PubMed Health⌕ Search

Biomedical subjects

G Capovilla

Publications and source records attributed to G Capovilla.

26 records · Page 2Linked to original sources
PubMed1998

Utility of Tc 99m HMPAO SPECT in the early diagnosis of Rasmussen's syndrome.

Neuroradiological examinations are important in the diagnosis of Rasmussen's syndrome (RS), but they frequently result normal in the early phase of the disease. We performed Tc 99m HMPAO SPECT (single photon emission computed tomography) on an 11-year, 5-month-old, right-handed girl, two months since the first seizure. SPECT showed a reduced uptake of HMPAO in the left temporal region despite normal results with magnetic resonance imaging (MRI). After six months, she developed epilepsia partialis continua, neurological signs appeared, and MRI showed cortical atrophy on the left side. SPECT seems to be a useful, inexpensive and diffuse technology able to give information in the early phases of RS useful in the prevention of serious consequences of the disease within a medical therapeutic program (ganciclovir or intravenous immunoglobulins).

Child↗
PubMed1998

Benign partial epilepsy in infancy with complex partial seizures (Watanabe's syndrome): 12 non-Japanese new cases.

International epilepsy classification includes different epileptic syndromes with favourable outcomes in pediatric age. In addition to these, other forms probably exist and in various papers in international literature they are proposed as new entities. This article presents a survey of benign complex partial epilepsy in infancy, a new epileptic syndrome first proposed by Watanabe, in 1987. Our work represents the only description of non-Japanese cases although similar but familial cases had been referred by Vigevano in 1992. We present data for 12 children (aged up to 9 years) followed over 2 years who had all the typical clinical features characterizing Watanabe's cases. For all of them we obtained EEG seizure recordings demonstrating the partial nature of their fits, arising from occipital or temporal regions. Interictal EEG were completely normal, both in waking and sleep. Evolution demonstrated benign outcome and all the children are seizure-free (eight of them have already stopped all medication) and all have normal psychomotor development.

Age of Onset↗
PubMed1997

Rasmussen's syndrome: longitudinal EEG study from the first seizure to epilepsia partialis continua.

PURPOSE: The aim of our work was to describe the initial electroencephalographic characteristics of Rasmussen's syndrome (RS). METHODS: We performed repeated EEG recordings in an 11-year 5-month-old girl affected by RS, as demonstrated through the progressive evolution of the illness. All EEGs were carried out in polygraphy and videorecordered, both in waking and in sleep. RESULTS: In our opinion, our patient's EEG picture is absolutely unusual in childhood partial epilepsy form without any neuroradiologic perturbation. Delta focal activity persistence in such a clinical context should be considered an RS sign, among the possible causes. CONCLUSIONS: We are not aware of any early-stage RS EEG description. We think that the initial RS EEG picture is so unusual as to suggest such pathology. We hope that analogous reports can confirm our belief.

Child↗
PubMed1989

Progressive myoclonic encephalopathy in X-linked hypogamma-globulinemia. Case report, review of the literature and its relationship with progressive encephalopathy in children with A.I.D.S.

A 7-year-old boy suffering from X-linked hypogammaglobulinemia and progressive myoclonic encephalopathy is reported. The onset of neurological disturbances is at four years of age with ataxic gait and myoclonic jerks. The EEG shows a progressive slowing of background activity, bilateral diffuse and repetitive, pseudoperiodic, high amplitude slow waves, myoclonic jerks polygraphically documented. The CT-scan shows generalized cerebral atrophy, white matter hypodensity--principally in the frontal regions -, multiple nodular calcifications, also in the basal ganglia. Two years after the onset of neurological signs, the boy is completely bedridden, spastic, dement and blind; the myoclonic jerks persist. Finally the relationship is discussed with both the previously reported patients with the same affection, and with similar progressive encephalopathy in children suffering from A.I.D.S.

Acquired Immunodeficiency Syndrome↗
PubMed1982

[Severe infant myoclonic epilepsy (author's transl)].

Twenty children (15 males and 5 females) suffering from a particular type of myoclonic epilepsy were submitted to a longitudinal study. All children were neurologically normal. Familial antecedents existed for epilepsy in 25% of the cases (5/20) and for febrile convulsions in 15% (3/20). The first fit appeared with fever at the mean age of 6 months in all cases but one of clonic type. Frequent similar febrile or afebrile clonic seizures recurred in all subjects before the age of 12 months. At this time the EEG was normal in 14 cases and brief discharges of generalized spike-waves during ILS or during sleep were present in 6 cases only. Later, frequent non-febrile clonic unilateral or generalized fits, frequent atypical 'absences' often accompanied by jerks, high photosensitivity and non-epileptic erratic myoclonias appear. Nevertheless, atomic and/or tonic seizures did not appear. The evolution is characterized by the persistence of fits and the appearance of severe language disorder and light cerebellar and pyramidal signs. The authors present their results and discuss the nosological problems of this severe infant myoclonic epilepsy.

Electroencephalography↗
PubMedDate not supplied

Early myoclonic epileptic encephalopathy (E.M.E.E.).

The authors describe the electroclinical aspects and evolution of nine cases of myoclonic epileptic encephalopathy which began between two days and ten weeks of life. At onset it is associated with: myoclonic jerks, partial fits and periodic paroxysmal EEG abnormalities. Repeated spasms coexisting with partial fits and 'suppression-bursts' (both appearing later) complete the electroclinical picture. The neurological status (initially normal) progressively deteriorates leading within a few months to a decerebrate posture with opisthotonos. In spite of thorough neuroradiological, biochemical, cytological, metabolic, and ultrastructural investigations, the etiology remained unknown. However, the electroclinical and evolutive patterns are similar to those of some metabolic diseases (Polyodystrophy, Non-Ketotic Hyperglycinemia, etc.). All these observations display a homogeneous electroclinical pattern for which the authors propose the name of Early Myoclonic Epileptic Encephalopathy. This type deserves to be classified as a particular electroclinical entity among the epileptic encephalopathies of the first year of life; since its course is regularly downhill in all cases there may be a familial recurrence due to the possibility of a metabolic etiology.

Age Factors↗