw-lowering movement may play a primary role in increasing sucking strength during the suckle-feeding period in infants.

'prevalence of tourette syndrome in a mainstream school population'. authors' reply

Abstract: The aim of this study was to ascertain accurately the prevalence of Tourette syndrome (TS) in a mainstream school population. All year 9 pupils (aged 13 to 14 years) in a mainstream secondary school were investigated using a two-stage procedure. Standardized questionnaires were completed by parents, teachers, and pupils. Class observations were also carried out to identify tics. Those pupils identified as having tics underwent a semistructured interview to determine whether they had TS according to DSM-III–R criteria. Data were available from at least one source (parent, teacher, or self-reports) on 166 of the 167 pupils in the year. Five subjects were identified as having TS according to DSM-III-R criteria, yielding a prevalence estimate of 299 per 10 000 pupils in this age group. The results of this study suggest that TS in the community as a whole is more common and milder than those prevalence estimates and descriptions of the disorder based on TS encountered in secondary or tertiary health-care service settings.

How much brain does a mind need? Scientific, clinical, and educational implications of ecological plasticity.

Abstract: Plasticity is defined as the brain's capacity to modify its structure or function as a reaction to learning and to brain damage. The term has a variety of meanings: sometimes only microanatomic changes are regarded as neuroplastic1, but some authors use plasticity in its broadest sense to mean the capacity of an organism to adapt, as opposed to being a static, fixed structure2. Although Lashley4 can be seen as one of the research pioneers of plasticity as far back as 1944, neurobiological understanding is fairly recent. The number of plasticity studies is rising exponentially: from seven studies between 1966 and 1974, to 1139 between 1995 and 1997. Mechanisms of plasticity include: an increase in synaptic connections, axon sprouting and thickening, neurochemical moditications, construction of new functional pathways4-8, and neuroprotein synthesis9. These phenomena also occur in normal brain development associated with learning9. However, plasticity need not necessarily imply an anatomical change, as in functional compensation10, 11. In this case an impaired function is compensated for by another system, for example, orientation by a blind man through compensatory auditory attention. Functional repair is a term reserved to indicate restoration of functional neural connections after injury. In this article, the tern plasticity is used in its broadest sense.

Assessment of preterm infants in the intensive-care unit to predict cerebral palsy and motor outcome at 6 years.

Abstract: One hundred and fifty-three infants were assessed by a method specific to preterm infants and appropriate to the intensive-care nursery environment. The presence or absence of six atypical features (coarse jitters, dominant asymmetrical tonic neck reflex, paucity of movement, 'contradictory' development, hypotonia, or hypertonia) resulted in neonatal classification. There were 116 'usual' (an absence of atypical features with progressive development), 26 'unusual' (a transient occurrence of any atypical feature), and 11 'suspect' (dominance of one or more atypical features). At 6 years of age the motor subsystems of balance, coordination, fine and gross motor, and associated movements were assessed. Nine of 11 children with cerebral palsy had neonatal categories of 'suspect' (7) and 'unusual' (2), of the remaining four other 'suspect' children, two failed two motor subsystems and two failed motor items. A 'usual' neonatal assessment predicted normal motor outcome for 72 of 116 (62%) whereas a 'suspect' assessment predicted major motor dysfunction for seven of 11 of the children.

Juvenile familial amyotrophic lateral sclerosis: four cases with long survival.

Abstract: Four cases are reported of juvenile familial amyotrophic lateral sclerosis (JFALS) with exceptionally long survival (mean=27 years), and consequent development of dementia. Subjects' mean age at onset was 15.7 years. Their clinical features and electrophysiological findings support the diagnosis. One subject's MRI scan showed severe atrophy to the cortex and brain stem; wallerian degeneration in the pyramidal pathway, as reported in other studies, could not be found. JFALS is characterized by the involvement of other neuronal systems not present in the adult form and by long survival after disease onset.

'Reflections on the hypothesis for the etiology of spastic cerebral palsy caused by the "vanishing twin" syndrome'.

