Cases reported "Seizures, Febrile"

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1/21. Hemiconvulsion-hemiplegia-epilepsy syndrome: characteristic early magnetic resonance imaging findings.

    We report three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and were shown to have striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere, including extensive diffusion-weighted imaging abnormalities in two cases. Two patients subsequently developed progressive and extensive atrophy of the involved hemisphere. These findings are consistent with earlier descriptions of the classic neuroradiologic features of this syndrome and are helpful in the differential diagnosis of acute infantile hemiplegia. Further, the findings support the previously proposed pathogenetic mechanism of neuronal injury caused by status epilepticus.
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ranking = 1
keywords = status epilepticus, epilepticus, status
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2/21. 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.
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ranking = 5.0000478495872
keywords = status epilepticus, epilepticus, status, complex
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3/21. 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.
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ranking = 1
keywords = status epilepticus, epilepticus, status
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4/21. Uncommon complication of herpes simplex encephalitis.

    A 9-year-old-girl who had herpes simplex encephalitis developed impending uncal herniation requiring surgical decompression. This case highlights the development of an uncommon complication despite the early initiation of treatment with acyclovir.
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ranking = 0.00013988719650634
keywords = simple
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5/21. 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.
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ranking = 0.00011962396793983
keywords = complex
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6/21. 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.
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ranking = 2.3924793587966E-5
keywords = complex
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7/21. 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.
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ranking = 2.3924793587966E-5
keywords = complex
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8/21. A missense mutation in SCN1A in brothers with severe myoclonic epilepsy in infancy (SMEI) inherited from a father with febrile seizures.

    Severe myoclonic epilepsy in infancy (SMEI) is an age-dependent epileptic encephalopathy occurring in the first year of life and is one of the intractable epilepsies. Heterozygous mutations in the voltage-gated sodium channel alpha subunit type1 gene (SCN1A) are frequently identified in patients with SMEI; two-thirds of these mutations are truncation mutations (non-sense and frameshift), and one-third are missense mutations. Although most reported SMEI cases arise as sporadic mutations, close relatives of SMEI patients have also been shown to manifest other types of epilepsies at a higher rate than that in the general population. Here, we report a familial case of SMEI, in which two brothers were affected with SMEI while their father had previously experienced simple febrile seizures. A gene-based analysis identified a novel missense mutation in the SCN1A gene (c.5138G>A, S1713N) in both brothers and in their father. Clinically, both siblings showed failure in locomotion, an impairment of the sleep-wake cycle after late infancy, and the subsequent appearance of frontal foci. The similarity in clinical manifestations in both brothers suggests that the impairment of elements of the brainstem, particularly aminergic neurons, develops after late infancy in SMEI. However, the siblings differed in age at onset of SMEI and of myoclonic seizures, as well as in the severity of speech delay. Our molecular and clinical findings suggest that different genetic backgrounds and/or environmental factors may critically affect the clinical features of patients with SCN1A mutations, consistent with the heterogeneity prevalent in this disorder.
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ranking = 2.7977439301268E-5
keywords = simple
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9/21. Beta-ureidopropionase deficiency presenting with febrile status epilepticus.

    Beta-ureidopropionase is the third enzyme in the catabolic pathway of uracil and thymine. To date, only three other patients are reported with this inborn error of metabolism. We report the clinical presentation of a male patient who presented at the age of 4 months after an ALTE-like event (ALTE = acute life-threatening event) with febrile status epilepticus. Such a clinical presentation has not been reported before in this condition. Diagnosis was based on biochemical, enzymatic and molecular studies. MRI (magnetic resonance imaging) at the age of 11 months demonstrated large subdural hematomata and global supratentorial atrophy. At that time the patient showed severe psychomotor retardation with muscular hypotonia, extremely limited visual contact and poorly controlled epilepsy. CONCLUSIONS: Pyrimidine degradation defects should be included in the differential diagnosis of convulsions, (febrile) status epilepticus, psychomotor retardation and possibly also ALTE-like events.
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ranking = 6
keywords = status epilepticus, epilepticus, status
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10/21. Low frequency electrical stimulation through subdural electrodes in a case of refractory status epilepticus.

    OBJECTIVE: We delivered low frequency stimulation through subdural electrodes to suppress seizures in a case of refractory status epilepticus (RSE). methods: A 26-year-old female developed RSE after several days of febrile illness. Seizure control required continuous infusion of two anesthetics plus high doses of 2-4 enteral antiepileptic drugs. After 3 months of RSE, subdural strips were placed to determine surgical candidacy. Five independent ictal onset zones were identified. Because she was a poor candidate for epilepsy surgery and had a poor prognosis, the implanted subdural electrodes were used to administer 0.5 Hz stimulations to the ictal onset zones in 30 min trains daily for 7 consecutive days in an attempt to suppress seizures. RESULTS: After 1 day of stimulation, one anesthetic agent was successfully discontinued. seizures only returned by the 4th day when the second anesthetic had been reduced by 60%. Upon returning, seizures arose from only one of the 5 original ictal onset zones. Unfortunately, RSE persisted, and she eventually died. CONCLUSIONS: In this case of RSE, low frequency stimulation through subdural electrodes transiently suppressed seizures from all but one ictal onset zone and allowed significant reduction in seizure medication. SIGNIFICANCE: Low frequency cortical stimulation may be useful in suppressing seizures.
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ranking = 5
keywords = status epilepticus, epilepticus, status
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