Search PubMed⌕ Search

Biomedical subjects

J Roger

Publications and source records attributed to J Roger.

At least 55 records · Page 3Linked to original sources
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

The Ramsay Hunt syndrome revisited: Mediterranean myoclonus versus mitochondrial encephalomyopathy with ragged-red fibers and Baltic myoclonus.

Among progressive myoclonus epilepsies (PME), the nosography of the Ramsay Hunt syndrome (RHS) has been much debated. The authors report on a homogeneous group of 43 patients originating from around the western Mediterranean, with a large number of northern African subjects, who were followed up for a mean period of 11.6 years. Onset is between 6 and 17 years (mean: 11.2) and the transmission appears to be recessive. The clinical features include: action myoclonus, generalized epileptic seizures, mild cerebellar signs and lack of dementia. EEG features include normal background activity, spontaneous fast generalized spike-wave discharges, photosensitivity, lack of activation during nREM sleep and vertex/rolandic spikes during REM sleep. The prognosis is variable, even within families, but the progression seems to be slow in a majority of patients. This condition can be distinguished from mitochondrial encephalomyopathy and is less severe than Baltic myoclonus. The authors propose that this form of PME, formerly reported as RHS, be more properly described as Mediterranean myoclonus.

Adolescent↗
PubMed1989

[Electroencephalographic findings in severe symptomatic partial epilepsies in childhood].

The EEG records of 64 patients affected by a symptomatic severe partial epilepsy have been reviewed retrospectively. The epilepsy started before age 12. The follow-up was at least 5 years after the onset of the disease. In a longitudinal study of interictal EEG, the following parameters were studied: normal or slightly abnormal EEG records at the onset, late appearance of abnormalities of background, multifocal abnormalities and generalized spike and wave discharges. The study of ictal EEG was performed in 32 patients: a good correlation with the interictal and ictal site of the discharges was found. The EEG semeiology of the seizures persisted unchanged throughout the evolution.

Adolescent↗
PubMed1989

Dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome): a condition unrelated to mitochondrial encephalomyopathies.

Thirteen patients with dyssynergia cerebellaris myoclonica (Ramsay Hunt syndrome) had full clinical and neurophysiological study as well as muscle biopsy. The patients had action myoclonus, generalised epileptic seizures, and mild cerebellar syndrome. The disease was inherited in an autosomal recessive pattern in five patients, and occurred as isolated cases in the remaining eight patients. The age at onset of symptoms ranged from 6 to 15 years (mean, 10.4 years). The EEG and polygraphic findings included normal background activity in most patients, spontaneous fast generalised spike-and-wave discharges, photosensitivity, no activation during slow sleep, and vertex and rolandic spikes in REM sleep. Results of muscle biopsy, performed an average of 14 years after onset of the disease, were normal and showed no mitochondrial abnormalities. These findings suggest that Ramsay Hunt syndrome is a condition with distinctive clinical and neurophysiological features and unrelated to mitochondrial encephalomyopathies.

Adolescent↗
PubMed1989

Semi-late onset and rapidly progressive case of Lafora's disease with predominant cognitive symptoms.

The authors report a sporadic case of Lafora's disease, unusual for the comparatively late age at onset and atypical evolution. Discrete visual phenomena that may be considered as partial seizures occurred at age 19 years. A generalized tonic-clonic seizure occurred at 20 years of age and myoclonus became apparent a few weeks later. A massive cognitive dysfunction was clearly apparent 3 months after the first seizure and further mental deterioration occurred although seizures were controlled by medication and myoclonus remained minimal. The EEG showed the typical association of generalized and focal (occipital) changes. Axillary++ skin and muscle biopsies were positive and easily confirmed the diagnosis. The clinical presentation of Lafora's disease is considered by the authors to be sufficient for a clinical diagnosis, even in such an atypical case. Confirmation by skin biopsy is easily obtainable.

Adult↗
PubMed1988

Familial encephalopathy with permanent periodic breathing: 4 cases in 2 unrelated families.

