Biomedical subjects
F Sellal
Publications and source records attributed to F Sellal.
Nocturnal paroxysmal dystonia: a clinical form of focal epilepsy.
Different paroxysmal movements occur during sleep. They correspond either to epileptic seizures of sleep, or to parasomnia. Recently, other nocturnal motor phenomena have been described in the literature as nocturnal or hypnogenic paroxysmal dystonia (NPD), paroxysmal arousal, episodic nocturnal wanderings, etc. The NPD are involuntary nocturnal movements characterized by the association of dystonic postures, tonic movements of the four limbs and the body axis, automatisms, affective mimicry, and vocalization. In certain patients, the EEG records show abnormalities characteristic of epilepsy; in others, the EEG appears normal. A large proportion of the patients present epileptic seizures as antecedents. Typical generalized tonic-clonic seizures can follow an NPD. The NPD are improved with anti-epileptics. The considerable similarity of the clinical and paraclinical signs and of the effects of anti-epileptic treatments do not seem to justify the individualization of different subgroups as a function of the EEG patterns: the NPD are always the result of focal epilepsy, and never of a pathology of movement or of parasomnia. Numerous arguments based on the symptoms and the EEG suggest that these seizures involve the mesial frontal regions.
[Encephalopathy and Hashimoto thyroiditis].
A generalized epileptic seizure revealed a subactute encephalopathy which was attributed to Hashimoto's thyroiditis. Primary biliary cirrhosis was also discovered. The encephalopathy rapidly followed a favourable course under corticosteroid therapy, which confirms that it was an immune disease.
[Memory and benzodiazepines].
Benzodiazepines (BZs) affect acquisition of new information, while retrieval of already learned information is unimpaired. The variability of this effect is important and depends on the nature of the BZ, its dose, the route of administration and the susceptibility of the subject taking the drug. This last factor depends itself on the anxiety level, the age, and a less known idiosyncratic susceptibility of the patient. Finally, there is probably a partial tolerance for the amnestic action of BZs, which explains the fact that the most dramatic amnesias have been described after administration of a single dose of BZ, taken by a patient unaccustomed to BZs. The value of pharmacocinetic and pharmacodynamic characteristics in predicting cognitive impairment remains misunderstood, even though in clinical practice the greatest amnestic effects have been described with short-acting BZs. The interest of studying BZs induced amnesia rely upon several arguments: first, it can be an harmful side-effect, which could be avoided or at least predicted by a better knowledge of BZs and the synthesis of new and more specific drugs; secondly it is an interesting model of organic amnesia, which could allow a better understanding of normal memory.
Electroclinical signs of benign neonatal familial convulsions.
Benign neonatal familial convulsions comprise a distinct epileptic syndrome with an autosomal mode of transmission. The electroclinical signs of seizures in this syndrome are not yet well defined. In 3 children from two families presenting with benign neonatal familial convulsions, 14 seizures were recorded during electroencephalographic (EEG)-video sessions. All seizures occurred during sleep, after a short arousal reaction. Seizures started with bilateral, symmetrical flattening of the EEG for 5 to 19 seconds; simultaneously there was apnea and tonic motor activity. The EEG flattening was followed by a long (1-2-minute) bilateral discharge of spikes and sharp waves; simultaneously, there were vocalizations, chewing, and focal or generalized clonic activity. The prominence of EEG and motor abnormalities varied between the left and the right from one seizure to the next in any given child. The seizures stopped without EEG or clinical postictal depression. These electroclinical observations suggest that the convulsions of benign neonatal familial convulsions are a form of generalized tonic-clonic seizure whose expression may be asymmetrical, probably because of the immaturity of the corpus callosum or other structures ensuring seizure synchronization.
Nocturnal paroxysmal dystonia.
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A case of symptomatic hemidystonia improved by ventroposterolateral thalamic electrostimulation.
A 16-year-old boy presented with a left anterothalamic lesion secondary to an extradural hematoma. A few months later, a right hemidystonia developed. The dystonic posture, which predominated in the right hand, was not associated with any motor deficit or sensory loss. Superficial sensory stimulation of the right palm and forearm and proprioceptive stimuli induced by passive movements of the right thumb decreased the dystonic posture. Electrical stimulation of the left ventroposterolateral nucleus of the thalamus induced a dramatic improvement in the dystonic postures and movement of the upper right limb. This finding suggests that the role of tactile and proprioceptive stimulation should be analyzed in patients presenting with a symptomatic hemidystonia. When such sensory stimulation is effective, thalamic stimulation may be tried in patients whose condition is incapacitating.
