PubMed Health⌕ Search

Biomedical subjects

R Guerrini

Publications and source records attributed to R Guerrini.

At least 91 records · Page 5Linked to original sources
PubMed1994

Occipitotemporal seizures with ictus emeticus induced by intermittent photic stimulation.

We recorded occipitotemporal seizures induced by intermittent photic stimulation in three children with brain injuries, aged 10 to 13 years. All had a history of seizures and showed occipital spikes on EEG, but were seizure free and were not being treated at the time of investigation. In all, photic stimulation induced seizures in the right occipital lobe. They were followed by clinical and EEG signs suggesting infrasylvian spreading to ipsilateral mesiotemporal limbic structures and by vomiting, appearing at late stages of the attacks. Seizure spread was very slow in two patients in whom attacks lasted 16 and 25 minutes. Patients with occipital epileptiform abnormalities presenting with ictal vomiting are often diagnosed as having vague migraine-epilepsy syndromes. We conclude that vomiting can be a late ictal phenomenon resulting from temporal lobe spread of seizures originating in the occipital lobe.

Adolescent↗
PubMed1994

The epileptic spectrum in the congenital bilateral perisylvian syndrome. CBPS Multicenter Collaborative Study.

We studied the frequency, clinical and EEG characteristics, and outcome of the epileptic syndrome in 31 patients with a congenital neurologic syndrome characterized by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian polymicrogyria. Seizures were present in 27 of 31 patients (87%) and usually began between the ages of 4 and 12 years; they commonly consisted of atypical absence, atonic/tonic, and generalized tonic-clonic seizures. Partial attacks were present in 26%. EEG demonstrated generalized spike and wave abnormalities and, less frequently, multifocal discharges, predominantly in centro-parietal regions. Seizures were poorly controlled in 65%, with the remaining patients well controlled. Seven patients underwent callosotomy, which resulted in seizure improvement. This study indicates that the epileptic spectrum in this syndrome is broad but follows predictable patterns. Callosotomy is a valuable treatment strategy in those with intractable drop attacks.

Adolescent↗
PubMed1993

Congenital bilateral perisylvian syndrome: study of 31 patients. The CBPS Multicenter Collaborative Study.

Advances in neuroimaging techniques have enabled the recognition of developmental malformations of the brain during life. Careful correlation of clinical and imaging features has identified several new syndromes. We have studied 31 patients with a congenital neurological syndrome characterised by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian abnormalities on imaging studies. All patients had diplegia of the facial pharyngeal, and masticatory muscles, of variable severity. Some patients had slight dysarthria, whereas others were unable to speak. 85% of patients had mental retardation, ranging from mild to severe. Epilepsy was present in 27 (87%) and commonly consisted of atypical absence, atonic/tonic, tonic-clonic seizures, and, less frequently, partial attacks. Seizures were poorly controlled in 55%. Magnetic resonance imaging showed bilateral perisylvian cortical malformations consistent with polymicrogyria, confirmed at necropsy. Division of the corpus callosum in several patients resulted in seizure improvement. This congenital bilateral perisylvian syndrome can be clinically diagnosed and confirmed by imaging studies. Further studies are necessary to elucidate its cause.

Adolescent↗
PubMed1993

Epileptic negative myoclonus.

Five patients with partial epilepsy of diverse etiology insidiously developed action-activated jerks. The disorder was limited to one arm in two patients and to the legs in another, and was multifocal in the remaining two. Each jerk was related to an EMG silent period lasting 100 to 400 msec, causing a lapse followed by resumption of posture. Simultaneous EEG-EMG recording showed each postural lapse to be time-locked with a sharp or spike and slow-wave transient over the contralateral sensorimotor cortex, where almost continuous paroxysmal activity occurred. The three patients who were able to cooperate during neurologic evaluation also exhibited motor neglect in the most affected body segment and decreased awareness of the disorder. In three patients, the phenomenon was medically resistant, and in two of them it was continuous and could be defined as epilepsia partialis continua. In the other two, medical treatment induced remission of EEG, motor, and neuropsychological abnormalities. This disabling movement disorder can be classified as "epileptic negative myoclonus" and may result from focal-discharge-related transient disruption of cortical function in the sensorimotor cortex.

