[Sensory disorders screening in learning disabilities]. / Interprétation du dépistage sensoriel dans les troubles des apprentissages ?

Inserm French collective expert's report describes sensory disorders screening. However, the causality link with learning disorders remains questionable. In auditory disorders, there are high-level proof recommendations: early and intensive treatment improves language development, at least partially. For visual disorders, consensus conferences recognize their high frequency in learning disorders but there is no proof of a direct causality link. Currently, in learning disorders, orthoptic treatment is not recommended as a specific therapy. In France, despite medical ignorance about orthoptic assessment, absence of reference values and lack of therapy benefits evaluation, orthoptic treatment is usually prescribed, without any objective criteria. This article makes a literature review concerning the link between learning and sensory disorders. It also describes a typical orthoptic assessment with vision and optic musculature evaluation, and reports its results in a prospective comparative study in three populations (controls, dyslexic and Developmental Coordination Disorders [DCD] children). Strabismus or binocular vision disorders are frequent in DCD. Combined ocular motor function is almost constantly disturbed in DCD (90 %), whereas 34 % of dyslexic children and only 13 % of controls are concerned. Visual disorders are therefore present in learning disorders but also in normal population. Orthoptic assessment results must be interpreted in a multidisciplinary evaluation context.

[Attention deficit disorder: multidisciplinary observational study over 1 year]. / Le trouble déficitaire de l'attention, avec ou sans hyperactivité (TDA/H) : une approche pluridisciplinaire longitudinale croisée de 36 enfants.

INTRODUCTION: In France, attention deficit disorder (ADHD) has traditionally met with two opposing approaches (biological and psychoanalytic). This conflict led us to conduct a multidisciplinary observational study, on a group of 36 children over a period of 1 year. METHODS: Thirty-six children with ADHD diagnostic (DSM IV), not treated by MPH were included. Initial "multi-field" evaluation (T0) consisted of: neuro-paediatric consultation (Conners questionnaire, Child Behaviour Checklist, reading and writing scores by French tests); semi-structured child psychiatric interview (DSM-IV axis I), structural hypothesis (CFTMEA), existence of narcissistic fragility, parents/child interactions; neuropsychological standardized evaluation (attention and executive functions); psychodynamic interview and projective tests (Rorscharch, CAT or TAT). A therapeutic project is proposed combining MPH and psychotherapy according to the results. A new evaluation 1 year later (T1) included a consultation and a neuropsychological evaluation. RESULTS T0: All parental questionnaires appreciating attention deficit and hyperactivity/impulsivity were significantly pathological. The neuropsychological evaluation showed usual characteristics of ADHD with individual differences. The psychiatric evaluation revealed the frequency of comorbidity in axis I (23% of children with more than two diagnoses, 57% with anxiety disorder, 23 and 3% with oppositional and conduct disorder). FOLLOW UP (T1): Thirty-one children were re-examined (20 treated by MPH and 11 not treated because of parental refusal or particular psychopathological situations). Psychoanalytical psychotherapy, proposed to 28 children, was undertaken with only 19. An improvement in scores for attention and executive tests was registered only in the treated group. DISCUSSION: The tests confirm deficits of attention and executive functions without correlation with the scores of questionnaires, underlining the need for a neuropsychological evaluation to objectify attention disorders. Projective tests refine and enrich psychiatric evaluation and showed that half of the children had borderline organization. However, structural hypotheses were heterogeneous, suggesting the need for specific therapeutic projects to be devised according to each child. The treated children were the only ones to improve attention deficit. On the other hand, the scores of anxiety are not improved by MPH, emphasizing the indications of psychotherapy if comorbidity is present. Psychotherapeutic care was carried out only among part of the population, because of parental reservations, exacerbated by differences of opinion among professionals and lack of access. CONCLUSION: This study is innovative, providing precise data on ADHD from a multidisciplinary perspective. Psychopathological comorbidity is high in this population, so the concept of ADHD cannot be limited to a cognitive point of view. These elements and doubts regarding the efficacy of behavioural therapies suggest the need for a rigorous evaluation of analytical psychotherapies independent of MPH to treat attention deficit.

[Dyslexia: clinical characteristics]. / Clinique de la dyslexie.

