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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Parry-Romberg syndrome (PRS) is a sporadic disease of unknown etiology with typical onset in childhood or in young adults. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. The neurological symptoms usually include focal epilepsy, migraine, and unilateral brain lesions on the same side as the atrophy. A common neuroimaging finding of the syndrome is white matter high signal intensity on brain magnetic resonance (MR) imaging. Rasmussen encephalitis (RE) is a rare and chronic inflammatory disease of the brain that begins in the first decade of life and more rarely in adolescents and adults. It usually involves one hemisphere with focal cortical inflammation. Neurologic symptoms are intractable seizures and progressive hemiplegia. Both PRS and RE are often associated with other inflammatory or autoimmune disorders and only 1 case of both syndromes has been reported in literature. We report the clinical and neuroradiological findings in a 6-year-old boy, presenting with focal hemifacial and arm motor seizures and progressive facial hemiatrophy. Serial MR imaging studies revealed progressive brain hemispheric signal alterations and atrophy. This would thus suggest acoexistence of PRS and RE.
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PMID:Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features. 1955 4

Rasmussen encephalitis (RE), initially described half a century ago, is an inflammatory unihemispheric brain disorder. Its two clinical key facets are the progressive tissue and function loss and the epilepsy, often in form of epilepsia partialis continua. For both, treatment options are available. Anti-seizure effect of anti-epilepsy drugs is usually limited to secondarily generalized seizures and complex partial seizures whereas epilepsia partialis continua usually is totally refractory. Hemispherectomy in one of its modern variants offers a very high chance of seizure freedom, however at the price of irreversible loss of functions located in the affected hemisphere. In a proportion of patients, long-term immunotherapy is able to prevent or slow down hemispheric tissue loss and the associated functional decline. It does, however, mostly not improve the epilepsy. Whereas for many patients unequivocal treatment proposals can be readily made, a dilemma may emerge in those with severe epilepsy but still preserved hemispheric function.
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PMID:Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. 1961 63

Epileptic seizures can be induced by a variety of underlying diseases. One of the causes is brain inflammation or encephalitis. In this review, we focus on three different kinds of encephalitis known to occur with seizures: Rasmussen encephalitis, paraneoplastic encephalitis, and nonparaneoplastic encephalitis. We describe pathogenic similarities and differences in the immunological response and cell death in the central nervous system of these three types of encephalitis and discuss the present ideas regarding the underlying etiology of these diseases.
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PMID:Encephalitis and epilepsy. 1976 76

Rasmussen encephalitis (RE) is characterized by chronic inflammation of one cerebral hemisphere which causes intractable epileptic seizures and progressive neurological deficits. Since antiepileptic pharmacotherapy is often ineffective the traditional therapy for Rasmussen encephalitis is hemispherectomy in one of its modern variants which renders the patient seizure free but leads to a severe deficit. To escape this dilemma, immunomodulatory therapeutic approaches such as rituximab, a monoclonal anti-CD20 antibody, offer an alternative and bear promising therapeutic potentials in Rasmussen encephalitis.
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PMID:New hope for Rasmussen encephalitis? 1983 59

Peri-insular hemispherotomy is a disconnective procedure that enables functional isolation of single or multiple epileptogenic regions largely involving one hemisphere. This report is based on a consecutive series of 13 pediatric patients affected by refractory hemispheric epilepsy studied and operated on at the Neurological Institute "C. Besta" of Milan. In all patients seizures were focal with polymorphic features and daily frequency. Preoperative electro-clinical and MRI data were suggestive of Rasmussen's encephalitis in 6 cases, hemimegalencephaly in 2, hemispheric cortical malformation in 3, perinatal stroke in 2 cases. Mean age at surgery was 7.3 years. The postoperative course was good for all patients. Mean follow-up period was 4.5 years (range 2-7 years). After hemispherotomy, 8 patients were Engel Class I and five continued to have seizures and were classified as Engel Class II (1 case) III (3 cases) or IV (1 case). The outcome results varied according to the etiology; the best results were achieved in patients with Rasmussen encephalitis and perinatal vascular strokes with 75% classified in Engel Class I. Three patients underwent a second surgery because of persistent seizures and evidence of incomplete disconnection; two out of three patients had hemimegalencephaly, and did not improve; the third patient with perinatal stroke resulted seizure free. We conclude that hemispherotomy can be performed successfully to treat medically intractable hemispheric epilepsy, providing remarkable results in terms of seizure outcome and quality of life.
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PMID:Hemispherotomy and functional hemispherectomy: indications and outcome. 1988 95

The aim of this study was to investigate the correlation between magnetic resonance imaging (MRI) and histopathologic findings in Rasmussen syndrome. Serial MRIs were obtained for five patients who had histologically proven Rasmussen syndrome. The histopathologic grades of the lesions were subdivided into phases: active 1-3, resolving 1-3, and chronic inflammatory. The images were then correlated with histopathologic findings. Neuropathologic findings in the central areas on MRI demonstrated only the chronic and resolving grades, but active inflammatory abnormalities were present not only at the margins of the lesions, but also in areas of subtle signal abnormality on MRI. Atrophic areas on MRI exhibited all grades of histopathologic abnormalities, but chronic and resolving grades were predominant. Seizure duration of less than 6 months was associated with very active grades, duration of 1-2 years with variable grades, and duration greater than 6 years with chronic and resolving grades only. The MRI images correlated highly with histopathologic analysis. These findings suggest that the lesions initially arise from one site in the brain, and so support the centrifugal spreading theory of this disease. Findings also suggest that the margin rather than the center of the MRI abnormality may be the most ideal site for biopsy.
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PMID:Correlation between magnetic resonance imaging and histopathologic grades in Rasmussen syndrome. 2015 25