Adolescent↗
PubMed1993

[Partial trisomy of the long arm of chromosome 6. A clinical case].

The authors present the case of a child with partial trisomy of the long arm of chromosome 6 who was observed aged between 1 and 5 months and 5 years and 5 months. The kariotype shows a duplication of the distal tract (6q22.36-->qter) deriving from the imbalance of a maternal malsegregation. The phenotype is that characteristic of the syndrome: microbrachycephalia with a high and prominent forehead, "full" orbits, exophthalmos, antimongoloid palperbral fissures, hypertelorism, flattened root of the nose, antiverted nostrils, small mouth with thin lips, micrognathy, auricles with a low implantation, short and thick neck with anterior palmature, low implantation of hair at nape, reduced diameter of chest, articular retraction, clinodactilia of 5th finger, bilateral single palmar groove, hypospadia. Weight, height and cranial circumference were below the 3rd percentile. The cases of dup (6q) reported in the literature generally describe similar somatic characteristics so much so that these may represent a syndrome. However, there is considerable variation in the level of visceral and cerebral impairment, only partly attributable to the degree of chromosome imbalance. The general conditions of the probabd were good and there was no malformation of internal organs. Magnetic resonance scan of the brain showed a slight increase in the volume of the ventricular system and a slight dilatation of the subarachnoid spaces with a dysmorphic cortical pattern.(ABSTRACT TRUNCATED AT 250 WORDS)

Chromosomes, Human, Pair 6↗
PubMed1992

Continuous focal spikes during REM sleep in a case of acquired aphasia (Landau-Kleffner syndrome).

We report a girl 3 years and 6 months old with onset of aphasia at age 3 years and 3 months. There was no evidence of brain damage and there were no seizures. The neuropsychological evaluation showed that the girl tended to be right-handed, that aphasia was global and that other higher cortical functions seemed to be preserved. Isolated spikes and spikes-and-wave were recorded during wake over the right temporal region with rare independent contralateral abnormalities. During polysomnography (PSG), the physiological patterns of sleep were preserved and right temporal epileptiform discharges were significantly increased in all sleep stages. Maximal activation was obtained at sleep onset and during rapid eye movement (REM) sleep periods, when focal abnormalities became continuous and spread contralaterally. Repeat PSGs showed that the activation profile retained this particular trait, although subclinical discharges tended to increase during slow wave sleep (SWS). This pattern of subclinical temporal status epilepticus during REM sleep differs from the characteristic activation profile found in the syndrome of continuous spikes-and-waves during SWS. However, this profile was transient and all epileptiform changes disappeared during clinical recovery at 18 months of follow-up.

Anticonvulsants↗
PubMed1992

Synthesis and pharmacological activity of the N-terminal dermorphin tetrapeptide analogs with CH2-NH peptide bond isosteres.

The synthesis of pseudotetrapeptides H-Tyr-D-Ala-Phe-NH-(CH2)2--NH2 (1a), H-Tyr-D-Ala-Phe-psi (CH2--NH)-Gly-NH2 (2a), H-Tyr-D-Ala-psi (CH2--NH)-Phe-Gly-NH2 (3a), and H-Tyr-psi (CH2--NH)-D-Ala-Phe-Gly-NH2 (4a), representing the N-terminal tetrapeptide sequence of dermorphin, in which amide bonds are replaced by CH2--NH bond, is described. N-acetyl-Tyr and desamino-Tyr pseudopeptide analogs (1-4b), (1-3c) are also described. The analogs were assayed in binding studies based on displacement of mu and delta-receptor selective radiolabels from rat brain membrane and in a bioassay using guinea pig ileum (GPI). Pseudopeptides in which the C-terminal (1a) or D-Ala-Phe (3a) amide bond are substituted, exhibit higher mu-affinities and mu-receptor selectivity than the corresponding Phe-Gly or Tyr-D-Ala analogs (2a, 4a). Acetyl-and desamino-Tyr pseudopeptide analogs (1-4b) and (1-3c) did not exhibit mu and delta-opioid receptor affinity at nM concentration. The relevance of the single peptide replacement and of its association to acetylation or amino group elimination of Tyr, is discussed on the basis of a receptor model for mu and delta opioids.

