We report two young patients presenting with acute homonymous hemianop
ia and acute severe hemiparesis involving the face. The diagnostic wor
k-up led to the diagnosis of multiple sclerosis. Clinically symptomati
c retrochiasmal demyelinating lesions are rare even in the long-term c
ourse of multiple sclerosis and those affecting the pyramidal tract us
ually produce only mild partial dysfunction with bilateral symptoms du
e to brainstem or spinal cord involvement. Acute homonymous hemianopia
or motor hemiparesis first suggest another etiology than multiple scl
erosis, particularly a vascular lesion, MRI is the investigation of ch
oice in this clinical setting, showing an unusually large lesion in mo
st cases where a demyelination is the cause. Clinical recovery neverth
eless is usually excellent, despite persistence of the lesion on repea
ted imaging, This contrasts with that of an infarction and also with t
hat of acute monophasic demyelinating disease (Marburg, Bale), variant
s of multiple sclerosis that need to be considered initially.