Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cclinical and electroencephalographic aspects of twenty seven (27) patients with Lennox-Gastaut syndrome were studied (20 without previous West syndrome, group A, and 7 with this antecedent, group B). The epileptic seizures were characterized through descriptions made by relatives who were in close contact with the patients, and also by direct observation by the author in the clinic. The direct observation was possible due to high frequency rates of the seizures. Different clinical patterns were observed in patients who had and who had not previous history of West syndrome. The analysis of these differences permitted the identification of two groups of patients, although both of them had sharp and slow waves in the EEG. The clinical picture of each group was interpreted as the result of the stage of cerebral maturation at the time the diffuse epileptic encephalopathy occurred.
 ...
PMID:[Comparison between the epileptic manifestation of the Lennox-Gastaut syndrome with and without previous West syndrome]. 82 82

On 62 cases with Lennox-Gastaut's syndrome aged four to 31, the clinical-electroencephalographic findings were summarized as follows; (1) Age of onset was over 10 years in 10 cases (16.1%). (2) Mental deficiencies were more severe in those with onset earlier than age three. (3) Behavioral problems were observed in 34 cases (54.8%); 21 with hyperactive and 13 with hypoactive ones -- 18 hyperactive cases (85.7%) with the onset taking place before age six, and 12 hypoactive cases (92.3%), all whose age is now over 10. (4) The number of clinical seizures showed a tendency in which monoictal manifestation decreased from 25 to three whereas polyictal one increased from 13 to 59 cases during the course of a decade. (5) Interictal EEG findings were pseudorhythm of slow spike-wave with or without focal spikes, and so-called runs of rapid spikes during sleep recording. Focal spikes were observed in 25 cases (40.3%); mainly in the frontal area among those under 10 years old, and in the anterior temporal area among those over 20. The rapid spikes were demonstrated in 19 cases (30.6%) in the over-15 age group and appeared to be correlated with epileptic drop seizures and atypical complex absences.
 ...
PMID:Lennox-Gastaut's syndrome--prognosis of the secondary generalized epilepsies. 82 21

Childhood epilepsies (not including the first 2 years of life) are outlined and discussed; particular emphasis is laid upon the variety of certain forms of epileptic conditions and their clinical course. These forms are divided as follows: a) The Lennox-Gastaut syndrome: a poly-etiological condition with distinct clinical-ictal and electroencephalographic characteristics, mostly associated with mental defects and prognostically unfavorable. b) "Common generalized epilepsy" (also called "centrencephalic" epilepsy), characterized by petit mal absences or a combination of petit mal and grand mal and with a predominantly favorable prognosis. c)Childhood epilepsies with focal spikes in the EEG, in most cases a very benign form with an excellent prognosis. These 3 forms of seizure disorders may be divided in subgroups. The distinction of fine diagnostic nuances is quite helpful but requires well integrated epileptological and EEG experience. The special role of temporal lobe epilepsy is briefly discussed. Furthermore, several etiologies of childhood etiologies are singled out such as inborn errors of metabolism (lipidoses, amino-acidurias), essential hereditary myoclonus epilepsy, tuberous sclerosis, Sturg-Weber's disease, encephalitis, brain tumor and brain abscess. The fringe of the seizure ("borderland of epilepsy") is briefly delineated.
 ...
PMID:[Epilepsies in childhood: differential diagnosis of their forms and courses (author's transl)]. 82 45

