Biomedical subjects
Publications and source records attributed to C Diebler.
A review of cranial CT studies of 233 patients for the Second Medulloblastoma Trial of the International Society of Paediatric Oncology showed "typical" CT appearances in only 30% of patients. The varied appearances encountered are described with particular emphasis on atypical CT features.
A large fistula between the left circumflex coronary artery and the distal branches of the right pulmonary artery resulting in myocardial ischemia in a 52 year old woman with no angiographic atheromatous coronary lesions, was occluded by a detachable balloon introduced percutaneously and positioned at the origin of the fistula. A control angiogram performed one month later showed the balloon in position and the disappearance of the fistula. The Thallium myocardial scintigraphic abnormalities regressed after occlusion of the fistula. This is the second case (the first one in France) of radical non-surgical treatment of a congenital coronary fistula.
Regional cerebral blood flow (rCBF) was studied using SPECT (single photon emission computed tomography) with 133-Xenon in 13 patients with confirmed Sturge-Weber disease, aged 9 months to 18 years. CT scan, performed at the same time, showed evident cerebral angioma in 10 but not in three. A marked hypoperfused area was found in all patients, ranging from -32% to -72% and of the same location as the CT signs. The hypoperfusion seems to result from post ictal phenomenon as well as from chronic ischaemia. SPECT imaging is therefore a sensitive method for visualising intracranial angioma in Sturge-Weber disease and it provides an aid for diagnosis when a CT scan is not reliable.
Among 174 cases of infantile spasms examined by CT-scan between January 80 and December 85, 17 (9.8%) had a focal lesion without microcephaly. It consisted of an angioma (1 case), a cortical dysplasia (1 case) and porencephalic lesions (15 cases). In 11 cases with porencephaly, an acute event had occurred between 28 weeks of gestation and 3 months of life. In 7 cases, the lesion extended to the major part of one hemisphere. In the 10 others, it was localized either to the rolandic or to the temporo-occipital regions. In all the cases, the lesion concerned at least one of these areas. It involved the cortex in 15 cases but in the 2 others it seemed to only concern the white matter. The lesion was on the left in 14 cases. After steroids, the epilepsy recurred in 6 cases of which 5 involved the frontal lobe (P less than 0.05). This study seems to show that it is mainly the rolandic and the temporo-occipital areas that favour the occurrence of infantile spasms, whereas extension to the frontal area is responsible of a later partial epilepsy.
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A study of 31 observations of congenital toxoplasmosis shows that there is a clear relationship between the cerebral lesions as observed on CT scan, neurological symptoms, and the date of maternal infection. The appearance of the CT scan is characteristic of cases with early maternal seroconversion (before the 20th week of pregnancy), of cases with maternal infection between the 20 and 30th week of pregnancy, and of cases with late maternal seroconversion (after the 30th week of pregnancy). Preventive maternal treatment does not change the pattern of the cerebral lesions observed on CT scan and thus proves ineffective in conferring real protection.
Among 13 infants with apparently cryptogenic status epilepticus occurring between two and four months of age, two shared several features of 'poliodystrophy' encephalopathy. For the other 11, an anoxic-ischaemic event was suggested by the history, the seizure and EEG characteristics, and clinical and CT-scan evolution. The possible relationship with 'near-miss' sudden infant death is discussed.
Brain CT scans of 40 neonates with bacterial meningitis showed that the main lesions consisted of obstacles to CSF flow resulting in hydrocephalus during the second month; single or multiple foci of ischaemia of very early onset appearing as hypodense areas sometimes contrast-enhanced, and abscesses consecutive to haematogenous septic necrosis. The organisms responsible for these abscesses usually were Proteus spp., more rarely other enterobacteria. Attention is drawn to the usefulness of CT in detecting the nature and size of lesions which are often clinically silent, except for convulsions.
Hematomas of the brainstem are revealed by signs of brainstem dysfunction. Sudden onset is characteristic but only demonstration of subarachnoid hemorrhage and typical CT-Scan appearance are diagnostic. Surgical evacuation of the hematoma may help in the acute stage but does not avoid recurrence; pronosis is guarded.
Indications of CT examinations should be carefully established. Undisputed emergency indications in pediatric neurology are specified. Acute intracranial hypertension, whether isolated or associated with neurological signs or fever, is the principal emergency indication. Its main causes are posterior fossa tumors, but other space-occupying lesions may be found. The main radiological features in each etiology are described. Hemorrhage and trauma are the other essential emergency indications. Emergency CT examination can also be performed in unexplained coma. The indications of CT and ultrasonography in neonates are discussed.
The hamartoma of the tuber cinereum, a midline dysraphic syndrome, corresponds to a mass of normal neuronal tissue implanted in the area of the mamillary bodies. It has no evolutionary potential, but association to other cerebral malformations has been observed. On the basis of 18 personal cases and a review of the literature, the authors evaluate the actual diagnostic criteria and the incidence of different clinical signs such as precocious isosexual puberty, seizures, and mental impairment.
Cephaloceles are congenital malformations with herniation of intracranial structures through a defect in the cranium. On the basis of a review of the literature and 31 personal observations the authors discuss the clinical and neuroradiological presentation of their various anatomical locations: sphenoidal, ethmoidal, frontal, occipital and parietal.
Four infants had agyria confirmed by CT scan. All were mentally retarded, microcephalic, 3 of them having characteristic facial dysmorphy and 2 infantile spasms. EEG was characterized by very high amplitude rhythms in the alpha range, associated with delta waves and with infrequent spikes. The tracing was poorly modified by sleep. Drugs reduced its amplitude and rapid rhythms appeared with benzodiazepines. The authors point to the usefulness of the EEG in suggesting diagnosis before the CT scan, as was the case in 2 patients. They point to the urgent need of studying infantile epilepsies according to etiology, and not only to seizure types.
The authors report 7 new cases of children with laughing seizures associated with hamartoma of the hypothalamus. Precocious puberty is present in 4 cases and seizures of another type (clonic or tonic) in 5 cases. The diagnosis is confirmed by histology (2 cases) or by CT scan (5 cases). The interictal EEG abnormalities are detailed as well as those found during the laughing seizures which were registered (3 cases). The literature was reviewed and 18 cases were found with posterior hypothalamic lesions and laughing seizures: they have been studied and compared with ours. The role of hamartoma is discussed in the production of these seizures, either isolated laughing seizures or else laughing seizures accompanied with other symptoms.
Seven cases of hypothalamic hamartomas with gelastic seizures are reported. A precocious puberty was found in 4 cases. The normal neurologic examination and lack of sign of intracranial hypertension were in contrast with the severity of the epileptic seizures, of the mental impairment and of the behavioral disorders. The fact that the presenting symptom may be gelastic seizures is stressed. CT scan is the best means to assess the diagnosis and to follow the evolution of these tumors. Except for the management of the precocious puberty, the treatment is disappointing and neurosurgical indications are quite exceptional.
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The CT appearance of adrenoleukodystrophy is discussed on the basis of 16 personal observations and a review of the literature. CT appearance was typical in 10 of the 16 cases, atypical but suggestive of adrenoleukodystrophy in four cases, and misleading in two cases. Atypical CT presentations were most often observed at an early phase of the disease and included unilateral lesions or lesions without opacification at the periphery of the edemalike areas after contrast enhancement. In three cases, neurological signs, CT scans, and conjunctival and skin biopsies were highly suggestive of adrenoleukodystrophy, but there was no adrenal insufficiency. These cases may correspond to an evolutive or phenotypic variant of adrenoleukodystrophy.