1/9. Clinical and EEG analysis of initial status epilepticus during infancy in patients with mesial temporal lobe epilepsy.This study investigated the clinical and EEG characteristics of initial status epilepticus (SE) during infancy in patients with mesial temporal lobe epilepsy (MTLE). The subjects were six patients who had been brought to our emergency clinic and treated for their initial SE between 1977 and 1988, and later developed MTLE. We reviewed the medical records and laboratory findings at the time of the initial SE, and the clinical evolution up to the development of MTLE. The six patients included four females and two males. The initial SE developed at ages ranging from 7 months to 2 years and 9 months with a mean of 1 year and 2 months. These episodes were characterized by an elevated temperature of more than 38 degrees C (4/6 cases), clusters of prolonged seizures during one episode of SE (4/6 cases), long-lasting SE (120-380 min, mean 227 min, 6/6 cases), postictal prolonged loss of consciousness (median 5 h, 6/6 cases), and the presence of Todd's paralysis (3/6 cases). The lateralization of the ictal or postictal EEGs of the SE in five of the six cases was identical to that of the hippocampal atrophy later confirmed by MRI. Follow-up EEG examinations at a 6 month interval demonstrated temporal spike discharges appearing only after the onset of complex partial seizures. Two patients, who had no fever at the initial SE, were characterized by a very early appearance of epileptic EEG abnormality and a short interval between the initial SE and the development of complex partial seizures, suggesting that the SE was the first epileptic manifestation. The result of this study showed that SE progressing to MTLE tends to have complicated clinical manifestations characterized by clusters of unilateral or generalized SE followed by prolonged postictal unconsciousness, generalized clinical manifestations despite lateralized ictal EEG discharges, and the Todd's paresis in addition to the prolonged seizure duration.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/9. A family of generalized epilepsy with febrile seizures plus type 2-a new missense mutation of SCN1A found in the pedigree of several patients with complex febrile seizures.We report a family with complex febrile seizures (FS). The proband is a 15-year-old boy with seizures that persisted beyond 6 years of age. His father, aunt, and cousin also have the histories of FS until 8, 9, and 8 years old, respectively. A base substitution 5569G-->T of voltage-gated sodium channel alpha-1 subunit gene was found in dna derived from the affected members of this family.- - - - - - - - - - ranking = 2.5keywords = complex (Clic here for more details about this article) |
3/9. Hippocampal sclerosis in severe myoclonic epilepsy in infancy: a retrospective MRI study.PURPOSE: Severe myoclonic epilepsy in infancy (SMEI; Dravet's syndrome) is a malignant epilepsy syndrome characterized by early prolonged febrile convulsions (PFCs) with secondary psychomotor delay and a variety of therapy-resistant seizures. Although the initial symptoms are repeated PFCs, the MRI performed at the onset of disease shows no hippocampal structural abnormalities. We aimed to assess clinical and serial MRI data of patients with SMEI with a special attention to the temporomedial structures. To our knowledge, this is the first systematic MRI study in this disease. methods: Clinical and MRI data of all SMEI patients treated in our hospitals between 1996 and 2004 were reviewed. RESULTS: Twenty-eight MRIs from 14 children (one to four images/patient) were included. Age at disease onset was between 3 and 9 months; age at initial MRI was 5 months to 13 years. Ten of 14 patients showed hippocampal sclerosis (HS) during the course of the disease (nine unilateral, one bilateral). Six of these 10 had a normal initial MRI. Age at the first verified HS was between 14 months and 13 years. Neither complex partial seizures nor anterior temporal irritative zone was recorded in these children. CONCLUSIONS: After initially normal structures, in most patients with SMEI, HS develops several months or years after the first PFC. These data support the hypothesis that PFC might be responsible for HS, but other factors and individual sensitivity should play a role in this process.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
4/9. Convulsions with fever as a presenting feature of bacterial meningitis among preschool children in developing countries.The authors report 522 infants and young children aged between one month and six years who presented with convulsions and fever as emergencies in nigeria. 22 had bacterial meningitis, six of whom lacked the usual signs of meningitis. Although features of complex febrile convulsions were significantly associated with bacterial meningitis, it is concluded that, as an aid to the early diagnosis of bacterial meningitis, all preschool children convulsing with fever in developing countries should have a lumbar puncture. This may reduce the contribution of meningitis to chronic neurological disabilities. The necessity for such a policy is illustrated by a case report of a young infant with convulsions and fever caused by meningitis, seen at a general hospital.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
