Timing of Surgery in Rasmussen Syndrome: Is Patience a Virtue?

Hartman, Adam L.; Cross, J. Helen

doi:10.5698/1535-7511-14.s2.8

Cited by 15 publications

(12 citation statements)

References 26 publications

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“…Functional recovery usually depends on such factors as age of operation and the preceding course of the disease, and it is important to determine what age is most preferable for performing the operation (Hartman & Cross, 2014;HYPERLINK \l "bookmark23" Moosa et al, 2013). Resting state fMRI analysis, performed both before and after the operation, can be used to assess network autonomy and the potential for rearrangement.…”

Section: Discussionmentioning

confidence: 99%

Intrinsic functional organization of putative language networks in the brain following left cerebral hemispherectomy

Ivanova

Zaidel

Salamon³

et al. 2017

Brain Struct Funct

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In rare cases of severe and intractable epilepsy, cerebral hemispherectomy is performed to arrest seizure activity and improve quality of life. The remaining hemisphere is often capable of supporting many cognitive functions post-surgery, although the outcome depends on the underlying etiology, hemisphere removed, and age of resection. The mechanisms underlying this massive reorganization are at present unknown. Here we examined intrinsic functional connectivity of putative language brain networks in 4 children after left cerebral hemispherectomy using resting state functional magnetic resonance imaging (rsfMRI). We compared these functional systems to intrinsic language networks in 15 neurotypical controls using region-of-interest (ROI) based functional connectivity analyses. In 3 out of 4 hemispherectomy patients, the ROI placed in the right inferior gyrus revealed a functional network that strongly resembled the right hemisphere intrinsic language network observed in controls. This network typically comprised inferior frontal gyrus, superior temporal sulcus, and premotor regions. Quantitative ROI-to-ROI analyses revealed that functional connectivity between major nodes of the language network was significantly altered in all 4 examined patients. Overall, our data demonstrate that the pattern of functional connectivity within language networks is at least partially preserved in the intact right hemisphere of patients who underwent left hemispherectomy.

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Section: Discussionmentioning

confidence: 99%

Intrinsic functional organization of putative language networks in the brain following left cerebral hemispherectomy

Ivanova

Zaidel

Salamon³

et al. 2017

Brain Struct Funct

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“…Mutations in GNAQ, a gene governing angiogenesis, have recently been identified in SWS (Shirley et al 2013). Some authorities feel that early surgery (hemispherectomy) affords a better prognosis in hemispheric epilepsy syndromes (Hartman and Cross 2014).…”

Section: Childhood Hemispheric Epilepsy Syndromesmentioning

confidence: 99%

Seizures and Epilepsy: An Overview for Neuroscientists

Stafstrom

Carmant²

2015

Cold Spring Harbor Perspectives in Medicine

655

421

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Epilepsy is one of the most common and disabling neurologic conditions, yet we have an incomplete understanding of the detailed pathophysiology and, thus, treatment rationale for much of epilepsy. This article reviews the clinical aspects of seizures and epilepsy with the goal of providing neuroscientists an introduction to aspects that might be amenable to scientific investigation. Seizures and epilepsy are defined, diagnostic methods are reviewed, various clinical syndromes are discussed, and aspects of differential diagnosis, treatment, and prognosis are considered to enable neuroscientists to formulate basic and translational research questions.T his article provides an overview of seizures and epilepsy for neuroscientists. We focus on broad concepts, rather than clinical details, and raise questions related to mechanisms, epileptogenesis, and therapeutic approaches that might generate interest among basic researchers. Further information about differential diagnosis, drug doses, and clinical management are available from numerous resources (Engel and Pedley 2008;Duchowny et al. 2012;Engel 2013).We first define seizures and epilepsy and summarize their classification, pathophysiology, and genetics. Diagnostic methods are then considered, including the importance of an accurate historical description of an event suspected to be a seizure and the appropriate use of ancillary/confirmative tests, such as electroencephalogram (EEG), neuroimaging, and genetic studies. These modalities enable the clinician to differentiate epilepsy from numerous clinical conditions that mimic seizures, but have a nonepileptic pathophysiological basis. Examples of epilepsy syndromes are then described, selected based on their frequency in the population or because they embody scientific questions that warrant elucidation. Finally, we provide an overview of treatment options and prognosis, including a consideration of conditions that accompany epilepsy (comorbidities) and complicate the daily lives of people with epilepsy. Subsequent articles in this collection explore the scientific basis of many of the clinical concepts introduced here. DEFINITIONS AND EPIDEMIOLOGYA "seizure" is a paroxysmal alteration of neurologic function caused by the excessive, hyper-

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“…Hemi‐disconnection of the affected side of the brain is the only effective cure for seizures, with long‐term seizure freedom in 70%‐80% of cases . Consequences, however, include hemiplegia, hemianopia, and aphasia . Severity of seizures is one of the major factors that informs surgical decision making, although loss of motor skills, reorganization of language functions to the nondominant hemisphere, and the neuropsychological profile are also considered .…”

Section: Introductionmentioning

confidence: 99%

“…3 Consequences, however, include hemiplegia, hemianopia, and aphasia. 4 Severity of seizures is one of the major factors that informs surgical decision making, although loss of motor skills, reorganization of language functions to the nondominant hemisphere, and the neuropsychological profile are also considered. 2 The neuropsychological trajectory of RS during the period of disease progression has yet to be formally elucidated.…”

Section: Introductionmentioning

confidence: 99%

Pre‐ and postsurgical cognitive trajectories and quantitative MRI changes in Rasmussen syndrome

et al. 2018

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During the chronic phase of the disease, parallel decline of verbal and nonverbal abilities suggest progressive bilateral hemispheric involvement, supported by evidence from MRI morphometry. Postsurgical cognitive losses are predicted by greater presurgical ability and continuing seizures. A shorter duration from seizure onset to surgery could reduce the postoperative cognitive burden by minimizing the decline in functions supported by the unaffected hemisphere.

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Timing of Surgery in Rasmussen Syndrome: Is Patience a Virtue?

Cited by 15 publications

References 26 publications

Intrinsic functional organization of putative language networks in the brain following left cerebral hemispherectomy

Intrinsic functional organization of putative language networks in the brain following left cerebral hemispherectomy

Seizures and Epilepsy: An Overview for Neuroscientists

Pre‐ and postsurgical cognitive trajectories and quantitative MRI changes in Rasmussen syndrome

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