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Query: UMLS:C0014547 (focal epilepsy)
 1,627 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To study the pathophysiologic mechanism of astatic seizures in a patient with myoclonic astatic epilepsy of early childhood, ictal magnetoencephalography was recorded and the neuronal pathway involved was analyzed. The patient was a 12-year-old female who developed myoclonic and astatic seizures including nodding and sudden falling at the age of 4. The current source of spikes during nodding attacks was located in the bilateral frontal area with left predominance, possibly in the premotor cortex. Although we could not claim, on the basis of our findings, that myoclonic astatic epilepsy of early childhood is a type of focal epilepsy, it seems likely that the premotor cortex might be more excitable than other areas. Thus we speculate that the functionally altered premotor-reticulospinal tract which normally controls postural adjustment might play an important role in the generation of myoclonic astatic seizures. Furthermore, the underlying mechanism in the brainstem seems to be common, at least in part, for infantile spasms when considering the efficacy of synthetic adrenocorticotropic hormone for nodding seizures.
Pediatr Neurol 2004 Sep
PMID:A magnetoencephalographic study of astatic seizure in myoclonic astatic epilepsy. 1535 Oct 21

The neurocutaneous melanosis (NCM) is a rare, neuroectodermal dysplasia defined by the association of giant or multiple, nonmalignant pigmented cutaneous nevi with leptomeningeal melanosis or melanoma. As a rule, the cerebral pathological substratum is characterized by a melanocytic infiltration of the leptomeninges, often leading to hydrocephalus. The most frequent clinical symptomatology starts early in life, with convulsive seizures, psychomotor delay, intracranial hyperpression: the prognosis is severe. Malignant melanomas can also occur. One 21 years-old patient affected by NCM with a giant bathing nevus and epilepsy is reported. Her psychomotor development was slightly delayed. Academic progress was disturbed by the frequency of seizures and the multiple dermatological surgeries, and she remained at the elementary school level. Her epilepsy appeared at seven years and became pharmacoresistant. It was a focal, left temporal epilepsy. Neuroimaging investigations were performed repeatedly, and demonstrated the progressive appearance of parenchymal lesions with T1 and T2 shortening, without contrast enhancement, at the pons (11 years), the two hippocampi (14 years), and of an atrophy of the cerebellum and the brainstem (19 years). No hydrocephalus, tumoral aspect, or meningeal involvement were demonstrated. This patient's case is peculiar because her neurological symptomatology consists only of focal epilepsy, unrelated to a tumor, with moderate cognitive impairment despite a rather long course of the disease. Her evolution raises the question of condidency to surgical treatment.
Epileptic Disord 2004 Sep
PMID:An unusual case of neurocutaneous melanosis. 1574 Nov 43

Stuttering is characterized by involuntary syllabic repetitions and interruption in the smooth flow of speech. The exact cause of primary stuttering remains a matter of debate but a frontal dysfunction has been evoked. On the other hand, acquired stuttering is uncommon. We report a case of reflex epilepsy in which seizures were triggered by reading aloud or stressful conversation. Each paroxysmal event in left frontal region was associated clinically with a language disorder mimicking stuttering. Our observation suggests that reflex frontal focal epilepsy could be a putative etiology for acquired stuttering.
Epileptic Disord 2004 Sep
PMID:Stuttering or reflex seizure? A case report. 1550 17

Topiramate was assessed in an open-label trial as broad-spectrum antiepileptic monotherapy, independently from the epilepsy type or syndrome. Adults and children aged 2 years and older, who were diagnosed with epilepsy within the last 5 years, treatment-naive or failing prior treatment with one antiepileptic drug (AED), received individually adjusted doses of topiramate, after escalation to 100mg/day over 4 weeks (maximum 400mg/day) or 3mg/kg/day over 6 weeks (maximum 9 mg/kg/day), respectively. Patients were followed for >or=7 months and optionally up to a maximum of 13 months. Data were analysed for all patients (n=692), as well as for focal (n=421) and generalized epilepsies (n=148). The median topiramate dose used was 125 mg/day in adults and 3.3mg/kg/day in children (<or=12 years). Overall, 80% of patients completed the 7-month study. During this period, 44.3% were seizure-free, while 76.3% achieved >or=50% reduction in mean monthly seizure frequency. Patients with focal and generalized epilepsies alike responded to treatment (73.9 and 83.8% with at least 50% seizure reduction): 39.4% of patients with focal epilepsy and 61.5% of those with generalized epilepsy were seizure-free. The mean monthly seizure frequency was significantly reduced versus baseline at all visits (p<0.001). Similar response rates were obtained from the 237 patients completing the 1-year observation period. During the mandatory 7-month period of study, 8.8% of patients reported insufficient tolerability as a reason for dropout. The most frequent adverse event was paraesthesia. Our results support findings that emerge from controlled studies that topiramate is effective and well tolerated when used as initial or second monotherapy. They also suggest that in a naturalistic setting, overall good retention on treatment and seizure freedom are observed at low doses in a broad spectrum of epilepsies.
Seizure 2005 Sep
PMID:Topiramate monotherapy as broad-spectrum antiepileptic drug in a naturalistic clinical setting. 1596 26

The therapy of focal epilepsy remains inadequate. Many patients who have localization-related seizures find themselves either overmedicated with anticonvulsants or suffering from frequent seizures. While surgical resection can lead to excellent outcomes in up to 60% of patients with neocortical epilepsy, there are obviously many who either fail surgery or are deemed inappropriate surgical candidates. We are currently determining the efficacy of local cooling for the therapy of certain focal epilepsies. We have attempted to adapt new technologies borrowed from electrical and mechanical engineering to develop cooling devices that will ultimately improve the diagnosis and therapy of these focal epilepsies. The present review describes the rationale for this research and our progress to date.
Epilepsy Behav 2005 Sep
PMID:Focal cooling for epilepsy: an alternative therapy that might actually work. 1604 77

In this report a patient with episodic depersonalization is described. As the depersonalization episodes had been attributed to partial seizures, this patient was treated with antiepileptic medication. However, clinical evaluation with long-term video/EEG revealed no evidence of seizure activity during the depersonalization episodes. On the other hand, further evaluation revealed findings that are frequently associated with focal epilepsy. In addition to episodic depersonalization, this patient had secondary generalized seizures. The relationship between episodic depersonalization, temporal lobe pathology, and epilepsy is discussed against the background of this case.
Epilepsy Behav 2005 Sep
PMID:Episodic depersonalization in focal epilepsy. 1604 78

Using the wavelet mapping of sleep spindles we investigated influence of focal epilepsy on spindle generation. We found that the maximum of sleep spindle intensity is usually localized away from the epileptic focus. We discuss the possibility of the application of wavelet mapping for localization of epileptic foci prior to epileptic neurosurgery.
J Physiol Pharmacol 2005 Sep
PMID:Wavelet mapping of sleep spindles in young patients with epilepsy. 1620 72

Mental retardation, facial dysmorphisms, seizures, and brain abnormalities are features of 6q terminal deletions. We have ascertained five patients with 6q subtelomere deletions (four de novo, one as a result of an unbalanced translocation) and determined the size of the deletion ranging from 3 to 13 Mb. Our patients showed a recognizable phenotype including mental retardation, characteristic facial appearance, and a distinctive clinico-neuroradiological picture. Focal epilepsy with consistent electroencephalographic features and with certain brain anomalies on neuroimaging studies should suggest 6q terminal deletion. The awareness of the distinctive clinical picture will help in the diagnosis of this chromosomal abnormality.
Am J Med Genet A 2006 Sep 15
PMID:Clinical phenotype and molecular characterization of 6q terminal deletion syndrome: Five new cases. 1690 58

There is currently increasing interest in identifying and classifying pediatric benign epilepsy syndromes and recently several new syndromes have been recognized. Benign epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to therapy, and remit without sequelae. The large majority of children with benign epilepsy syndromes follow a truly benign course. The concept of benign epilepsy syndromes has, however, been challenged by the minority of patients who continue to have seizures despite therapy, develop new seizures after initial remission, or exhibit neuropsychological abnormalities. Without long-term follow-up, benignity can not be truly ascertained a priori. Thus it may be preferable to use the terms possible and probable before the name of a specific syndrome until such time that the diagnosis of a definite benign syndrome is confirmed on long-term follow-up. In this review of the pediatric benign localization-related epilepsy syndromes, we address the concept of benignity and the process of diagnosis of a benign epilepsy syndrome. In addition we review the epidemiology, clinical manifestations, EEG findings, work-up, diagnostic criteria, differential diagnosis, genetics, management and prognosis of benign infantile familial convulsions, benign partial epilepsy in infancy with complex partial seizures, benign partial epilepsy in infancy with secondarily generalized seizures, benign infantile convulsions associated with mild gastroenteritis, and benign infantile focal epilepsy with midline spikes and waves during sleep.
Epileptic Disord 2006 Sep
PMID:Benign pediatric localization-related epilepsies. Part I. Syndromes in infancy. 1698 37

The objective of this study was to evaluate the safety and efficacy of clobazam in children with refractory focal epilepsy. We investigated 100 consecutive patients concerning etiology of epilepsy, previously used antiepileptic drugs, seizure frequency and adverse events. Clobazam was introduced as add-on therapy in patients with previous failure of at least two monotherapies. Mean age was eight years-old and 39 patients were girls. Clobazam mean dosage was 23.6 mg/day. Mean use of clobazam was 18.6 months. Twenty-two patients had adverse events. Twenty-six patients became seizure-free, 11 had an improvement of >75% and in 58 there was no modification in seizure frequency. Five patients had an increase in seizure frequency. Clobazam efficacy lasted for more than one year in 42% of the seizure-free patients. Clobazam seems to be safe and effective in the treatment of focal epilepsy in childhood and should be considered in patients with refractory seizures.
Arq Neuropsiquiatr 2006 Sep
PMID:Effectiveness of clobazam as add-on therapy in children with refractory focal epilepsy. 1705 71


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