Amino Acid Sequence↗
PubMed1992

Neurological findings and seizure outcome in children with bilateral opercular macrogyric-like changes detected by MRI.

The authors studied 10 patients aged between six and 23 years (mean age 14 years 5 months) with magnetic resonance imaging, which detected bilateral 'macrogyric-like' maldevelopment of the insulo-opercular regions. The data confirm that biopercular gyral anomaly, associated with mental retardation, pseudobulbar palsy (cortical or central) and epilepsy, represents an anatomo-clinical syndrome. Nevertheless, a wide clinical spectrum was found varying from pictures correlating with the topography and extent of the MRI-detected anomaly to conditions indicating wider cerebral involvement. Epilepsy, varying greatly in age at onset and severity, consistently influenced the prognosis for five patients with diffuse EEG abnormalities and intractable seizures with falls. Anterior callosotomy relieved such seizures in one case.

Adolescent↗
PubMed1992

Epilepsy and focal gyral anomalies detected by MRI: electroclinico-morphological correlations and follow-up.

The authors studied 10 patients (mean age 15 years 6 months) with localized developmental gyral disorder detected by MRI. There were two groups of major malformations. Seven patients (group 1) had unilateral 'macrogyric-like' insulo-opercular changes, one of whom died early in life and had extensive microgyria. The six others had mental retardation and epilepsy, three of whom had focal neurological signs. Age at onset of epilepsy varied greatly. Clinical and EEG data suggested a wider cerebral involvement than recognized on MRI. The remaining three patients (group 2) had abnormal gyri of variable topography and extension, with bulging grey matter and ventricular deformity. One had mental retardation, another had neurological signs. All had intractable complex partial seizures and focal EEG anomalies correlating with the MRI lesion site, pointing to a well-defined epileptogenic zone. No clinical or EEG evidence of significant malformation in the remaining brain tissue was observed. Ablative surgery was beneficial for one patient; focal cortical dysplasia was the pathological substrate.

Adolescent↗
PubMed1992

Epilepsy, antiepileptic drugs, and malformations in children of women with epilepsy: a French prospective cohort study.

We conducted a prospective study of teratogenic effects of antiepileptic drugs (AEDs) in pregnant women with epilepsy in southeast France, comparing malformation rates with those collected by a birth defects registry. We evaluated isolated microcephalies separately. Malformations were seen in 7% of infants of mothers with epilepsy (IME) and in 1.36% of the general population. No significant relationship was found between type and severity of epilepsy and occurrence of malformations or isolated microcephaly. Valproate and phenytoin were the most teratogenic (all malformations). None of the malformations observed in IME whose mothers received valproate, phenytoin, or phenobarbital was seen in IME not exposed to the respective AEDs. Phenytoin plus phenobarbital was more teratogenic than phenobarbital alone. Benzodiazepines, prescribed only in combinations, had a borderline, nonspecific effect on microcephaly.

Abnormalities, Drug-Induced↗
PubMed1991

Double-blind placebo-controlled trial of flunarizine as add-on therapy in refractory childhood epilepsy.

