Title : Susac syndrome: Case report
Abstract:
Introduction:
Susac syndrome; It is a rare disease that is characterized by the triad of encephalopathy, sensorineural hearing loss and branch retinal artery occlusion, which is thought to develop due to immune-mediated microangiopathy. It is more common in patients aged 16-40 years and in women. Microocclusions that can be seen in the brain, retina and inner ear due to non-inflammatory vasculopathy are responsible for the pathogenesis. The diagnosis is primarily made by clinical findings, visualization of branch retinal artery occlusion by fluorescent angiography (FFA), presence of characteristic lesions often observed in the corpus callosum on cranial magnetic resonance (MR) imaging, and sensorineural hearing loss on audiometric examination.
Case Presentation:
A 39-year-old female patient presented with complaints of dizziness, headache, tinnitus and hearing loss in the left ear. The dizziness was in attacks, sliding from underground, lasting for half an hour to an hour. No nausea or vomiting. She had a throbbing headache, usually in the form of a left-sided headache, occasionally. It was accompanied by photophobia, phonophobia and kinesiophobia. Neurological examination was normal. Cranial Magnetic Resonance (MRI) examination revealed thinning and atrophy in the corpus callosum. Numerous millimeter-sized microinfarcts were observed in the head and body parts of the corpus callosum, and in the pericalllosal-periventricular white matter. Carotid-vertebral doppler ultrasound, brain and cervical MRI angiography were normal. Transthoracic echocardiography was normal. Electroencephalography was normal. Hearing test revealed mild sensorineural hearing loss on the right and moderate-severe on the left. Optical coherence tomography showed thinning and atrophy in the left superior temporal posterior region, and Fluorescein Fundus angiography (FFA) showed delayed choroidal filling in left eye, arterial venous occlusion was not observed. As a result of the examinations, the patient was evaluated as compatible with Susac syndrome.
Discussion:
Susac syndrome is a rare disease characterized by encephalopathy, retinopathy and hearing loss. It should be considered in the diagnosis and should be considered in the differential diagnosis of vasculopathy.
