Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0009952 (febrile convulsions)
 1,215 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy. 141 73

A random cluster sample survey of approximately 18,000 people in 11 villages was performed in Ulanga, a Tanzanian district with a population of approximately 139,000 people. Well-instructed fourth-year medical students and neurologic and psychiatry nurses identified persons with epilepsy using a screening questionnaire and sent them to a neurologist for detailed evaluation. Identified were 207 subjects (88 male, 119 female) with epilepsy; of these, 185 (89.4%) (80 male, 105 female) had active epilepsy. The prevalence of active epilepsy was 10.2 in 1,000. Prevalence among villages varied, ranging from 5.1 to 37.1 in 1,000 (age-adjusted 5.8-37.0). In a 10-year period (1979-1988) 122 subjects living in the 11 villages developed epilepsy, with an annual incidence of 73.3 in 100,000. Generalized tonic-clonic seizures (GTCS) accounted for 58% and partial seizures accounted for 31.9%, whereas in 10.1% seizures were unclassifiable. Of the partial seizures, secondarily generalized seizures were the most common. Possible etiologic or associated factors were identifiable in only 25.3% of cases. Febrile convulsions were associated in 13.4 of cases. Other associated factors included unspecified encephalitis (4.7%), cerebral malaria (1.9%), birth injury (1.4%), and other (3%). In 38% of the cases, there was a positive family history of epilepsy.
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PMID:Prevalence and incidence of epilepsy in Ulanga, a rural Tanzanian district: a community-based study. 146 63

We examined the clinical significance of bisynchronous spike-wave complexes which appear in EEGs of some patients with focal spikes in one or both temporal lobes. Few features distinguished patients with bisynchronous spike-waves from those with only focal temporal spikes: a higher incidence of patients with more than two grand mal seizures a year (43 vs. 7%) and a younger age at last recording (22.5 vs. 28.3 years) were the only statistically reliable factors. Proportion of patients with complex partial seizures (CPS) (100%), age of CPS onset, incidence of febrile convulsions, intellectual level, and normal neurologic examination were features common to the two groups. In contrast to other studies which did not exclude influencing variables such as extratemporal spike foci, our group with bisynchronous spike-waves fared equally well after temporal lobectomy as did patients with temporal spikes alone. All 6 patients with spike-waves obtained a reduction of at least 50% in seizure frequency and 5 of the 6 (83%) had a greater than 90% reduction. Sixteen of 17 patients (94%) without spike-waves had a reduction of at least 50% and 14 of the 17 (82%) had a greater than 90% reduction of seizures.
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PMID:Significance of bisynchronous spike-waves in patients with temporal lobe spikes. 249 40

The present study classified epilepsies in Nigerians and compared them with the profile of epilepsies as found in other countries: India, France, and Denmark. Partial epilepsy formed the largest group (76.6%) in this study and in that in India (80%), but these incidences were higher than that found in France (62%), owing to a higher frequency of birth injury, CNS infections, and childhood febrile convulsions in developing countries. In contrast to our study and that in France, where partial epilepsy with complex symptomatology formed the largest subgroup, partial epilepsy with elementary symptomatology formed the largest subgroup in India. The reason for this is not totally clear, though etiological factors and criteria for categorization are contributory. The incidence of partial epilepsy was lower in children than in adults owing to a relatively lower incidence of partial epilepsy with elementary symptomatology in children in the present series and a lower incidence of complex symptomatology in children in France and Denmark. Nigerian children seem more vulnerable to complex symptomatology owing to a high incidence of febrile illness (e.g., from malaria) and febrile convulsions. The incidence of generalized epilepsies in children was higher than in adults. Grand mal formed the largest subgroup of generalized epilepsies in children in this series and in Denmark, whereas petit mal formed the largest subgroup in France and India. Petit mal was relatively rare in children in our series (2.5%) compared with children in the French study (17.5%). Secondary generalized epilepsy was peculiar to children in all the series.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Classification of the epilepsies: an investigation of 945 patients in a developing country. 392 51