Clonazepam or 5-(2-chlorphenyl)-1, 3-dihydro-7-nitro-2H-1,4benzodiazepin-2-one, is a close structural and pharmacological relative of nitrazepam. It has a broad spectrum of activity against the various types of epilepsy, and is effective in many patients whose condition has proved resistant to other antiepileptic drugs. Its chief uses are in status epilepticus, in which intravenous clonazepam may replace diazepam as the drug of first choice, and in the minor motor seizures of childhood, particularly petit mal absences, the Lennox-Gastaut syndrome and infantile spasms. Clonazepam is also at least as effective as current treatment in psychomotor and myoclonic epilepsies, but seems unlikely to replace phenytoin and the barbiturates in the treatment of grand mal or focal motor seizures except in patients resistant to standard therapy. Initial success with clonazepam can be followed by loss of effect, but benefit can often be restored, at least initially, by temporary interruption and re-institution of treatment. Side-effects are common with clonazepam. Most patients experience drowsiness and fatigue, which are frequent causes of withdrawal, together with lesser incidences of ataxia, dystonia, hypotonia, and hyperactivity. These effects usually disappear with continued therapy, and are minimised by gradual introduction of the drug over 2-4 weeks. Hypersalivation and excessive bronchial secretion may be a problem in children and infants.
 ...
PMID:Clonazepam: a review of its pharmacological properties and therapeutic efficacy in epilepsy. 97 34

Polygraphic recordings were performed 22 times during a period of about two years on a 25-years-old man with the Lennox-Gastaut syndrome of the adult type. He had several generalized convulsions initially when he was eight years old, and had the Lennox-Gastaut syndrome since the age of 17 years. The paroxysmal fast rhythm shown 214 times in the recordings, which appeared only in the light stage of sleep, was analyzed. The pattern of the paroxysms, and the relationship between the pattern and the clinical seizures was studied. The parozysms were composed of a series of spikes and slow spike and wave. They appeared diffuse and bilaterally synchronous. And so, were classafied these paroxysms into four types. A through D, from the differences of basic activity before appearance of the paroxysms, amplitude, frequency and fluctuation of amplitude and frequency. Consequently D type was thought to be different from the other types of the pattern in spite of the differences in frequency, fluctuation of that and duration of the discharge. All of the clinical seizures which appeared with these paroxysmal fast rhythms was local tonic spasm in the lip. But D type never failed to associate with local tonic spasms and usually developed generalized tonic seizure. Except for D type, it was shown obviously that the same local tonic spasm appeared when the duration of the paroxysm was longer than 4.9 sec. We proposed the paroxysmal fast rhythm of a name "tonic seizure discharge" on the basis of the findings of this patient.
 ...
PMID:The electroencephalographic study on adult-type Lennox-Gastaut syndrome. 99 14

Electroencephalographic and clinical findings are reported for 100 patients with the Lennox-Gastaut (LGS) triad of slow bilateral spike and wave (BSW), retardation and multiple seizures. Neurological and mental deficits were frequently observed, especially in patients who developed seizures before age 1 yr. More than half of the patients had focal epileptiform discharges that peaked in occurrence at age 4-6 yrs. EEG follow-up showed that background frequency slowed when patients developed the LGS pattern, and increased after recovery. Only 2 patients developed normal EEGs on follow-up, although 22 patients no longer showed the LGS pattern after an average of 3 yrs 3 mos follow-up.
 ...
PMID:The Lennox-Gastaut syndrome: electroencephalographic characteristics, clinical correlates, and follow-up studies. 139 56

We studied all adult patients who between 1984 and 1989 were initially diagnosed at our hospital as having nonconvulsive status epilepticus. Thirty-two patients fulfilled the criteria, which included ictal EEG recordings. The annual incidence was 1.5 in 100,000 inhabitants. The median age at onset of status was 51 years. Ten patients had status as their first epileptic manifestation, but most patients had a previous history of epilepsy. Median duration of epilepsy at onset of status was 4 years. Fourteen patients had focal ictal seizure activity on EEG and thus met the criteria for complex partial status. Eighteen patients had generalized seizure activity on EEG, but only 6 of these had a history of absence epilepsy or juvenile myoclonic epilepsy. None had Lennox-Gastaut syndrome. The clinical features of status in the remaining 12 patients were in some respects similar to those of the patients with complex partial status. We hypothesize that the EEG seizure activity in these patients may have been generalized from an initial focus.
 ...
PMID:Nonconvulsive status epilepticus in adults: thirty-two consecutive patients from a general hospital population. 139 25

