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Biomedical subjects

T Deonna

Publications and source records attributed to T Deonna.

At least 73 records · Page 4Linked to original sources

Prognostic value of neonatal CT scans in asphyxiated term babies: low density score compared with neonatal neurological signs.

Twenty-five asphyxiated term babies were investigated in order to evaluate the prediction of their neurodevelopmental outcome by means of computerized tomography (CT) as compared to neurological symptoms during the neonatal period. Low density (LD) areas, thought to represent hypoxicischaemic lesions, were assessed quantitatively by means of a LD score based on the extent and degree of LD, the total score ranging from 0-36. Neonatal scans were defined according to the time span elapsed between asphyxia and CT as (1) early CT (day 1-7, n = 15), and (2) intermediate CT (day 9-23: n = 14; day 29: n = 1). The newborns were classified according to the neonatal neurological findings as having mild (n = 8, 32%), moderate (n = 9, 36%), and severe (n = 8, 32%) encephalopathy, following the definition of Sarnat and Sarnat (1976). Among the twenty-two survivors, the follow-up (mean age 19.2 +/- 6.0 mts) revealed fourteen (56%) with normal outcome, two (8%) with transient neurodevelopmental anomalies during the first year, and nine (36%) with permanent abnormalities such as cerebral palsy and/or retardation (mainly global) and/or epilepsy. Early CT scans had no predictive value. Intermediate CT, however, showed distinct variations of LD areas which resulted in an LD score well correlated with the later outcome. In particular, a LD score below 14 characterized every baby who developed normally; a prediction not possible in a reliable way be means of neonatal neurological signs. For all abnormal children, the score correlated with the severity of the later neurodevelopmental disorder, except for one with the latest intermediate CT (day 29).

Asphyxia Neonatorum↗

Idiopathic fluctuating dystonia: a case of foot dystonia and writer's cramp responsive to L-dopa.

A forme fruste of progressive idiopathic dystonia is reported in a girl, starting at eight years and followed to 24 years of age. The first symptom was isolated dystonia of the left foot and later writer's cramp developed in the hands. Fluctuations of the dystonia occurred later in the course of the disorder, which were not related to the sleep-walking cycle. Complete remission of symptoms was obtained with low dosage of L-dopa. This case broadens the spectrum of fluctuating dopa-sensitive progressive childhood dystonias and provides evidence for writer's cramp being a form of focal dystonia.

Adult↗

[Functional neurosurgery of cerebral palsy].

In 1983, approximately 40 000 patients in France and 5 760 patients in Switzerland suffered from cerebral palsy, representing more than 0.1% of their respective populations. The functional disability of these patients is particularly impressive and emphasizes the medical, social and economic importance of this problem. The term cerebral palsy is restricted to non-progressive disorders of motor function, already observed at an early age and due to cerebral lesions. These motor disorders can be of paretic, dystonic and dyskinetic nature. Their epidemiology, classification, etiology, pathology, early diagnosis and evolution are extensively reviewed by Th. Deonna. The difficulty in evaluation of treatment is the absence of a generally accepted rating scale. G. Broggi has proposed one on the basis of a large experience which could serve in the future for more objective evaluation. This monograph is devoted to the functional neurosurgical treatment of cerebral palsy. Physiotherapy and rehabilitation are part of the basic treatment of cerebral palsy, and must be continued after any neurosurgical treatment. Various conservative methods of treatment and their neurophysiological rationale are mentioned by P. Claverie. Some technical devices which improve the neurological deficits and facilitate rehabilitation are presented. Radiculotomies and neurotomies are probably the oldest neurosurgical operations for the treatment of spasticity. The neurophysiological and neuroanatomical basis of this therapeutic approach are treated in the review of the material from the neurosurgical department of Montpellier. Sixty cases were collected and the results analysed according to the type of operation (posterior radiculotomy, anterior radiculotomy, mixed) performed. Stereotactic thalamotomies and subthalamotomies are believed to be the best neurosurgical method to treat the tremor and improve other dyskinesias and hyperkinesias. The technique and a personal review of 49 cases of cerebral palsy are presented. The long-term follow-up in this study demonstrates that this type of operation markedly improves the functional disability of patients with moderate hyperkinesias, moderately improves patients severely affected, but also demonstrates that possible side effects cannot be ignored. Review of the literature indicates the difficulty in interpretation of results due to a lack of objective evaluation. Nevertheless, stereotactic thalamotomy can still be recommended when tremor and rigidity are the most prominent symptoms. Stereotactic dentatotomies in the treatment of spasticity were very popular 20 years ago, but have been largely forgotten for nearly a decade.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

Agenesis of the corpus callosum: real-time ultrasonographic diagnosis and autopsy findings.

Ultrasonographic appearances of agenesis of corpus callosum and its associated abnormalities are described in four cases. Clinical data, ultrasonographic diagnostic criteria, and correlation with neuropathological findings are presented. Ultrasound is the method of first choice in the neonatal period for the detection of cerebral malformations and more particularly of agenesis of corpus callosum.

Agenesis of Corpus Callosum↗

Paroxysmal visual disturbances of epileptic origin and occipital epilepsy in children.

