Early-onset benign occipital seizure susceptibility syndrome.

PURPOSE: Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. METHODS: This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. RESULTS: One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. CONCLUSIONS: Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

[EEG anomalies in the prodromic phase of Rasmussen's syndrome. Report of two cases]. / Anomalies EEG dans la phase prémonitoire du syndrome de Rasmussen. A propos de deux observations.

Electroencephalographic (EEG) recordings were studied at disease onset in two subjects presenting with Rasmussen's syndrome. Particular attention was paid to abnormalities detected during the prodromic phase before clinical outcome suggested the existence of chronic encephalitis. EEG recordings showed focal, polymorphic abnormalities associated with slow biphasic complexes (SBC). These complexes that are composed of two slow waves with opposite polarity, a 150- to 250-mV peak-to-peak amplitude and a 500-ms duration have only been described in inflammatory syndromes of the central nervous system. Their occurrence at onset of Rasmussen's syndrome are discussed.

[Effect of antiretroviral treatment on early electroencephalographic and otoneurologic manifestations in HIV infection and prognostic importance of verified perturbations]. / Effet du traitement antirétroviral sur les manifestations électroencéphalographiques et otoneurologiques précoces de l'infection à VIH et signification pronostique des perturbations constatées.

Electrophysiologic tests may be abnormal in asymptomatic HIV-1-infected individuals. Our study was aimed at determining whether these findings have a prognostic value and could be corrected by antiviral treatment. In 18 patients, followed for 34 or 43 months, these findings were not progressive. Only one patient developed Aids dementia complex (ADC). Three have died (one with normal, two with abnormal tests at baseline). To study the effect of antiviral treatment, another group of seven asymptomatic patients was included into a cross-over double-blind study with either eight weeks zidovudine or eight weeks placebo, separated by eight more weeks without treatment. Electrophysiological evaluation was also performed in a group of 15 patients before antiviral therapy with zidovudine or didanosine was started and again after a mean of three and 13 months treatment. Results did not suggest that treatment reverses early electroencephalographic and otoneurological changes seen in HIV-1 infection.

Special types of periodic lateralized epileptiform discharges associated with confusional state in cerebral circulation insufficiency.

A peculiar type of periodic lateralized epileptiform discharges (PLEDs) was recorded on 22 EEGs performed in 11 patients. They were characterized by bursts of sinusoid activity around 7-9 Hz, usually followed by a slow wave, lasting less than 500 ms, with a period less than 2 s, and these sequences could be continuous over several days. We propose to name this pattern 'periodic sinusoid paroxysmal activity' (PSPA). PSPA was recorded mainly over both parieto-temporo-occipital regions, although asymetrically. Sensory stimulations and anticonvulsants had no effect on PSPA. In three cases, mobilization of the head blocked PSPA. The spontaneous disappearance of PSPA was sometimes followed by classical PLEDs. PSPA was always associated with a confusional state and behavioural disturbances, and erratic distal clonic movements were seen in 6 cases. These symptoms disappeared as soon as PSPA ceased. Patients had either vascular risk factors or a previous cerebrovascular insult. The confusional state was associated with either lowered carotidian bloodflow or probable global cerebral hypoperfusion due to a systemic pathology. A vascular origin of PSPA is likely, due to the presence of vascular risk factors, to the morphology of PSPA and to the provoking and inhibiting factors.

Epilepsy or paroxysmal kinesigenic choreoathetosis?

Epileptic seizures induced by sudden movement and paroxysmal kinesigenic choreoathetosis (PKC) have often been confused in the past, owing to the close similarity of the attacks, the equally good response to anticonvulsants, and the frequent occurrence of epilepsy and PKC in the same family, or even in the same patient. The pathophysiology of PKC is still unclear and its relationship with epilepsy open to discussion. The sparing of consciousness and the lack of postictal phenomena are constant features of PKC, thus differentiating this syndrome from epilepsy. We report the case of an 8-year-old boy with frequent brief tonic attacks, without loss of consciousness, triggered by sudden movement. The neurologic examination, EEG and MRI did not help to differentiate between epilepsy and PKC. Only the occurrence of a longer seizure with clouding of consciousness and the recording of the postictal abnormalities on the EEG supported a diagnosis of reflex epilepsy induced by movement.

[EEG in HIV infection]. / L'EEG dans l'infection par le VIH.

Since 1985, early changes in the EES have been demonstrated in the course of HIV infection. This method can be considered one of the accurate tests for the assessment of evolution of the disease. Abnormal anterior changes are present in about 30% of HIV+subjects with at least stage II disease. EEG abnormalities during the disease form a spectrum ranging from minor changes to slow diffuse activity, usually found in cortical lesions. These are associated in some cases with a periodic or pseudo-periodic activity as often found in white matter lesions.

