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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cases of the Lennox syndrome which began at the age between 14 and 16, namely in puberty, were reported. The first case, a 22-year-old woman, was attacked with grand mal seizure at the age of 15, and one year later "Juchzer" (ref. Doose) appeared. This continued for two years, and then astatic seizures appeared. The second case, a 20-year-old woman, was attacked with grand mal seizure at the age of 14, and soon after astatic seizure appeared. Thus both are closely related with grand mal seizures. The first case is idiopathic, and the intelligence developed normally till the onset of the Lennox syndrome. but after that it became disturbed. The second case is residual state of arsenic toxicosis, and so the intelligence was already disturbed before the onset. On EEG both of them at first showed diffuse slow waves of high voltage, and in several years after the appearance of the Lennox syndrome, slow spike-waves were found. On therapy, scarcely no beneficial effects were noted by conventional antiepileptics, and by nitrazepam only the temporal beneficial effect was obtained. The symptoms of our cases are generally similar to the Lennox syndrome in infancy. Though we observed only two cases, we presented our cases as the Lennox syndrome with late onset.
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PMID:On the Lennox syndrome with the onset in puberty. 82 22

The long-term clinical and electroencephalographic follow-up studies were carried out for more than three years, up to 14 years in the longest, on 116 cases with Lennox syndrome. And the results of s-ystematic study on changing patterns and out come have been reported. The follow-up examination was performed also on West syndrome which is closely related with Lennox syndrome; especially the relationship between both syndromes regarding prognosis has been clarified. (1) According to the long-term follow-up on 116 cases with Lennox syndrome, there were 98 cases (84.5%) having mes (61.2%) and persisted as Lennox syndrome except for one case. (2) Generally speaking, the cases with age of onset before two years old showed unfavorable outcome. (3) There were 42 cases (36.2%), which were converted from West syndrome and showed markedly unfaborable prognosis in regard to intelligence as well as the remainging of seizure. (31 cases, 77.5%) (4) On thehand, in 23 idiopathic cases, which showed no developmental retardation before onset of seizure, had favorable outcome, and the remaining of seizure was observed in eight cases (34.8%). However, even in such cases, it was noticed that those displaying mental defect at the follow-up attained 14 cases (60.9%). That is to say, it was clarified that persistence of even minor seizures induced mental deterioration. (5) The cases with favorable prognosis showed usually a typical slow spike-and-wave pattern electroencephalographically, whereas those with poor prognosis showed mostly an asymmetric or disorganized slow spike-and-wave pattern. (6) In many cases displaying signs of brain atrophy with pneumoencephalograpm and accompanying overt neurological signs at the initial examination, prognosis is obviously poor. (7) From the follow-up examination on 94 cases with West syndrome for three to 15 years transformed into Lennox syndrome Among them, those cases with the remaining of seizure at the time of follow-up were 44 (46.8%) out 94 cases, of which 37 cases (83.8%) had remaining seizure as Lennox syndrome. (8) From the above results, it is emphasized that Lennox and West syndromes show close relationship with each other and that a study should be done on the interrelation between their prognosis.
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PMID:Prognosis of the Lennox syndrome-long-term clinical and electroencephalographic follow-up study, especially with special reference to relationship with the West syndrome. 99 12

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.
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PMID:Myoclonic-astatic epilepsy. 141 79

We studied the clinical efficacy of allopurinol as add-on therapy in 31 patients with intractable epilepsy. When administered for a short time, allopurinol was effective in 17 patients (55%); 8 were seizure-free, 8 had 75% decrease in seizure frequency, and 1 had greater than 50% decrease. Allopurinol was most effective in patients with localization-related epilepsy, especially in secondarily generalized tonic-clonic seizures. Allopurinol was not as effective in patients with Lennox syndrome or West syndrome, or in severe myoclonic epilepsy in infants. When allopurinol was administered greater than 1 year, its initial effectiveness continued in 8 of 14 patients who exhibited initial improvement. In 2 of the remaining 6 patients, the initial improvement disappeared during the course of treatment but control was regained by increasing the dosage of allopurinol. Mild side effects were observed in 4 patients (13%): drowsiness in 3 and abdominal pain in 1. Allopurinol may be a useful antiepileptic drug (AED), and a double-blind placebo-controlled trial should be performed.
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PMID:Clinical effects of allopurinol on intractable epilepsy. 190 Jul 91

A clinical and electroencephalographic study was undertaken on 215 children with complex partial seizures as selected on the basis of the International Classification of Epileptic Seizures (1981). Complex partial seizures were noted in 7.8% of the epileptic children. The ictal symptoms of complex partial seizures closely resembled those of psychomotor triad described by Lennox. Interictal EEG revealed seizure discharges from the temporal or frontal focus in 57.2%. There existed a group with automatism as a main symptom having both diffuse slow spike-waves and focal temporal spikes. This type should be interpreted to be one of secondary generalized epilepsies and be a variant of the Lennox syndrome.
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PMID:Complex partial seizures in childhood. 383 Aug 38