Dyslexia is characterized by a severe, persistent reading disorder occurring in an intelligent child. In the large field of learning disabilities, dyslexia is related to a cerebral dysfunction well described with Imagery and genetic studies. Nevertheless the diagnosis of dyslexia cannot be done by another way than clinical symptoms. Optimizing the management of children with dyslexia is a critical issue and is now possible, thanks to the improvement of neurosciences data and the mobilization of the key stakeholders. The knowledge of the precise symptoms is essential in order to lead the child's doctor able to improve coordination and harmonization of teaching and care and guidance of parents.

[Validation of BREV: comparison with reference battery in 173 children with learning disorders]. / BREV, une batterie d'évaluation des fonctions cognitives: validation dans les troubles des apprentissages.

INTRODUCTION: The BREV battery (Battery for rapid evaluation of cognitive functions) is a tool which can be used for the rapid neuropsychological evaluation of children aged between 4 and 9 years. OBJECTIVES: After standardization (700 unaffected children) and validation by comparison with a reference battery (202 children with epilepsy), the aim of this study was further validation in 173 children with learning disorders. POPULATION AND METHODS: The study protocol included administration of the BREV, precise neuropsychological examination and evaluation of oral and written language. Statistical analysis was used to compare the findings of the BREV with those of the reference method, and the recommendations indicated by the BREV with the final diagnoses, and to define the sensitivity and the specificity of the BREV battery. RESULTS: All the correlations between BREV tests and reference tests were significant. Recommendations after the BREV were in agreement with the conclusions of the reference evaluation in 168/172 children for language, 145/173 for the psychometric evaluation. For only 4 chidren, the results of the BREV were false negative. Diagnoses corresponded in 168/173 children for oral language, in 102/110 for written language, 166/173 for praxis disorders and 157/173 for intellectual deficit. The most predictive subtests of the BREV and sensitivity and specificity of verbal and non-verbal scores were calculated. CONCLUSION: The BREV is a reliable examination, in learning disorders, to determine the most complementary investigations both in terms of language disorders and for non-verbal or global learning disabilities.

[Specific remedial therapy in a specialist unit: evaluation of 31 children with severe, specific language or reading disorders over one academic year]. / Evaluation ouverte de l'efficacité de la prise en charge en milieu spécialisé de 31 enfants avec un trouble spécifique sévère du langage oral/écrit.

INTRODUCTION: Up to 3 percent of the children in France present severe and specific language and/or reading disorders, despite regular remedial therapies. Few studies have measured the effectiveness of treatment administered in a specialist unit. PATIENTS AND METHOD: The aims of this study, focusing on children diagnosed as dysphasic and/or dyslexic, were: During the academic year 2001-2002, 31 children (18 dyslexic and 13 dysphasic) were attending school in our unit. The teaching program and intensive speech therapy (3 hours/week) were tailored for each child according to his/her specific disorders. Reading, spelling and numeracy developmental skills of each child were evaluated by appropriate tools at the beginning and at the end of the year. Impairment was defined by measuring the gap between the observed and the expected skills, according to each child's age. Using a self-control method, progress achieved by each child throughout the year was calculated with each tool, in each subject, by subtracting the impairments disclosed at the beginning from those disclosed at the end of the year. Progression was classified within three groups according to the progress normally expected over an academic year (i.e. nine months) from children with no disabilities attending school regularly; a progression fewer than three months was considered as no progression. Uni- and multivariate analyses including age (< or= or />9), type of pathology (dysphasia/dyslexia), and intellectual quotient (IQ) as covariates was carried out to search for independent prognosticators. RESULTS: The entire group demonstrated during the year significant progress for reading (p = 0.0001), spelling (p = 0.0001) and numeracy (p = 0.0001). Nineteen children (61 percent) showed more progress in reading than normally expected over nine months. Out of the remaining 12 children, 10 demonstrated more progress in spelling and/or numeracy than normally expected over nine months. All three reading evaluation tools disclosed a progression although one was less efficient (p = 0.05). Multivariate analysis disclosed age< or=9 and dysphasia as independent progress prognosticators. CONCLUSION: Placement in a specialist unit allows children suffering from severe dyslexia and dysphasia to lessen the gap in reading, spelling and numeracy. The two prognosticators disclosed highlight the importance of early diagnosis (i.e. before nine years old) and treatment of specific language and/or reading disorders.

Infantile spasms with basal ganglia MRI hypersignal may reveal mitochondrial disorder due to T8993G MT DNA mutation.

PURPOSE: To report three cases of infantile spasms (IS) with an abnormal magnetic resonance imaging signal in the basal ganglia (Leigh-like syndrome), due to T8993G mt DNA mutation. PATIENTS AND RESULTS: The first sign was, at the end of the first year of life, IS in one case and the combination of IS with behavior changes in the two other cases. Video EEG polygraphy demonstrated both spasms and hypsarrhythmia, but no other kind of seizures. Vigabatrin or steroids controlled the spasms with a follow-up of several years. All 3 patients had hyperlactatorrhachia (3.47 to 7 mmol/l). Axial hypotonia and dystonia appeared by the end of the first year of life. As in cases with the NARP mutation and onset later in life, neuropathy and retinopathy could also be demonstrated. DISCUSSION: Although it is well established that symptomatic IS with hypsarrhythmia mainly result from cortical lesions, this epileptic encephalopathy may also be generated by lesions in the basal ganglia without evidence of cortical damage. This finding suggests that West syndrome is likely to be caused by age-related dysfunction at any level of a cortico-putaminal loop of hyperexcitability.

Hemispheric specialization using SPECT and stimulation tasks in children with dysphasia and dystrophia.

Developmental dysphasia, a severe childhood learning disorder, is thought to result from problems in hemispheric specialization involving both left and right cerebral hemispheres. Regional cerebral blood flow (rCBF) was measured at rest and during stimulation of both hemispheres independently: dichotic listening for the left, dichaptic palpation for the right. Eight right-handed boys with expressive dysphasia, aged 8 to 12 years, were investigated using SPECT and compared with eight right-handed age-matched boys with Duchenne muscular dystrophy with reading disorders but normal speech. rCBF values at rest were also compared with those of five right-handed age-matched normal boys. In the dichotic task, children with dysphasia differed from children with dystrophia by failure to increase rCBF in the left hemisphere, in Broca's area, but rCBF increased in the right hemisphere, in the region homologous to Broca's area. In the dichaptic task, rCBF increased bilaterally for children with dysphasia whereas in children with dystrophia rCBF increased only in the right hemisphere. At rest the physiological asymmetry was reversed in favor of the right hemisphere in all areas except Broca's area. Surprisingly, the same applied at rest and for all areas in children with dystrophia. These results confirm that functional specialization of both hemispheres is impaired in developmental dysphasia. Moreover, they suggest that learning disabilities associated with Duchenne muscular dystrophy could also be related to abnormal hemispheric specialization.

[Progressive convulsive encephalopathy: considering a abnormality of biopterin metabolism]. / Encéphalopathie progressive convulsivante: penser aux anomalies du métabolisme des bioptérines.

BACKGROUND: Symptomatic forms of hyperphenylalaninemia are rare in France since neonatal screening began. CASE REPORT: A child born in Algeria from consanguinous parents was referred at 2 years of age for a severe epileptic encephalopathy with hypotonia. Amino acid chromatography revealed hyperphenylalaninemia due to a dihydropteridine reductase deficiency. Dietary restriction of phenylalanine and oral administration of amine precursors, L-dopa and 5-hydroxytryptophan had poor efficiency on epilepsy and psychomotor delay. CONCLUSION: Diagnosis of hyperphenylalaninemia must be evoked in any children with progressive encephalopathy born in country where neonatal screening is not performed.

Early single photon emission computed tomography in Sturge-Weber syndrome.

OBJECTIVES: Functional cerebral imaging PET and SPECT have shown hypometabolism and hypoperfusion in the area of vascular malformation in children with epilepsy due to Sturge-Weber syndrome. However, data are scarce in infants and do not exist in patients with Sturge-Weber disease without epilepsy. The pattern of perfusion during the first two years of life was studied including patients before the onset of seizures. METHODS: Twenty two infants with later confirmed Sturge-Weber disease underwent SPECT examination using TOMOMATIC 564 (Medimatic) and xenon-133 at ages ranging from 8 days to 25 months. Twelve had never had seizures before SPECT and seven underwent a second SPECT a mean seven months later. Cerebral blood flow (CBF) was measured in the whole hemisphere and in the part of the cortex involved in the vascular malformation on both sides as well as a "pathological to normal" index for the hemisphere and vascular malformation. These values were compared with normal age paired values. RESULTS: Compared with controls, CBF and the indices in the hemisphere and vascular malformation were significantly decreased in patients who already had had seizures before SPECT, whereas they were significantly increased in 75% of the patients who had never had any seizures. On second SPECT, the indices were decreased in all patients, including the four who still remained non-epileptic. CONCLUSIONS: SPECT therefore detects CBF asymmetry in infants with Sturge-Weber disease, which tends to shift with age. The cortex involved in the vascular malformation is hyperperfused during the first year of life before first seizures. The classic hypoperfusion appears after one year of age, even in non-epileptic patients.

Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations.

The prognosis of Sturge-Weber syndrome (SWS) is partly related to early occurrence of seizures but the diagnosis of this phakomatosis may be difficult during the 1st year of life. We have performed a retrospective study of seven patients with confirmed SWS (age 7 days to 3 months). None of the patients was asymptomatic at the time of the study. They all underwent MRI (T1 and T2 sequences) and single photon emission computed tomography (SPECT) at the same time. Regional cerebral blood flow was measured using xenon-133. In all cases, myelination appeared to be accelerated in the areas underlying the leptomeningeal angioma on both MRI sequences. In five cases, SPECT showed hyperperfusion in the damaged hemisphere. In one case, the SPECT was symmetrical and in another it showed hypoperfusion in the damaged hemisphere which was already atrophied. These data suggest that the accelerated myelination is not related to ischemia but to transient hyperperfusion. This MRI pattern can be helpful for the early diagnosis of SWS, which is of utmost importance for preventive antiepileptic treatment.

Brain functional imaging SPECT in agyria-pachygyria.

Agyria-pachygyria or lissencephaly type I, a diffuse cortical malformation, provides infantile spasms (IS) which are refractory and persisting after the first decade, an age at which IS have disappeared in the other causes. In order to study the functional postnatal development of the lissencephalic cortex, we measured regional cerebral blood flow (rCBF) using SPECT (Single photon emission computed tomography) and 133Xe in 14 children with lissencephaly, aged from 4 months to 12 years (mean = 40 months) compared to normal children of the same age range and to children with cryptogenic IS aged from 3 months to 3 years (mean = 13 months). rCBF was calculated in frontal (FR) and parieto-temporo-occipital (PTO) cortex as well as the ratio FR/PTO. FR/PTO was higher in lissencephalic patients than in controls (P < 0.001) due to higher FR rCBF (P < 0.001), particularly in patients aged less than 3 years. FR/PTO was also higher in lissencephalic patients than in patients with cryptogenic IS (P < 0.001) also due to higher FR rCBF (P < 0.001). The values of FR/PTO and FR rCBF remained stable during the first years of life and did not exhibit any age- or topography-related changes as they do in controls or in patients with cryptogenic IS. There results suggest that the normal process of postnatal development in the brain is lacking in agyria-pachygyria. That could play a role in determining the persistence of epileptic spasms, the specific seizure type of this malformation.

[Pleural lipoma]. / Lipome pleural.

Reviewing the clinic cases of two patients bearing with asymptomatic pleural lipoma, whose general course and treatment of their disease were very different, it seemed useful to us to focus on such a benign uncommon tumor. We therefore analysed this tumour's features, especially its scanographic characteristics, its spontaneous evolution, and its potential complications, in order to avoid diagnostic thoracotomy as far as possible.

[Brain abscess complicating dental caries in children]. / Abcès cérébraux de l'enfant compliquant des caries dentaires.

Brain abscess following dental or periapical infection is rare in childhood. This report describes brain abscesses found in two children with dental caries. Case 1.--A 12 year-old boy was admitted because he had suffered from acute meningitis for 3 days. Clinical examination showed symptoms of meningitis plus palsy of the right third and fourth cranial nerves and of the left facial nerve, and a defect in the left temporal field. Funduscopic examination showed papilledema; CT scan and MRI showed a ring-shaped lesion in the right occipital area. The patient was given cefotaxime and thiamphenicol. The abscess was drained; bacteriological examination showed Actinomyces viscosus and Peptostreptococcus magnus. The neurological condition and the CT scan lesion improved, but intracranial pressure increased again on the 17th day after the onset, requiring replacement of the antibiotics by rifampicin and ampicillin plus clavulanic acid for 2 months. This brain abscess appeared to be metastatic, derived from the infection of a large dental cyst due to a dental infection that had been treated 6 months earlier. Case 2.--A 8 1/2 year-old girl was admitted because she was suffering from palsy of the left facial nerve and left arm. She had had headaches and fever for a few days. Clinical examination showed the palsies and drowsiness. CT scan showed two brain abscesses. The patient was given ceftriaxone, fosfocin and metronidazole. She had been treated for a gingival abscess 1 month earlier, and had two infected teeth extracted. Improvement of the intracranial pressure was transient and the antibiotics were changed on the 12th day of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)