This patient has a rare disease with an estimated incidence of one case per million per year. Symptoms started two weeks ago with a steady gnawing or boring pain behind the left eye. This was followed by double vision. Your extraocular movement exam is shown above (the center picture is neutral, the patient looks in the direction of the image relative to the center picture).
Labs including CBC, electrolytes, glucose, hemoglobin A1c, liver function tests, ACE, ANA, Anti-dsDNA, Anti-Sm, ANCA, FTA/ABS, lyme serologies, SPEP, ESR, and CRP are normal. A lumbar puncture is performed and all CSF studies are normal.
Contrast-enhanced coronal T1 MRI shown below:
Since you don't know what's going on, you give a trial of steroids, and within 48 hours, the patient's pain has resolved. Within a month, all the eye findings have resolved as well.
Challenge: Now you suspect the diagnosis is...what?
First image shown under Creative Commons Share-Alike License. Second image shown under Fair Use.
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1 comment:
Artemis
Artemis is the Hellenic goddess of the hunt; this is Tolosa-Hunt syndrome, characterized by painful ophthalmoplegia from idiopathic granulomatous inflammation of the cavernous sinus. Involvement of CN III is most frequent, followed by CN VI, the ophthalmic division of CN V, and CN IV; Horner’s syndrome is also possible. MRI shows the enlargement and enhancement of the left cavernous sinus. This is a diagnosis of exclusion.
Sources: UpToDate; www.ajronline.org; Wikipedia.
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