'Pinocchio syndrome': a peculiar form of reflex epilepsy?
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[Tick-borne encephalitis in Alsace].
Central European tick-borne encephalitis is mainly found in Central European countries and Austria where hundreds of cases are reported each year. Apart from 2 cases diagnosed in Alsace in 1968 and 1970 respectively, this disease was hitherto unknown in France. We report 8 new cases observed in Alsace between 1985 and 1990. Clinical presentation in these 10 patients was a pure meningitis syndrome in 4 cases and meningo-encephalitis in 6 cases, very severe in 3 of them. All patients recovered rapidly, and only 3 have slight sequelae. In a seroprevalence survey conducted in 1989 among 619 professional foresters of Eastern France, 8% were found to be seropositive, which suggests that the disease is often unrecognized. A study of the large series published in Austria and in other Central European countries has shown that the prognosis of tick-borne encephalitis is not always as favourable as it was in the Alsatian cases: severe sequelae or death occur in 1 to 2% of the patients. The need for a better detection of the disease and for vaccination of the subjects at risk must be emphasized.
Antineuronal antibodies and subacute paraneoplastic neuropathy.
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Differential effects of diazepam and lorazepam on repetition priming in healthy volunteers.
The effects of two benzodiazepines, diazepam (15 or 20 mg orally) and lorazepam (1.75 or 2.5 mg orally), and a placebo on explicit memory, lexical priming and perceptual priming were assessed using a free-recall, a word-completion and a picture-completion test. The picture-completion test included two different study conditions intended to manipulate the magnitude of the priming effect. Sixty healthy volunteers took part in this double-blind study. Free-recall performances were altered by both drugs. Lorazepam impaired word-completion and picture-completion performance, whereas diazepam only exhibited a deleterious effect on the more sensitive of the two measures of the picture-completion test. These results indicate that the two benzodiazepines have differential amnestic effects. It is suggested that these differential effects could be accounted for by a different cortical distribution of the two benzodiazepines.
Contralateral disappearance of parkinsonian signs after subthalamic hematoma.
A man with Parkinson's disease (PD) suddenly developed a left hemiballismus, and the CT showed a hematoma of the right subthalamic nucleus. After the ballistic movements had disappeared, akinesia and the other parkinsonian signs did not reappear on the left. This clinical case confirms the involvement of the subthalamic nucleus in the akinesia of PD, as suggested by recent experimental data.
[Paroxysmal neurological manifestations disclosing panic attacks].
Thirty-seven patients presented with paroxysmal neurological manifestations attributed to anxiety attacks. The manifestations included loss of consciousness, focal sensorimotor deficits, diffuse dysesthaesiae, visual disorders and tremor. They lasted 10 to 45 minutes and occurred once per day to once per week. Organic pathology was dismissed on the basis of normal examinations and atypical course. In all patients questioning revealed symptoms that were those of acute anxiety. The fact that these attacks took place in suggestive (circumstances e.g. in crowds and car driving), and that they could be induced by challenge tests hyperpnoea, infusion of lactate) suggested that these disorders were consecutive to panic attacks.
[Myelitis caused by toxocara canis (larva migrans)].
A young woman presented with recurrent myelitis associated with persistent eosinophilia in blood and CSF. Specific serological procedures in blood and CSF led to the diagnosis of visceral larva migrans. The patient recovered after 21 days of treatment with diethylcarbamazine. To our knowledge, this is the second report of myelitis in the course of visceral larva migrans.
[Recurrent aseptic meningitis in periodic disease or Mollaret's meningitis?].