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 this study, we report the cases of five unrelated patients with Klinefelter's syndrome and seizures or EEG epileptiform abnormalities; the karyotype was 47,XXY in four, and 47,XXY/46,XX in one. They were aged 13-25 years and followed up both clinically and by means of EEG. Two of the patients had epilepsy, one had only one isolated generalized tonic-clonic seizure, one had febrile convulsions and one presented focal epileptiform EEG abnormalities without seizures. In two of the patients, it was possible to classify the epilepsy (childhood epilepsy with occipital paroxysms and cryptogenic or symptomatic generalized epilepsy). Although the electroclinical patterns appeared to be rather heterogeneous in our patients, it is possible to infer the relative good evolution of seizures in Klinefelter's syndrome.
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PMID:Seizures in Klinefelter's syndrome: a clinical and EEG study of five patients. 759 75

A family history of epileptic seizures including febrile convulsions was found in 15 of 103 patients (15%) with localization related epilepsy with partial seizures with and without secondary generalization, who were operated on because of drug resistance. This rate was significantly higher than that of the cumulative incidence in the general population (4%). The localization of the brain damage did not play a role (temporal lobe resection left: 15%, right: 17%, extra-temporal lesion excision: 20%, hemispherectomy: 11%). Various family members were involved. Some patients had more than one relative with seizures. Thus, 21 relatives suffered from seizures. Eleven of them had generalized tonic-clonic seizures (one grand mal on awakening), 7 had febrile convulsions (4 complicated), and in 1 patient the grand mal seizures on awakening were preceded by absences; 1 had generalized tonic-clonic and complex partial seizures; 1 after complicated febrile seizures likewise had complex partial seizures; another mentally retarded patient suffered from generalized tonic-clonic, axial tonic and myoclonic-astatic seizures. The seizure type of 3 remote relatives was not known. The first seizure occurred in 16 family members during childhood, in 3 in adolescence and in only 1 in adulthood (1 unknown). Eight showed mental retardation of slight degree in most. It is interesting that only one-third of the patients with a family history with seizures were seizure-free after the operation; 5 still had seizures, mostly reduced in frequency, 3 had seizures and isolated auras and 2 had only isolated auras. On comparing the findings in patients with and without a family history with seizures, those with family members with epileptic seizures showed a lower rate of an intellectual deficit (7 vs 47%) and brain tumours (13 vs 44%). Our earlier findings with a different group of patients are thus confirmed: that genetics play a role in symptomatic epilepsies.
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PMID:The genetics of localization-related symptomatic epilepsy: risk of a family history with seizures in patients who have undergone surgery. 926 63

Generalized tonic-clonic seizure activity in infants and children frequently leads to an emergency department visit, often after emergency medical service personnel, such as paramedics, provide initial evaluation and treatment. Important subsets of patients who present to the emergency department include those with non-seizure-mediated movements, those with nongeneralized seizure activity, those with complications of anticonvulsant therapy, and those with status epilepticus. Recognizing, diagnosing, and treating these conditions and minimizing complications are key issues to be considered in the refinement of emergency department practice. Of the children with seizures who are seen in the emergency department, those with febrile convulsions or exacerbations of underlying seizure disorders predominate, while those with new-onset epilepsy or other seizure disorders account for a smaller proportion. Current issues in the emergency department management of seizures in children include: (1) modifying interventions to stabilize patients and simultaneously minimize the physiologic deterioration accompanying generalized seizures; (2) selection, initiation, administration, and refinement of anticonvulsant therapy; (3) minimizing complications of prolonged seizures and their treatment; (4) rapid recognition and treatment of life-threatening illnesses that underlie seizure presentations; (5) selection of appropriate diagnostic measures; and (6) use of electroencephalography in selected patients.
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PMID:Clinical issues in acute childhood seizure management in the emergency department. 979 45

