Rasmussen encephalitis: Incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins

Bien, Christian G.; Tiemeier, Henning; Sassen, Robert; Kuczaty, Stefan; Urbach, Horst; Lehe, Marec von; Becker, Albert; Bast, Thomas; Herkenrath, Peter; Karenfort, Michael; Kruse, Bernd; Kurlemann, Gerhard; Rona, Sabine; Schubert‐Bast, Susanne; Vieker, Silvia; Vlaho, S; Wilken, Bernd; Elger, Christian E.

doi:10.1111/epi.12042

Cited by 135 publications

(130 citation statements)

References 27 publications

(30 reference statements)

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“…Antiepileptic drugs, immunoglobulins and high doses of steroids have been demonstrated to be ineffective in controlling the associated seizures and stopping the progression of RE [6,15,16,21]. Focal excision and callosotomy are unsatisfactory in many cases.…”

Section: Discussionmentioning

confidence: 99%

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Surgical Treatment of Patients with Rasmussen Encephalitis

Guan

Zhou

Luan

et al. 2014

Stereotact Funct Neurosurg

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Background: Rasmussen encephalitis (RE) is a rare progressive encephalitis that results in intractable seizures, cognitive decline and hemiparesis. Surgery seems to be the only effective way to control seizures in RE patients. Objective: To describe the clinical, electrophysiological, neuroradiological and histological findings of our patients with RE and to evaluate the outcome of their surgical treatment. Methods: A total of 20 patients were identified by the criteria of RE. Surgery was conducted in the left hemisphere in 6 patients and in the right hemisphere in 14. The surgical methods included anatomical hemispherectomy, functional hemispherectomy, hemispherotomy, lesion resection, multilobar resection, selective resection and bipolar electrocoagulation of functional cortexes. Results: The mean follow-up period was 5.45 years (range 3-8). After surgery, 16 patients (80%) were evaluated as being Engel class I. All of the patients had increases in cognitive abilities after surgery except 1 patient with bilateral RE. After surgery, most patients could walk independently, but the fine movement of the hands was lost. Postoperative hydrocephalus was observed in 1 patient after functional hemispherectomy, and there was no death in this series. Conclusion: Hemispherectomy and hemispherotomy were both confirmed as beneficial procedures in controlling seizures and improving quality of the life in RE cases.

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Section: Discussionmentioning

confidence: 99%

“…To date, no antiepileptic drug has been demonstrated to be effective for controlling the disease or stopping its progression [6]. Immunoglobulins and high doses of steroids may be transiently helpful in some patients at an early stage [13,14,15,16]. Focal excision and callosotomy have been unsatisfactory in many cases [17].…”

Section: Introductionmentioning

confidence: 99%

Surgical Treatment of Patients with Rasmussen Encephalitis

Guan

Zhou

Luan

et al. 2014

Stereotact Funct Neurosurg

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“…Investigators in a German studyestimated the countrywide incidence at 2·4 cases per 10 million people aged 18 years and younger per year. 6 Similarly, researchers in a recent UK surveillance study estimated an incidence of 1·7 per 10 million people aged 16 years and younger per year (a prevalence of 0·18 per 100 000 people).…”

Section: Case Reportmentioning

confidence: 98%

Rasmussen's Encephalitis - Chronic Focal Encephalitis (CFE) - A Rare Entity

Kumar¹

2017

jmscr

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Rasmussen's encephalitis, also known as chronic focal encephalitis (CFE), is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motorskills and speech, hemiparesis (paralysis on one side of the body), encephalitis (inflammation of the brain), and CASE REPORTA 12 year old male child presented with history of focal seizures in the form of jerky movements of left upper limb and left lower limb associated with history of up-rolling of eyeball, frothing from mouth and deviation of angel of mouth to right side. There was no associated history of tongue bite, loss of consciousness, altered sensorium, post-ictal confusion, involuntary urination or defecation during episode. These complains were slowly progressive with increasing weakness on left half of body. There was no significant past history and family history related to patients complains.

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“…Immune suppressive therapy was initiated, consisting of tacrolimus, methylprednisolone, and intravenous immunoglobulin (IVIG) [9]. Immediately after surgery, she experienced worsening of her right upper-extremity weakness, which we …”

Section: Follow-up and Treatmentmentioning

confidence: 99%