Presentation
Acute onset of right eye vision loss. No prior medical history.
Patient Data
Multiple hyperintense punctate lesions on T2-FLAIR sequences and hypointense in T1W sequences in the supra- and infratentorial white matter, predominantly affecting the corpus callosum (large size, tumefactive), subcortical white matter, centrum semiovale, left internal capsule and middle cerebellar peduncles.
Some of the lesions show restricted diffusion.
On gadolinium-enhanced T1W sequences, faint enhancement of some lesions is identified, particularly in the left corona radiata.
The optic nerves show normal morphology and signal intensity, without signs of optic neuropathy.
Ophthalmological examination showed 0,2/1 vision on the right eye and 1/1 on the left eye. Funduscopic examination showed retinal ischemia with multiple retinal artery branch occlusions but with sparing of the central retinal artery.
No other abnormalities were found in the neurological examination.
Case Discussion
These MRI findings display a characteristic pattern of periventricular white matter lesions arranged in a series of hyperintense "snow-balls" within the central fibers of the corpus callosum (rather than the callososeptal interface, which is more typical of multiple sclerosis). “Snowball” lesions of the corpus callosum on MRI are pathognomonic for Susac syndrome and represent multifocal callosal microinfarcts; these lesions can evolve into callosal “holes”, usually called “punched-out” lesions.
The main differential diagnosis of Susac syndrome includes multiple sclerosis and acute disseminated encephalomyelitis (ADEM).
When the MRI was performed, the patient only presented ophthalmological symptoms. Following the findings described in the MRI, a complete auditory examination was conducted, revealing bilateral sensorineural hearing loss. The patient met the diagnostic criteria and was diagnosed with Susac syndrome. After receiving immunosuppressive treatment, the number of lesions on the follow-up MRI decreased.
Unable to process the form. Check for errors and try again.