New‐Onset Refractory Status Epilepticus in an Adult with an Atypical Presentation of Cat‐Scratch Disease: Successful Treatment with High‐Dose Corticosteroids

Abstract

New-onset refractory status epilepticus (NORSE) is defined as a sudden onset of refractory status epilepticus in patients who do not have a history of epilepsy. It is a neurologic emergency, and determining the underlying etiology is an important factor for effectively managing and predicting the prognosis of NORSE. We describe the case of a 28-year-old woman who was hospitalized with NORSE secondary to an unknown etiology. She did not respond to traditional anticonvulsant therapy, including benzodiazepines, fosphenytoin, propofol, and levetiracetam. The patient was placed on continuous electroencephalography (EEG) monitoring and was treated further with multiple antiepileptics, which were titrated aggressively based on EEG readings and therapeutic drug levels; despite this treatment, EEG monitoring revealed continued seizures. Thus, high-dose corticosteroids were started for seizure control. Her workup included computed tomography and magnetic resonance imaging of the head, a lumbar puncture, toxicology screening, and extensive testing for multiple infectious and inflammatory etiologies. The patient's history revealed recent exposure to a new cat. Serologic results were positive for Bartonella henselae, and she was diagnosed with cat-scratch disease (CSD). She did not have the typical presentation of symptoms of lymphadenopathy, however, which is common in CSD. Doxycycline 100 mg and rifampin 300 mg twice daily were added to the patient's anticonvulsant and corticosteroid therapy. She was hospitalized for a total of 26 days and discharged with only minor neurologic impairment (short-term memory deficits and minor cognitive problems). The patient was discharged receiving antiepileptics, antibiotics, and a corticosteroid taper. To our knowledge, this is the first clinically known case of NORSE secondary to CSD without typical CSD symptoms in the adult population. The patient failed to respond to traditional anticonvulsant therapy alone. With the addition of high-dose corticosteroids to aggressive anticonvulsant therapy, seizure control was achieved. Further studies are needed to determine the effectiveness of high-dose corticosteroid and anticonvulsant therapy followed by antibiotics in patients with NORSE secondary to CSD.

New onset refractory status epilepticus (NORSE)

“…NORSE treatment algorithm: Commonly used drugs in NORSE and FIRES with most frequently reported doses (expert opinion) [66]. Adapted from Gaspard et al, 2018 [39] and van Baalen et al, 2017 [10]. IV = intravenous; IVIG = intravenous immunoglobulin; RSE: refractory status epilepticus, SE: status epilepticus.…”

“…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.…”

Sporadic Creutzfeldt–Jakob disease with tau pathology mimicking new‐onset refractory non‐convulsive status epilepticus: Case report and review of the literature

“…Treatment of NCSE is based on the administration of benzodiazepines and other anti‐epileptic drugs [9,10]. In recent years, increasing evidence of autoimmune epilepsy has led to the use of corticosteroids in new‐onset refractory status epilepticus (NORSE) [11]. The prognosis of NCSE depends on etiology, age and duration, among other factors [12].…”

The etiology and mortality of new‐onset refractory status epilepticus (NORSE) in adults: A systematic review and meta‐analysis

“…21,28,[43][44][45][46][47] Other systemic autoimmune antibodies were categorized under non-neoplastic autoimmune causes, such as thyroid or antinuclear antibodies. 24 Specified infectious causes included viral etiology such as severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) in five cases, 35,[48][49][50] Herpes simplex virus (HSV) in four cases, 31,[51][52][53] bacterial such as Bartonella in one case, 54 neurosyphilis in three cases, 5,10,55 and the infectious prion disease, Creutzfeldt-Jakob disease (CJD) in three cases. 10,56,57 Unspecified autoimmune etiology was classified as such when investigation panels rendered seronegative results, but where an autoimmune basis was strongly suspected: a diagnosis based on the "holistic" view of the clinical presentation, taking into consideration the history, imaging result, cerebrospinal fluid (CSF) analysis, and exclusion of other etiologies.…”