1/122. apomorphine and levodopa challenge in patients with a focal midbrain lesion.Three patients who presented with parkinsonian signs resulting from a focal midbrain lesion are reported. In all patients parkinsonian features occurred acutely and improved following acute challenge with apomorphine but not with levodopa. Remission of parkinsonian signs occurred spontaneously to a different degree. Inconsistent clinical response following administration of levodopa has been well documented in patients with focal midbrain lesions associated with parkinsonian signs; however, the efficacy of apomorphine has not been tested before. Anatomic or etiologic features do not allow us to predict in which cases parkinsonian signs secondary to a midbrain lesion would respond to levodopa or to dopamine agonists. A trial with apomorphine is warranted in all such cases.- - - - - - - - - - ranking = 1keywords = focal (Clic here for more details about this article) |
2/122. Folinic acid-responsive neonatal seizures.We report three cases of folinic acid-responsive intractable neonatal seizures. All patients were born at term following normal gestation and delivery. In the first infant, seizures began on the 5th day of life and were unresponsive to phenobarbital, pyridoxine, and valproate, but stopped within 24 hours of initiation of folinic acid treatment at the age of 6 months. Her sibling had died at age 6 months with intractable seizures. In the second infant, seizures began in the 2nd hour of life. These were initially controlled with phenobarbital; however, at 3 months of age she developed status epilepticus refractory to anticonvulsants, steroids, and pyridoxine and she required repeated induction of pentobarbital coma. seizures stopped within 24 hours of starting folinic acid. seizures and encephalopathy were noted in the third infant on the 2nd day of life. These were controlled with phenobarbital, but at 8 weeks of age seizures recurred and were difficult to control despite the addition of phenytoin. Immediately after folinic acid was initiated the seizures stopped. Breakthrough seizures in all patients have responded to increases in folinic acid; two of the three remain on standard anticonvulsants. All patients have global developmental delay. Cranial magnetic resonance imaging in the second patient shows diffuse atrophy, and in the third patient shows increased signal on T2 images in the white matter of the frontal and parietal lobes. Analysis of cerebrospinal fluid from these patients using high-performance liquid chromatography with electrochemical detection has consistently revealed an as-yet unidentified compound, which can be used as a marker for this condition. We suggest that cerebrospinal fluid be analyzed for the presence of this compound and a trial of folinic acid be considered in neonates with unexplained early onset intractable seizures.- - - - - - - - - - ranking = 103.08685681029keywords = seizure (Clic here for more details about this article) |
3/122. Encephalopathy as the presenting symptom of Hashimoto's thyroiditis.In recent years, encephalopathy has increasingly been recognized as a complication of Hashimoto's thyroiditis. It can begin abruptly as a stroke-like event, acute seizures, or confusion, or as an insidious decline in cognitive function. Most reported cases have been on adult patients, although this encephalopathy does affect children as well. This form of encephalopathy should be considered in the differential diagnosis of children and adults with unexplained neurologic deterioration. We describe the case of a child in whom acute encephalopathy was the presenting symptom of Hashimoto's thyroiditis.- - - - - - - - - - ranking = 8.5905714008571keywords = seizure (Clic here for more details about this article) |
4/122. A case of atypical absence seizures induced by leuprolide acetate.We report a case of a 13-year-old female with atypical absence seizures induced by prolonged administration of long-acting leuprolide acetate (LA). This patient had brain involvement resulting from chemotherapy and radiotherapy for a medulloblastoma. At 13 years of age, administration of long-acting LA was started. After the third dose of long-acting LA, atypical absence seizures appeared. After discontinuing long-acting LA, the seizures stopped without administration of any antiepileptic drugs. However, 2 years, 6 months later, the same seizures again appeared. On the basis of the findings of endocrinologic investigations and the reported data of pharmacokinetics of LA, we speculate that her seizures were induced by LA and that the seizures were associated with the presence of brain damage in the patient. Care should therefore be taken when using long-acting LA or other gonadotropin-releasing hormone analogues for pediatric patients with diffuse brain damage.- - - - - - - - - - ranking = 85.905714008571keywords = seizure (Clic here for more details about this article) |
5/122. Ring chromosome 20 epilepsy syndrome in children: electroclinical features.Ring chromosome 20 mosaicism is associated with dysmorphic features, mental retardation, and intractable seizures, including recurrent episodes of nonconvulsive status epilepticus. The authors' findings in four children, all without dysmorphic features, indicate that mental deterioration and frequent subtle nocturnal frontal lobe seizures, associated with a characteristic EEG pattern, represent prominent additional clinical features not previously described in this syndrome. This emphasizes the importance of full-night video-EEG in children with frontal lobe seizures and cognitive deterioration.- - - - - - - - - - ranking = 164.47452617644keywords = epilepsy, seizure (Clic here for more details about this article) |
6/122. perfusion brain scintigraphy studies in infants and children with malformations of the vein of Galen.Cerebral perfusion brain scintigraphy obtained in six unselected patients (age newborn to 14 years) from among 50 children with vein of galen malformations was used in conjunction with magnetic resonance imaging to determine the basis of the neurological and cognitive abnormalities in patients with vein of galen malformations (VGMs). Five had a hemiparesis - persistent, transient, or alternating. Four were developmentally delayed. Two had so far been cognitively normal and acquired a neurological deficit, following an embolization procedure. The school age patient had a nonverbal learning disability. Three had epilepsy and/or an abnormal electroencephalogram. magnetic resonance imaging documented only the VGM, hydrocephalus and atrophy; one child with perinatal asphyxia had periventricular leukomalacia. perfusion brain scintigraphy was normal in two (a normal infant, and a toddler with a hemiparesis and aphasia). Abnormal findings included: left parietal hypoperfusion, fronto-temporal atrophy, patchy flow; left fronto-temporal hypoperfusion, left hemiatrophy, bilateral medial temporal hypoperfusion, right cerebellar hypoperfusion; right temporal hypoperfusion, patchy flow; right hemiatrophy, occipital hypoperfusion. perfusion brain scintigraphy findings correlated better with focal neurological and cognitive defects than did magnetic resonance imaging.- - - - - - - - - - ranking = 34.842369660135keywords = epilepsy, focal (Clic here for more details about this article) |
