Table 4.

Summary of the 2-case series on ADEM/MDEM followed by optic neuritis. ADEM: Acute disseminated encephalomyelitis; AQP4: Aquaporin4; GA: Glatiramer acetate; INF-beta: interferon beta; IVIG: Intravenous immunoglobulins; MDEM: Multiphasic disseminated encephalomyelitis; MOG: Myelin oligodendrocyte; ND: No data; OCB: Oligoclonal bands; ON: Optic neuritis; PLEX: Plasma exchange; TDL: Tumefactive demyelinating lesions.

	Hupke et al., 2012 [188]	Baumann et al., 2016 [191]
N of patients	7	8
Median Age (range) years	6 (4–8)	3 (1–7)
Gender (F/M)	6/1	5/3
Clinical presentation ADEM MDEM ON	n = 7 n = 3/7 Unilateral in n = 6; Bilateral in n = 1 Always following ADEM/MDEM N of attacks: 1–7 Inter-attack intervals: 3 weeks-2 years	n = 7 Unilateral/Bilateral: ND; n = 2
Median inter-attack interval	Minimum 4 weeks for ON	4 months for MDEM
Preceding febrile illness; N; (weeks prior)	ND	Yes; n = 4; 4 weeks prior
Median follow up (range) years	6 for n = 4/7	4 (1–8)
Autoantibody	ADEM stage: MOG-antibody (+) 3/7 and ND 4/7 MOG- antibody (+) with ON AQP4-antibody (-)	MDEM stage: MOG- antibody (+) 8/8 AQP4-antibody (-)
CSF	Pleocytosis Negative OCB	Pleocytosis Negative OCB in n = 7/8
MRI Brain MRI spinal cord	Classical ADEM findings New lesions with MDEM No new brain MRI lesions during ON ND	Classical ADEM findings with TDL and cortical GM lesions. New lesions with MDEM LETM n = 2 SSTM n =2
Treatment	Improvement with corticosteroids Azathioprine: Partial effectiveness IFN-beta and GA: Not effective	Corticosteroids during attacks (n = 8) IVIG (n = 1) during attack PLEX (n = 1) during attack IVIG Monthly (n = 4)
Outcome	Minimal to no relapses, n = 2 Continuous relapses, n = 2 Mild vision loss, n = 4	Normal n = 4 Mild-moderate deficit n = 4 (psychomotor and/or seizures)