Flunarizine (FLN) has been suggested as an add-on treatment in drug-resistant epilepsy patients. In view of the discordant experiences and of the paucity of controlled trials in children, we studied its effectiveness in 20 patients aged 6 to 18 years (10 males and 10 females), affected by drug-resistant epilepsy. 14 had symptomatic generalized epilepsy (the Lennox-Gastaut syndrome in 10; other forms in 4); 3 had cryptogenic generalized epilepsy (the Lennox-Gastaut syndrome in 2; myoclonic absences epilepsy in 1); 3 had symptomatic partial epilepsy (temporal lobe epilepsy). 7 of them were withdrawn: only 1 because of side effects. An initial four-month baseline pretrial period was followed by two four-month periods of administration of FLN or a placebo, under double blind conditions, in a randomized sequence. Preexisting antiepileptic (AEDs) medication was maintained at a constant dose throughout the study. FLN was administered as drops in a single evening dose of 5 mg (patients less than 10 years) or 10 mg. (patients greater than 10 years). During the pretrial phase, after phase 1 and phase 2, a waking EEG was recorded and blood samples were taken for hematology, hepatic-function tests, and AED serum levels. The evaluation of the activity of FLN was based on the total number of seizures. A 30-60% reduction in seizure frequency was found in 5 out of the 13 patients completing the trial (no changes occurred in the remainders). This result did not appear to be due to changes in the plasma levels of the AEDs. No significant differences were seen in the EEG paroxysmal activity in the three phases of the study. Side effects were rare. The serum FLN levels ranged between 16.4 and 109 ng/ml. It seems that the antiepileptic properties of FLN need further validation, particularly in childhood.

Adolescent↗
PubMed1990

[Reducing and stopping treatment].

It is nowadays well-established that a large number of patients with epilepsy can experience a full remission of their condition. In some cases, seizures that were resistant at the onset of the disease may become responsive to therapy over the years. A good knowledge of the classification of epilepsies and epileptic syndromes may give important clues to the overall prognosis of particular epilepsy case. In subjects controlled by therapy, a slow and careful discontinuance of drugs may be initiated after a 2-year seizure-free period. If seizures recur, it will most probably be possible to return to full control with a lesser treatment. Even if seizures fail to be totally controlled and therapy cannot be discontinued, it is often possible to reduce polypharmacy and thus lessen unwanted side-effects.

Anticonvulsants↗
PubMed1990

Brainstem auditory evoked potentials in alternating hemiplegia: ictal vs interictal assessment in one case.

The authors report a case of alternating hemiplegia (AH) in a 3yr 6m old boy who had presented, from the age of 4 months on, episodes of alternating hemi- or quadriplegia. Brainstem auditory evoked potentials were recorded both in the interictal state and, for the first time, during an attack. There was no significant difference between the two states. These findings suggest that a massive involvement of the posterior vascular territory is not likely to be associated with attacks in AH.

Brain Stem↗
PubMed1990

Antimyoclonic effects of alcohol in progressive myoclonus epilepsy.

We studied the effect of a single oral intake of 30 to 40 grams of alcohol in 4 consecutive patients presenting with Ramsay Hunt syndrome, the most common type of degenerative progressive myoclonic epilepsy (PME) encountered in southern Europe. Clinical and polygraphic monitoring demonstrated abatement of myoclonus in all patients, although the degree of improvement varied among patients. These findings show that alcohol is a potent antimyoclonic agent. In the particular context of PME, occasional alcohol intake may help patients in their social life.

Administration, Oral↗
PubMed1989

[Contribution of ambulatory EEG recording (Medilog 9000) in a population of epileptics].

Thirty-four epileptic patients, aged 9 to 36, were submitted to A/EEG between May 1987 and July 1988. All patients had a thorough clinical and EEG work-up including long-term conventional EEG, afternoon polygraphic sleep recording and, in some cases, full-night EEG and video monitoring. Patients were divided into 2 groups: group I included 19 patients (18 with symptomatic partial epilepsy (SPE) and 1 with idiopathic generalized epilepsy (IGE) in whom no seizure had ever been recorded in spite of EEG recordings averaging a total of 16 hrs 10 min, awake and asleep); group II included 15 subjects (6 with SPE, 5 with IGE, 3 with symptomatic GE and 1 with undetermined epilepsy) in whom one or several seizures had been recorded. A/EEG was performed in order to: 1) obtain better clinical and EEG characterization of seizures, 2) study the circadian distribution of seizures, 3) verify the efficacy of drug treatment and, 4) establish the epileptic or non-epileptic nature of some ictal events. The results of A/EEG were considered positive in 52.63% of group I patients and in 93.33% of group II patients. The authors discuss the specific advantages of A/EEG vs conventional EEG: recording of seizures with random occurrence, of seizures accompanied by falls, checking the remission of seizures.

Adolescent↗