Medical treatment of Rasmussen's Encephalitis: A systematic review

Lagarde, Stanislas; Boucraut, José; Bartolomei, Fabricē

doi:10.1016/j.neurol.2022.01.007

Cited by 22 publications

(13 citation statements)

References 86 publications

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“…However, surgery can lead to complications such as comprehending homonymous hemianopia, hemiplegia, or aphasia. Immunotherapy can slow disease progression, but does not halt or cure the disease and thus, may be used in early stages of the disease or in patients with slow disease progression, with mild deficits, and/or not suitable for surgery (Lagarde, 2022). Intravenous methylprednisolone and oral prednisone given for up to 24 months in a tapering schedule may help to diminish the intractable focal seizures and motor deficits during the first year of onset and before hemiplegia develops.…”

Section: Current Management/treatmentmentioning

confidence: 99%

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence‐Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue

Connelly‐Smith

Alquist

Aqui

et al. 2023

J of Clinical Apheresis

218

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The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis (TA) in human disease. In the Ninth Edition, the JCA Special Issue Writing Committee has incorporated systematic review and evidence-based approaches in the grading of evidence and categorization of apheresis indications to make recommendations on the use of apheresis in a wide variety of diseases and conditions. This edition has largely maintained the general layout and concept of a fact sheet introduced in the Fourth Edition (2007). Each fact sheet succinctly summarizes the evidence for the use of TA in a specific disease or medical condition. The Ninth Edition of the JCA Special Issue comprises 91 fact sheets and

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Section: Current Management/treatmentmentioning

confidence: 99%

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence‐Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue

Connelly‐Smith

Alquist

Aqui

et al. 2023

J of Clinical Apheresis

218

View full text Add to dashboard Cite

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“…Although this procedure alone may not be sufficient to achieve seizure freedom due to continuous antibody cytotoxicity, 5 a probability higher than 70% of long-term seizure-freedom has been described for RE. 15 This case broadens the spectrum of anti-Hu-associated neurologic disorders in children. We highlight the importance of considering antineuronal antibody testing in children presenting with EPC, focal cerebral atrophy, or hypersignal suggestive of RE and the need for an ongoing surveillance for malignancy in the presence of anti-Hu antibodies.…”

Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 99%

Child Neurology: Anti-Hu Encephalitis in an Adolescent With a Mediastinal Seminoma

Cabreira,

Ferreira,

Melo

et al. 2023

Neurology

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Anti-Hu antibodies are associated with autoimmune syndromes, mainly limbic encephalitis, encephalomyelitis and painful sensory polyneuropathy (Denny-Brown). We report a case of a 15-year-old boy presenting withepilepsia partialis continua(EPC) found to have a right middle frontal gyrus brain lesion without atrophy or contralateral involvement. Following partial resection, neuropathology revealed neuronal loss, reactive gliosis and astrocytosis, as well perivascular mononuclear inflammatory infiltrate and features of neuronophagia resembling Rasmussen encephalitis. Suboptimal response to antiseizure drugs and surgery prompted further workup with identification of positive serum anti-Hu antibodies and a mediastinal seminoma. The patient was treated with immunotherapy including steroids, IVIG, azathioprine, rituximab and plasmapheresis as well as mediastinal lesion resection. However, he continued to experience EPC, psychomotor impairment along with left hemiparesis and dysarthria. Given clinical progression with failure to respond to immunotherapy and anti-seizure polytherapy, hemispherotomy was attempted and seizure freedom achieved. A review of the literature found only sixteen cases of neurological presentations associated with anti-Hu antibodies in children, confirming the rarity of EPC in these cases. Thus, this report provides a new observation of germ cell mediastinal tumour associated with anti-Hu antibodies in children broadening the spectrum of anti-Hu–associated neurologic disorder in children and highlighting the importance of considering antineuronal antibody testing in children presenting with EPC and brain lesions suggestive of Rasmussen encephalitis.

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“…The median age of onset is six years but can range from infancy to adulthood [ 1 ]. In the view that Rasmussen's encephalitis is an immune-regulated process, immunotherapy treatments such as long-term corticosteroids, intravenous immunoglobulins, and tacrolimus have been used to alleviate symptoms [ 7 ]. However, for patients with refractory symptoms, surgery remains the only cure with procedures that disconnect the affected hemisphere.…”

Section: Discussionmentioning

confidence: 99%

Endocrinopathies in a Pediatric Patient Post-anatomical Hemispherectomy for Rasmussen’s Encephalitis Treatment: A Case Report

Sanchez,

Belmar,

Sanchez

et al. 2024

Cureus

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Hemispherectomy is a neurosurgical procedure that is frequently performed in pediatric patients diagnosed with Rasmussen's encephalitis. Postoperative complications include immediate complications such as hydrocephalus and hemorrhage and behavioral complications such as language impairments and contralateral weakness. However, there are limited studies or case reports that address the potential endocrinopathies associated with this and other pediatric epileptic surgeries. This case report describes the endocrinopathies following an anatomical hemispherectomy procedure. A four-year-old African-American female had a right anatomical hemispherectomy for the treatment of Rasmussen's encephalitis in 2020. The postoperative course was immediately complicated by central diabetes insipidus which was stabilized with desmopressin. The patient’s labs in 2021 were consistent with central precocious puberty with elevated luteinizing hormone (LH) and follicle-stimulating hormone (FSH). Additionally, the patient was found to have secondary adrenal insufficiency in which she failed a low-dose adrenocorticotropic hormone (ACTH) stimulation test. Oral hydrocortisone therapy was initiated for secondary adrenal insufficiency in addition to initiating leuprolide injections for central precocious puberty. Furthermore, at the age of seven years, the patient had her first menarche. This case report emphasizes the need for closer and long-term surveillance for endocrine issues in postepileptic surgical pediatric patients as well as a surveillance plan for the development of other potential endocrine abnormalities throughout the patient’s life.

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Medical treatment of Rasmussen's Encephalitis: A systematic review

Cited by 22 publications

References 86 publications

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence‐Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice – Evidence‐Based Approach from the Writing Committee of the American Society for Apheresis: The Ninth Special Issue

Child Neurology: Anti-Hu Encephalitis in an Adolescent With a Mediastinal Seminoma

Endocrinopathies in a Pediatric Patient Post-anatomical Hemispherectomy for Rasmussen’s Encephalitis Treatment: A Case Report

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