Biomedical subjects

U Aguglia

Publications and source records attributed to U Aguglia.

At least 73 records · Page 4Linked to original sources

PubMed1990

Sensory evoked potentials in Creutzfeldt-Jakob disease.

Eight patients presenting with intermediate or terminal evolution of Creutzfeldt-Jakob disease (CJD) were investigated by means of evoked potentials. Fifteen age-matched healthy subjects served as controls. The 8 patients had well-recognizable but simplified flash evoked potentials (FEPs) consisting of P1 and N2 waves followed by a single late positive (P2) deflection. Enlarged FEPs were found in 2 of the 8 patients. The somatosensory central conduction time was normal in 3 of 5 patients, and it resulted in upper normal limits or was moderately slowed in 2 patients. No enlarged somatosensory scalp potentials were recorded. Cortical somatosensory responses were characterized by an unrecognizable (4 patients) or delayed (2 patients) N33 wave. Brainstem auditory evoked responses, recorded in 6 patients, were normal. In CJD very important functional impairment of the sensory cortical areas is associated with absent or mild dysfunction of the subcortical sensory pathways.

Acoustic Stimulation↗

PubMed1990

Nonmetabolic causes of triphasic waves: a reappraisal.

Two more patients with triphasic waves (TW) on their EEGs in the absence of metabolic disturbances are described. One patient had coma associated with cerebellar hematoma, the other had mild dementia associated with idiopathic calcifications of the basal ganglia and normal auditory brainstem responses, subcortical and cortical somatosensory evoked potentials. Neurologic examination failed to show asterixis in both patients. The literature on nonmetabolic causes of TW was also reviewed, and the clinical and anatomic reports of 10 patients have been analyzed: 7 patients had focal brainstem-diencephalic lesions (craniopharyngioma: 2 patients; thalamic gliomas: 3 patients; pontine stroke: 2 patients), and 3 patients suffered from diffuse subcortical or multifocal encephalopathies (Binswanger's encephalopathy: 1 patient; cerebral carcinomatosis: 1 patient; multifocal cerebral lymphoma: 1 patient). From the clinical point of view, patients with nonmetabolic diseases causing TW presented either disturbance of higher cerebral functions with no asterixis or sudden onset of coma. It is concluded that TW may result from focal brainstem/diencephalic lesions or from diffuse subcortical or multifocal encephalopathies in the absence of concomitant metabolic abnormalities. Nonmetabolic causes of TW should be suspected in patients presenting with neurologic disturbances not associated with asterixis.

Aged↗

PubMed1990

Neurocutaneous syndromes with pigmentary abnormalities and central nervous system involvement. II. Two cases with atypical incontinentia pigmenti.

Two cases are reported of a boy and a girl with similar neurocutaneous syndromes clinically characterized by hyperpigmented skin patches and severe CNS involvement. The diagnosis of atypical cases of Incontinentia Pigmenti is suggested. The different syndromes with mental and motor retardation associated with skin abnormalities (Incontinentia Pigmenti, Ito's hypomelanosis, phakomatoses, etc.) are reviewed.

Child, Preschool↗

PubMed1989

Combined neurophysiological studies in Creutzfeldt-Jakob disease: a case report.

EEG and brain mapping (on basal conditions and after i.v. administration of diazepam) and visual, somatosensory and auditory evoked potentials have been performed on a patient at terminal evolution of CJD. These combined neurophysiologic studies indicated that very important functional impairments of either thalamic-frontal circuits and frontal, parietal and occipital cortex were associated with absent or mild dysfunction of the visual, somatosensory and auditory subcortical pathways. Combined neurophysiologic studies are important to better understand the pathophysiologic mechanisms of neurodegenerative diseases.

Aged↗

PubMed1987

Subacute spongiform encephalopathy with periodic paroxysmal activities: clinical evolution and serial EEG findings in 20 cases.

Evolution of both clinical and EEG abnormalities was analyzed in 20 (16 pathologically confirmed) patients suffering from subacute spongiform encephalopathy with periodic paroxysmal activities (PPA) on the EEG. Illness duration was less than 4 and greater than 17 months in 65% and 10% of cases, respectively. All data but EEG were utilized to assess 3 conventional clinical stages in 20 patients. The early clinical stage was characterized by gradual presentation of gait disturbances, mental deterioration, sensory or autonomic disorders. In contrast with other reports, no PPA were observed in 10 EEG recordings from 7 patients examined at the early clinical stage. Both clinical and EEG findings were not in contrast with a hypothetic subcortical onset of disease. Similar to recent data in the literature, early PPA appeared within 12 weeks of disease evolution in 88% of patients who underwent EEG recordings in the first 3 months of disease. Nonetheless, these early PPA always occurred at an intermediary stage, when our patients showed a marked worsening of the clinical picture. Focal, segmental and/or generalized myoclonic jerks were observed in 15%, 53% and 100% of cases at prodromal, intermediary and terminal stages respectively. Different kinds of PPA were observed: bi-tri-phasic periodic complexes (PC), periodic complexes with multiphasic configuration (PPC) and periodic polyspiking discharges (PPD). Abnormal "pacing" of PC by slowly repeated flashes was found in 4 patients presenting visual hallucinations or cortical blindness. Burst-suppression activity was frequently found at the terminal stage in decorticate patients.

