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Biomedical subjects

T Deonna

Publications and source records attributed to T Deonna.

At least 91 records · Page 5Linked to original sources
PubMed1980

Temporal lobe epilepsy and hemianopsia in childhood of perinatal origin. An overlooked and potentially treatable disease? Report of two cases, one with a demonstrable etiology.

Two children with a history of traumatic delivery developed severe temporal lobe epilepsy starting at age 4 and 10 respectively. The main neurological finding was total homonymous hemianopsia. The CAT Scan showed a large localised hypodensity in the occipital region on one side consistent with an old infarction in that region. A similar clinical syndrome was described in 1974 by Remillard as temporal lobe epilepsy and perinatal occlusion of the posterior cerebral artery (PCA). In one of our cases, the clinical course suggested that vascular occlusion in the PCA territory resulted from temporal lobe herniation related to a subdural hematoma at birth. It is proposed that this mechanism may apply to some instances of that condition. This clinical syndrome is now increasingly diagnosed as a large number of epileptics are now submitted to CAT. The diagnosis can be delayed until a seizure disorder develops because of the frequent absence of obvious neuromotor signs or visual complaints in the first years of life. Early recognition of this particular type of neonatal cerebral injury could possibly lead to prevention or early treatment.

Epilepsy, Temporal Lobe↗
PubMed1979

Benign hereditary (dominant) chorea of early onset.

Two siblings (one girl 7 1/2 and one boy 6 1/2) are described with a choreic syndrome dating from early childhood without other neurological abnormalities. The father was similarly affected but has markedly improved. Extensive neurological work-up and family history permitted to rule out numerous conditions known to cause abnormal movements in childhood. The clinical entity corresponds to the recently described syndrome of hereditary benign non progressive chorea. To our knowledge it is the first described family with this entity in Switzerland. The relevant literature is reviewed particularly as it pertains to the genetic unity of the syndrome. Its importance lies in its differentiation from the other more severe hereditary abnormal movements of childhood, its non progression and tendency to improve with time. The possible reasons for the only very recent recognition of this purely clinical syndrome are also discussed.

Adult↗
PubMed1979

Myoclonic encephalopathy of infants or "dancing eyes syndrome". Report of 7 cases with long-term follow-up and review of the literature (cases with and without neuroblastoma).

We describe 7 children with myoclonic encephalopathy of infants (MEI). MEI is a clinical entity characterized by an acute or subacute onset of polymyoclonia, cerebellar ataxia and opsoclonus ("dancing eyes"). It occurs either spontaneously, following an infectiuos illness or in association with an occult neuroblastoma. It is likely that immunological factors play a role in the pathogenesis. Steroid therapy resulted in rapid dramatic improvement of the neurological symptoms in 4 cases. However, this initial response did not correlate with the eventual outcome. We reviewed the literature to compare 45 reported cases of MEI associated with a neuroblastoma with 48 children without such a tumor to identify possible differences in clinical presentation, response to steroid medication and long-term prognosis of the neurological syndrome. In this respect we found no differences. Impairment of motor, verbal or intellectual performance were reported in at least half the cases. Although an immediate and marked response to steroids occurs in many cases of both groups, it remains unclear whether the long-term outcome is favourably influenced by this medication. The two-year-survival rate (90%) in the neuroblastoma group and the percentage of mediastinal localisation of the tumor (49%) are much higher compared with neuroblastomas without MEI. The reasons for these remarkable differences are not known. Diagnostic, therapeutic and prognostic implications justify the separation of MEI from the more common and benign syndrome known as acute cerebellar ataxia of childhood.

Adrenocorticotropic Hormone↗
PubMed1979

[Mental deficiency: organic aspects].

This review of the organic aspects of mental retardation considers this handicap among the other chronic neuropediatric disorders and is one of the numerous possible manifestations of a cerebral damage occurring in a developing nervous system. The relative frequency of the different etiologies is discussed in relation to the severity of the mental handicap. The prenatal causes of mental deficiency which are the most frequent are reviewed and a distinction is made between malformations and pseudomalformations. Particular mention of the fetal alcohol syndrome is made. The role of perinatal medicine in the epidemiology of mental retardation is discussed and the diagnostic approach of a particular child with mental retardation is presented with the personal experience of the author in the pediatric department, CHUV, Lausanne. The possible measures to prevent mental retardation are finally discussed.

