Susac syndrome (SS) is a rare autoimmune microangiopathy characterized by the triad of encephalopathy, branch retinal artery occlusions (BRAO) and sensorineural hearing loss. The authors present two cases with this syndrome.
Methods
Case 1: 54-year-old woman presents with a 3-year history of auditory complaints and a 2-month history of fatigue, incoherent and apraxic speech, headaches and decreased visual acuity (VA). Neurological exam revealed altered mental state and pyramidal signs. Brain MRI showed snowball lesions in the corpus callosum. Ophtalmological examination revealed VA of 20/32 (right eye [RE]) and 20/25 (left eye) and attenuated peripheral retinal arteries. Fluorescein angiography (FA) showed multiple nonperfused arterial segments. SS was suspected and 1g IV methylprednisolone (mPDN) for 3 days was iniciated. IV immunoglobulin and Rituximab were started and improvement ensued.
Results
Case 2: 30-year-old man presented with 3 day history of decreased VA of the RE and 2-month history of tinnitus, hearing loss and headache. Neurological examination revealed cognitive impairment. The audiogram confirmed right-sided sensorineural hearing loss. FA showed multiple bilateral BRAO, while brain MRI revealed several T2/FLAIR hyperintensities in the corpus callosum and white matter. He was treated with 1g IV mPDN for 3 days, hyperbaric oxygen therapy and cyclophosphamide, followed by Rituximab for 2 years, with substantial and sustained improvement.
Conclusion
Early recognition and treatment of SS are crucial to prevent irreversible neurological, auditory and visual damage, but its varied presentation often leads to a diagnosis delay. The ophthalmologist often plays a key role in its early diagnosis, enabling timely treatment and improved prognosis.
Conflict of interest
No
1
Last name
ROMANA
Initials of first name(s)
J
Department
Hospital Egas Moniz, Lisboa, Unidade Local de Saúde Lisboa Ocidental
City
Lisboa
Country
Portugal
2
Last name
Tavares Correia
Initials of first name(s)
M
Department
Hospital Egas Moniz, Lisboa, Unidade Local de Saúde Lisboa Ocidental
City
Lisbon
Country
Portugal
3
Last name
Madeira
Initials of first name(s)
M
Department
Hospital Egas Moniz, Lisboa, Unidade Local de Saúde Lisboa Ocidental
City
Lisbon
Country
Portugal
4
Last name
Cordeiro
Initials of first name(s)
M
Department
Hospital Egas Moniz, Lisboa, Unidade Local de Saúde Lisboa Ocidental
City
Lisbon
Country
Portugal
5
Last name
Guedes
Initials of first name(s)
M
Department
Hospital Egas Moniz, Lisboa, Unidade Local de Saúde Lisboa Ocidental
City
Lisbon
Country
Portugal
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