1/159. Improvement in adult-onset Rasmussen's encephalitis with long-term immunomodulatory therapy.OBJECTIVE: To study the immediate and chronic effects of high-dose, long-term human i.v. immunoglobulin (h i.v.Ig) therapy in two patients with advanced adult-onset Rasmussen's encephalitis (RE). BACKGROUND: Despite advances in our understanding of the autoimmune pathogenesis of RE, medical options for chronic treatment are limited. methods: In an open-label treatment trial, treatment started with monthly cycles of high-dose h i.v.Ig (0.4 g/kg/d for 5 days) followed by maintenance therapy (0.4 g/kg 1 day each month) after the patients' conditions began to improve. Outcome measures included clinical, psychological, functional, and laboratory assessments before and at relevant intervals throughout 1 year of treatment. RESULTS: In both patients, unrelenting pretreatment deterioration halted, and after this they displayed striking improvements in seizure control, hemiparesis, and cognition that produced useful recovery of function. Improvements were delayed until after 2 to 4 monthly cycles of high-dose h i.v.Ig and continued when patients switched to maintenance treatment. Their recoveries were accompanied by increased cerebral perfusion on interictal SPECT and suppression of inflammatory markers in CSF. CONCLUSIONS: h i.v.Ig can be a useful, possibly disease-modifying, long-term therapy for adult-onset RE that should be considered before radical surgery is performed. Because improvements can be delayed, we propose guidelines for intensive and prolonged trials of immunomodulatory therapy in adults with this syndrome.- - - - - - - - - - ranking = 1keywords = seizure (Clic here for more details about this article) |
2/159. plasma exchange in Rasmussen's encephalitis.The authors observed a 4-year-old girl who has Rasmussen's encephalitis. She started with frequent localized and generalized seizures. Standard antiepileptic treatment was almost ineffective. The frequency of the generalized seizures decreased, but the myoclonic jerks of the left part of the body persisted. An EEG showed partial status epilepticus. The results of the CT scan were normal. antibodies to viruses were absent from the blood and cerebrospinal fluid. An MR scan showed a T2-weighted hypersignal zone in the right frontal region. Intravenous bolus injections of corticosteroids and drips of immunoglobulins were inefficient, and we started plasma exchanges which have continued for 9 months. The clinical state stabilized, and the images on the MR scan improved, but the results of the EEG did not improve. The authors discuss the effect of the plasma exchange, the use of which is questionable in this disease.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
3/159. Reproducible localization of interictal epileptiform discharges using EEG-triggered fMRI.We report preliminary experiences using fMRI triggered by EEG to localize the site of interictal epileptiform activity. EEG was recorded in the scanner and monitored on-line; the recording quality was good enough to allow the clear identification of spikes in the EEG. Snap-shot EPI was performed 2-4 s after an epileptiform discharge ('spike') or after at least 10 s of background activity ('rest') was observed. A pixel-by-pixel t-test was performed between the 'rest' and the 'spike' images to determine areas of significant activation. Significant activation was obtained in a patient with epilepsy. To assess the reliability and reproducibility of the technique, the patient was scanned on four separate occasions with similar areas being activated in all the studies, confirming the validity of the result.- - - - - - - - - - ranking = 0.27987739905623keywords = epilepsy (Clic here for more details about this article) |
4/159. Rasmussen encephalitis in childhood.Six patients admitted to the Department of Pediatric neurology at Hacettepe University Children's Hospital between 1992 and 1997 with a clinical diagnosis of Rasmussen encephalitis received surgical treatment for their intractable epilepsy. MRI, SPECT and WADA tests were performed in patients with an epileptic focus demonstrated on routine or long-term video EEG monitoring. Viral studies using the PCR methodology were performed in cases with histopathological evidence of Rasmussen encephalitis. The ages of these patients ranged between 7 and 16 years, and the mean age at onset of seizures was 7.1 /-2.2 years. In four patients seizures presented as epilepsia partialis continua and were refractory to anticonvulsive drug therapy. In three cases intravenous immunoglobulin therapy yielded temporary and partial improvement in seizure control. The mean presurgical follow-up duration was 2.04 1.74 years, and early surgical intervention for epilepsy was performed in one case. The surgical approach selected for the treatment of epilepsy was resective surgery with electrocorticography. The mean postoperative follow-up duration was 32.3 17.2 months. seizures were fully controlled in one patient, in whom surgery was performed 3 months after the seizures first started. Early surgical intervention may provide histopathological evidence for diagnosis as well as effective seizure control.- - - - - - - - - - ranking = 5.8396321971687keywords = seizure, epilepsy (Clic here for more details about this article) |
