Idiopathic generalized epilepsy of late onset.

Most idiopathic generalized epilepsies have an onset in childhood or adolescence, with a moderate second incidence peak in the presenium predominantly in women. This study addressed the question of a later onset. The available literature and the records of four personal data sets (two prospective incidence surveys of epileptic seizures, one prevalence study of epilepsy, and one clinical series of individuals with epilepsy) were screened for patients who had experienced a first generalized convulsive seizure with bilateral spike-wave complexes on EEG after 60 years of age. Reports of first idiopathic generalized tonic-clonic seizures occurring after age 60 were extremely rare and none was found in our four cohorts regardless of the methodology involved. Only five case reports were found, all involving a woman. Two had a family history of seizure disorders and two had had at least one seizure earlier in life. Idiopathic generalized epilepsy of late onset, if this condition actually exists, is likely to be the consequence of a genetic predisposition triggered by acquired epileptogenic factors.

Absence epilepsies.

Individuals fulfilling diagnostic criteria for childhood absence epilepsy (CAE) and juvenile absence epilepsy (JAE) were selected from a large group of patients who were born between 1945 and 1973 and had presented with absence seizures (AS). Updated data allowed an analysis of 52 patients with CAE and of 62 patients with JAE age > or = 20 years. In CAE, complete control was achieved in 90% of patients (95%, AS only; 77%, AS + generalized tonic-clonic seizures, GTCS). Only 16% of patients with an onset < 9 years had developed GTCS. In JAE, complete control was achieved in 37% of patients (47%, AS; 37%, GTCS). These figures support the validity of the International Classification of Epilepsy (ICE). Stricter diagnostic criteria are discussed.

Pontine myelinolysis following liver transplantation: a report of two cases.

Two cases of central pontine myelinolysis (CPM) following orthotopic liver transplantation (OLT) are reported. Several months after the onset of this neurological syndrome, the two patients are still alive but with severe neurological sequelae. Some patients undergoing emergency OLT present a high risk of CPM because of pre-existing malnutrition, encephalopathy, and hepatic insufficiency. All of these are associated with an inevitable abrupt rise in sodium serum concentration due to intraoperative compensation of blood losses with high-sodium content blood products. Whenever the renal capacity to excrete sodium is impaired by the surgical procedure, continuous intraoperative venovenous hemofiltration is recommended.

[Vascular hemi-parkinson disease]. / Syndrome hémiparkinsonien vasculaire.

A case of unilateral dopa-responsive parkinsonism of gradual onset in a 65 year-old woman suffering from severe ischemic lesions of the contralateral striatum is reported. This case appears to be an example of the rare entity of vascular parkinsonism.

[Central pontine myelinolysis after hepatic transplantation]. / Myélinolyse centropontine après transplantation hépatique.

We report two cases of central pontine myelinolysis (CPM) following liver transplantation. The incidence may well be underestimated as in the past the diagnosis of CPM was based on postmortem findings. Malnutrition, poor clinical condition, encephalopathy are common features of transplanted patients developing CPM. The clinical course is characterized by a biphasic pattern; after normal recovery from anesthesia, there is a subsequent and gradual deterioration in the neurological state. The complex syndrome associates loss of consciousness, flaccid quadriplegia and pseudobulbar palsy. Among the many factors suspected of inducing CPM, a rapid correction of natremia (> 12 mmole/l/day) seems most probable. With regards to liver transplantation, CPM presents rather specific problems. Delaying transplantation to correct hyponatremia carries a risk of severe hepatic encephalopathy. On the other hand, the intraoperative compensation of blood losses with high sodium content blood products tends to induce an abrupt rise in sodium serum concentration. Moreover, renal capacity to excrete sodium is often impaired, due to hepatic insufficiency and surgical procedure. Transplantation should not be delayed, but as infusion of large amounts of sodium cannot be avoided (fresh frozen plasma, human albumin, red blood cells), natremia may be controlled by continuous veno-venous hemofiltration with low sodium content substitution fluids.

[Facio-linguo-masticatory diplegia and epilepsy. Cortical dysplasia]. / Diplégie facio-linguo-masticatrice et épilepsie. Dysplasie corticale.

Neuronal migration anomalies are caused by insults occurring during the third to fifth gestational months when neuroblasts migrate from the germinal zone to the cortical plate. They lead to several cerebral malformations such as macrogyria, identified nowadays by MRI. We describe a case of bilateral parieto-rolandic macrogyria responsible for a bi-opercular syndrome resulting in a facio-linguo-masticatory diplegia associated with mental retardation and severe epilepsy.

The prognosis of benign localized epilepsy in early childhood.

