Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen’s encephalitis

Pradeep, K.; Sinha, Sanjib; Mahadevan, Anita; Saini, Jitender; Arivazhagan, A; Bharath, Rose Dawn; Bindu, Parayil Sankaran; Rajan, Jamuna; Rao, Malla Bhaskara; Govekar, S.; Ravikumar, B. V.; Chandramouli, Bangalore A.; Satishchandra, P

doi:10.1016/j.jocn.2015.05.062

Cited by 40 publications

(17 citation statements)

References 35 publications

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“…The clinical presentation in our patient with almost continuous unilateral movements predominating in the upper limb may resemble that of epilepsia partialis continua (EPC), however the polymorphous phenomenology combining dystonia, choreoathetosis, myorhythmia and fast frequency and low amplitude clonic-like movements is not a usual feature in the latter. EPC is rather characterized by repetitive, monomorphic, simple and brief myoclonic jerks with regular or irregular occurrence [6], [7]. In our patient, some additional key features supported the diagnosis of movement disorder instead of seizures the including non-stereotyped clinical presentation, the fact that movements systematically disappeared during sleep, and persistence of both, clinical and electrophysiological findings despite antiseizure drugs but with a response to immunotherapy [8], [9], [10].…”

Section: Discussionsupporting

confidence: 52%

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Benjumea-Cuartas¹,

Eisermann²,

Simonnet³

et al. 2017

Epilepsy & Behavior Case Reports

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Anti-NMDA receptor encephalitis is a treatable autoimmune disease characterized by cognitive, motor and psychiatric features that primarily affects young adults and children. We present a case of a 7-year-old boy with asymmetrical (mainly right hemibody) and abnormal polymorphic movements without concomitant scalpictal EEG changes but had background slowing predominating over the left hemisphere. This report illustrates previous descriptions of asymmetric presentation of abnormal movements in pediatric anti-NMDA receptor encephalitis and emphasizes the importance of video-EEG interpreted within the overall clinical context, to differentiate epileptic from non-epileptic abnormal movements in patients with autoimmune encephalitis.

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Section: Discussionsupporting

confidence: 52%

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Benjumea-Cuartas¹,

Eisermann²,

Simonnet³

et al. 2017

Epilepsy & Behavior Case Reports

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show abstract

“…The mainstay in treatment remains functional hemispherotomy, which has been shown to have benefit in quality of life. [65][66][67] Opsoclonus Myoclonus Ataxia Syndrome Opsoclonus myoclonus ataxia syndrome (OMS) is a rare, immune-mediated disease that occurs primarily in young children. It affects both sexes equally, and mean age of onset is 1.6 years.…”

Section: Rasmussen's Encephalitismentioning

confidence: 99%

Autoimmune Neurologic Diseases in Children

Sweeney

2018

Semin Neurol

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Autoimmune diseases of the nervous system in children are composed of a heterogeneous group of rare disorders that can affect the central or peripheral nervous system at any level. Presentations may occur in children of any age and are typically acute or subacute in onset. Consideration of an autoimmune process as the etiology of neurologic diseases in children is important, as it may lead to early initiation of immunotherapy and an improvement in long-term neurologic outcomes. The developing nervous and immune systems in children create unique challenges in diagnosis and treatment of these rare diseases. In this review, autoimmune diseases affecting the brain, spinal cord, nerve roots, peripheral nerves, neuromuscular junction, and muscle in children are described.

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“…This result indicates that IFITM3 rs12252-C is related to the disease progression of RE patients by promoting persistent HCMV infection in brain tissue (Wang et al, 2021). Drug-resistant epilepsy is one of the most important clinical features of RE (Bien et al, 2002;Pradeep et al, 2016). TLE is a common and frequently intractable seizure disorder.…”

Section: Discussionmentioning

confidence: 89%

Genetic Factors in Rasmussen’s Encephalitis Characterized by Whole-Exome Sequencing

Wang

Liu

et al. 2021

Front. Neurosci.

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Rasmussen’s encephalitis (RE) is a rare chronic neurological disorder characterized by unihemispheric brain atrophy and epileptic seizures. The mechanisms of RE are complex. Adaptive immunity, innate immunity and viral infection are all involved in the development of RE. However, there are few studies on the role of genetic factors in the mechanisms of RE. Thus, the objective of this study was to reveal the genetic factors in the mechanisms of RE. Whole-exome sequencing (WES) was performed in 15 RE patients. Ten patients with temporal lobe epilepsy (TLE), which is a common and frequently intractable seizure disorder, were used as the controls. Thirty-one non-silent single nucleotide variants (SNVs) affecting 16 genes were identified in the RE cases. The functions of the genes with SNVs were associated with antigen presentation, antiviral infection, epilepsy, schizophrenia and nerve cell regeneration. Genetic factors of RE were found first in this study. These results suggest that RE patients have congenital abnormalities in adaptive immunity and are susceptible to some harmful factors, which lead to polygenic abnormal disease.

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Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen’s encephalitis

Cited by 40 publications

References 35 publications

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

Autoimmune Neurologic Diseases in Children

Genetic Factors in Rasmussen’s Encephalitis Characterized by Whole-Exome Sequencing

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