5/159. Coexistence of a ganglioglioma and Rasmussen's encephalitis.A small number of recent reports have documented coexisting cerebral pathologic entities in patients with Rasmussen's encephalitis. We report the case of a 4-year-old boy who presented with refractory seizures and was found to have both a ganglioglioma and Rasmussen's encephalitis of the same hemisphere. The patient ultimately underwent a functional hemispherectomy with excellent seizure control. We also review the clinical and pathologic hallmarks of both gangliogliomas and Rasmussen's encephalitis. We hope that the addition of this patient to the small but growing literature on dual pathology in Rasmussen's encephalitis may help shed some light on the etiology of this mysterious disease.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
6/159. Activation of the left motor cortex during left leg movements after right central resection.A patient with Rasmussen's encephalitis underwent a right central resection at the age of 6 as a treatment for status epilepticus. She became seizure free, but suffered a left hemiplegia which improved so that she could walk. Because of the recurrence of seizures an enlargement of the resection to a hemispherectomy was carried out 17 years after the first operation. Various examinations, including H(2)(15)O PET and amytal testing, performed before this second operation indicated that a compensatory reinforcement of the ipsilateral uncrossed corticospinal and spinocortical pathways had taken place. This was confirmed postoperatively. The patient had no new sensorimotor deficits.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
7/159. Unusual presentation and MRI findings in Rasmussen's syndrome.Rasmussen's syndrome is a chronic disorder characterized by uncontrollable focal seizures and eventually epilepsia partialis continua, ipsilateral hemiparesis, developmental arrest, and cerebral inflammation. Viral and autoimmune etiologies have been postulated. A patient is presented who illustrates the wide variability of clinical and radiographic presentations in this disorder. The patient is an 8-year-old female who developed intermittent facial twitching at 2 years of age that eventually progressed to epilepsia partialis continua. electroencephalography demonstrated clinical seizures that emanated from the right parasagittal area. Cranial magnetic resonance imaging revealed pronounced atrophy of the right caudate nucleus, globus pallidus, and putamen, with mild increased T2-weighted signal in the right striatum, without accompanying cortical atrophy. Ictal single-photon emission computed tomography revealed markedly reduced uptake in the right hemisphere that was maximum in the right basal ganglia. cerebrospinal fluid, blood, and urine collected for metabolic and immunologic screening and dna testing for a wide variety of disorders were all unremarkable. Neuropsychologic testing demonstrated difficulties in memory, attention, and calculation. Brain biopsy revealed mild microglial activation, rare glial nodules, and collections of lymphocytes and histiocytes, consistent with the clinical diagnosis of Rasmussen's syndrome. After a modified hemispherectomy, she demonstrated marked clinical improvement.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
8/159. [11C](R)-PK11195 positron emission tomography imaging of activated microglia in vivo in Rasmussen's encephalitis.This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.- - - - - - - - - - ranking = 1keywords = seizure (Clic here for more details about this article) |
9/159. Potential hepatotoxicity of lamotrigine.Lamotrigine is a new antiepileptic drug that is effective for a broad range of seizures in adults and children. Three children with seizures of different causes who were treated with lamotrigine and developed reversible hepatotoxicity are reported. In one child, this therapy led to relatively severe hepatic failure that required and responded to aggressive therapy. Unlike most of the previously reported six patients with similar severe hepatic involvement, this patient's liver function and blood hepatic enzymes became normal. All three patients were on multiple drugs, and two were in epilepsia partialis continua secondary to encephalitis. Two of the patients had relatively rapid medication titration schedules. The close time relationship between the initiation of the lamotrigine therapy and the reversal of the liver abnormalities with lamotrigine discontinuation argues against a cause other than the lamotrigine; however, because of the complexity of the reported cases, the causality remains an assumption. review of the literature revealed six other previously reported patients (five adults and one child) who had hepatotoxicity during lamotrigine therapy, with or without associated multisystem failure, and similar patient profiles. Lamotrigine is generally a safe and effective medication; however, it should be used with caution in patients on polytherapy and in those with complicated acute systemic and central nervous system conditions, such as fever, status epilepticus, epilepsia partialis, and encephalitis.- - - - - - - - - - ranking = 2keywords = seizure (Clic here for more details about this article) |
10/159. Unusual encephalitic illnesses in a child with acute leukaemia in remission: possible role of measles virus and toxoplasma gondii.We report a girl who developed an encephalitic illness with visual loss after two years treatment for acute lymphoblastic leukaemia. The visual loss was found to be due to bilateral macular degeneration. She later developed radiological evidence of intracranial calcification and temporal lobe epilepsy. A second episode of encephalitis occurred when she had been off all antileukaemic treatment for three years and this left her with a right hemiparesis. Investigation suggested involvement with both measles virus and toxoplasma gondii as a cause for these illnesses.- - - - - - - - - - ranking = 0.27987739905623keywords = epilepsy (Clic here for more details about this article) |
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