Abstract: 'Reflections on the hypothesis for the etiology of spastic cerebral palsy caused by the \"vanishing twin\" syndrome' Sir-I read withgreat interest the intrigiritigpaper by Pharoah and Cooke'. T1.e essence of their proposed Iiypotbesis is that single ettibryotiic dettrise in twins may be intplicared in the etiology ofspastic cerehriilpalsy (CP). Singgle en~bryonic demise, ulso knouvi as the 'vanishing tuJin'pbcnornenon bas been, until recetitljf, only recognized bjl sonogrrrphy. The initial image oftroo distinct gestations (one of ivhich disq@?ars on ci sirhseqirent sonogram) is h@h!y srrgsestive ofdichorionic (DC) t winning Hence, the sonographic inmage oftbe imiishing trvin .yyndro?tIe miry cast serioirs rloribts on the mithors ' hj'potbesis. Brain dunmage in the suwiiw folloudng single fetal demise is uglniost esclrisively seen in nrotiocboriotiic (111C) twit is in uhicl! inter1 it fit I t t-ansplrrceritul vascular ritrtistontoses are iiivmiLihly foirnd atid associuted udtb the tuh-tiuin trmzsjrrsiotz s)wdrottzr (T7XS)'. Folloruing the rleirrh of one twin. tbroinbopl~rstiti-li~~~ tticzterirrl is transfirsed through the iviscIrIcir connection into tbe sirri~iiwr's circulation, rtwrlt ing in ettil-orgri)t rlirnuige (the 'mzbolic ' theoql). Alternutioely~ it bas beeti proposed lbnt blood is sl~i~nted viu an open mzustoniosis into tbe lori~-resisicrrice ixisciilar systetn clft lk deud fetus, resulting in uciite Iqpoi-otentia, ischemia, atid eird-orgcitt i i ~ i u y (the 'ischemic' theoty)'. Despite the otigoing cotrtroaersy about iclhicb of lbe md..atrisms is correct, both reqiritr the opening of (in existing finastornosis. Accorditzg to the t!!ypott!Jcsis ofYhnrouh ancl Cboke, some forni of twitr-licit1 trcitis/rrsioti shorrlcl occiir (it mi early stage qfprqnnttcy To settle the cliscrepatrcy betuiecw the pntboguiesis of CP mid the sonogrcrpbic image ofthe vanishing twiti sytidrottie, two possibilities exist:first-tritnestcv TTlSor a coninion intertwin circulation in UC twim. T ~ J C idea tlkit the ucr~iishitig twin sjwdrottie might be the result of erirly-onset ]\"ITS bm beeti inil~liedprevioirsly und is based o t z the follouJrzg mgirnzents' ( I ) The placenta of 7TTJ' arid of the survivor of the iunishitig tiuiti syndrome lire chracterizeri by iin extrendy bigh prevalence of iiekinienlorrs insertion of the umbilical cord. 7;be fact thut the two s-vndronies share the same (rme) pliicenttil anomaly tnay suggest a coninion etiology. (2) T77X way occiir earlier that1 preuious3, thoright. Cmdiac deconipensatiori of the r&pietit twin bcis beetr obseroed b3~ ecbocardiograpby as earij as 12 to 13 weeks'gestation. T ~ s , TTTS sboirld t i 0 longer be considered a niid-pregtiatic~' phetionietrotI but niiiy, in fact, develop during the first triniester. (3) Sonograpbic accotirits of the vanislXrrg twin syndrome rarely consider placental chorionicity U I I ~ , therefiire, the possibility tbut some cases may huve been IMC twins cuttnot be excluded. .b our krioidedge of TTiT is deriued entirely from cases which develop during ttrid-triniestec one can speculate that some unastonioses tnay h u e occiirred in cases of DCplacentatiotr, but sitbseqrrently disappeared and were tnissed udwn second and third trimester placentas were exaniined. In f m t , anastonioses are found, albeit rarely, b i term DC placentas, but are not iissociated with 7\"KCS2.