A young lady with a history of episodic migraine with visual aura, presented with transient weakness of arm, and word-finding difficulty associated with headache. She has intermittent vertigo with transient blurry vision and nonpulsatile tinnitus for several months. She has got hearing loss with a robotic quality of auditory perception. John O. Susac, described the first case in the mid-1970s in a young woman with a previously unreported triad of encephalopathy, branch retinal artery occlusions, and deafness.
Suz axe syndrome a young lady with the history of episodic migraine with visual aura presented with transient weakness of arm and word finding difficulty associated with headache she has intermittent vertigo with transient blurry vision and non-pulsatile tinnitus for several months she has got hearing loss with a robotic quality of auditory perception jonno
Susak described the first case in the mid-1970s in a young woman with a previously unreported triad of encephalopathy branch retinal artery occlusions and deafness susac syndrome is a rare disorder caused by occlusions of micro vessels in the brain retina and inner ear with an autoimmune cause more than 300 cases of susak syndrome have been reported in the
Literature worldwide with female individuals aged 20 to 40 years affects females more frequently than male individuals the prevalence of this disease may not be as rare as once thought given that the full clinical triad rarely exists a time of initial presentation and many cases are often being misdiagnosed as multiple sclerosis the most common manifestation is
Central nervous system symptoms followed by visual and hearing or vestibular disturbances headache is present in more than 80% of patients and often resemble a migraine central nervous system symptoms are focal neurological deficits owing to transient ischemic attack horse stroke later in the disease patients can develop global deficits such as encephalopathy or
Dementia magnetic resonance imaging of the brain typically shows punctate hyper intense deep white and gray matter lesions consistent with micro infarctions some of which may have restricted diffusion suggestive of acute ischemia and contrast enhancement involvement of the central portion of the corpus callosum rather than the periphery as in multiple sclerosis as
Evidenced by presence of so called snowball lesions is considered a characteristic sign of sue’s acts in jerome echocardiography is usually low yield because the deep location of the micro infarctions include being the corpus collosum makes cardio embolism less likely cerebral spinal fluid studies may show pleaaase ptosis and elevated protein typically without ala
Gatlin or band’s catheter based cerebral angiography results are usually normal an audiogram usually shows bilateral sensorineural hearing loss retinal fluoresce an angiography reveals unilateral or bilateral branched retinal artery occlusions and our arterial wall hyper fluorescence so into micro vasculitis even in patients who are visually asymptomatic fundus
Coupee typically shows branched retinal artery occlusions as well as retinal arterial ethera meatus plaques is chamak fluffy white patches and/or tiny hemorrhages susac syndrome is frequently misdiagnosed as multiple sclerosis owing to the presence of white matter lesions corpus callosum involvement and csf a plyo see ptosis however absence of all ego clonal
Bands involvement of deep gray matter and presence of retinal an auditory pathology argue against the diagnosis of multiple sclerosis recurrent oral and genital ulcers uveitis or retinal vasculitis for the g and skin or vascular lesions our chair act ik features of basic disease but lack of ulcers and presence of corpus callosum ethology makes the diagnosis of
Behcet’s disease unlikely the imaging phenotype and involvement of hearing do not support the diagnosis of cerebral autosomal dominant arteria pathy with subcortical in pharr sand luc encephalopathy genetic testing for cerebral autosomal dominant arteria pathy with subcortical in pharr sand luke encephalopathy may be considered in patients with cryptogenic stroke
With appropriate mri findings based on the idea that susak syndrome may have an inflammatory causative mechanism immunosuppressive agents such as mycophenolate mofa tail and a tapering dosage of oral prednisone may stabilize the patient good response to immunosuppressive and immunomodulatory therapies are reported there is no consensus on the treatment of susak
Syndrome because of the lack of large well-designed studies addressing this rare underreported disease thank you
Transcribed from video
Susac Syndrome By Dr. Zulfiquar Ahmed