Síndrome de Rasmussen

Caraballo, Roberto; Tenembaum, Sílvia; Cersósimo, Ricardo; Pomata, Hugo; Medina, Carlos Alberto Hernández; Soprano, Ana María; Fejerman, Natalio

doi:10.33588/rn.26154.98701

Cited by 8 publications

(12 citation statements)

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“…These findings (absolute and relative increases in theta-delta predominantly in one hemisphere) may be in correspondence with the anatomopathological changes that charac terize Rasmussen encephalopathy: inflammation and progressive unilateral cerebral necrosis (progressive hemiatrophy), with neuronal loss and gliosis. 3,23 Generally these disorders affect one hemisphere, in our patient the left hemisphere was the most damaged, mainly centrotemporal regions (reported as the most affected areas in the international literature). These results were reflected in the qEEG analysis by an increase in delta frequencies.…”

Section: Discussionmentioning

confidence: 52%

“…Age of onset, neurological and cognitive evidence of deterioration, hemiparesis and intellectual impairment; supported the diagnosis. [1][2][3] Structural MRI study was normal, but EEG findings supported the diagnosis. As described in the literature, polymorphism in the EEG tracing is typical of this entity.…”

Section: Discussionmentioning

confidence: 94%

“…As described in the literature, polymorphism in the EEG tracing is typical of this entity. 2,3 Presence of a single focus of paroxysmal activity indicates a better prognosis for surgery (frequently in central and medial temporal regions), or there may be multifocal discharges that usually affect one hemisphere. With progression of the disease, although less prominent, epileptic discharges may be seen in the healthy hemisphere.…”

Section: Discussionmentioning

confidence: 99%

“…Nowadays surgery (hemispher ectomy) is the most effective treatment to control convulsions and progressive worsening. [1][2][3] Epilepsy surgery requires accurate localization of the epileptogenic focus or area. For this purpose different neuroimaging techniques (anatomical and functional) have been used.…”

Section: Introductionmentioning

confidence: 99%

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Noninvasive Multimodal Neuroimaging for Rasmussen Encephalopathy Surgery: Simultaneous EEG-fMRI Recording

Bravo¹,

Iturria

Praderes

et al. 2010

Clin EEG Neurosci

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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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Section: Discussionmentioning

confidence: 52%

Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Noninvasive Multimodal Neuroimaging for Rasmussen Encephalopathy Surgery: Simultaneous EEG-fMRI Recording

Bravo¹,

Iturria

Praderes

et al. 2010

Clin EEG Neurosci

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“…Despite the uncertainties surrounding the presence or absence of antibodies to GluR3, 50 patients have thus far been reported in whom efforts were made to treat Rasmussen's encephalitis with immunomodulatory therapy using plasma exchange, intravenous IgG, and, in a few patients, cyclophosphamide or other immunosuppressive agents. [69][70][71][75][76][77][78][79][80] Of these 50 patients, improvement was stated to be sustained in only 7. Five patients treated with corticosteroids by Chinchilla et al developed sustained improvement, 76 and two patients treated with monthly intravenous IgG by Leach et al developed sustained improvement after several months of treatment.…”

Section: Rasmussen's Encephalitis: Is It An Infectious Disease An Aumentioning

confidence: 99%

Controversies in Neurological Infectious Diseases

Greenlee

Rose²

2000

Semin Neurol

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The past several years have seen major advances in our understanding of neurological infectious diseases, their diagnosis, and their treatment. Along with these advances, however, new information about infectious agents and new therapeutic options have also introduced both uncertainty and controversy in the approach and management of patients with diseases of the central nervous system. Here, we discuss six such areas: the long-term efficacy of HAART therapy in treatment of HIV infection; the role of viral infection in chronic fatigue syndrome; Rasmussen's encephalitis as an infectious or autoimmune disease; the spectrum of neurological diseases caused by rickettsial infection; the role of Mycoplasma pneumoniae in human central nervous system disease; and the possible association of Chlamydia pneumoniae and human herpesvirus 6 with multiple sclerosis.

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