2. BBE. Data from series of 5 or more patients.

Study ID / Name	Treatment	N	n(%)Asymptomatic 6m	n(%)Asymptomatic 1m	n(%)Ventilated 1m	n(%)Dead 6m	n(%)Fluct / relapse	? Adverse event
Odaka 2003	PE	14	8 (57%)	‐	2 (14%)	1 (7%)	1 (7%)	1 cardiac arrest
	Steroids	13	11 (85%)	‐	0	0	0	0
	IVIg	2	2 (100%)	‐	0	0	0	0
	PE + steroids	16	6 (38%)	‐	4 (25%)	0	0	0
	IVIg + steroids	5	3 (60%)	‐	0	0	0	0
	PE + IVIg	2	0	‐	2 (100%)	0	0	0
	PE + IVIg + steroids	3	1 (33%)	‐	1 (33%)	1 (33%)	0	1 respiratory obstruction
	Natural history	7	1 (14%)	‐	1 (14%)	1 (14%)	0	1 meningitis
Yaqub 1989	Natural history	6	6 (100%)	1 (17%)	1 (17%)	0	0	0
Kuntzer 1987	Steroids (1), acyclovir (1)	2	2 (100%)	0	0	0	‐	‐
	Natural history	5	5 (100%)	1 (20%)	1 (20%)	0	‐	1 pneumonia
Al‐Din 1982	ACTH or prednisolone	3	2 (67%)	1 (33%)	0	0	0	0
	Natural history	10	7 (70%)	0	1 (10%)	1 (10%)	0	1 chest infection + pulmonary embolism