Table 1.

Comparison of data of the different reported cases of Susac syndrome with onset in pregnancy.

Cases	1	2	3	4	5	6	7	8
Published by	Gordon19918	McFayden19879	Hua201410	Ionnides201311	Engelholm201312	Antulov201413	Feresiadou201414	Our case
Origin	US	Canada	US	Australia	Germany	Croatia	Sweden	Mexico
Age	28	31	25	28	32	21	35	34
Gestational age at onset (weeks)	28	No specified	14	13	32	35	37	15
Previous medical history	None	None	None	Epilepsy from a perinatal ischaemic event	None	None	Apparent similar clinical picture at 12 yo, treated with steroids	None
System of onset	Eye	Eye	Auditory	Neurologic	Neurologic	Neurologic	Auditory	Auditory
Neurologic symptoms	Unilateral weakness, dysarthria and apathy	Ataxia and dysarthria	Amnestic syndrome, Gait disorder, Bilateral severe weakness	Bilateral severe weakness and dysarthria	Encephalopathic syndrome and Unilateral weakness	Bilateral severe weakness and progressive cognitive affection	None	Cognitive affection (frontal medial syndrome) and bilateral weakness
Ophthalmologic symptoms	Visual field deficit	Visual field deficit	Loss of visual acuity	Loss of visual acuity	Visual field deficit	None	Loss of visual acuity and visual field deficit	Loss of visual acuity
Auditory symptoms	Bilateral neurosensorial hearing loss	Bilateral tinnitus and neurosensorial hearing loss	Tinnutus	Right neurosensorial hearing loss	Neurosensorial hearing loss	Left neurosensorial hearing loss	Tinnitus and left neurosensorial hearing loss	Tinnitus and bilateral neurosensorial hearing loss
Additional or atypical affection	None	None	Cervical cord involvement	None	Livedo racemosa	None	None	None
Time until fully triad (months)	1	2	6	4	1.5	Not completed	Not completed	6
MRI findings
Deep grey matter	No	Not done	Yes	Yes	Yes	Yes	No	Yes
White matter	Callosal and periventricular	Not done	Callosal and periventricular lesions	Callosal	Callosal and periventricular lesions	Callosal and periventricular lesions	No	Callosal
Posterior fossa involvement	No	Not done	Yes	No, but also reported meningeal enhancement	Yes	Yes	No	No
Gadolinium enhancement	No reported	Not done	Yes	No reported	Yes	No reported	No	Yes
CSF findings
Proteins mg/dl	No reported	252	95	2000	1800	1009	No performed	77
Cells (Mono)	No reported	0	6	9	No reported	No reported	No performed	0
Treatment
Initial treatment	Heparin	None	IVMP × 5	IVMP × 3	IVMP × 5	IGIV × 5	IVMP × 5	IVMP × 5
Response	Partial		Partial	No response	Partial	Complete response	Partial	No response
2nd line treatment	None	None	Oral prednisone	PLEX and IVIg	Oral prednisone	None	None	IgIV
Chronic treatment	Warfarin	Oral prednisone	MMF	MMF	MMF + MTX	AZA	Oral prednisone	CCF
Response	Almost complete recovery	Partial remission	Partial remission	No response	Almost complete recovery	Almost complete recovery	Almost complete recovery	Partial remission
Prognosis
Follow up (years)	0.2	4	1.5	3.5	1	0.2	3	0.5
Sequels	Visual deficit	Mild hearing loss	Cognitive deficit with visuospatial and word recall	No specified	Mild cognitive deficit	Subtle weakness	Mild left hearing loss	Cognitive deficit
Course of disease		Monocyclic	Monocyclic	Chronic continuous	Probably Monocyclic	Probably Monocyclic	Monocyclic	Probably Monocyclic
Final pregnancy state	Healthy product	Healthy product	Healthy product	Therapeutic abortion at 15 weeks GA	Healthy product	Healthy product	Healthy product	Therapeutic abortion at 17 weeks GA