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Biomedical subjects

P Curatolo

Publications and source records attributed to P Curatolo.

At least 37 records · Page 2Linked to original sources
PubMed1998

Frontal lobe epilepsy associated with tuberous sclerosis: electroencephalographic-magnetic resonance image fusioning.

We studied the topographic relationships between cortical and subcortical lesions shown on magnetic resonance images (MRI) and sources of epileptiform activity in a series of nine children with intractable epilepsy and tuberous sclerosis complex. Although video-electroencephalographic (EEG) monitoring was suggestive of a frontal seizure onset, interictal EEG was, in seven of nine cases, in the form of apparently bisynchronous discharges. In all cases, the use of a short time lag estimation procedure based on a nonlinear correlation function between surface recorded EEG signals allowed the detection of a lateralized onset of EEG paroxysmal activity. Furthermore, a computerized method based on a source localization EEG-MRI image fusioning procedure, has revealed a topographic concordance between well-defined frontal cortical lesions shown on MRI and site of onset of paroxysmal discharges. Lennox-like EEG patterns frequently reported in children with tuberous sclerosis complex could be the result of the tendency of frontal tubers to induce secondary bilateral synchrony, with implications in the medical and eventually surgical management of the often drug-resistant associated seizures.

Child↗
PubMed1996

Neurological manifestations of tuberous sclerosis complex.

CNS lesions of tuberous sclerosis complex (TSC) are due to a developmental disorder of neurogenesis and neuronal migration. MRI studies provide excellent in vivo demonstration of the various pathologic lesions. Symptoms of cortical tubers may include seizures, mental retardation, learning disabilities, and abnormal behavior. Seizures have a focal or multifocal origin, this clinical feature depending on the localization of the cortical tubers. Epilepsy associated with TSC is often intractable, but seizure control has benefited from the introduction of the new antiepileptic drugs. Carefully selected drug-resistant patients can be assessed with intensive monitoring as candidates for surgical removal of epileptogenic lesions. The success of epilepsy surgery is predicated on the clear identification of epileptogenic foci.

Anticonvulsants↗
PubMed1995

Risk factors for the co-occurrence of partial epilepsy, cerebral palsy and mental retardation.

A case-control study (64 cases and 209 controls) was carried out to identify risk factors for the co-occurrence of early-onset partial epilepsy, cerebral palsy and mental retardation in children with and without cerebral malformations. History of epilepsy in first-degree relatives, maternal diseases in the two years before pregnancy, placental pathologies, low gestational age, being small for dates, neonatal convulsions and the need for cardiopulmonary resuscitation were associated with partial epilepsy, cerebral palsy and mental retardation. A family history of epilepsy in first-degree relatives was surprisingly frequent in both groups, suggesting that genetic factors play an important role for children with and without cerebral malformations.

Adolescent↗
PubMed1994

Early clinical and imaging (high-field MRI) diagnosis of Hallervorden-Spatz disease.

In a 10-year-old girl, with progressive motor disturbance since the age of seven years and dominant pyramidal signs, MRI revealed marked overall low signal from the globus pallidus on each side, with central zones of high signal. There was also strikingly low signal in the zona reticularis of the substantia nigra, indicative of Hallervorden-Spatz disease (HSD).

Child↗
PubMed1993

Familial white matter hypoplasia, agenesis of the corpus callosum, mental retardation and growth deficiency: a new distinctive syndrome.

We have observed in two different families two pairs of male siblings born from normal, non-consanguineous parents having the same syndrome, characterized by severe cerebral white matter hypoplasia, agenesis or extreme hypoplasia of the corpus callosum, mental retardation, failure to thrive and minor midline facial abnormalities. This seems to be a previously unreported genetic syndrome.

Abnormalities, Multiple↗
PubMed1991

Tuberous sclerosis: relationships between topographic mapping of EEG, VEPs and MRI findings.

A group of 10 patients suffering from tuberous sclerosis (TS) and epilepsy was studied by means of topographic mapping of EEG and visual evoked potentials. The localizing ability of the technique was compared to the topography of hyperintense T2-dependent areas seen on magnetic resonance images (MRI). Data were collected from 19 electrodes, free from interictal EEG transients and artifacts, spectral analysis was performed using the FFT algorithm and color maps were produced by specialized equipment. Data were analyzed with respect to the presence of interhemispheric asymmetries and significant differences with age-matched normal controls. The topography of slow frequency components was the best clue for localization, with a concordance between imaging and spectral EEG data in 76% of the lesions detected by MRI. In 7 patients topographic mapping revealed spectral abnormalities in areas where MRI was not able to detect morphological lesions. These abnormalities were characterized by an increased delta (5 patients), theta (4 patients), or lowered ipsilateral alpha (1 patient) power. Although full agreement between imaging and electrophysiological data was not observed, we believe that in patients with TS the study of EEG rhythm activity can add valuable information to the visual inspection of the tracings.

Adolescent↗
PubMed1991

Neuropsychological aspects of tuberous sclerosis in relation to epilepsy and MRI findings.

The neuropsychological performances, outcome of epilepsy and MRI topography of tubers of 23 children with tuberous sclerosis were reviewed. Seven children had normal intelligence, 10 had mental retardation, and six mental retardation and autism. An adverse association was found between the number of lesions and IQ, behaviour and severity of epilepsy. Posterior lesions, in addition to frontal-lobe dysfunction, were observed in children with autism. Tuberous sclerosis with well-defined cerebral lesions may represent a model for the relationship between different neuropsychiatric problems.

Adolescent↗
PubMed1991

Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy.

The epidermal nevus syndrome (ENS) is a sporadic neurocutaneous disorder that consists of epidermal nevi and congenital anomalies involving the brain and other systems. From among over 60 patients with ENS presenting with neurologic manifestations, we identified 17 who had hemimegalencephaly based on pathologic or radiologic studies. Associated brain and neurologic abnormalities included gyral malformations in 12 of 12, mental retardation in 13 of 14, seizures in 16 of 17 (including 9 with infantile spasms), and contralateral hemiparesis in 7 of 12. All had ipsilateral epidermal nevi of the head, and several had ipsilateral facial hemihypertrophy. We concluded that these abnormalities comprise a recognizable neurologic variant of ENS that we believe represents the full expression of primary brain involvement. Several patients also had evidence of acquired brain lesions such as infarcts, atrophy, porencephaly, and calcifications, which are best explained by prior ischemia or hemorrhage. Given repeated observations of blood vessel anomalies in ENS patients, we hypothesize that underlying vascular dysplasia predisposes to these acquired lesions. The same cause may be invoked to explain the wide variety of neurologic symptoms reported in ENS patients without hemimegalencephaly. While the cause of ENS remains unknown, several observations suggest a somatic mutation.

Bone and Bones↗
PubMed1990

Hemimegalencephaly and neurofibromatosis.

Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features: no presence of seizures before one month, seizures controlled or absent, no focal neurological signs and peculiar EEG findings. In our opinion the absence of heterotopias and hamartomas can be related with less severe outcome.

Brain↗
PubMed1989

Polymicrogyria: a case detected by MRI.

We report a 3-year-old child affected by severe encephalopathy, partial seizures with early onset and microgyria whom polymicrogyria was detected by means of magnetic resonance imaging (MRI). We believe that MRI first allow an early and confident in vivo diagnosis of this severe and probably not so rare condition.

Cerebral Cortex↗
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

[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↗