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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

EEG findings and the course of epileptic seizures in two patients with neuronal ceroid lipofuscinosis (Batten Spielmeyer Vogt syndrome) are presented. Both patients, during the course of disease, developed therapy resistant epileptic reactions with myoclonicastatic seizures. These seizures in connection with diffuse encephalopathy and EEG pattern with 2.5 to 3.5/sec slow-spike-wave meet the criteria of the Lennox syndrome. Pathogenetic questions regarding possible additional genetic predisposition for epileptic seizures are discussed. Since therapeutic effect of different medications is uncertain hormonal therapy may be considered.
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PMID:[Myoclonic-astatic seizures (Lennox syndrome) in the course of juvenile neuronal ceroid-lipofuscinosis (M. Batten-Spielmeyer-Vogt) (author's transl)]. 21 38

Neuropathological considerations were performed on a case, who went into Lennox syndrome after an acute encephalopathy at the infantile period, and moreover who fell into an akinetic-mute state derived from brain damage by herniation caused by a head injury and subsequent status epilepticus. Neuropahtological background in the present case of Lennox syndrome is thought to be based on the widespread unilateral cerebral lesions and the basal ganglional, especially thalamic, degenerations derived secondarily from the diffuse cerebral damage. The patient revealed akinetic mutism with the disappearance of the epileptic seizures and the desynchronization of the EEG's, when the brain lesions formed at the adult period spread over the opposite hemispheric limbic system and the brain stem tectum.
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PMID:Neuropathology of secondary generalized epilepsy--(Lennox-Gastaut syndrome)--a case report. 65 45

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

Convulsions or status epilepticus in 11 infants after pertussis vaccination are reported. In 3 cases grand mal epilepsy persisted and 2 children developed infantile epileptic encephalopathy (Lennox syndrome). On the basis of our own experience, the incidence of seizures approximates 1:4800 infants vaccinated or 1:12 800 vaccinations. According to a recent prospective study from the USA, the incidence of seizures may be closer to 1:600 infants. Since there is a significant difference between the incidence of spontaneous fits in children of the same age group and the incidence after vaccination, a causal relationship between the seizures and vaccination appears to be confirmed. The following conclusions are drawn from these observations: 1. In view of the usually benign course of whooping cough today, current vaccination against pertussis is hardly satisfactory. Improvement of the available vaccines is an urgent necessity. The protection should include the population most at risk, i.e. infants during the first few months of life. 2. Parents should be better informed about the risks involved in pertussis vaccination. 3. Booster inoculations should be abandoned. 4. Health authorities should decide whether the current pertussis vaccination program should be continued. 5. Complications following vaccination should be registered at a national centre.
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PMID:[Convulsions after whooping-cough vaccination]. 679 99

A longitudinal clinico-electroencephalographic study was conducted on 484 cases of age-dependent epileptic encephalopathy; early-infantile epileptic encephalopathy with suppression-burst (Ohtahara) (EIEE), the West and Lennox syndromes. 1) The three types had the same kind of etiologic heterogeneity. 2) An evolutional change with age was noted from EIEE to the West and from the West to the Lennox syndromes, with the change from suppression-burst to hypsarhythmia, and from hypsarhythmia to the diffuse slow spike-waves. 3) The core ictal EEG pattern of tonic spasms was the desynchronization in EIEE and the West syndrome, with the rapid synchronization and recruiting rhythm in the Lennox syndrome. In the same cases, the changes from desynchronization to hypersynchronization were observed with age. These facts suggest that the three syndromes are based on the same pathophysiology, strongly related to the subcortical mechanism, and that their clinico-electrical manifestations are modified by the degree of brain maturation.
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PMID:Age-dependent epileptic encephalopathy: a longitudinal study. 732 68

Lennox-Gastaut syndrome (LGS) is a rare epileptic encephalopathy with a peak age of onset of 3-5 years of age. Reported prevalence rates for LGS vary widely from 1-10% of all childhood epilepsies. Incidence rates are much lower. LGS is characterized by intractable, multiple, generalized seizure types and an interictal electroencephalogram showing bursts of slow spike-and-wave, paroxysmal bursts of generalized polyspikes, and a slow background. All patients have tonic seizures during sleep that may be subtle, and nearly all have treatment-resistant, lifelong epilepsy. Cognitive stagnation and behavioral problems are seen in almost all patients and lead to a life of dependency. The differential diagnosis includes other symptomatic generalized epilepsies and pseudo-Lennox syndrome. Misdiagnosis is common. Children and adults with LGS have an enormous impact on their families, and efforts to improve the quality of life for these patients are complex.
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PMID:Definition and natural history of Lennox-Gastaut syndrome. 2179 May 60