'De novo' absence status of late onset: report of 11 cases.

Absence status (AS) is a heterogenous epileptic syndrome that can occur at any age, usually in a context of prior epilepsy. Eleven cases of AS occurring in middle-aged patients who had no history of epilepsy were retrospectively collected over a 10-year period (10 women and one man; mean age, 58.6 years). Eight patients were receiving high doses of psychotropic drugs. Clinical and EEG presentation was similar to AS occurring in patients with prior epilepsy. Evaluation of precipitating factors revealed that AS coincided with benzodiazepine withdrawal in eight cases. Cofactors included excessive use of other psychotropic drugs, nonpsychotropic treatment, hypocalcemia, hyponatremia, and chronic alcoholism. CT demonstrated mild cerebral atrophy in six cases. There was no recurrence, even without chronic antiepileptic treatment. These data indicate that (1) most cases of "de novo" AS of middle age or late onset result from the addition of various epileptogenic factors; (2) AS can be considered a new and uncommon complication of benzodiazepine withdrawal, and (3) long-term administration of anticonvulsant medication may not be required.

A controlled study of early neurologic abnormalities in men with asymptomatic human immunodeficiency virus infection.

BACKGROUND: Although neurologic complications are frequent in the acquired immunodeficiency syndrome, their incidence and progression in early human immunodeficiency virus (HIV) infection remain controversial. The goal of this study was to assess neurologic manifestations in asymptomatic carriers of HIV. METHODS: We studied 29 HIV-seropositive homosexual men and 33 seronegative homosexual controls by means of neurologic and neuropsychological examinations, magnetic resonance imaging (MRI), and electrophysiologic tests (electroencephalography, multimodal evoked-potential tests, and otoneurologic tests). After six to nine months, the tests were repeated in 27 seropositive men and 30 controls. The investigators were blind to the serologic status of the subjects. RESULTS: The seropositive subjects had a mean CD4+ lymphocyte count of 635 X 10(6) per liter. Neurologic and neuropsychological examination, MRI, and measurements of pattern visual evoked potentials did not show significant differences between the two groups. The latencies of the median-nerve somatosensory evoked potentials were slightly prolonged in the seropositive men. The initial electroencephalogram was judged abnormal in 8 of 27 of the seropositive subjects (30 percent) as compared with none of the controls, with a slowing of fundamental activity, anterior spread [corrected] of alpha rhythm, subnormal reactivity, and unusual anterior theta activities. These findings were confirmed by computerized spectral analysis. The second electroencephalogram was abnormal in 10 of 25 of the seropositive men (40 percent). The otoneurologic evaluation identified abnormalities in the central auditory or vestibulo-ocular pathways in 34 percent of the seropositive men (10 of 29), as compared with 6 percent of the controls (2 of 33), on the first examination and in 44 percent (12 of 27) and 7 percent (2 of 30), respectively, on the second examination. Altogether, electrophysiologic abnormalities were found in 67 percent of the seropositive men (18 of 27) and 10 percent of the controls (3 of 30) (P less than 0.00005). CONCLUSIONS: In persons with asymptomatic HIV infection, electrophysiologic tests may be the most sensitive indicators of subclinical neurologic impairment. Electrophysiologic abnormalities are far more common in asymptomatic carriers of HIV than in controls and tend to progress over time.

[EEG recordings in 19 cases of AIDS with encephalic involvement]. / EEG dans 19 cas de SIDA avec atteinte de l'encéphale.

Between 1983 and 1987, 19 patients presenting with encephalic involvement of AIDS had an EEG recording during the early neurological examination. In 8 patients, EEGs were repeated 1 to 11 times during the course of the disease. All recordings were abnormal. A good EEG correlation existed with clinical, virological and bacteriological (SF), radiological, and histological data (10 cases). EEG recordings are useful to study the encephalic pathology of AIDS, not only for its secondary, but even more for its primary involvement.

[Confusional states of epileptic origin. Value of emergency EEG]. / Etats confusionnels d'origine épileptique. Intérêt de l'eeg fait enurgence.

Fifty-two patients having suffered 60 episodes of non convulsive Status Epilepticus (SE) proven by electroencephalography between 1976 and 1986 are reported. According to electroclinical criteria, these SE were classified into three groups: Petit Mal Status (PM St), Psychomotor Status (Ps M St) and Frontal Polar Status (F St). The exact diagnosis could not be accurately established by the clinical examination alone. It required the analysis of the ictal EEG. However, some clinical signs might suggest the correct diagnosis. Thus, a fluctuating confusional state associated with myoclonus suggested a PM St. A state of confusion with alteration of the emotional sphere evoked especially a Ps M St. A confusional state associated with behavioural disorders of euphoric type and to programmation difficulties was seen mainly in F St.