Rasmussen syndrome is characterized by continuous partial seizures with progressive neurological/cognitive impairment. Currently the only effective treatment is surgery (hemispherectomy). The objective of our study is to detect the exact epileptogenic focus through the analysis of multimodal noninvasive and innocuous functional neuroimaging. The subject is a 5-year-old female patient with Rasmussen encephalopathy. Continuous and simultaneous electroencephalography-functional magnetic resonance imaging (EEG-fMRI) was recorded. The sources of background and paroxysmal activity of EEG were computed by low resolution electromagnetic tomography (LORETA). Image analysis (SPM: statistic parametric mapping) was obtained for the areas where statistically significant differences in the fMRI BOLD response were computed, and the results from both techniques were compared. The main source of paroxysmal activity by EEG analysis was found in the anterolateral left hemisphere, with a significant increase in absolute and relative energies of slow frequency bands (theta-delta): Z > or = 3. The fMRI BOLD signal (basal vs. paroxysmal activity) was significantly different in the same region (t-test > or = 2.39). The generators of propagated paroxysmal activity were found in similar areas for both techniques. In conclusion, simultaneous EEG-fMRI recording allows the analysis of two harmless functional neuroimaging techniques separately and together in the same time period. In our case, it allowed the accurate delineation of epileptogenic foci and areas of spread with high spatiotemporal resolution, which is crucial for epilepsy surgery.
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PMID:Noninvasive multimodal neuroimaging for Rasmussen encephalopathy surgery: simultaneous EEG-fMRI recording. 2072 52

Rasmussen encephalitis (RE) is a rare, chronic, inflammatory neurological disorder that usually affects one hemisphere of the brain. RE is characterized by frequent and severe seizures, progressive neurological dysfunction, and unilateral brain atrophy. This article documents a case of RE with bilateral involvement occurring at the age of 2. The patient's seizures consisted of focal motor seizures (epilepsia partialis continua in the fingers on the right hand); tonic convulsions of the right arm and head turning to the right with impairment of consciousness; and secondarily generalized tonic-clonic seizures. Antiepileptic drugs did not effectively control the seizures. The pathology of the left frontoparietal lobe biopsy of the abnormal MRI signal showed typical neuronal loss, abundant lymphocytic infiltration into the perivascular space, and numerous microglial nodules. Bipolar electrocoagulation on functional cortexes, which failed to alter the course of the illness, was followed by high-dose prednisone (about 2 weeks worth), without improvement. Both functional hemispherectomies and anatomical hemispherectomies failed to halt the course of the illness, and contralateral seizures developed and became apparent after the operation, suggesting bilateral RE.
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PMID:Bilateral Rasmussen encephalitis. 2121 75

To detect change in mental development or intelligence over two years following hemispherectomy in children with pharmacologically intractable epilepsy. Seventeen infants and preschoolers (median age at epilepsy onset of 0.0 years and at hemispherectomy 1.5 years; epilepsy duration of 0.2-2.6 years) and 12 older children (median age at onset of 1.0 year and at hemispherectomy 8.3 years; epilepsy duration of 1.1-11.7 years) with pharmacologically intractable seizures due to developmental, acquired or progressive pathology. Prospective study with consecutive inclusion of children, fixed assessment intervals (shortly before and 6, 12 and 24 months after hemispherectomy) and assessment using developmental scales and intelligence scales. Dependent variables included mental developmental index (MDI), mental age (MA) and mental developmental delay (MDD) in younger children and intelligence quotient (IQ) in older children. Mental development had arrested or deteriorated prior to hemispherectomy in 14 children (82%) assessed with developmental scales. In 14 children, it was not possible to more precisely determine MDI than "below the lowest MDI that the test manual provided" either before or after hemispherectomy. MA, however, increased in 16 children. Overall, IQ changed negligibly over two years after hemispherectomy, although an individual approach revealed variability. Children with Rasmussen encephalitis did not recover from the significant presurgical deterioration of intelligence. Removal of the affected hemisphere enables epileptic children, even those with severe mental delay, to further develop mentally.
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PMID:Hemispherectomy: a basis for mental development in children with epilepsy. 2139 89

Brain magnetic resonance imaging (MRI) studies in patients with Dravet syndrome and SCN1A mutations have shown abnormal findings in a small minority of patients. The origin of the structural abnormalities--such focal brain atrophy, cortical dysplasia, and hippocampal sclerosis--observed in some children remains unclear. There seems to be no correlation between the presence of MRI abnormalities and duration of epilepsy, age at seizure onset, or the frequency of episodes of status epilepticus having occurred early in life. Recent descriptions of Rasmussen syndrome and of the hemiconvulsion-hemiplegia syndrome in isolated patients with SCN1A mutations are of uncertain meaning but might indicate that co-occurring immunomediated or seizure-induced structural changes can, in turn, become a substrate for the severe epileptic encephalopathy. The few available neuropathologic studies of Dravet syndrome have provided inconsistent findings, including evidence of subtle brain malformation. However, the underlying dysfunction of the SCN1A gene might confer to the brain a unique profile of vulnerability whose consequences are not easily disclosed by neuropathology and require specific experimental settings to be fully appreciated. There would seem to be value in studies in animal models of these aspects, as well as prospective studies in humans, with a particular view to establishing if earlier diagnosis and efforts at seizure control may influence the development of any clinical, imaging, or pathologic deterioration.
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PMID:Neuroimaging and neuropathology of Dravet syndrome. 2146 76


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