A Case of Rasmussen Encephalitis: The Differential Diagnoses and Role of Diagnostic Imaging

Varghese, Binoj; Aneesh, M K; Singh, Navdeep; Gilwaz, Percival

doi:10.5001/omj.2014.15

Cited by 13 publications

(9 citation statements)

References 11 publications

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“…Subsequent visual inspection of 3D FLAIR images (Figure 1) verified left-hemispheric atrophy in the frontal regions (co-authors: MT, SC, and SK). Further, in agreement with the observation that stage 4 encephalitis occurs approximately 8 years after seizure onset and leads to hemispheric atrophy (Varghese et al, 2014), the brain regional volume in our patient, who had experienced seizures for 15 years, showed that the left anterior frontotemporal region was the most affected area (volume of atrophy/damage: 15.13 cm 3 , mean ± standard deviation: 248.05 ± 126.34). The volume of atrophy was estimated using OsiriX Lite (v.10.0.3, Switzerland) 3D medical image processing software.…”

Section: Left-hemisphere Atrophy and Left-lateralized Atrophy In Igt supporting

confidence: 92%

Functional Cerebral Specialization and Decision Making in the Iowa Gambling Task: A Single-Case Study of Left-Hemispheric Atrophy and Hemispherotomy

et al. 2020

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The Iowa Gambling Task (IGT) is a decision-making task that preferentially involves the right prefrontal cortex (PFC). However, the performance of the task is driven by two attributes: intertemporal (long vs. short-term) and frequency-based processing of rewards-punishments, and differs over the two phases of uncertainty (early trials) and risk (later trials). Although intertemporal decision making involves the right PFC, the extent of hemispheric specialization in attribute and phase-specific decision making is unknown. Therefore, the current study assessed decision making in a patient with a unihemispheric disease, who underwent hemispherotomy surgery, comparing pre-surgical IGT performance (3 days prior to surgery) with post-surgical performance (1 month, and 12 months post-surgery). The patient's pre-and post-surgical IGT performances were analyzed to examine changes in attribute and phase-specific decision making, including the widely reported deck B phenomenon. The results for the two attributes of deck selection at the pre-and post-surgical assessments suggested marked changes in the two IGT phases of risk and uncertainty. Pre-surgery, the patient made more intertemporally disadvantageous choices, and task-progression contributed to it; within 1 month of surgery, intertemporal disadvantageous deck choices were contingent on task progression, after 1 year, disadvantageous choices were independent of task progression. Intertemporal attribute alteration was unresponsive to uncertainty and risk phase. The effect of task progression on frequency attribute remained unchanged before and immediately after the surgery, and preference for infrequent decks was observed only after 1 year. Further, pre and post surgery alteration in frequency attribute was phase-specific: within 1 month of surgery, infrequent deck choices decreased in uncertainty and increased in risk, whereas the reverse was observed after 12 months. Deck B choice increase was in the uncertainty phase. Results are discussed in reference to valence-linked hemispheric specialization and its potential role in attribute and phase-specific IGT decision making.

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Section: Left-hemisphere Atrophy and Left-lateralized Atrophy In Igt supporting

confidence: 92%

Functional Cerebral Specialization and Decision Making in the Iowa Gambling Task: A Single-Case Study of Left-Hemispheric Atrophy and Hemispherotomy

et al. 2020

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“…Our patient had a late-onset RE. Both sexes are equally affected [2,3]. It is characterized by focal intractable seizures, progressive neurological deficit, and cognitive decline, with unihemispheric brain atrophy, found in our patient [2].…”

Section: Discussionmentioning

confidence: 48%

“…The etiopathogenesis of RE is unknown. Suggested etiologies include viral infections, an autoimmune phenomenon involving circulating antibodies against glutamate receptors, and cytotoxic T cells [ 3 , 4 ]. Diagnosis of RE is based on characteristic clinical, radiological, and pathological features.…”

Section: Discussionmentioning

confidence: 99%

“…Various abnormalities are seen in patients with RE. Some unihemispheric findings such as impairment of background activity with persistent polymorphic delta waves and sleep spindles, focal slow activity, subclinical ictal discharges, and multifocal ictal discharges are strongly suggestive of RE [ 2 , 3 ]. The EEG of our patient showed intercritical left frontotemporal discharge persisting during sleep.…”

Section: Discussionmentioning

confidence: 99%

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Rasmussen’s Encephalitis: A Report of a Tunisian Pediatric Case and Literature Review

Klaa

Younes

Benrhouma

et al. 2020

Case Reports in Neurological Medicine

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Rasmussen’s encephalitis (RE) is a rare progressive inflammatory disease of the central nervous system. It is characterized by unilateral hemispheric atrophy, pharmacoresistant focal seizures, and progressive neurological deficit. The exact etiopathogenesis still remains unknown. Brain imaging plays an important role in diagnosis and follow-up. Fluctuation of lesions in brain imaging was reported in few cases. Herein, we report an additional pediatric case of Rasmussen encephalitis with fluctuating changes in brain MRI.

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“…Rasmussen encephalitis is an immune-mediated brain disorder that affects children between 6 and 8 years of age, leading to unilateral hemispheric atrophy, intractable focal epilepsy, and progressive neurological dysfunction. This condition can be very well differentiated on the basis of imaging features, which include unihemispheric focal cortical atrophy and ipsilateral head of the caudate nucleus, without any calvarial changes 21 . HHE is characterized by unilateral prolonged clonic seizures followed by the development of hemiplegia in younger children 22 .…”

Section: Discussionmentioning

confidence: 99%

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

Kalaskar

2018

J Korean Assoc Oral Maxillofac Surg

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Dyke-Davidoff-Masson syndrome is a non-inherited rare condition that presents during childhood and is characterized by seizures, hemiplegia, mental retardation, cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. The present article highlights a case of a 12-year-old male child with additional clinical findings of café-au-late pigmentation and ocular lipodermoid. This is the first case report of Dyke-Davidoff-Masson syndrome to describe oral manifestations, such as unilateral delayed eruption of teeth, hypoplasia, and taurodontism, which could be unique and characteristic of this condition. Oral health care providers and physicians should be aware of these oral observations as dental referrals could warrant early dental prophylactic care and can be useful in diagnosing the possible time of injury and type of Dyke-Davidoff-Masson syndrome.

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A Case of Rasmussen Encephalitis: The Differential Diagnoses and Role of Diagnostic Imaging

Cited by 13 publications

References 11 publications

Functional Cerebral Specialization and Decision Making in the Iowa Gambling Task: A Single-Case Study of Left-Hemispheric Atrophy and Hemispherotomy

Functional Cerebral Specialization and Decision Making in the Iowa Gambling Task: A Single-Case Study of Left-Hemispheric Atrophy and Hemispherotomy

Rasmussen’s Encephalitis: A Report of a Tunisian Pediatric Case and Literature Review

Classical oral manifestations of Dyke-Davidoff-Masson syndrome: a case report with review of the literature

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