A 33 year-old Sephardic Jewish man with familial mediterranean fever (FMF), presented during a 7 year period, 6 episodes of aseptic meningitis, improving within less than 24 h after spinal tap. Cerebrospinal fluid analysis showed a mixed leucocytic pleocytosis ranging from 100 to 1,000 cell/mm3. Spinal fluid cultures for bacteria, viruses and viral antibodies were always negative. Our case supports other reports showing that recurrent aseptic meningitis, although rare, may occur in FMF. It usually responds to treatment with colchicine, like other manifestations of the disease. FMF meningitis has been compared to Mollaret's meningitis whose cause is undetermined. However, Mollaret's meningitis, unlike FMF, is sporadic and ubiquitous, is not transmitted genetically and affects men and women equally. Moreover, in Mollaret's meningitis transient neurological abnormalities, such as signs of encephalitis have often been reported: polyserositis or associated amylosis are absent, there is no biological inflammatory syndrome, and in 65% of the patients the CSF contains specific large mononuclear-derived cells called endothelial cells. Such abnormalities have not been described in FMF.
[Postures and abnormal paroxysmal movements during sleep: hypnogenic paroxysmal dystonia or partial epilepsy?].
In recent years, a differentiation has been made between two syndromes that are characterized by brief abnormal paroxysmal movements occurring principally at night: 1, hypnogenic paroxysmal dystonia (HPD), sometimes considered a particular form of dystonia similar to paroxysmal kinesigenic choreoathetosis, and 2, mesiofrontal epilepsy. Whether HPD is a distinct syndrome is not clear. Twenty-three patients, 11 men and 12 women, were hospitalized between 1985 and 1989 for examination of this type of abnormal paroxysmal movements (APM) occurring at night. In order to clarify the physiopathology of these abnormal nocturnal movement as focal epilepsy or a particular form of dystonia, we analyzed the personal and familial antecedents of all 23 patients, the polygraphic records during waking and sleep periods, and the results of neuroradiological examinations. Four patients were examined by positron emission tomography (PET) using i8F deoxyglucose. Symptoms first appeared between 3 and 28 years of age (M, 10.1) and developed over 1 to 20 years (M, 10.1). APM clearly occurred more commonly (greater than 90%) during sleep, usually during phases of slow-wave sleep. The sleeping patient opened his eyes and the motor signs then variously associated affective facial expression; axial postural modifications; tonic, dystonic or choreic postural movements of the limbs; pedalling; automatisms; disordered agitation and vocalization. The seizure was abruptly interrupted after 10 to 60 seconds. There was usually no postictal confusion. Thirteen patients clearly had clear epileptic antecedents: in 9, generalized tonic-clonic seizures; in 4, focal epileptic status. During nocturnal polygraphic recording, 6 patients presented a generalized seizure following a period of APM.(ABSTRACT TRUNCATED AT 250 WORDS)
[Meningoradiculitis after a tick bite. Study of 31 cases].
A retrospective study covering a period of 20 years identified reports on 31 cases of meningoradiculitis of the Garin-Bujadoux-Bannwarth type (MRGBB). Clinical, biological, electromyographic characteristics and course of the disease were studied. The most recent cases (n = 8) in 1984 and 1985 had serological tests for Borrelia Burgdorferi and half of the cases had negative results. Conversely, in some patients with meningoradiculitis, even in the absence of a tick bite or of migrating chronic erythema, serology was positive for Borrelia Burgdorferi antigen. The efficacy of antibiotic therapy against pain and on the quality and time of functional recovery justifies the use of this therapy under these two circumstances.
Landau-Kleffner syndrome: a pharmacologic study of five cases.
Five children with Landau-Kleffner syndrome (epilepsy, acquired aphasia, and continuous spike-wave discharges during sleep), were treated with antiepileptic drugs (AEDs), sleep-modifying drugs, and corticosteroids. The pharmacologic profiles differed from those observed in focal epilepsies, resembling instead those of certain generalized epilepsies, such as West or Lennox-Gastaut syndromes. Phenobarbital (PB), carbamazepine (CBZ), and phenytoin (PHT) were ineffective or worsened the EEG and neuropsychological symptoms, whereas valproate (VPA), ethosuximide (ESM), and benzodiazepines were partially or transiently efficacious. Dextroamphetamine produced a dramatic but transient improvement in waking and sleep EEG in one of two children; aphasia did not change. Corticosteroid treatment resulted in improved speech, suppression of seizures, and normalization of the EEG in three of three children. Our own experience and data from the literature suggest that corticosteroids should be given in high doses as soon as the diagnosis is firmly established and should be continued in maintenance dose for several months or years to avoid escape. Early diagnosis, before mutism or global deterioration develops, appears to be essential for effective therapy with minimal neuropsychological sequelae.