A Can of Worms - Slides

A 67 year-old man presented to the ophthalmology clinic with one day of binocular horizontal diplopia. He also complained of one week of bilateral periorbital pain which was worse on the right than the left. Four days prior, he had undergone partial right nephrectomy for grade II papillary renal cell carcinoma. He denied vision loss; other neurologic symptoms including weakness, numbness, or imbalance; or constitutional symptoms such as fever, chills, or weight loss. Visual acuity was 20/20 in each eye with normal color vision and full visual fields to confrontation. Pupils were equally reactive without afferent pupillary defect. External examination was unremarkable without proptosis, ptosis, or eyelid retraction. Motility was mildly limited in abduction bilaterally, and he had a comitant 16 prism diopter esotropia. MRI brain, acetylcholine receptor antibodies, and TSH were ordered, and the patient was referred to neuro-ophthalmology. Two weeks later, he presented to neuro-ophthalmology clinic with worsening diplopia and was found to have complete bilateral abduction deficits and 50 PD esotropia. He was admitted to the neurology service and during his hospital stay developed right upper and lower facial weakness, distal vibratory loss, and mild sensory ataxia. MRI brain revealed a questionable fullness of the right cavernous sinus, but on further review this was reported to be normal. Lumbar puncture revealed an opening pressure of 21cm, WBC 1/mL, RBC 1/mL, glucose 64 mg/dL, protein 98 mg/dL, and normal cytology. Serum GQ1b antibodies were negative, and EMG/NCS revealed evidence of axonal polyneuropathy without demyelinating features. He was treated empirically for Miller-Fisher syndrome with IVIG 2g/kg. Despite treatment, his diplopia continued to worsen over the next three months and he developed a new left supraduction deficit in addition to his bilateral abduction deficits (Figure 1). Further diagnostic testing was then performed.SMpolyradiculopathie