Severe myoclonic epilepsy in infants (SME) is one of the most malignant epileptic syndromes recognized in the latest classification of epileptic syndromes. The clinical details and electroencephalographic (EEG) characteristics have been elucidated by Dravet et al. The diagnosis of SME depends largely on the combination of clinical and EEG manifestations at different ages, of which the presence of myoclonic seizures appears to be the most important. However, because of the inclusion of different types of myoclonic attack and the lack of strict criteria for diagnosing SME, there has been some confusion as to whether patients without myoclonic seizures or myoclonus should be classified as SME, despite other identical clinical symptoms (SME borderlands (SMEB) group). Among the various clinical manifestations characterizing SME, special attention has been paid to seizures easily precipitated by fever and hot baths in Japan. We have demonstrated that the onset of myoclonic attack in these patients is very sensitive to the elevation of body temperature itself rather than its etiology. Using simultaneous EEG and rectal temperature monitoring during hot water immersion, we showed that epileptic discharges increased in frequency, and eventually developed into seizures at temperatures over 38 degrees C. We believe that the unique fever sensitivity observed in SME is similar to, but more intense than that of febrile convulsions. We have also identified a group of cases who have had innumerous myoclonic and atypical absence seizures daily which were sensitive to the constant bright light illumination. In these cases, spike discharges increased or decreased depending on the intensity of constant light illumination. Although these cases form the most resistant SME group, they lost the constant light sensitivity with increasing age, leaving only relatively common types of fever-sensitive grand mal seizures (FSGM) at the age of around 5 years. In the long run, only convulsive seizures continue, while myoclonic or absence seizures and photosensitivity disappear with advancing age, thus it is conceivable that SMEB constitutes a basic epileptic condition underlying SME. There is a clinical continuum that extends from the mildest end of SMEB to the severest end of SME with constant light sensitivity, with intermediates of frequent or infrequent myoclonic and absence seizures in-between. This spectrum concept appropriately explains the clinical variabilities between SME and SMEB during early childhood.
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PMID:Severe myoclonic epilepsy in infants--a review based on the Tokyo Women's Medical University series of 84 cases. 1170 Dec 88

A retrospective case note study of the aetiology and course of children in convulsive status epilepticus (CSE) admitted to a large paediatric intensive care unit (PICU) was undertaken between January 1999 and April 2004. Status epilepticus was defined as a prolonged (>30 min) tonic-clonic seizure irrespective of whether the seizure had stopped prior to admission to PICU. During this period, 137 (74 male) children aged 1 month to 15 years were admitted to PICU with 147 episodes of status epilepticus. Forty-seven of the 137 children (34%) were admitted following a prolonged febrile seizure. Thirty-eight of the 137 children (28%) had a remote symptomatic cause for the CSE, 24 (18%) were admitted for an acute symptomatic cause and 15 (11%) were admitted with an acute exacerbation of a pre-existing idiopathic/cryptogenic epilepsy. Six children had a progressive encephalopathy and no cause was identified in the remaining 7 of the 137 children (5%). Forty-nine (36%) of the 137 children had pre-existing epilepsy. The mean duration of CSE was 44 min. Forty-nine (36%) children admitted to PICU who had received a benzodiazepine with either phenobarbital or phenytoin, required further treatment to terminate the presenting episode of CSE. Forty-two of these 49 were treated with thiopentone anaesthesia and the remaining 7 with a continuous infusion of midazolam, successfully terminating status in all. No child died. Of the 70 children considered to be previously neurologically and developmentally normal prior to admission, only 1 child demonstrated a new gross neurological abnormality at the time of latest follow-up. Seven patients (5%) developed new or de novo epilepsy.
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PMID:Aetiology, course and outcome of children admitted to paediatric intensive care with convulsive status epilepticus: a retrospective 5-year review. 1729 36


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