Aged↗

PubMed1987

Prolonged ictal paralysis: electroencephalographic confirmation of its epileptic nature.

Somato-inhibitory status epilepticus is a rare condition which may pose some problems of differential diagnosis. The authors describe the case of a girl who suffered from episodes of prolonged ictal paralysis lasting from some minutes to some hours. One seizure was described 2 hours from onset. The EEG showed a continuous spike activity on the right side, predominantly on the parieto-rolandic areas. Status was stopped by the administration of clobazam per os. The physiopathological mechanism of this rare form of status epilepticus is discussed.

Adolescent↗

PubMed1986

Benign versive or circling epilepsy with bilateral 3-cps spike-and-wave discharges in late childhood.

Twenty-eight of 920 patients seen between the ages of 8 and 20 years for a first seizure manifested versive or circling events associated with bilateral rhythmic 3-cps spike-and-wave discharges. In 21 (75%), the seizures appeared between the ages of 10 and 14 years. Paroxysmal adversion or gyration was always toward the same side in a given patient. "Break of contact" during the ictus was found in 70% of the seizures, and secondary generalization occurred in half. Ictal electroencephalographic recordings failed to show a focal hemispheric origin. Additional features were: absence of prior major diseases; normal results of neurological and neuroradiological examinations; excellent response to anticonvulsant treatment with sodium valproate and/or phenobarbital; frequent family history of epileptic seizures (25% of patients); and association with generalized seizures (57.2% of patients). We conclude that versive or circling epilepsy with bilateral rhythmic 3-cps spike-and-wave discharges represents a benign form of primary generalized epilepsy in late childhood.

Adolescent↗

PubMed1986

Use of clobazam in certain forms of status epilepticus and in startle-induced epileptic seizures.

Sixteen patients with status epilepticus (SE) and 13 patients suffering from startle-induced epilepsy were treated with clobazam (CLB). Status epilepticus was of the absence (six cases), myoclonic-absence (one case) myoclonic (one case), tonic (one case), and single or complex partial (seven cases) type. In all patients, consciousness was sufficiently retained during SE to allow CLB ingestion. Clobazam was administered in a single oral dose (0.5 to 1.70 mg/kg, mean 1.08 mg/kg) under electroencephalographic monitoring. In all patients but one, SE terminated within 15 to 31 min (mean 24.4 min) after CLB administration without significant side effects. Treatment of SE depends on the clinical context and type of SE. We conclude that CLB is a useful drug in the management of some forms of SE. In 13 patients suffering from startle-induced epileptic seizures, CLB was added to the previous ineffective treatment protocol. Of these 13 patients, 15.4% were completely unresponsive, 23.1% experienced drug resistance, and 61.5% achieved a good control (91.5% reduction of the reflex seizures) for a mean of 22.7 months. Drowsiness was observed in four patients, whereas favorable side-effects were observed in four patients.

Adolescent↗

PubMed1985

Absence status appearing on eye-closure.

Two personal observations of absence status appearing on eye closure are described and similar cases in the literature considered. Treatment of such an absence status with clobazam is proposed.

Adolescent↗

PubMed1985

Occipital seizures in Lafora disease: a further case documented by EEG.

The authors present the case of a patient with Lafora disease proven by skin biopsy, who suffered two occipital seizures recorded on EEG and provoked by intermittent photic stimulation. On the basis of data in the literature and their present experience, the authors suggest the involvement of the occipital lobes as a whole in the epileptogenic activity.

Adolescent↗

PubMed1985

Brainstem auditory evoked responses in Lafora disease.

Brainstem auditory-evoked responses (BAERs) have been studied in five patients suffering from Lafora-type of progressive myoclonus epilepsy proven by skin biopsy and in ten healthy volunteers. At the time of examination the patients were not taking benzodiazepines and showed myoclonic jerks. In all patients the central conduction time (interpeak latencies I-V, I-III, III-V) and the amplitude (amplitude ratio I/V) of BAERs were within the +/- 2 S.D. limits of the normal values. Since Lafora disease is a neuropathologically prevalent grey-matter illness (typical inclusion bodies are stored intraneuronally) these data indicate that diseases primarily affecting the brainstem grey-matter are usually associated with normal BAERs.

Adolescent↗

PubMed1984

[Treatment of certain forms of status epilepticus by means of a single oral dose of clobazam].