[Lennox-Gastaut syndrome in the adult]. / Le syndrome de Lennox-Gastaut de l'adulte.

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.

Monotherapy with valproate in primary generalized epilepsies.

Sodium valproate enteric-coated tablets were administered as monotherapy to 118 patients (median age, 19 years) with primary generalized epilepsies. More than half (56%) of these patients were transferred from prior drug therapy, most of them because of inadequate seizure control, and some because of adverse effects. Seventy-one percent of the patients experienced tonic-clonic seizures, either alone or in combination with other types of seizures, principally absences. Mean duration of follow-up was 18 months (median, 17 months; range, 1-68 months). At a mean daily dosage of less than 20 mg/kg, 83% of the patients became seizure-free. Therapy was equally effective against tonic-clonic seizures, absences, and myoclonic seizures. Tonic-clonic seizures were suppressed in 85% of cases (89% when patient had only one seizure type), absences in 82% (95% when patient had only one seizure type), and myoclonic seizures in 82%. Paroxysmal activity was present in 88% of the electroencephalogram (EEG) records before valproate monotherapy, and in 32.4% at the study's end. These results were achieved with generally mild and mostly transient side effects; side effects were reported by 16% of patients during the first month, and 2% at the last follow-up. No hematologic or hepatic toxicity was observed. The lag time between attaining steady-state serum concentrations and achieving maximal clinical improvement suggests that sodium valproate monotherapy should be given an adequate trial to ensure that patients derive the greatest possible benefit before adding or switching to another drug.

Plasma prolactin and cortisol concentrations in epileptic patients during the night.

Plasma cortisol and prolactin concentrations were determined every four hours, from 8 PM to 8 AM, in 19 epileptic patients during EEG recording of sleep. Data were compared with those obtained from 12 healthy young male volunteers studied under similar conditions. Patients had normal cortisol rhythm, with peak levels at 4 AM or 8 AM and trough at midnight. A sleep-related increase in prolactin concentration was observed in all patients. The range of prolactin concentrations in the patients was also normal. Treatment with valproic acid (ten patients) and frequent abnormal EEG discharges (five patients) did not affect cortisol and prolactin secretion.

[Benign partial epilepsies: 11 cases of frontal partial epilepsy with favorable prognosis]. / Les épilepsies bénignes partielles: 11 cas d'épilepsie partielle frontale à évolution favorable.

The authors report a retrospective study of 11 observations of partial frontal epilepsy. Cases have been selected on the basis of a complete lack of any associated neurological or psychiatric sign and a disappearance of seizures with normalization of the EEG trace for more than 5 years. This peculiar type of focal epilepsy characterized by an EEG frontal spike focus is first shown to share clinical and electroencephalographic signs with other forms of benign epilepsy of childhood and adolescence. Then the predictive value of some electro-clinical criteria to indicate a favourable outcome is examined.

Infantile epilepsy with occipital focus and good prognosis.

The authors studied 18 cases of infantile epilepsy with occipital focus and good prognosis. The patients were selected according to clinical and EEG criteria. Those criteria are: neurological and neuroradiological examinations must not show any abnormality; the patients must be free from seizures for at least 3 years and 6 months; the fundamental EEG activities must be normal, and a spike and/or spike and wave occipital focus has to be present. The medical story, the clinical and EEG follow-up, the seizures semiology (clinical and EEG) during the awake and sleep stages are described. The data show a new primary benign epileptic entity which is probably associated to maturation disturbances.

[Indication of tele-electroencephalography coupled to videoscopy in the study of higher functions in the epileptic child]. / Indication de la télé-électroencéphalographie couplée à la vidéoscopie dans l'étude des fonctions supérieures de l'enfant épileptique.

An examination procedure, including psychometric tests performed during a tele-encephalographic recording coupled to videoscopy, has been applied twice to 32 epileptic children in a double-blind study appreciating the clinical and electroencephalographic effects of an antiepileptic drug. The psychometric test scores were influenced by the presence of subclinical spikes and spike-and-wave discharges or by seizures of very short duration eluding investigation of the psychologist. It is shown that psychometric test results can be interpreted in a different way according to the procedural choice. Without simultaneous tele-encephalographic and videoscopic documents, the anti-epileptic drug tested in the study (sodium valproate) would have been considered as having a direct favorable action on superior functions appreciated by performance on the tests used. However, it has been possible to demonstrate that the improvement is consecutive to the beneficial effect of the drug in relieving seizures and subclinical spikes and spike-and-wave discharges.