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Biomedical subjects

T Deonna

Publications and source records attributed to T Deonna.

At least 55 records · Page 3Linked to original sources
PubMed1992

[Transverse myelitis and optic neuromyelitis in children. Apropos of 3 case reports].

We report on three children with acute transverse myelitis. One of them also had optic neuritis. In connection with these three cases, we discuss etiology, diagnosis and outcome of transverse myelitis in childhood and possible involvement of the optic nerve. Our observations show the variability of spinal cord deficit and the fact that there can be a good prognosis, despite severe initial neurological deficits and massive elevation of basic myelin protein in children with ATM.

Adolescent↗
PubMed1992

Cranial CT and MRI in malignant phenylketonuria.

Malignant phenylketonuria is a rare disease caused by a deficiency in dihydropteridine-reductase which induce a hyperphenylalaninemia and a deficiency of neurotransmitters such as 3,4,dihydroxyphenylalanine (DOPA) and 5 hydroxytryptophan. The case of a patient with malignant phenylketonuria (PKU) who underwent both CT and MR Imaging is reported. CT demonstrated the characteristic calcifications of the basal ganglia. MRI demonstrated areas of hypersignal on T1 images in the basal ganglia, subcortical frontal and occipital white matter and cortex probably corresponding to calcifications. The MR findings are not specific but could be useful in monitoring the diet and neurotransmitter substitution therapy.

Brain↗
PubMed1991

Acute fatal myeloencephalopathy after combined intrathecal chemotherapy in a child with acute lymphoblastic leukemia.

We report a case of a fatal toxic encephalomyelopathy in a 12-year-old girl due to prophylactic intrathecal injection of methotrexate and cytosine arabinoside, with a characteristic progressive symptomatology leading to death after 28 days. The location and type of neuropathological changes support the hypothesis of a direct toxic effect of methotrexate and/or cytosine arabinoside on structures directly exposed to the cerebrospinal fluid.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Primitive megalencephaly in children: natural history, medium term prognosis with special reference to external hydrocephalus.

We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.

Brain↗
PubMed1990

Benign paroxysmal tonic upgaze of childhood--a new syndrome.

A child with intermittent upward deviation of the eyes starting at 9 months of age, compensating bending forward of the head, nystagmus on attempted downward gaze and a mild gait ataxia is described. The symptoms gradually disappeared between 3 and 4 years of age. Four cases with identical clinical findings have been initially described by Ouvrier in Australia (5) as "benign paroxysmal tonic upgaze of childhood". It is apparently a new syndrome.

Child, Preschool↗
PubMed1990

Does migraine-related stroke occur in childhood?

This report concerns seven children who had at least one episode of infarct, possibly during an attack of migraine. They fulfilled the following criteria: presence of acute neurological deficit associated with headache or other symptoms characteristic of migraine attacks; a history of migraine; evidence of infarct on CT scan; and no other evident cause of the stroke. CT showed that the area of infarction was in the distribution of the posterior cerebral artery in three cases. Four of the children have been followed for at least 23 months and none has severe residual deficit. An aetiological relationship between migraine and stroke could not be demonstrated. However, epidemiological data suggest that childhood migraine can be a contributory risk-factor for strokes.

Aphasia↗
PubMed1990

Hidden intelligence of a multiply handicapped child with Joubert syndrome.

A child with Joubert syndrome (episodic hyperpnoea, abnormal eye-movements and ataxia) was followed from birth to eight years of age. Although severe mental retardation was suspected until approximately five years of age, unexpected and exceptional mental capacities were evident at later follow-up. Adequate testing and appropriate stimulation of children with such a severe early motor disorder are difficult, so they may give a false impression of mental retardation. The history of this child challenges the notion that mental retardation is part of Joubert syndrome.

Abnormalities, Multiple↗
PubMed1989

Adult follow-up of the acquired aphasia-epilepsy syndrome in childhood. Report of 7 cases.

The authors report at adult age 7 patients (6 men, one woman) with the syndrome of "acquired aphasia-epilepsy", 6 of which had been previously studied as children. The results of the language, neuropsychological and socio-educational evaluation detailed many years after the onset of the aphasia are the subject of this report. One man has recovered completely, one has a normal oral language but is severely dyslexic, one has recovered normal comprehension but has severe expressive language problems. Four have absent language comprehension and lack of expressive speech, and only one of them has learned and is using sign language with some efficiency. None has developed functional written language. Attempts to offer a substitutive language to children with prolonged inability to understand and use oral language appears important but is fraught with problems. Although there are no conclusive data about the role of the continuous paroxysmal EEG discharges and the effect of their suppression with drug treatment on the prognosis of the aphasia, the definite fluctuations of the aphasia in some cases, the isolated recent case reports of definitive improvement with drug treatment justify further trials in this potentially severe and chronic condition.

Adolescent↗
PubMed1987

Childhood epileptic speech disorder: prolonged, isolated deficit of prosodic features.