Onset of seizures during early childhood is a not infrequent possibility in benign partial (localization-related) epilepsy (BPEC) when all these syndromes and not only benign partial epilepsy with centrotemporal spikes are considered. In patients followed up for long periods of time, temporal changes in the EEG often make impossible a distribution into discrete syndromes. The electroclinical patterns overlap and the determining factor is not the location but the morphology of the sharp waves. A complete remission is observed in all patients, with rolandic and/or extrarolandic foci. In rare patients, seizures occur during adolescence or later and an early onset is a possible risk factor for such an outcome. It is not a relapse of BPEC but another epileptic syndrome, usually a generalized idiopathic epilepsy. BPEC may be considered as a risk factor for late epilepsy. Patients with an early onset of BPEC tend to have a longer active period of epilepsy and a higher total number of seizures, whatever is the EEG pattern. But neither frequent seizures nor a long seizure period impair the children's abilities. Nonetheless, when BPEC begins in early childhood, the patient is prone to experience frequent seizures during several years. Drug therapy is advisable more often than in BPEC with a later onset.

Classification of epilepsies and epileptic syndromes in two different samples of patients.

We attempted to classify, according to the International Classification of Epilepsies and Epileptic Syndromes, 986 patients consecutively examined during a 13-month period either in a specialized private practice (n = 642) or in an adult neurology unit in a university hospital (n = 344). Without major difficulty, we classified 97% of patients in more or less clearly defined syndromes. Benign frontal and benign psychomotor epilepsies of childhood were represented in this sample of patients. In either partial or generalized idiopathic epilepsies, a diagnosis of epilepsy appears justified even after a single epileptic event when sufficient electroclinical characteristics are present. Patients with symptomatic generalized epilepsies often have to be classified under two or three headings. Many children with a symptomatic generalized epilepsy also experience partial seizures. Alcoholic epilepsy is described as a veritable epileptic syndrome. The distribution of epileptic syndromes was clearly different in the two samples, casting doubt on the value of some epidemiologic surveys based on selected groups of patients.

A randomised double-blind placebo-controlled crossover add-on trial of lamotrigine in patients with treatment-resistant partial seizures.

Efficacy and safety of lamotrigine (LTG) as add-on therapy was assessed in a randomised double-blind placebo-controlled trial of this drug in 23 adult patients with refractory partial seizures. Fifteen patients showed an improvement on LTG treatment, with a greater than 50% decrease in total seizure count in 7 patients. Fourteen patients experienced fewer simple and complex partial seizures, with 8 patients benefitting by more than a 50% decrease in seizure frequency. The drug was well tolerated over the 2 month treatment period. The plasma concentration of concomitant antiepileptic drugs remained unchanged. No haematological or chemical abnormalities were noted.

Local amyloid deposits in a primary central nervous system lymphoma. Study of a stereotactic brain biopsy.

A 37-year-old woman presented with epileptic fits and monoclonal IgG lambda type on CSF examination. A stereotactic brain biopsy disclosed a lymphoplasmacytic lymphoma with local amyloid deposits. The pathogenesis of this unusual localized amyloidosis is discussed.

Survey of seizure disorders in the French southwest. I. Incidence of epileptic syndromes.

An epidemiologic survey began on March 1, 1984, and ended on February 28, 1985. During this period, all neurologists and electroencephalographers of the department of Gironde, an administrative district of the French Southwest (1,128,164 residents in 1982) obtained information by questionnaire from all persons who had experienced an epileptic seizure for the first time in their lives. Recurrent, isolated, and situation-related seizures were included. Febrile convulsions and neonatal seizures were excluded. The global incidence rate of diagnosed epileptic seizures was 71.3/100,000. The incidence rates per year and per 100,000 persons by type of epileptic syndrome were 1.7 for idiopathic and 13.6 for symptomatic localization-related epilepsies, 5.6 for idiopathic and 1.1 for symptomatic generalized epilepsies, 1.9 for undermined epilepsies, 29.0 for situation-related seizures, 18.3 for isolated seizures, and 0.3 for television epilepsies. Other epileptic syndromes were not represented. Using a classification of epileptic syndromes and not of epileptic seizures reduces difficulties in an epidemiologic survey. Diagnosis of an epileptic syndrome is time dependent, however, and at follow-up some patients shift from one group to another.

A survey of epileptic disorders in southwest France: seizures in elderly patients.