Brain Damage, Chronic↗
PubMed1979

Nonsurgical treatment of interhemispheric subdural empyemas.

Interhemispheric subdural empyema complicating sinusitis was diagnosed in two children by CAT scan. One of them presented with intracranial hypertension and paresis of one foot (syndrome of the falx cerebri). Antibiotic treatment alone without surgery (in one case with brief initial steroid therapy) brought rapid and complete clinical and radiological cure. A nonsurgical approach can now be considered in certain cases of intracranial local suppurations, given the possibility of earlier and more precise initial diagnosis and follow-up with CAT scan.

Adolescent↗
PubMed1978

Klinefelter syndrome and neurological disease.

Six patients with Klinefelter syndrome (47,XXY) and different neurological disease are described. Essential tremor has been reported repeatedly but its significance deserves further studies. The prevalence of epilepsy with the Klinefelter syndrome is higher in comparison with the normal poplation, but it is not greater than expected in the population of a mental hospital. Therefore we suggest that the neurological symptoms with the Klinefelter syndrome should basically be regarded as coincidental findings not related to the chromosome abnormality.

Adolescent↗
PubMed1977

Acquired aphasia in childhood with seizure disorder: a heterogeneous syndrome.

The authors report six children with acquired aphasia of unknown etiology. The clinical picture was clearly different from that seen in the usual childhood aphasias and resemble other cases initially reported as "syndrome of acquired aphasia with convulsive disorder". All had associated paroxysmal EEG abnormalities, and 5 have had clinical seizures. The language disorder has improved or remained stationary and no other neurological signs have developed. Our review of the literature and the study of our personal cases show no uniform clinical picture in these children. Three different clinical patterns seem to emerge. The first group show rapid onset and recovery of aphasia, frequent fluctuations in the severity of the language deficit typical of so-called epileptic aphasia. These children appear to have a better prognosis. The second group show worsening of the aphasic deficit after repeated seizures or episodes of aphasia. In the third group progressive deficit in language comprehension (auditory agnosia) with a variable degree of recovery and rare or no clinical seizures. The possible significance of the EEG abnormalities has been discussed and the importance of the aphasia on general behavior and the problems of differential diagnosis have been stressed.

Adult↗
PubMed1977

Hypoglycorrhachia in neonatal intracranial hemorrhage. Relationship to posthemorrhagic hydrocephalus.

The level of cerebrospinal fluid (CSF) glucose may be lowered after subarachnoid hemorrhage. This was observed in each of 18 cases of proven posthemorrhagic hydrocephalus in infants (study group). In one of these children with a hemorrhagic spinal fluid and hypoglycorrhachia unaccompanied by clinical signs of intracranial hemorrhage or hydrocephalus, the axial tomography showed a significant although asymptomatic hydrocephalus. To further evaluate the significance of this finding (hypoglycorrhachia), we compared the incidence of hypoglycorrhachia (CSF glucose less than 40 mg) and lowered CSF glucose/blood glucose ratio (ratio less than 0.4) at three similar time intervals from the presumed time of the intracranial hemorrhage in the study group with that of a control group of 40 neonates with similar neonatal associated pathology (mainly premature infants with hyaline membrane disease) but who did not later develop posthemorrhagic hydrocephalus or cerebral palsy. There was a statistically greater frequency of these anomalies in the hydrocephalic group. Only 3 of the 40 control patients had hypoglycorrhachia and low ratio. Hypoglycorrhachia in the absence of other known causes for decreased CSF glucose is a good index of a probably significant meningeal hemorrhage with a high risk of secondary hydrocephalus which may or may not be symptomatic. Hypoglycorrhachia may be used as an indication of the frequency of clinically inapparent subarachnoid hemorrhage in these high risk newborns.

Cerebrospinal Fluid Proteins↗
PubMed1975

[Plasma concentration of phenytoin in epileptics--importance for the clinician].

Plasma concentrations of diphenylhydantoin were determined by the method of Wallace (double extraction, spectrophotometry) in 150 samples taken from 121 epileptic patients. They correlated well with those determined by gas chromatography, were not dose-dependent and were often below 10 mul/ml. They were above 20 mul/ml in 6 patients with CNS intoxication. These determinations were also useful for detecting patients who did not take the drug as prescribed (14 suspected cases, 4 confirmed). However, blood levels did not seem to offer an accurate index of the effectiveness of diphenylhydantoin.

Adult↗