A special form of partial occipital epilepsy clinically resembling migraine and possibly related to the benign focal epilepsies of childhood has recently attracted attention (Gastaut 1982) but its existence is still debated. To approach this problem, in a group of 195 children with idiopathic partial or generalized epilepsy we have studied those who had visual complaints as part of their seizures (twelve children) and those who also had migraine (four children). The clinical and electroencephalographic features of these children were analyzed together with those of another group of thirty children diagnosed as migraine accompagnée in which an EEG had been obtained (3/30, i.e. 10% had paroxysmal spike-waves: one centrotemporal focus, two generalized spike-waves). One child with the type of epilepsy described by Gastaut (1982) as partial benign occipital epilepsy (phosphenes, moving lights, headaches and occipital high voltage biphasic spike-waves blocked by eye opening on the EEG) was found in the epileptic group whereas the other children of this group, including those with associated migraine, had other types of epilepsy. This "new" type of epileptic syndrome can be distinguished from symptomatically resembling entities but its place needs to be further defined.

Adolescent↗

Early diagnosis and evolution of deafness in childhood bacterial meningitis: a study using brainstem auditory evoked potentials.

Fifty-one children with bacterial meningitis were studied prospectively using serial recordings of brainstem auditory evoked potential (BAEP) from the earliest phase of the disease, according to a standardized protocol. The objectives were to make an early diagnosis and follow the evolution of deafness in the course of meningitis and evaluate the prognostic value of BAEP. Thirty-five children (68.6%) always had normal recordings; 11 children (21.6%) had transient BAEP abnormalities (prolonged wave V latency or elevated threshold for wave I), and five children (9.8%) had persistent pathologic BAEP recordings from the first examination at 48 hours until discharge from the hospital and have a persistent deafness. All recordings that were normal or pathologic at discharge were confirmed by behavioral audiometry 3 months later. These results show the early occurrence of deafness in the course of meningitis with a crucial phase of possible recovery or worsening occurring during the first 2 weeks. There were no cases of "late" deafness or "late" recovery (there was sometimes slight improvement) occurring after discharge; thus BAEPs have a prognostic value. However, observation of a child with clinically proven selective high-frequency postmeningitic deficit but without a hearing handicap, a disorder that was diagnosed early with BAEP (which tests only the high frequencies), is a warning that this method alone is insufficient and that clinical auditory surveillance and conventional audiometry remain necessary.

Adolescent↗

Prenatal diagnosis of genetic microcephaly.

True microcephaly can be diagnosed at an early stage of gestation by serial measurements of fetal head growth as demonstrated by this case report in which the diagnosis of genetic microcephaly was made but termination refused. True microcephaly was evident at birth.

Consanguinity↗

Pachygyria and congenital nephrosis disorder of migration and neuronal orientation.

A case of pachygyria with associated nephrosis has been studied. Several microscopic abnormalities have been identified: cytoarchitectonic disorders including neuronal ectopies in the molecular layer and in the meninges, improperly oriented neurons shown with Golgi stain, fetal aspect of inferior olives. The mechanism of the disorder of migration and neuronal and dendritic orientation are discussed. The significance of the association of microcephaly and nephrosis is also reviewed in light of recent literature.

Brain↗

Congenital hemiparesis: the spectrum of lesions. A clinical and computerized tomographic study of 37 cases.

Cerebral computerized tomography (CT) was obtained in 37 children with congenital hemiparesis to document the type of cerebral anomalies found in this group, correlate them with various features of the neurological handicap and clinical history and determine the prognostic and practical value of this examination in a newly diagnosed case. The CT-findings were: 1. normal CT: 4 children (11%); 2. unilateral ventricular enlargement: 9 children (24%); 3. others (cortical and subcortical lesions): 24 children (63%). In this latter group, special patterns were found: a) focal ischemic lesions: 7 cases; b) cystic porencephaly: 2 cases; c) ventricular deformities with hydrocephalus: 7 cases; d) varia: 8 cases. The children with normal CT or unilateral ventricular enlargement had mild or moderate hemiparesis without epilepsy or mental retardation. The third group comprised the more severe cases but the prognosis was very variable. The different types of CT-lesions, their possible origin and neuropathological correlates are described and compared with two previous studies. The variety of lesions found in this series and the relative prognostic value of the CT-Scan justify this examination in a newly diagnosed case.

Age Factors↗

Temporary regression during language acquisition: a linguistic analysis of a 2 1/2-year-old child with epileptic aphasia.

A 2 1/2-year-old child suffered a brief and temporary regression of speech, with complete recovery, on two separate occasions. During the second episode, clinical evaluation and electroencephalographic data led to the diagnosis of epileptic aphasia. The child's conversations at home were tape-recorded by the mother during the acute phase of the disorder and the recovery period, and transcripts of the conversations were analysed by means of a linguistic profile. These analyses confirmed the clinical improvement in the child's language abilities, which occurred within a few days. This case illustrates the remarkably specialised and localised cerebral function which underlies speech production at an early age. It indicates that this function can be interfered with selectively and temporarily, without any significant effect on general behaviour.

Aphasia↗

Persistent toe-walking in children. A comprehensive clinical study of 28 cases.

In 28 children with persistent toe-walking, in whom no etiologic diagnosis was evident from the beginning, we have studied peri- and postnatal history, details of locomotor development and the evolution of the symptom (toe-walking). A detailed neurological and orthopaedic examination was performed. Four main groups could be identified: 1. minimal spastic diplegia (5 children), 2. habitual toe-walking (9 children), 3. congenital short tendo calcaneus (4 children), 4. mixed or unclassified (10 children). A detailed reevaluation of this latter group has enabled us to reclassify them in one of the three major categories even if in certain cases several factors seemed to play a role in the toe-walking. Diagnostic criteria, the evolution and consequences of the symptom, and the familial aspect are discussed. Regarding habitual toe-walking, the history of pre-walking locomotor development, the characteristics of behaviour and the knowledge of normal development of independent gait do not offer a general explanation for this particular motor behaviour.

Adolescent↗