7/122. The use of a skill-based activity in therapeutic induction.This paper describes a hypnotherapeutic intervention for a brain damaged 36-year-old male who has suffered from asthma since infancy and seizure disorder from the age of eight. In early sessions it was discovered that conventional "passive-relaxation" induction techniques seemed to exacerbate certain disturbing somatic experiences, which he refers to as scary feelings. It was found that his performance of a previously learned skilled activity (the playing of the computer game Tetris) permitted the experience of a highly focused but relaxed state that was conducive to therapeutic interaction. This approach to induction bears similarity to "active-alert" procedures but may be more importantly related to Mihaly Csikszentmihalyi's principle of flow, in that it involves engagement in a subjectively meaningful, skill-based activity.- - - - - - - - - - ranking = 8.5905714008571keywords = seizure (Clic here for more details about this article) |
8/122. Operative results without invasive monitoring in patients with frontal lobe epileptogenic lesions.PURPOSE: To further explore the still controversial issues regarding whether all or most candidates for epilepsy surgery should be investigated preoperatively with invasive long-term video-EEG monitoring techniques (ILTVE). methods: We studied five patients with intractable seizures since early childhood using the same protocol: clinical evaluation, magnetic resonance imaging (MRI) with fluid-attenuated inversion recovery (FLAIR) sequences, long-term video-EEG (LTVE) monitoring with scalp electroencephalogram (EEG), interictal single photon emission computed tomography (SPECT), positron emission tomography (PET), and neuropsychological testing. The patients' seizures had clinical features suggesting a frontal lobe (FL) origin. MRI scans revealed focal cortical dysplasia (CD) in four patients and a probable gliotic lesion in the fifth. The findings in both PET and SPECT images were congruent with those of the MRI. scalp LTVE failed to localize the ictal onset, although the data exhibited features suggestive of both CDs and FL seizures. On the basis of these results, surgery was performed with intraoperative corticography, and the cortical area exhibiting the greatest degree of spiking was ablated. RESULTS: Histopathologic study of four of the resected specimens confirmed the presence of CD, whereas in the fifth, there were features consistent with a remote encephaloclastic lesion. There were no postoperative deficits. seizures in three of the patients were completely controlled at 2-3.5 years of follow-up; a fourth patient is still having a few seizures, which have required reinstitution of pharmacotherapy, and the fifth has obtained > or =70% control. All patients have had significant improvement in psychosocial measures. For comparison, five patients with generally similar clinical and neuroradiologic features to the previous group underwent preoperative ILTVE monitoring. The surgical outcomes between the two groups have not differed significantly. CONCLUSIONS: We conclude that patients with FL epilepsies may be able to undergo successful surgery without preoperative ILTVE monitoring, provided there is high concordance between neuroimaging tests (MRI, SPECT, PET) and the seizure phenotypes, even when routine EEGs and scalp LVTE fail to localize ictal onset unambiguously. The surgical outcomes of these patients generally paralleled those of the other subjects who also had FL epilepsy but who were operated on only after standard ILTVE monitoring.- - - - - - - - - - ranking = 112.47092965789keywords = epilepsy, seizure, focal (Clic here for more details about this article) |
9/122. Long-term results of functional hemispherectomy for intractable seizures.OBJECTIVE: From May 1989 to April 1997, functional hemispherectomy was performed in 8 cases of intractable seizures. We retrospectively analyzed our experience to evaluate the seizure control and complications of this surgical technique. methods: Following Dr. Rasmussen's model of functional hemispherectomy or performing a modification of this operation, we removed the sensorimotor cortex and temporal lobe associated with disconnection of the remaining portions of the frontal lobe and parieto-occipital lobe. RESULTS: All the patients were followed up for 3-11 years (mean 6.7 years). Satisfactory seizure control was obtained in all the cases. life quality improved and patients worked or studied well after the operations. No cases of superficial cerebral hemosiderosis were found. CONCLUSION: Modified functional hemispherectomy may allow the patients to lead more independent lives by leading to a cessation or reduced frequency of seizures.- - - - - - - - - - ranking = 68.724571206857keywords = seizure (Clic here for more details about this article) |
10/122. Dutch patients with progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome.PEHO syndrome (progressive encephalopathy, peripheral edema, hypsarrhythmia, and optic atrophy) is a neurodegenerative disorder first characterized in Finnish patients. Subsequent reports have occasionally identified cases of PEHO occurring in some other countries. We describe two Dutch children who represent the first reported cases with PEHO syndrome from western europe. Both of these children showed typical characteristics of the syndrome, including generalized hypotonia and edema of their faces and extremities, profound psychomotor retardation, progressive cerebellar atrophy, and severe epilepsy which initially started as infantile spasms. Our experience has shown that distinguishing cases with true PEHO from those with other, clinically similar disorders requires a firm demonstration of both the presence and the progress of typical neuroradiological findings. Our present cases suggest that 1) PEHO syndrome is not limited to Finnish heritage, and that 2) many more patients with PEHO could be identified with increased knowledge of the syndrome.- - - - - - - - - - ranking = 34.675702993468keywords = epilepsy (Clic here for more details about this article) |
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