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Biomedical subjects

R Cusmai

Publications and source records attributed to R Cusmai.

At least 37 records · Page 2Linked to original sources
PubMed1988

Symmetrical bithalamic hyperdensities in asphyxiated full-term newborns: an early indicator of status marmoratus.

Three severely asphyxiated full-term newborns showed tomographic findings of bilateral symmetrical hyperdensities restricted to the thalamic region. All these patients had a strikingly similar poor neurological outcome characterized by dystonia, severe mental retardation and acquired microcephaly. We presume that these bithalamic hyperdensities could be an early predictor of the later status marmoratus.

Asphyxia Neonatorum↗
PubMed1988

[Focal lesions in infantile spasms].

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.

Brain↗
PubMed1988

[Magnetic resonance imaging in Bourneville's disease: relation to the EEG].

Six children affected by tuberous sclerosis and severe multifocal epilepsy were studied by MRI. In all cases, MRI disclosed multiple cortical lesions and the relationship between these lesions and the EEG localization of epileptogenic foci has been studied. In all cases, a relationship between the localization of persistent EEG foci and the site of the major MRI lesions was found. The presence of frontal cortical tubers, mainly localized in the parasagittal region, was related to more intractable seizures.

Adolescent↗
PubMed1986

Increased chromosomal breakage in epileptic children after long-term treatment.

To investigate the mutagenic effects of antiepileptic drugs (AED), 39 epileptic children treated by long-term monotherapy (10 cases with Pb, 11 with Cbz, 9 with Vpa, 8 with Pht) have been studied. The long-term administration was monitored by measurement of AED serum concentrations by gaschromatography. Metaphase chromosome observations were performed using short time culture of peripheral blood lymphocytes and 100 mitoses from each proband were analyzed. A significant increase of CA in the group of patients with Pb (0.23), Cbz (0.19), Vpa (0.25), Pht (0.18) as compared with those of nine epileptic children without treatment (0.08) has been found. Because unrepaired damage of DNA may act as a possible carcinogenic potential, the shortest possible duration of AED treatment is recommended.

Adolescent↗
PubMed1984

Gelastic epilepsy and true precocious puberty due to hypothalamic hamartoma.

A new case of gelastic epilepsy and precocious puberty due to hypothalamic hamartoma is reported. After long-term medical treatment there was no observable neurological or endocrinological improvement and the clinical outcome was poor. The authors consider that early surgery for hamartoma should be reconsidered.

Diseases in Twins↗
PubMedDate not supplied

Congenital X-linked ataxia, progressive myoclonic encephalopathy, macular degeneration and recurrent infections.

We report on 2 boys (maternal cousins), with severe congenital ataxia with generalized hypotonia, psychomotor retardation and recurrent bronchopulmonary infections. Later, they developed myoclonic encephalopathy and macular degeneration. Serial brain imaging investigations showed a cyst of the septum pellucidum, persistence of the cavum vergae, corpus callosum and cerebellar vermis hypoplasia without cortical atrophy. In the maternal pedigree, 5 males had recurrent bronchopneumonia associated with severe congenital hypotonia and died during the first years of life. Neurophysiological studies, including nerve conduction velocities, brainstem auditory evoked responses, somatosensory evoked potentials were normal. Electroretinogram showed normal wave morphology. Visual evoked potentials were mildly impaired. Extensive screening for metabolic disease gave normal results. Immunologic investigations showed normal T and B cell number, T cell function and immunoglobulin levels in both patients with a reduced level of IgG2 subclass in one.

Brain Diseases↗
PubMedDate not supplied

Is there a partial benign epilepsy in infancy?

Benign idiopathic neonatal convulsions and partial benign childhood epilepsy, the most frequent types of childhood benign epilepsy, are both characterized by partial seizures. This study was performed to determine whether any kind of benign epilepsy with partial seizures occurs in the age range between these two syndromes. We identified 17 cases of benign epilepsy with partial seizures from a review of 442 records collected in a 5-year period in which seizures occurred between 8 days and 3 years of age. Six patients with onset before 6 weeks of life had clinical features characteristic of benign idiopathic neonatal convulsions, and appeared to be a delayed type of this syndrome. Eleven patients had clinical features characteristic of partial benign childhood epilepsy. In the age range between occurrence of these two recognized epileptic syndromes, we identified no case of partial epilepsy with favorable outcome.

Age Factors↗
PubMedDate not supplied

Topographic comparative study of magnetic resonance imaging and electroencephalography in 34 children with tuberous sclerosis.

