1/5. Prolonged febrile convulsions and mesial temporal lobe epilepsy in an identical twin.The authors report a 30-year-old identical twin who had prolonged febrile convulsions (FC) at the age of 8 months, left mesial temporal lobe epilepsy beginning at the age of 2 years, and left mesial temporal lobe sclerosis (MTS). The unaffected twin had short FC and meningitis at the age of 4 years but remained seizure free. Thus, prolonged FC in children younger than 4 years may precipitate later development of MTS.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/5. From swelling to sclerosis: acute change in mesial hippocampus after prolonged febrile seizure.Mesial temporal sclerosis (MTS) has been linked to prolonged febrile seizures. The sequence of changes in the temporal lobe/hippocampus following prolonged febrile seizures and status epilepticus is beginning to be elucidated. We obtained repeated magnetic resonance imaging (MRI) volumetric analysis of the hippocampi in a 23-month-old boy after a prolonged focal febrile seizure. Three days after a prolonged left focal febrile seizure, brain MRI showed increased T2 weighted signal and increased volume (swelling) of the right hippocampus. Repeat MRI 2 months later demonstrated sclerosis of the right hippocampus. review of the literature shows four other children with prolonged focal seizures associated with the MRI sequence of temporal lobe swelling followed by sclerosis. All had left focal seizures followed by right MTS. Our patient demonstrates a shorter interval for the radiologic development of hippocampal sclerosis compared to other reports.- - - - - - - - - - ranking = 8keywords = sclerosis (Clic here for more details about this article) |
3/5. 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 = 5keywords = sclerosis (Clic here for more details about this article) |
4/5. Coexistence of focal and idiopathic generalized epilepsy in the same patient population.PURPOSE: To review the clinical, electrographic, radiological, and pathological findings of patients with coexistent idiopathic generalized and partial epilepsy syndromes. methods: We performed a medical record review and a phone interview with all patients hospitalized to the Cleveland Clinic epilepsy monitoring unit (EMU) between 1992 and 2002 who fulfilled clinical and EEG criteria of coexistent partial and generalized epilepsy syndromes. RESULTS: Seven patients were identified. Two (29%) were men with a mean age of 26 years. Four had a history of febrile seizures. family history was positive in five. Mean duration of the generalized epilepsy syndrome was 11 years, and of the focal epilepsy 18 years. An equal number of patients developed focal versus generalized epilepsy first. Interictal EEG activity was predominantly generalized. Four had video-EEG documentation of both types of seizures. In the rest, only focal seizures were recorded but interictal activity strongly suggested a coexistent generalized epilepsy. MRI showed hippocampal atrophy in all, and hippocampal dysplasia in three. Five patients had PET imaging, all with hypometabolism in areas corresponding to the ictal onset on EEG. Four patients underwent epilepsy surgery with good surgical outcome and pathological confirmation of hippocampal sclerosis in all. CONCLUSION: We found a 0.2% incidence of coexistent focal and primary generalized epilepsy. Febrile seizures and a positive family history were common. Good seizure control was achieved after temporal lobectomy, even when interictal generalized activity predominated.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/5. Postictal cerebral hemiatrophy: with a contribution to the problem of crossed cerebellar atrophy.A case is presented of a 4.5-year-old child who died 5 days after the onset of a continuous hemiconvulsion and compared with three cases of established cerebral hemiatrophy. The laminar necrosis in the first case was strikingly similar in severity and distribution with the laminar cell loss in the others, thus producing additional evidence in support of the postictal aetiology of diffuse cerebral hemiatrophy. Particular attention was focussed on the pontocerebellar lesions. Three types of lesions were observed in the ipsilateral nuclei pontis: (1) focal neuronal necrosis in the acute stage, (2) focal neuronal loss, presumably the end result of the preceding lesion and (3) transneuronal atrophy. The contralateral cerebellar hemisphere showed the following abnormalities, singly or in combination: (1) necrosis of purkinje cells in the acute stage, (2) loss of purkinje cells and lobular sclerosis in advanced cases and (3) reduction in bulk of the hemisphere due to lesions in the nuclei pontis and degeneration of pontocerebellar fibres. These findings of the so-called crossed cerebellar atrophy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |