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Treatment responsive GABA(B)-receptor limbic encephalitis presenting as new-onset super-refractory status epilepticus (NORSE) in a deployed U.S. soldier Volume 16, numéro 4, December 2014

TEST YOURSELF


(1) Autoimmune encephalitides are known to be associated with antigens targeting which of the following structures?
A.NMDA-receptor
B. AMPA-receptor
C. GABA(B)-receptor
D. LGI1-protein
E. CASPR2-protein
F. All of the above


(2) Which cancer is most often associated with GABA(B) limbic encephalitis?
A. Ovarian teratoma
B. Breast cancer
C. Small cell lung cancer
D. Testicular cancer
E. Hodgkin’s disease


(3) Which of the following is NOT a modality of immune-suppressive therapy?
A. IV methylprednisolone 1000 mg IV x 5 days
B. Intravenous immunoglobulins (IVIG) 0.4 g/kg/day x 5 days
C. IV cyclophosphamide 750 mg/mˆ2 once monthly
D. Therapeutic plasma exchange (PLEX) x 5-7 treatments
E. IV rituximab (375 mg/Mˆ2) weekly x 4 weeks
F. Tumour resection (if discovered)
G. IV midazolam 0.20 mg/kg bolus, then 0.05 to 0.60 mg/kg/hr continuous infusion

 

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Answers


(1) F. All of the above are potential neuronal cell surface targets for immune-mediated neurological disorders of the central nervous system.

 

(2) C. Small cell lung cancer is associated with about 50% of all case of anti-GABA(B)-receptor limbic encephalitis.

 

(3) G. IV midazolam is an anti-seizure anesthetic, not an immune-modulatory therapy. The other choices are potential immunosuppressive treatments for autoimmune encephalitis.

 

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