A series of 34 children with confirmed tuberous sclerosis (TS) were studied prospectively by both EEG and magnetic resonance imaging (MRI) at ages ranging from 5 months to 18 years. Size and topography of the cortical tubers were analyzed on axial and coronal views, in T2 sequences, and the large tubers greater than 10 mm were studied. In addition, EEG follow-up data were reviewed retrospectively. Twenty-six patients (76%) had both MRI large cortical tubers and EEG foci, 3 had normal EEG, 2 had normal MRI, and the remaining 3 had only small tubers. The number of large tubers was significantly related to EEG foci. A topographic MRI/EEG correspondence was observed for at least one tuber in 25 of the 26 patients, and correspondence was complete in 10 patients. Large cortical tubers without corresponding EEG foci were observed in 11 patients; these tubers mainly involved the frontal regions and were found before the patients were 2 years old. EEG foci without corresponding tubers were observed in 4 patients. In addition, secondary bilateral synchrony was preferentially observed in patients with frontal lesions and after the age of 2 years. These data confirm that the cortical tubers are epileptogenic and that their expression may be influenced by regional cortical maturation.

Adolescent↗
PubMedDate not supplied

West syndrome due to perinatal insults.

Clinical, neuroradiological data and outcome of epilepsy of 32 patients with symptomatic infantile spasms caused by perinatal insults are reported. Neuroradiological investigations showed focal as well as diffuse cerebral lesions. Fifteen children had a porencephalic lesion, 12 had periventricular leukomalacia and five had diffuse bilateral cerebral atrophy, associated with status marmoratus in one child and bilateral ulegyria in another. Eight patients (25%) had epilepsy following West syndrome. In the group of 15 children with porencephalic cysts, the lesion was always unilateral, but topography and extension varied. Eight children had circumscribed cysts involving the rolandic or parieto-occipital regions, and the outcome of epilepsy was favorable. Seven patients had large lesions involving the frontal lobe, associated with unilateral hemispheric atrophy in four cases. These four children had an unfavorable epilepsy outcome. In the 12 patients with periventricular leukomalacia, all premature, eleven had no seizure relapse after the spasms; one of these children had epilepsy. In the five full-term patients with bilateral and diffuse cerebral lesions, three children had epilepsy at the last observation. In children with leukomalacia and in patients with localized porencephalic lesions the outcome of epilepsy appears to be better than in patients with diffuse cerebral lesions or in children with extensive porencephalic cysts, particularly those involving the frontal lobe.

Atrophy↗
PubMedDate not supplied

The idiopathic form of West syndrome.

The clinical and electroencephalographic data of 31 children with cryptogenic West syndrome (WS), selected from a series of 103 WS patients, with a follow-up between 4 and 12 years, were studied retrospectively to verify whether this group included patients who fulfilled the criteria for an idiopathic etiology. The results identified a possible idiopathic etiology in 17 patients (55%), who had a family history of other forms of idiopathic epilepsy or febrile convulsions, or who developed, during the follow-up, an EEG genetic trait such as a photoconvulsive response or spike-and-wave discharges, or rolandic spikes. All 17 children had a favorable outcome and all had normal neuropsychological development. Four children (13%) fulfilled the criteria for a true cryptogenic etiology, a causative lesion being suspected, but never proved. At the end of the follow-up all four had seizures, or developmental delay or both, all signs that suggest an underlying cerebral lesion. The other 10 children, representing 32% of the cryptogenic cases, had a good prognosis, with early disappearance of spasms and hypsarrhythmia, and normal neurological development, but none had an EEG epileptic trait or family history of epilepsy or febrile convulsions; although they could have had an idiopathic WS, this was not proved. We conclude that among the children classified as having a cryptogenic WS, many--in our series at least 55%--fulfill the criteria for an idiopathic etiology.

Adolescent↗
PubMedDate not supplied

[Flunarizine in drug-resistant epilepsies of childhood and adolescence].

Therapeutic effects of flunarizine have been studied on 26 patients, aged from 9 months to 17 years, suffering for epilepsies resistant to common anticonvulsant treatment, despite proper plasmatic levels of drugs. All the cases were monitored with monthly clinical and EEG controls, neuropsychological evaluations and monitoring antiepileptic drugs plasmatic levels. At first, a study was performed on an intra-patient basis: after a basal observation during two months, an open clinical trial was started, using for three months flunarizine 5 mg a day in patients weighing over 10 kg, and 5 mg every two days in children weighing less than 10 kg. Later on, a simple blind clinical trial has been performed on 16 patients, using flunarizine or placebo for three months. The results, obtained in resistant epilepsy of children and adolescent, showed that flunarizine induced in 47.6% of cases a significant reduction of critical (stroke) frequency and intensity, together with normalization of sleeping-waking rhythm, and amelioration of attention performances (vigilance, reactivity, environmental participation). The only side-effect, noticed in 23.8% of cases, was a light diurnal sleepiness, spontaneously regressing after a few days of treatment.

Adolescent↗