In each of 2 unrelated Algerian families, we observed 2 sisters with a severe static encephalopathy which was detected in the first weeks of life. Anoxia at birth occurred in only one case. This previously unreported familial encephalopathy is characterized by severe mental retardation, hypotrophy, abnormal movements with unprovoked startles, major EEG abnormalities with undifferentiated sleep stages and a very particular periodic breathing pattern that persists during waking and sleep. EEG, polygraphic and video recordings were obtained for all patients. The evolution is chronic and stable. There are no major dysmorphic features. No metabolic or anatomic abnormality was found. Gynecotropy is uncertain and the transmission is likely to be recessive.

Brain Diseases↗
PubMed1988

[Napping sleep EEG in partial childhood epilepsy].

A nap sleep EEG with at least one full sleep cycle has been recorded in 3 groups of children free from diffuse encephalopathy and presenting with partial epilepsy with onset after the age of 3 years: 15 cases with typical benign epilepsy with centrorolandic spikes (BERS), 15 cases with partial symptomatic epilepsy without clinical or radiological evidence of a cerebral lesion, and 12 cases with partial epilepsy symptomatic of a proven cerebral lesion. The time course of paroxysmal abnormalities was quantified according to individual sleep stages; the number of foci was also quantified in the waking vs sleeping state. Paroxysmal abnormalities are significantly enhanced by sleep in all 3 groups: throughout sleep stages in BERS, in slow sleep stages only in the other groups. The enhancement is more striking in BERS, where a clear decrease of abnormalities is also found upon awakening. There is no major difference between the 2 symptomatic groups, with a greater enhancement of paroxysms in the proven lesional group. Contrary to the other groups, only the group including those patients with a documented cerebral lesion showed a significant increase in the number of foci during sleep: numerous foci were found in these patients that were distinct from the primary lesional focus. Under the conditions of our study, the nap EEG seems to provide an accurate evaluation of sleep-induced changes of paroxysmal activity in partial epilepsies of childhood.

Child↗
PubMed1987

[Idiopathic partial epilepsies in children (benign or primary partial epilepsies)].

The concept of idiopathic partial epilepsies in childhood is not yet accepted by all neurologists. We review the diagnostic criteria of these syndromes and stress both wake and sleep EEG characteristics. Different clinical entities have been individualized among other forms: benign partial epilepsy with centro-temporal spikes, benign epilepsy with occipital paroxysms, benign psychomotor epilepsy.

Adolescent↗
PubMed1987

[Lennox-Gastaut syndrome in the adult].

The authors used the definition of the Lennox-Gastaut syndrome (LGS) adopted by the Commission on Classification and Terminology of the International League against Epilepsy. Three hundred and thirty eight patients with childhood LGS have been followed until adulthood; 62.4 p. 100 had an unfavourable outcome. All began their LGS in childhood or infancy (between the ages of 1 and 8.80 p. 100 before the age of 4). In 46.9 p. 100 of cases, complete LGS persisted in the adult. Most cases were apparently primitive. In 15.6 p. 100 of cases, generally symptomatic, the LGS disappeared but an often severe, mostly multifocal epilepsy persisted. 37.6 p. 100 of cases had a more or less favourable outcome. In these cases, the LGS had often begun later (between 7 and 11 years of age) and lasted for less (between 2 and 6 years). Some patients (20.4 p. 100) still had fairly rare partial seizures and neurological or psychiatric symptoms. These cases were mostly symptomatic, 17.4 p. 100 of cases seemed to have been nearly completely cured. These were cases where the LGS had followed another type of epilepsy, mostly of the idiopathic generalized type. Fourty four patients with no prior epilepsy presented with LGS appearing between the ages of 13 and 23. They were divided into 2 groups: in 31 patients, the LGS was associated with focal signs. In all these cases, the evolution was for the worse, whatever the age at onset. In 13 cases, there were no focal signs. In these, there was a different prognosis between those with an onset between the ages of 13 and 15, where the entire syndrome persisted and the evolution is for the worse, and those with an onset after the age of 15, where the outcome was better.

Adolescent↗
PubMed1986

[Status epilepticus in the Lennox-Gastaut syndrome].

