Indian Journal of Ophthalmology (Jan 2013)
Susac′s syndrome: First from India and youngest in the world
- Gunjan Prakash,
- Shephali Jain,
- Mausam Gupta,
- Tirupat Nathi
Affiliations
- Gunjan Prakash
- Shephali Jain
- Mausam Gupta
- Tirupat Nathi
- DOI
- https://doi.org/10.4103/0301-4738.118446
- Journal volume & issue
-
Vol. 61,
no. 12
pp. 772 – 773
Abstract
A two and half year old female was admitted at the emergency room suffering from gradually worsening headache followed by nausea. The child presented with reduced level of consciousness and bilateral hypoacusis. The patient was lethargic. Ophthalmic examination showed branch retinal artery occlusion (BRAO). This finding was crucial to the diagnosis of Susac′s syndrome (SS), a rare autoimmune disease characterized by, endotheliopathy of retina, encephalic tissues and cochlea. Magnetic resonance imaging of the brain also showed typical features. Thorough blood investigations did not reveal any other abnormality. Patient was treated with immunosuppressive to prevent her from developing severe sequelae of this disease. The child showed dramatic improvement in her systemic condition within 48 h of starting the treatment. This is the youngest ever and first case report from India.
Keywords
- Amblyopia
- nasolacrimal duct obstruction
- probing
- Duane syndrome
- esotropia
- strabismus
- Bevacizumab
- mitochondrial function
- microvascular endothelial cells
- neurosensory retinal cells
- retinal pigment epithelial cells
- tissue culture
- Clinical donor tissue grading
- death to enucleation time
- death to preservation time
- eye bank specular microscopy
- optimizing donor tissue utilization
- DCR
- endodiathermy bipolar probe
- nasolacrimal duct obstruction
- recanalize
- Age-related macular degeneration
- carotenoids
- cross-sectional studies
- lutein
- Children
- GDx variable corneal compensation
- Indian
- normal
- retinal nerve fiber layer thickness
- Biological amniotic membrane
- 5-fluorouracil
- retina
- trabeculectomy
- Clinical features
- complications
- etiology
- retinal vasculitis
- Thailand
- Coloboma of the choroid
- coloboma optic disc
- indo cyanine green angiography
- vascular pattern
- Aphakia
- inadequate capsular support
- pediatric aphakia
- secondary intraocular lens implantation
- steel suture
- trans-scleral fixation
- vanadium steel
- Drug reaction
- drug-induced
- ranibizumab
- subacute cutaneous lupus erythematosus
- Prevalence
- rapid
- rapid assessment of refractive errors
- spectacles use
- uncorrected refractive errors
- visual impairment
- Bitemporal hemianopia
- Traumatic chiasmal syndrome
- visual field
- Phaeohyphomycosis
- pigmented fungi
- subretinal abscess
- subretinal biopsy
- Brimonidine
- combination
- glaucoma
- intraocular pressure
- timolol
- Congenital
- dermis fat graft
- orbital
- teratoma
- Familial hypercholesterolemia
- corneal arcus
- xanthomas
- Branch retinal arterial occlusion
- encephalopathy
- retinal vasculitis
- sensorineural hearing loss
- susac′s syndrome