Free amino acid patterns of cerebrospinal fluid in infants and children with various types of convulsive disorders were compared with those in age-matched normal subjects. The total free amino levels in Lennox syndrome were higher than the normal values, and those in infantile spasms controlled by ACTH were higher than those in uncontrolled infantile spasms. Although the levels of only one or two amino acids in tonic-clonic seizure, focal seizure and febrile seizure were higher or lower than those of the controls, the levels of 8 amino acids in infantile spasms were lower and those of 10 amino acids in Lennox syndrome were generally higher compared to the controls. Among amino acids in CSF of children with tonic-clonic seizure, infantile spasms or Lennox syndrome, only the ornithine level was commonly lower than that of the controls. After the treatment, in tonic-clonic seizure, the levels of taurine, asparagine and glycine were increased, and in infantile spasms, those of asparagine, glutamine, glycine, alanine, phenylalanine, lysine and arginine were increased while that of taurine was decreased. These results suggest that each type of convulsive disorder shows the specific amino acid pattern, and the effects of anticonvulsants may be partially understood through the changes of the free amino acid patterns in the brain.
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PMID:Amino acid metabolism in the brain with convulsive disorders. Part 3: Free amino acid patterns in cerebrospinal fluid in infants and children with convulsive disorders. 632 17

In Lennox syndrome the brainstem which plays important roles in regulating sleep and its parameters is thought to be disturbed. In order to clarify the importance of the dysfunction of the brainstem in Lennox syndrome, polygraphic examination were studied and their findings were assessed with prognosis. 8 patients aged from 6 to 17 years were subjected to this study. They were divided into two groups according to their prognosis. Group 1 showed good prognosis. Seizures were easily controllable and have not occurred for more than 24 months. In group 2 seizures were intractable and were uncontrollable by medication. In 4 normal children ranging in age from 4 to 10 years, the same studies were performed. Recordings were performed on two consecutive nights and the second night recordings were used for analysis. Polygraph consisted of EEG from C4 and P4, bipolar EOG from electrode attached to outer canthus, surface EMG from submental muscle and 5 or 6 other muscles including trunk and limbs. Sleep stages were determined in each minute according to the standard of APSS. Body movements were classified into two types on the basis of their distribution over body parts and on duration of movements. Gross movements (GM) involved the body trunk and lasted for more than two seconds. Twitch movements (TM) were localized in one muscle on surface EMG recordings lasting less than 0.5 seconds. In normal children, the rate of GM in sleep stage 1 and REM are significantly higher than slow wave sleep. And this is the same in TM of all muscles.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Body movements during sleep in Lennox syndrome]. 633 76

In 1981, Inanaga et al reported on the efficacy of DN-1417 (a TRH analog) in the treatment of degenerative myoclonus epilepsy and other forms of intractable epilepsy. Following this report, we studied the efficacy and safety of DN-1417 treatment in 10 intractable epileptic children ranging in age from 6 months to 11 years (mean 4 years), including 7 with Lennox syndrome (LS), 2 with West syndrome (WS) and 1 with degenerative myoclonus epilepsy (DME). The daily dose was from 0.02 to 0.05 mg/kg, initially, and then was increased to 0.05 mg/kg. Complete control of seizures was achieved in 2 patients with LS, a 50% or greater decrease in seizure frequency was observed in one patient each with LS and DME and a less than 50% decrease in 1 with LS and 2 with WS. There was no change in 2 LS cases, and 1 LS case became worse. Activation of psychic activities, such as psychomotor activity, facial expression and motivation, was also noted in 7 of the 10 patients. Furthermore, improvement of motor function was noted in 5 patients. Electroencephalographic abnormalities improved in 2 completely seizure free patients with LS in which EEG ameliorated along with clinical seizure control.
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PMID:A pilot study on the anticonvulsive effects of a thyrotropin-releasing hormone analog in intractable epilepsy. 641 1

Of various topics concerning convulsive disorders in children, long-term prognosis of childhood epilepsy and developmental aspects of age-dependent epileptic encephalopathy (ADEE) were described. Recent progress in epileptology and introduction of effective antiepileptic drugs has allowed marked improvement in the prognosis of epilepsy. According to our long-term observation of epileptic children over 10 years, the remission rate for over three years was as high as 81.7%. Intractable cases were notably high in the West and Lennox syndromes. One of the main targets of child epileptology is ADEE, i.e. the West and Lennox syndromes in addition to early-infantile epileptic encephalopathy with suppression-burst (EIEE). The concept and categorization of ADEE were outlined with special reference to the developmental aspects of EIEE by a long-term follow-up study. Six of 10 cases of EIEE evolved into the West syndrome at two to six months of age, and two cases showed further transition to the Lennox syndrome at one year one month and three years one month of age. In accordance with the evolutional change in clinical seizure pattern, EEG showed an evolutional change from suppression-burst to hypsarhythmia and further development to diffuse slow spike-waves. These facts suggest a close relationship among these three types of ADEE and the significance of developmental aspects in the study of epilepsy.
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PMID:Seizure disorders in infancy and childhood. 653 99

To investigate the significance of EEG findings relating to the appearance of psychic symptoms in epileptic children, a clinicoelectroencephalographic study was undertaken on 15 cases with psychotic episodes. 1) Psychotic episodes with hallucination and/or illusion were observed, though rarely, in childhood epilepsy. These seemed liable to occur in temporal lobe epilepsy. 2) Three of four patients in the hallucination and illusion group showed frequent epileptic discharges. However, no distinct relation existed between seizure discharges and psychotic episodes. 3) Eight of 11 patients in the dysphoria and excitement group were secondary generalized epilepsy. 4) In the dysphoria and excitement group, psychotic episodes occurred either as a result of the increase in epileptic discharges or conversely as a result of the suppression of epileptic discharges. The former was more frequent. Epileptic discharges tended to be suppressed in the Lennox syndrome and allied conditions relating to psychotic episodes. 5) A forced normalization-like phenomenon mostly resulted from the marked suppression of diffuse slow spike-waves. However, a complete suppression of epileptic discharges was not always noted.
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PMID:Psychotic symptoms in childhood epilepsy--an electroencephalographic study. 667 30


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