A 7 1/2-year-old left-handed boy developed partial complex seizures with a left temporal electroencephalographic focus, together with prolonged, isolated deterioration of speech. Seizures stopped completely with anticonvulsant therapy and speech quickly returned to normal. Quantitative analysis of speech production and the language examination when the disorder was at its worst and after recovery allowed the documentation of the slowing and monotony of speech, in the absence of aphasic disturbance in comprehension and expression. This case illustrates the possibility of prolonged yet rapidly reversible speech deficit as an isolated manifestation of epilepsy and documents an as yet unreported feature of speech disorders of epileptic origin.

Aphasia↗
PubMed1986

DOPA-sensitive progressive dystonia of childhood with fluctuations of symptoms--Segawa's syndrome and possible variants. Results of a collaborative study of the European Federation of Child Neurology Societies (EFCNS).

Twenty children with dystonia sensitive to L-Dopa were identified in a collaborative study by 11 European colleagues. Most cases showed clinical characteristics similar to those described by Segawa (age and mode of onset, marked diurnal fluctuations, predominant limb involvement with no or minimal axial dystonia and, in all cases, dramatic relief of symptoms with small doses of L-Dopa). The typical diurnal fluctuation of symptoms was not observed in 7 children. Significant differences in this respect were noted among affected siblings. There were 9 sporadic and 11 familial cases. The pedigrees observed do not contradict nor clearly confirm Segawa's hypothesis that this might be a dominantly inherited disorder with low penetrance. Response to low doses of L-Dopa was usually dramatic, the benefit was maintained over periods ranging from one to eight years without complications. Recurrence of symptoms on withdrawal was observed in all cases in which it was attempted. These cases represent a form of progressive, presumably hereditary, childhood dystonia, similar to that originally described in Japan and different from dystonia musculorum deformans. The absence of fluctuations of symptoms despite good L-Dopa response and the great variability in the severity of the disorder were important features which will require further study as will the homogeneity of the syndrome, the mode of genetic transmission and the need for persistent L-Dopa treatment in adult life.

Child↗
PubMed1986

Combined myoclonic-astatic and "benign" focal epilepsy of childhood ("atypical benign partial epilepsy of childhood"). A separate syndrome?

The authors have followed six children with atypical epilepsies but a favorable evolution, consisting in minor motor seizures of the myoclonic-astatic type (with diffuse slow spike-waves on the electroencephalogram) together with clinical and EEG features seen in benign focal epilepsy of childhood (BFEC), an association recently reported by Aicardi and Chevrie (1982). The maintenance of a normal neurological function despite severe epilepsy, the absence of tonic seizures and the marked activation of the spike discharges during sleep were described by these authors as important characteristics suggesting a good prognosis. The purpose of this study was to see if these children indeed represent a particular subgroup of idiopathic epilepsy and to draw attention to a special clinical and EEG combination indicating a possibly favorable ultimate outcome, in children usually diagnosed as suffering from Lennox-Gastaut syndrome. The six cases closely resembled the group described by Aicardi and Chevrie (1982), although the clinical and EEG features of BFEC were not as striking as in their cases. Also transient mental deterioration occurred during the active seizure periods. The therapeutic benefit of the various drugs tried was difficult to assess, but the behavior was often perturbed by medication. Although it is not possible to decide at the present time if these cases represent a particular epileptic syndrome, the special combination of clinical and EEG features seems characteristic enough to justify prospective studies of similar cases in the future.

Adolescent↗
PubMed1985

Temporal evolution of hypoxic-ischaemic brain lesions in asphyxiated full-term newborns as assessed by computerized tomography.

Hypoxic-ischaemic brain lesions may be detected as low density (LD) areas by means of computerized tomography (CT), but the clinical significance of such LD areas has been controversial. Since timing might be a critical factor, we studied the temporal evolution of LD areas in 9 asphyxiated term babies who had had two or more CT, and compared the changes to the neurodevelopmental outcome. Scans were classified according to the elapsed time after asphyxia as early (day 1-7, n = 6), intermediate (week 2-4, n = 7; week 4-7, n = 3) and late CT (3 months or more, n = 7). In early scans, no, or only ill defined, LD areas were seen in the periventricular region. In intermediate CT's, LD-zones were further diminished in those babies who later were normal. Sharply accentuated LD areas, however, appeared in those who later suffered from neurodevelopmental disorders. These LD areas, probably representing hypoxic-ischaemic lesions, were located periventricularly, extending into the subcortical white matter and the cortex, and usually involved both hemispheres symmetrically. They began to disappear at 4 to 7 weeks in some regions, possibly because of glial proliferation. LD persisting more than 4-7 weeks tended to transform into cyst-like lesions, or marked atrophy. We conclude (1) that hypoxic-ischaemic lesions appear as zones of low density on CT scans performed after the first week and (2) that the extent of such lesions can best be assessed between 9 to 23 days after asphyxia.

Asphyxia Neonatorum↗