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Thierry Deonna

Publications and source records attributed to Thierry Deonna.

11 recordsLinked to original sources
PubMed2006

Autistic spectrum disorder: evaluating a possible contributing or causal role of epilepsy.

The onset of epilepsy in brain systems involved in social communication and/or recognition of emotions can occasionally be the cause of autistic symptoms or may aggravate preexisting autistic symptoms. Knowing that cognitive and/or behavioral abnormalities can be the presenting and sometimes the only symptom of an epileptic disorder or can even be caused by paroxysmal EEG abnormalities without recognized seizures, the possibility that this may apply to autism has given rise to much debate. Epilepsy and/or epileptic EEG abnormalities are frequently associated with autistic disorders in children but this does not necessarily imply that they are the cause; great caution needs to be exercised before drawing any such conclusions. So far, there is no evidence that typical autism can be attributed to an epileptic disorder, even in those children with a history of regression after normal early development. Nevertheless, there are several early epilepsies (late infantile spasms, partial complex epilepsies, epilepsies with CSWS, early forms of Landau-Kleffner syndrome) and with different etiologies (tuberous sclerosis is an important model of these situations) in which a direct relationship between epilepsy and some features of autism may be suspected. In young children who primarily have language regression (and who may have autistic features) without evident cause, and in whom paroxysmal focal EEG abnormalities are also found, the possible direct role of epilepsy can only be evaluated in longitudinal studies.

Adult↗
PubMed2005

Acute brain perfusion disorders in children assessed by quantitative perfusion computed tomography in the emergency setting.

OBJECTIVE: Perfusion computed tomography (CT) is a simple imaging technique that allows accurate quantitative assessment of brain perfusion. Perfusion CT is an ideal imaging technique to be used in the emergency setting and has thus gained recognition in the early management of adult acute stroke patients. Perfusion CT can be applied to children successfully by using adequate imaging protocols. The goal of this article is to provide a pictorial essay of the perfusion CT features of diseases that affect brain perfusion as depicted in a population of children who were evaluated in the emergency CT unit of our institution. METHODS: During the period of September 2001 to October 2002, all the children, who were evaluated in the emergency CT unit of our institution and who were prescribed with a cerebral CT and an intravenous administration of iodinated contrast material, underwent a perfusion-CT examination. Perfusion-CT maps were reviewed in the patients diagnosed as abnormal on the basis of follow-up clinical/radiological examinations and correlated with the results of these tests. RESULTS: Brain perfusion-CT examinations have been performed in 77 children. Fifty-three patients were considered as normal, based on normal conventional cerebral CT and normal clinical/radiological follow-up. Perfusion-CT results showed major abnormalities in 14 cases among the 24 remaining patients, related to brain ischemia in 2, head trauma in 9, brain infection in 2, and sickle cell disease in 1. These abnormalities consisted in low regional cerebral blood flow and volume values, and in high mean transit time values. They demonstrated typical anatomical distribution, depending on the considered pathological condition. CONCLUSIONS: Perfusion CT provides quantitative assessment of child brain perfusion disorders. Its ability to be easily performed upon admission makes it an ideal emergency tool that advantageously competes with other imaging techniques such as perfusion-weighted magnetic resonance imaging, despite its limited spatial coverage. Its usefulness with respect to the impact on treatment and outcome, however, remains to be established in further studies.

Adolescent↗
PubMed2004

Intractable reflex audiogenic epilepsy successfully treated by peri-insular hemispherotomy.

We report a case of an infantile hemiplegia seizure syndrome (IHSS) that presented with intractable reflex audiogenic startle epilepsy which in itself is an uncommon form of seizure disorder. Peri-insular hemispherotomy provided complete seizure control. Also of particular interest was that this syndrome resulted from an iatrogenic brain injury sustained during the course of a caesarian section. We review the different mechanisms of birth injury reported in the literature and, discuss the physiopathogenesis of the hemispheric damage in this patient. We also review the literature on "reflex epilepsy" as it applies to this case. Intractable reflex audiogenic (startle) epilepsy in IHSS submitted to hemispherotomy has not previously been reported.

Cerebral Cortex↗
PubMed2004

Brain perfusion in children: evolution with age assessed by quantitative perfusion computed tomography.

OBJECTIVE: The objective of this study was to assess the age-related variations of brain perfusion through quantitative cerebral perfusion computed tomography (CT) results in children without brain abnormality. METHODS: Brain perfusion CT examinations were performed in 77 children, aged 7 days to 18 years. These patients were admitted at our institution for both noncontrast and contrast-enhanced cerebral CT. Only children whose conventional cerebral CT and clinical/radiologic follow-up, including additional investigations, were normal were taken into account for this study (53 of 77). RESULTS: The average regional rCBF amounts to 40 (mL/100 g per minute) for the first 6 months of life, peaks at approximately 130 (mL/100 g per minute) at approximately 2 to 4 years of age, and finally stabilizes at approximately 50 (mL/100 g per minute) at approximately 7 to 8 years of age, with a small increase of rCBF values at approximately 12 years of age. The rCBF in the gray matter averages 3 times that in the white matter, except for the first 6 months of life. The global CBF represents 10% to 20% of the global cardiac output for the first 6 months of life, peaks at approximately 55% by 2 to 4 years of age, and finally stabilizes at approximately 15% by 7 to 8 years of age. Specific age-related evolution patterns were identified in the different anatomic areas of the cerebral parenchyma, which could be related to the development of neuroanatomic structures and to the emergence of corresponding cognitive functions. CONCLUSIONS: Quantitative perfusion CT characterization of brain perfusion shows specific age variations. Brain perfusion of each cortical area evolves according to a specific time course, in close correlation with the psychomotor development.

