Updates in Refractory Status Epilepticus

Table 4

Diagnostic investigations in RSE. Adapted from the NORSE table of investigations on http://www.norseinstitute.org/definitions/. This is the basic workup suggested to be done in most patients with NORSE and is by no means an absolute list. For further workup and a complete list of tests, please refer to the NORSE Diagnostic Checklist which can be found on http://www.norseinstitute.org/definitions/ [25].

Basic workup for causes of refractory status epilepticus
Screen	Disease/agent tested
Infectious	Recommended in most or all patients
	(i) Serologic: bacterial and fungal cultures, RPR-VDRL, and HIV-1/2 immunoassay with   confirmatory viral load if appropriate
	(ii) CSF: cell counts, protein, glucose, bacterial and fungal stains and cultures, VDRL, PCR for HSV1,   HSV2, VZV, EBV, HIV, and Mycobacterium tuberculosis
	Recommended in immunocompromised patients in addition to above
	(i) Serologic: IgG Cryptococcus species, IgM and IgG Histoplasma capsulatum, and IgG Toxoplasma   gondii
	(ii) Sputum: Mycobacterium tuberculosis GeneXpert
	(iii) Serum and CSF: Toxoplasma IgG
	(iv) CSF: eosinophils, silver stain for CNS fungi, PCR for JC virus, CMV, HHV6, EEE, Enterovirus,   influenza A/B, WNV, Parvovirus, Listeria Ab, and measles (rubeola)
	(v) Stool: adenovirus PCR and Enterovirus PCR
Vascular	(i) CTA or MRA and MR venography
Autoimmune/paraneoplastic	Recommended
	(i) Serum and CSF paraneoplastic and autoimmune epilepsy antibody panel
	To include antibodies to VGKC with LGI-1 and CASPR2, Ma2/Ta, DPPX, GAD65, NMDA,   AMPA, GABA-B, GABA-A, glycine receptor, amphiphysin, CV-2/CRMP-5, neurexin-3 alpha,   adenylate kinase, anti-neuronal nuclear antibody types 1 (Hu), 2 (Ri), and 3, Purkinje cell   cytoplasmic antibody types 1 (Yo), Tr, and 2, and glial nuclear antibody type 1
	(ii) Serologic: also send for ANA, ANCA, anti-thyroid antibodies, anti-dsDNA, ESR, CRP, ENA,   SPEP, and IFE. Antibodies for Jo-1, Ro, La, Scl-70, RF, and ACE; anti-tTG and anti-endomysium   antibodies and cold and warm agglutinins
	Optional: consider storing extra frozen CSF and serum for possible further autoimmune testing in a research lab
Neoplastic	Recommended: CT chest/abdomen/pelvis, scrotal ultrasound, mammogram, CSF cytology, flow cytometry, and pelvic MRI
	Optional: bone marrow biopsy, whole-body PET-CT, and cancer serum markers
Metabolic	Recommended: LDH and ammonia
	Considered: vitamin B1 level, B12 level, folate, lactate, pyruvate, CPK, and troponin; tests for mitochondrial disorder (lactate and pyruvate); serum triglycerides
Toxicological	Recommended: benzodiazepines, amphetamines, cocaine, fentanyl, alcohol, ecstasy, heavy metals, synthetic cannabinoids, and bath salts
	Considered: extended opiate and overdose panel, LSD, heroin, PCP, and marijuana
Genetic	Considered: genetics consult, ceruloplasmin, and 24-hour urine copper