A 19-year-old right-handed man presented with a 3 year history of episodic headaches, right hemiparesis, and progressive vision loss OU. Beginning in the fall of 2015, he developed stepwise deterioration in vision OU in association with severe headaches. Initial evaluation at an outside hospital showed VA 20/40 OD and 20/50 OS with bilateral optic disc pallor. MRI of the orbits showed no abnormalities. VA worsened to 20/200 OU by January 2016 and fields showed central scotoma OU and a left homonymous hemianopia. LHON testing was negative. CSF analysis showed 1 WBC, protein 36 mg/d, IgG index 0.66 (<0.6), (+) oligoclonal bands, with negative AFP, cytology, and Lyme. He was started on IV followed by oral steroids in February 2016 with improvement in the visual field, but no improvement in VA. Mycophenolate was started on April 2016 with no improvement and therefore discontinued. He continued having attacks of nausea, headache, worsening vision, and then developed right hemiparesis. Repeat orbital MRI in March 2017 showed left optic nerve enhancement. In April 2017 he was started on rituximab, but became lethargic after the second infusion, and his neurological status deteriorated after the third infusion (October 2017). He began behaving unusually - saying inappropriate things and developed excessive thirst that led to drinking toilet water. In November of 2017 he developed left-sided weakness and became wheelchair bound. Repeat orbital MRI showed persistent left optic nerve enhancement and T2 hyperintensity of the right basal ganglia and cortical spinal tracts. His cognition declined thereafter to the point that he had difficulty expressing himself. In March 2018, on presentation to our institution, he was unable to follow commands, had severe cognitive impairment and spasticity throughout. VA was LP OU. Pupils were minimally reactive to light. Dilated funduscopic exam showed severe bilateral optic nerve pallor with normal retina.
Disease/Diagnosis
Germinoma with bilateral optic nerve infiltration
Date
2019-03
References
None.
Language
eng
Format
video/mp4
Type
Image/MovingImage
Source
2019 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of
NANOS Annual Meeting Frank B. Walsh Sessions; 2019