Adolescent↗
PubMed2003

Acquired epileptic dysgraphia: a longitudinal study.

A male presenting with benign partial epilepsy with rolandic spikes from the age of 7 years was evaluated at age 11 years for worsening of his epilepsy associated with a specific regression of graphomotor skills. A longitudinal study over nearly 2 years showed an improvement in handwriting to an almost normal level under modified antiepileptic therapy. A detailed analysis with a computer-monitored graphics table showed at first a rapid improvement of skills followed by protracted slower progress. We argue that the initial rapid recovery of skills was directly linked to the improvement of his epilepsy. The slower late acquisition of motor programmes that had never been fully established was due to long-standing interference by his epilepsy. The specificity of the deficit within the graphomotor system and its possible neurobiological basis are also discussed. The analytical method and approach used in a single patient might provide an example for other patients in whom epilepsy can interfere in the acquisition, progress, and maintenance of new skills and can be responsible for selective deficits.

Aging↗
PubMed2002

Preserved visual function in a case of occipitoparietal microgyria.

A 20-year-old man with bilateral parasagittal occipitoparietal polymicrogyria and epilepsy, from whom normal functional magnetic resonance imaging and electroencephalogram responses to visual stimuli were obtained, was found to have no visual perceptual deficits. This suggests that microgyric cortex can perform normal visual functions, despite its gross structural abnormalities.

Adolescent↗
PubMed2002

Early-onset absence epilepsy and paroxysmal dyskinesia.

PURPOSE: To report on the association of childhood absence epilepsy and paroxysmal dyskinesia (PD). METHODS: We describe six patients aged 6 to 27 years (mean, 14 years) who were identified in five centers participating in a European study group. Patients had been followed up clinically from the first symptoms and had been studied with video-EEG recordings of absence seizures, videotaping of dyskinetic attacks, and brain magnetic resonance imaging (MRI). RESULTS: Four patients were sporadic, and two were siblings. Age at onset of absence seizures was unusually early (range, 3 months to 3 years 6 months; mean, 16 months), with four children having their first episodes within the first year of life, and the remaining two by age 3 years 6 months. Resistance to multiple appropriate drugs was seen in five children, in four of whom absences improved remarkably when ethosuximide was added. Absences remitted between age 8 and 13 years in the three patients in whom follow-up was long enough. Different types of PD were seen including paroxysmal kinesigenic dyskinesia (one patient), paroxysmal exercise-induced dystonia (three patients), and paroxysmal tonic upgaze (two siblings). In most patients, PD appeared at a later age than, co-occurred with, and outlasted absence seizures. Only in the two siblings with tonic upgaze, dyskinetic attacks had an earlier onset. PD improved with increasing age and did not usually produce severe disability. CONCLUSIONS: There is a widening spectrum of epilepsy and PD syndromes, within which epilepsy has the characteristics of the common idiopathic syndromes, with some atypical features.

Adolescent↗
PubMed2002

A special type of head stereotypies in children with developmental (?cerebellar) disorder: description of 8 cases and literature review.

The authors report eight children, who presented in the first year of life with isolated head stereotypies, that corresponded neither to the usual normal 'rhythmic habit patterns of infancy', nor to various types of abnormal repetitive head movements described in young children. Their head stereotypies closely resembled those described in bobble-head doll syndrome. The neurological status evidenced axial hypotonia, ataxia, oculomotor abnormalities, motor and language delay. The patients were followed for several years clinically and with video recordings. No single aetiology was found. Computed tomography (CT) or magnetic resonance imaging (MRI) show a congenital cerebellar abnormality in two children but no hydrocephalus. The outcome of these children showed in all but one patient a normal cognitive and psychosocial development, even though the head stereotypies are still present in six of eight patients and all remain significantly clumsy. The association of head stereotypies and motor delay should prompt a search for cerebellar congenital malformation. The outcome of those patients was much better than originally anticipated and these head stereotypies are not related either to mental retardation, or to psychopathology. The possible mechanisms involved are also discussed.

Adolescent↗
PubMed2002

Epileptic falls and gait disturbance in two young children with a sharp wave focus at the vertex: a variant of benign partial epilepsy of childhood?

We describe two young children who presented with frequent falls and myoclonic jerks affecting the trunk and legs associated with a sharp and slow wave epileptic focus at the vertex. The initial neurological examination and brain magnetic resonance imaging were normal. Both patients had a persistent gait dysfunction, sometimes asymmetrical, fluctuating with the intensity of the epilepsy and the electroencephalogram abnormalities. The localization of the epileptic focus at the vertex, corresponding to the motor control of the legs and trunk, can explain this peculiar semiology. The seizures were difficult to treat, but one patient is currently in remission. Although epileptic falls are most often a feature of severe epilepsies of childhood, we think that these two patients present a variant of benign partial epilepsy of childhood.

Child↗