An underestimation of epileptic seizures occurring in aged patients is likely. In an epidemiological survey undertaken in Southwest France, the annual incidence rate per 100,000 persons 60 years or older was 127.2 for all seizures (101.3 in persons aged 60 to 69, 150.4 in those aged 70 to 79, and 139.9 in persons 80 years or older). It was 34.1 for symptomatic localization-related and undetermined epilepsies, 16.1 for isolated seizures, and 77.0 for seizures related to an acute situation, such as metabolic derangements, stroke, or head trauma. In this survey, seizures with an onset age of 60 or older represented 28.0% of the confirmed epilepsies, 17.5% of the isolated seizures, and 52.6% of the acute symptomatic seizures. These figures differ dramatically from those in most previous reports concluding that age-specific incidence declined through adult life. They are closer to the data from more recent reports. The main reason for such a high incidence of epileptic seizures in elderly patients is the number of acute symptomatic (or situation-related) seizures. However, even chronic epilepsies increase in frequency in aging persons. Cerebrovascular disease was the most frequently recognized origin (53.9% of the patients with confirmed epilepsies) and brain tumor was found in 32.9% of the patients.

[Classification and definition of epileptic syndromes]. / Classification et définition des syndromes épileptiques.

Epileptic syndromes are classified in order to supply the clinician with a useful terminology. A description of individual types of seizure is not relevant in practice since identical seizures occur in patients with very different outcomes. An epileptic syndrome is an epileptic disorder characterized by a cluster of signs and symptoms. It has therapeutic and prognostic implications. Our proposal for classification of epileptic syndromes is a compromise between scientific classification and practical arrangement.

[Stopping antiepileptic treatment]. / L'arrêt du traitement antiépileptique.

Antiepileptic drug withdrawal must be considered in seizure-free patients. It is theoretically possible. A seizure relapse is frequent. Numerous factors predictive of relapse are known, but they are of limited value. A favorable outcome depends mainly on the patient's epileptic syndrome. Drug reduction must be carried out stepwise, with a slow dose-tapering. A partial withdrawal: reduction of polytherapy to monotherapy, or reduction in daily doses, can also be wise.

[Juvenile myoclonic epilepsy]. / Epilepsie myoclonique juvénile.

Juvenile myoclonic epilepsy is an age-related form of idiopathic generalized epilepsy (mean age of onset: 12-14 years). The diagnosis is based on a cluster of clinical features: types of seizures, namely myoclonic jerks associated with generalized tonic-clonic or clonic-tonic-clonic seizures in 90% of the cases, absence seizures in one third of the cases; triggering factors and circadian rhythm of seizures on awakening or after sleep deprivation; a characteristic EEG pattern, i.e. bilateral symmetrical polyspike-waves. The clinical pattern is so suggestive that in clinical practice EEG is not necessary. Seventy percent of the patients are seizure-free with one-drug therapy. Treatment must be life-long, as relapse occurs in most cases after drug withdrawal, whatever the duration of control.

Prognosis of benign childhood epilepsy with centrotemporal spikes: a follow-up study of 168 patients.

A spontaneous and complete recovery of benign childhood epilepsy with centrotemporal or rolandic spikes (BECT) is taken for granted. However, some authors have reported the occurrence of generalized tonic-clonic seizures in a few adult patients and in some children who have seizures after a long period without problems. The aims of this study were (a) to search for early predictors of outcome and (b) to ascertain the long-term prognosis of BECT in a large group of patients. An attempt to relocate 268 patients born between 1941 and 1967 and consecutively seen as outpatients was undertaken. The outcome after age 20 is known for only 168. Being adults and cured, the others are no longer in touch with their clinics or have moved. Only one indicator of short-term prognosis was found: The earlier the onset of BECT, the longer the period with seizures. Of the 168 patients, 165 are seizure-free with follow-up ranging from 7 to 30 years. Three patients experienced generalized tonic-clonic seizures at age 18, 22-24, and 35. Two apparently had an isolated seizure. The occurrence of such seizures after recovery from BECT is a rare event (approximately 2% of cases) and a relapse with partial seizures is quite uncommon. These patients do not differ from patients remaining seizure-free.

[Subacute encephalopathy. Idiosyncratic reaction to carbamazepine?]. / Encéphalopathie subaiguë. Réaction idiosyncrasique à la carbamazépine?

A 81 year-old female, weighing 56 kgs, had taken carbamazepine (10.2 mg/kg/day) for simple partial motor seizures of recent onset during 15 days. EEG recording showed slow waves on the left hemisphere. CT was normal. The seizures were controlled for a few days and then reappeared beginning as simple partial seizures and progressing to impairment of consciousness. The patient became confused. On admission she was severely confused. Neurological examination was otherwise normal. Plasma sodium level was 134 meq/l and CBZ plasma level was 4.3 micrograms/ml. EEG showed high voltage bilateral delta waves and a left focus. The drug was withdrawn with relief of clinical symptoms and to a lesser extent, of EEG abnormalities. Phenytoin encephalopathy is a well known pathological entity but to our knowledge there has been no report of carbamazepine encephalopathy with neither excessive plasma level of the drug nor water intoxication. An idiosyncratic reaction is considered likely.