Thirty patients with the Lennox-Gastaut syndrome presenting one or more status epilepticus (the first before age 15) were studied. Triggering factors, semiology, duration, severity and EEG features were considered. A comparison was made between this group of patients and another group having the Lennox-Gastaut syndrome but without status. No significant difference appears in the long-term prognosis, even when status were prolonged. Only three patients died during a status. For this reason the authors recommend prudence and avoidance of very strong treatment. In their experience, intravenous diazepines, intravenous phenytoin and intramuscular ACTH were the most effective drugs.

Adolescent↗
PubMed1986

[Contribution of sleep EEG to epileptology: evaluation of a year-long study].

In order to confirm, or to deny, the diagnosis of epilepsy a sleep EEG recording as a supplement to the routine EEG investigation has been performed in 229 subjects in 1980 at Saint Paul Center. In 93% of the cases, spontaneous afternoon napping, and in 7% spontaneous nocturnal sleep was studied. Sleep EEG was analysed and correlated to the clinically suspected type of epilepsy. The occurrence of focal or generalized EEG abnormalities, clearly related to the type of epilepsy, confirmed anamnestic and clinical findings in 54% of the cases and gave a more accurate diagnosis in 20% of the cases. Sleep recordings were necessary for the diagnosis of the type of epilepsy in 2% of the cases.

Electroencephalography↗
PubMed1986

Benign myoclonus of early infancy or benign non-epileptic infantile spasms.

Lombroso and Fejerman (1983) described a syndrome which shares with West syndrome the clinical features of flexion spasm with onset in early infancy. However the syndrome differs from West syndrome in the absence of mental and psycho-motor involvement and having a normal EEG during wakefulness and sleep. We report four cases of these benign spasms of early infancy with polygraphic recording of the spasms providing the following information: the spasms are clinically similar to those often observed in West syndrome; namely they are characterized by a short (2-4 sec.) tonic contraction, there are no significant changes of the EEG concomitant to the spasm, series of spasms can occur not only during the daytime but also during sleep and immediately after awakening. Differential diagnosis is discussed with West syndrome and benign myoclonic epilepsy in infancy and with non-epileptic syndromes. The authors propose to name this syndrome: Benign Non-Epileptic Infantile Spasms.

Electroencephalography↗
PubMed1985

[Somatosensory evoked potentials and action myoclonus].

The authors studied the somatosensory evoked potentials (SEPs) in 16 cases of myoclonic encephalopathies: 8 cases of dyssynergia cerebellaris myoclonica (DCM); 2 cases of dyssynergia cerebellaris progressiva (DCP); 2 cases of Lafora's disease; 1 case of ceroid lipofuscinosis; 3 unclassifiable myoclonic syndromes. The mean age of the patients was 18 years and the mean duration of pre-study evolution was 10 years. All the patients had been treated by anticonvulsant drugs (phenobarbital, valproic acid, benzodiazepines). The amplitude of the complex P1N2 at the level of the contralateral parietal cortex, with stimulation of the median nerve at the wrist, was found to be enlarged in only 6 cases and giant responses (over 40 microV) were obtained in 2 cases. Only half of the patients with DCM presented a high amplitude response. There was no correlation either with clinical parameters (and in particular, certain patients with marked action myoclonic jerks have a normal SEP), or with the EEG data: on the contrary, the amplitude variations of the SEPs are most often similar to variations of the visual evoked potentials.

Adolescent↗
PubMed1985

[Case of epilepsy in an infant with fatal outcome during the 1st year of unknown etiology].

An 11-month-old infant, full-term born after normal pregnancy and delivery had a generalized short tonic-clonic seizure at the 7th hour of life. These seizures were repeated on the 7th, 8th, 9th and 22nd days, and they persisted like bilateral myoclonic fits once a week. The EEG recordings showed asynchronous spikes and spike-waves on the vertex and both frontal areas. The seizure's recording showed a brief burst of bilateral spike-waves. The psychomotor development was retarded but progressive. At 10 months 9 days the patient presented a status without any impairment to all therapeutic trials. Death occurred after 12 days of status. The unexpected severe evolution of this epilepsy with unknown etiology, which did not evoke any metabolic or degenerative diseases, is discussed.

Developmental Disabilities↗