Benign myoclonic epilepsy in infancy (BME) is characterized by the occurrence of brief myoclonic attacks in normal infants aged 4 months to 3 years. There is no prior personal history, although in some patients 1 or 2 isolated febrile convulsions may occur prior to the onset of myoclonias. A family history of epilepsy or febrile convulsions is present in 30% of cases. Myoclonic attacks are short and mild, they involve mainly the head and upper limbs. The psychomotor development continues normally after the onset of seizures. The EEG shows a normal background activity and generalized spike-wave or polyspike-wave discharges associated with the myoclonias. These abnormalities are activated by drowsiness and during the first stages of sleep. A clinical and EEG photosensitivity is present in one-third of the patients. Myoclonias can be easily controlled by valproate monotherapy. Rare grand mal seizures can occur during adolescence, after withdrawal of drug treatment. The psychomotor evolution is good if treatment is started early. When myoclonias begin during the first year of life, the diagnoses of cryptogenic infantile spasms and of non-epileptic benign infantile myoclonus must be eliminated. In cases with a later onset, the following diagnoses can usually be easily discarded: cryptogenic Lennox-Gastaut syndrome, myoclonic-astatic epilepsy and unclassified epilepsies with the association of myoclonias and other types of seizures.
 ...
PMID:Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy. 141 73

Myoclonic-astatic epilepsy (MAE) belongs to the group of epilepsies with primarily generalized seizures as absence epilepsies, and juvenile myoclonic epilepsy, as well as infantile and juvenile idiopathic epilepsy with generalized tonic-clonic seizures. Like these types of epilepsy, MAE is polygenically determined with little non-genetic variability. The disease is characterized by the following criteria: genetic predisposition (high incidence of seizures and/or genetic EEG patterns in relatives); mostly normal development and no neurological deficits before onset; primarily generalized myoclonic, astatic or myoclonic-astatic seizures, short absences and mostly generalized tonic-clonic seizures; no tonic seizures or tonic drop attacks during daytime (except for some rare cases with a most unfavourable course); generalized EEG patterns (spikes and waves, photosensitivity, 4-7/sec rhythms), no multifocal EEG abnormalities (but often pseudofoci). There is a partial overlap with other 'syndromes', such as benign and severe myoclonic epilepsy in infants (Dravet et al., 1985a, b), myoclonic epilepsy of infancy and early childhood (Aicardi and others). In differential diagnosis the Lennox-Gastaut syndrome in its stricter sense has to be considered, and also atypical benign partial epilepsy or pseudo-Lennox syndrome. Discussion is presented of possible pitfalls in the classical syndromic approach to classifying epilepsies of early childhood, and of the advantages of a neurobiological view for understanding the immense variability of clinical manifestations of epilepsy.
 ...
PMID:Myoclonic-astatic epilepsy. 141 79

Idiopathic epilepsies with generalized seizures of early childhood are based on a genetic predisposition. The onset takes place between the first and fifth years of age, boys are affected more often than girls. Dependent on the clinical symptomatology you have to distinguish: myoclonic seizures; atonic-astatic seizures; myoclonic-astatic seizures; absences; tonic-clonic seizures. In more than half of the cases a combination of these seizures can be observed. The differentiation of epilepsies with generalized seizures of multifocal origin (infantile spasms, Lennox-Gastaut syndrome and Pseudo-Lennox syndrome [atypical benign epilepsy]) may be difficult but is essential. Therapy of choice is valproate, often in combination with ethosuximide (in children with minor seizures) or with kaliumbromide or phenobarbital (in children with tonic-clonic seizures). Generally the prognosis is more unfavourable if epilepsy starts in the first year of life with afebrile and febrile generalized tonic-clonic or clonic seizures, if children are suffering from longlasting states of seizures and if development is disturbed before beginning of epilepsy.
 ...
PMID:[Idiopathic epilepsy with generalized seizures in early childhood]. 143 3


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>