new - onset refractory status epilepticus ( norse ) is a syndromic diagnosis recognized by de novo status , without a history of epilepsy , in which no underlying structural , metabolic , or infective cause is identified . the syndrome can have very prolonged status epilepticus ; it affects the young and healthy and frequently has a catastrophic outcome . increasingly early aggressive intervention with immunomodulatory treatment such as steroids , including cyclophosphamide is advocated , , , . a 31-year - old somali gentleman , with no prior history of epilepsy , presented with status epilepticus to his local district general hospital following a nonspecific prodrome of feeling generally unwell and weight loss for a few weeks . he was transferred to critical care , and initial treatment included phenytoin , ceftriaxone , and aciclovir . over the course of 5  days , his condition did not respond to treatment with four antiepileptic drugs ( aeds ) . additionally , the patient was sedated with midazolam , alfentanil , and propofol . despite these measures , daily eegs showed suboptimal control of seizure activity . the patient 's blood tests revealed a raised crp , negative viral pcr ( including hepatitis and hiv ) , negative blood cultures , and negative urine cultures . g / l , 0 polymorphs    10/l , 20 mononuclear cells    10/l , and a csf glucose level less than half the serum glucose level ( 4.2/10.2 ) , but no organisms were seen on the gram stain . head mri showed a slightly increased signal in the anterior left temporal lobe on axial flair sequence . the patient was given empirical treatment for suspected new - onset refractory status epilepticus syndrome ( norse ) with 3  days of high - dose methylprednisolone and subsequently maintained on 60-mg prednisolone . the treating neurological team made a clinical diagnosis of new - onset refractory status epilepticus ( norse ) syndrome . on day 9 of his admission , he was transferred to the itu department of the tertiary epilepsy center with the intention of giving plasma exchange and for consideration of cyclophosphamide treatment . a further review at the specialist center revealed a previous diagnosis of treated genital syphilis 3  years ago . a repeat csf examination was sent for treponemal serology ; this came back as strongly positive in serum and csf strongly indicating a diagnosis of neurosyphilis.initial treponemal serology screen:csf rpr ( rapid plasma reagin test ) 1:4 , tppa ( treponema pallidum particle agglutination ) 1:10,240,serum vdrl ( venereal disease reference laboratory ) 1:4 , serum tppa 1:20,480.the patient was treated for 21  days on high - dose iv benzylpenicillin 2.4  g qds for neurosyphilis in line with national guidelines . the patient 's eeg demonstrated dramatic improvement in the next 24  h with no further electrographic or clinical seizures.repeat syphilis screen 6  days later ( posttreatment):csf rpr negative , tppa negative,serum rpr 1:2 , tppa 1:10 , 240 . initial treponemal serology screen:csf rpr ( rapid plasma reagin test ) 1:4 , tppa ( treponema pallidum particle agglutination ) 1:10,240,serum vdrl ( venereal disease reference laboratory ) 1:4 , serum tppa 1:20,480 . csf rpr ( rapid plasma reagin test ) 1:4 , tppa ( treponema pallidum particle agglutination ) 1:10,240 , serum vdrl ( venereal disease reference laboratory ) 1:4 , serum tppa 1:20,480 . the patient was treated for 21  days on high - dose iv benzylpenicillin 2.4  g qds for neurosyphilis in line with national guidelines . the patient 's eeg demonstrated dramatic improvement in the next 24  h with no further electrographic or clinical seizures . repeat syphilis screen 6  days later ( posttreatment):csf rpr negative , tppa negative,serum rpr 1:2 , tppa 1:10 , 240 . csf rpr negative , tppa negative , serum rpr 1:2 , tppa 1:10 , 240 . the repeat axial head mri at 1  month demonstrated  marked interval reduction in cerebral volume showing a central pattern of atrophy with enlargement of the lateral and third ventricles. fig .  1 , fig . 2 show the initial and repeat axial brain mri scans at 1  month , respectively . at 12-month follow - up , the patient remains with gross functional deficits and marked cognitive impairment and requires full nursing care including percutaneous endoscopic gastrostomy tube for feeding . new - onset refractory status epilepticus syndrome affects the young and is often associated with a poor neurological outcome . increasingly , it is considered to have an immune basis , and therefore aggressive immunomodulatory therapy is advocated at an early stage , . however , clinicians must remember that norse syndrome is a diagnosis made by excluding other causes , some of which such as neurosyphilis are readily treatable ( see table 2 in the supplementary evidence for a list of known causes ) . the incidence of seizures in neurosyphilis from studies is reported to range from 14 to 60% , but status epilepticus ( se ) as the presenting symptom is very rare , , . furthermore , there are only a handful of cases of patients presenting with refractory status epilepticus secondary to neurosyphilis , . neurosyphilis presents diversely and is known to have the ability to infect the nervous system at any stage of infection . the standard dose given for the treatment of syphilis is insufficient in treating neurosyphilis , and treatment is with high - dose benzylpenicillin , , , . while this case had the clinical characteristics of other cases of norse syndrome seen at our center , the history of we would advocate that all patients with refractory status are tested for syphilis , allowing for early instigation of directed antibiotic therapy and avoidance of cytotoxic therapy . an etiological factor needs to be identified and be treated ; this case emphasizes the need to perform an extensive workup as early as possible , and given the complexities of managing and determining the cause of status epilepticus , we suggest such patients should be transferred to a regional neuroscience center early in their illness . new - onset refractory status epilepticus syndrome is a diagnosis of exclusion . before instigation of potentially harmful neuromodulatory therapies , treatable causes such as neurosyphilis should be considered . we advocate early transfer of refractory status patients to a specialist epilepsy center experienced in rapidly managing refractory epilepsy for both seizure management and cause determination . any patient that is admitted with refractory epilepsy should have a full extensive search done to find a causative agent . new - onset refractory status epilepticus syndrome is a diagnosis of exclusion and is increasingly treated with immunomodulatory therapies such as cyclophosphamide  always consider treatable causes such as neurosyphilis . we wish to confirm that there are no known conflicts of interest associated with this publication , and there has been no significant financial support for this work that could have influenced its outcome .
new - onset refractory status epilepticus ( norse ) is a syndrome of new - onset drug resistant status epilepticus that often has a catastrophic outcome . a 30-year - old man of somali origin presented with refractory status to a district general hospital . 
 a clinical diagnosis of norse syndrome was made , and he was transferred to the regional epilepsy center for immunomodulatory treatment and consideration for cyclophosphamide treatment . 
 after transfer to the regional epilepsy center , his repeat cerebrospinal fluid tested strongly positive for syphilis , indicating a diagnosis of neurosyphilis , and the patient was treated with high - dose intravenous ( iv ) benzylpenicillin . 
 his status epilepticus abated 24  h later.new-onset refractory status epilepticus syndrome is a diagnosis of exclusion . before instigation of potentially harmful neuromodulatory therapies , treatable causes such as neurosyphilis should be considered . 
 we advocate the early transfer of refractory status patients to a specialist epilepsy center for both seizure management and cause determination .