The authors administered clobazam (CLB) to treat status epilepticus in 16 patients from 3 to 62 years old. Status epilepticus was of absence type (6 cases), myoclonic-absence type (1 case), myoclonic type (1 case), tonic (1 case) and elementary or complex partial (7 cases). In all patients consciousness was sufficiently retained to allow ingestion of the drug. CLB administration was performed under EEG monitoring, in a single oral dose averaging 1.0 mg/kg. In 15 patients, the status was stopped after a period ranging from 19 to 31 min without significant sedation or other side effects. In one patient a somatomotor status epilepticus, lasting 8 days, terminated 6 h after drug administration. It is well known that the efficacy of a drug on status epilepticus depends on the clinical context and semiological form of the status. Nevertheless, the authors conclude that the pharmacokinetic properties of CLB, its mild sedative effects and ease of administration, make this a useful drug in the management of some forms of status epilepticus.

Adolescent↗

PubMed1984

[Absence status with unilateral EEG predominance (apropos of a personal case report)].

The authors describe a 33-year-old woman who, from the age of 15 years, presented many generalized epileptic seizures per year starting with right-sided adversion or gyration, several times per month long absences and occasional absence status lasting up to 3 days. In this patient, the hyperpnoea provoked a clinically typical absence status with 3 c/sec rhythmic continuous spike-and-wave discharges which were more evident on the left frontal regions of the scalp. This electro-clinical picture is compared to similar cases described in the literature and its nosological specificity is discussed.

Adult↗

PubMed1984

Startle-induced epileptic seizures.

The present study was undertaken in order to describe 16 more patients suffering from startle-induced epileptic seizures and to clarify further the possible therapeutic activity of benzodiazepines in this rare reflex epilepsy. The interictal and ictal electroclinical data of 16 epileptic children or adolescents have been detailed. A CT-scan was performed in 10 patients; six of them showed an atrophy of the mesial surface of one or both hemispheres ("mesial hypodensity"). Benzodiazepine was associated with the previous antiepileptic treatment in the 16 ineffectively treated patients. Clonazepam was administered in three patients; one of them was completely unresponsive and two became seizure-free for a mean of 16.5 months but complained of drowsiness or ataxia. Clobazam was administered in 13 patients; 15.4% of them were completely unresponsive, 23.1% experienced drug resistance, and 61.5% obtained a good control (91.5% reduction of the reflex seizures) for a mean of 22.75 months. In spite of a possible loss of therapeutic activity, the appearance of very few unfavorable side effects and the presence of favorable side effects ("psychomotor arousal") make clobazam therapy important in the treatment of patients suffering from startle epilepsy.

Adolescent↗

PubMed1984

Electroencephalographic and anatomo-clinical evidences of posterior cerebral damage in hypertensive encephalopathy.

The authors describe two patients suffering from hypertensive encephalopathy associated with epileptic seizures and anatomo-clinical evidences of posterior cerebral damage. The concordant electroencephalographic features with clinical signs and anatomic lesions of these two patients are discussed, in order to make some considerations about the role played by the blood-brain-barrier damage on the physiopathology of the hypertensive encephalopathy.

Adult↗

PubMed1983

[Vomiting: symptom of an epileptic seizure].

The authors present 3 cases of ictal vomiting with 2 seizures recorded on the EEG. The critical vomiting is always sudden, projectile, and lasts a short period. The interictal and ictal EEG recording showed a focus or a seizure involving the frontal temporal regions. The seizures were kept under control by a therapy with carbamazepine. Vomiting can be a particular semiological aspect of a possible seizure as are other vegetative features. The authors stress this critical aspect on one hand differentiating it from non-epileptic ictal phenomena (in particular children's cyclic vomiting), on the other hand from post-ictal vegetative phenomena of a major or partial seizure.

Adult↗

PubMed1983

[Prolonged confused seizure state of frontal origin in an aged patient].

The authors discuss the case of a man who, at the age of 63, presented episodes of prolonged confusional state de novo. The EEG recordings showed the existence of an electrical status with widespread spikes and polyspike-and-waves of the "absence status." Further recordings showed a focal onset of one of the episodes of prolonged confusional state. The electric and clinical characteristics of this observation indicate that a frontal localisation with secondary generalization of the discharge is probable. This electro-clinical picture is compared to other literature on the subject and its nosological specificity is discussed.

Cognition Disorders↗

PubMed1983

Prolactin secretion in man: a useful tool to evaluate the activity of drugs on central 5-hydroxytryptaminergic neurones. Studies with fenfluramine.

Acute oral administration of various doses of fenfluramine, a 5-HT releaser, induced a dose-related increase of PRL secretion in nine healthy volunteers. Fenfluramine reached the maximum effect on PRL secretion at 4 h after its administration. This effect was already significant at 2 h and lasted till 8 h. Metergoline, a 5-HT receptor blocker, when administered alone, decreased serum PRL levels in six healthy subjects. The pretreatment with this drug significantly antagonized the PRL-releasing action of fenfluramine (60 mg) suggesting that the effect of fenfluramine on PRL release may be mediated through a 5-HT mechanism in the brain. These findings suggest the possibility that serum PRL levels in humans may represent a useful tool to evaluate, in vivo, the activity of drugs possessing putative 5-hydroxytryptaminergic properties.

Adult↗