5/9. Periodic febrile confusion as a presentation of complex partial status epilepticus.A 75-year-old woman was evaluated for recurrent episodes of fever she experienced periodically every 4-5 weeks over the last 12 months, lasting 2-3 days each. The fever was associated with continuous complex partial seizures, paralleled the seizure activity and returned to normal after the seizures had ceased. The ictal EEG recordings showed rhythmic bitemporal 3-4 Hz activity; the interictal recordings showed a spike and wave discharge over the right fronto-temporal region. carbamazepine effectively controlled both the seizures and the fever; the latter was presumed to be an inherent manifestation of the seizure activity.- - - - - - - - - - ranking = 2.5keywords = complex (Clic here for more details about this article) |
6/9. Prolonged apnoea with severe arterial hypoxaemia resulting from complex partial seizures.Multi-channel tape-recordings of the EEG, breathing movements, arterial pulsed oxygen saturation and ECG were performed on a four-year-old child with a history of cyanotic episodes. These had started at nine months of age, resulted in cardiopulmonary resuscitation on unnumerable occasions, and were refractory to anti-epileptic medication. During each episode seizure activity appeared first, followed within a few seconds by sinus tachycardia, prolonged absence of inspiratory efforts and severe arterial hypoxaemia. As the seizure activity ended, breathing movements restarted and there was a gradual improvement in oxygenation.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
7/9. Benign epilepsy of children with complex partial seizures following febrile convulsions.Among the partial epilepsies with complex partial seizures (CPS) following febrile convulsions (FC), there is an idiopathic epilepsy with extremely benign outcome, characterized by: 1) no past history suggesting brain insult, no underlying brain lesions, no neurological abnormalities, no mental retardation; 2) a high incidence of a positive family history of FC or benign epilepsy; 3) no past history of prolonged febrile convulsions; 4) EEG spike foci other than anterior temporal ones; 5) CPS easily controlled with full recovery.- - - - - - - - - - ranking = 2.5keywords = complex (Clic here for more details about this article) |
8/9. Cerebral tuberculosis presenting as complex febrile convulsions.Complex febrile convulsions were the initial clinical manifestation of miliary tuberculosis in a 4-year-old immigrant girl. The cerebral lesions were visible only after contrast-enhanced cranial computed tomography (CT) while native CT scan as well as cell count and glucose concentration in the cerebrospinal fluid were normal. mycobacterium tuberculosis was cultured from gastric aspirate and liver biopsy tissue. Treatment with isoniazid and rifampin for 12 months, pyrazinamide for 9 months, and ethambutol for the initial 6 weeks resulted in resolution of the cerebral lesions but a retinal scar after granuloma formation in the right eye caused reduced visus. This case demonstrates the importance of thorough search for tuberculosis even in the absence of overt clinical pulmonary signs especially in high-risk individuals such as immigrants.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
9/9. Generalized epilepsy with febrile seizures plus. A genetic disorder with heterogeneous clinical phenotypes.The clinical and genetic relationships of febrile seizures and the generalized epilepsies are poorly understood. We ascertained a family with genealogical information in 2000 individuals where there was an unusual concentration of individuals with febrile seizures and generalized epilepsy in one part of the pedigree. We first clarified complex consanguineous relationships in earlier generations and then systematically studied the epilepsy phenotypes in affected individuals. In one branch (core family) 25 individuals over four generations were affected. The commonest phenotype, denoted as 'febrile seizures plus' (FS ), comprised childhood onset (median 1 year) of multiple febrile seizures, but unlike the typical febrile convulsion syndrome, attacks with fever continued beyond 6 years, or afebrile seizures occurred. seizures usually ceased by mid childhood (median 11 years). Other phenotypes included FS and absences, FS and myoclonic seizures, FS and atonic seizures, and the most severely affected individual had myoclonic-astatic epilepsy (MAE). The pattern of inheritance was autosomal dominant. The large variation in generalized epilepsy phenotypes was not explained by acquired factors. Analysis of this large family and critical review of the literature led to the concept of a genetic epilepsy syndrome termed generalized epilepsy with febrile seizures plus (GEFS ). GEFS has a spectrum of phenotypes including febrile seizures, FS and the less common MAE. Recognition of GEFS explains the epilepsy phenotypes of previously poorly understood benign childhood generalized epilepsies. In individual patients the inherited nature of GEFS may be overlooked. Molecular genetic study of such large families should allow identification of genes relevant to febrile seizures and generalized epilepsies.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |