New onset refractory status epilepticus due to primary angiitis of the central nervous system

Matar, Rawan K.; Alshamsan, Bader; Al-Saleh, S.; Alhindi, Hindi; Alahmedi, Khalid O.; Khairy, Sami; Baz, Salah

doi:10.1016/j.ebcr.2017.07.005

Cited by 18 publications

(15 citation statements)

References 22 publications

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“…New-onset refractory status epilepticus (NORSE) is a rare but devastating condition. Overall, approximately 200 cases of NORSE in adults [1][2][3][4][5][6][7][8][9][10][11][12] and 200 cases of FIRES in children [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] have been reported in the literature.…”

Section: Introduction and New Definitionsmentioning

confidence: 99%

New onset refractory status epilepticus (NORSE)

Sculier

Gaspard

2019

Seizure

108

109

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To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE. Method: Narrative review of the medical literature about NORSE and FIRES. Results: NORSE and FIRES mainly affect school-age children and young adults. A prodromal phase with flu-like symptoms precedes the SE onset in two third of NORSE cases, and by definition in all FIRES. Status epilepticus usually starts with repeated focal seizures with secondary bilateralization. Most cases evolve to super RSE (SRSE) and have unfavorable outcome, with short-term mortality of 12-27%, long-term disability and epilepsy. No specific imaging or laboratory abnormalities have been identified so far that allows an early diagnosis and half of adult cases remain of unknown etiology. A standardized diagnostic algorithm is provided and. Autoimmune encephalitis is the most frequent identified cause. In the absence of specific diagnosis, immunotherapy could be tried in addition to antiepileptic treatment. Conclusions: This review presents the rare but devastating syndrome of NORSE, including the subcategory of FIRES. Early recognition with complete work-up is primordial to identify the underlying cause and promptly start appropriate treatment.

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Section: Introduction and New Definitionsmentioning

confidence: 99%

New onset refractory status epilepticus (NORSE)

Sculier

Gaspard

2019

Seizure

108

109

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“…In the adult population, a literature search identified a case report of a 20-year-old man in nonconvulsive focal status epilepticus for 8 weeks who had a favorable outcome with long-term steroid treatment, surgical resection of his epileptic focus, and intensive rehabilitation. 12 Our patient's presentation is similar, but his treatment differed because he did not have a cortical lesion that was amenable to resection; instead, he received more intensive immunosuppression with intravenous immunoglobulin, cyclophosphamide, and mycophenolate mofetil.…”

Section: Discussionmentioning

confidence: 77%

Childhood Small Vessel Primary Angiitis of the Central Nervous System: A Treatable Cause of Super-refractory Status Epilepticus

Chiu

Datta

2019

J Child Neurol

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Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory disease of brain vessels. The small vessel subtype is diagnosed on brain biopsy and often presents with cognitive and behavioral changes, headaches, and seizures. However, there are few reported cases of super-refractory status epilepticus in children. We present a case of small vessel cPACNS complicated by super-refractory status epilepticus requiring burst suppression for 4 weeks in addition to multiple antiseizure medications and the ketogenic diet. Our patient was also treated with intravenous and oral steroids, intravenous immunoglobulin, and cyclophosphamide before starting maintenance therapy with mycophenolate mofetil. After prolonged rehabilitation, he recovered almost completely and has a normal neurologic examination with rare epileptiform activity on electroencephalogram (EEG). This is one of the longest cases of status epilepticus in small vessel cPACNS in the literature. We illustrate that super-refractory status epilepticus can be the first manifestation of small vessel cPACNS in previously healthy children and that symptomatic management of seizures with concurrent immunosuppression to treat the underlying pathology resulted in favorable neurologic outcomes.

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“…FIRES is applicable to any age and requires a prior febrile infection starting between 2 weeks and 24 hours before the onset of refractory status epilepticus whereas the presence of fever at the onset of status epilepticus is not a defining criterion 24 . Reports of meaningful results of biopsy in patients with NORSE are sparse and include rabies; primary angiitis of the central nervous system; lymphocytic infiltration with simian virus 40 (SV40) inclusion in oligodendrocytes; spongiform necrosis in limbic system associated with autoantibodies against GD1a, GT1b, and GQ1b; herpes simplex virus with negative polymerase chain reaction (PCR); Candida with negative culture; and acute disseminated encephalomyelitis (ADEM) 135-139 . In one case series, gliosis without infiltration was found in seven children 140 .…”

Section: Super-refractory Status Epilepticusmentioning

confidence: 99%

Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus

Trinka¹,

Leitinger²

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW: Status epilepticus is a serious condition caused by disorders and diseases that affect the central nervous system. In status epilepticus, hypersynchronous epileptic activity lasts longer than the usual duration of isolated self-limited seizures (time t1), which causes neuronal damage or alteration of neuronal networks at a certain time point (time t2), depending on the type of and duration of status epilepticus. The successful management of status epilepticus includes both the early termination of seizure activity and the earliest possible identification of a causative etiology, which may require independent acute treatment. In nonconvulsive status epilepticus, patients present only with subtle clinical signs or even without any visible clinical manifestations. In these cases, EEG allows for the assessment of cerebral function and identification of patterns in need of urgent treatment.RECENT FINDINGS: In 2015, the International League Against Epilepsy proposed a new definition and classification of status epilepticus, encompassing four axes: symptomatology, etiology, EEG, and age. Various validation studies determined the practical usefulness of EEG criteria to identify nonconvulsive status epilepticus. The American Clinical Neurophysiology Society has incorporated these criteria into their most recent critical care EEG terminology in 2021. Etiology, age, symptomatology, and the metabolic demand associated with an increasing duration of status epilepticus are the most important determinants of prognosis. The consequences of status epilepticus can be visualized in vivo by MRI studies. SUMMARY: The current knowledge about status epilepticus allows for a more reliable diagnosis, earlier treatment, and improved cerebral imaging of its consequences. Outcome prediction is a soft tool for estimating the need for intensive care resources. UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE:Drs Trinka and Leitinger discuss the unlabeled/investigational use of antiseizure medications for the treatment of status epilepticus.

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New onset refractory status epilepticus due to primary angiitis of the central nervous system

Cited by 18 publications

References 22 publications

New onset refractory status epilepticus (NORSE)

New onset refractory status epilepticus (NORSE)

Childhood Small Vessel Primary Angiitis of the Central Nervous System: A Treatable Cause of Super-refractory Status Epilepticus

Management of Status Epilepticus, Refractory Status Epilepticus, and Super-refractory Status Epilepticus

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