A 38-year-old male patient presented with horizontal diplopia, divergent strabismus and ptosis of the left eyelid. He also had an intense stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. Headache was continuous and demonstrated little response to common analgesics and nonsteroidal anti-inflammatories (NSAIDs). There was no complaint of nausea, vomiting, phonofobia or photophobia. The patient also complained of numbness in his left frontal and upper face (territory of ophthalmic and maxillary divisions of the trigeminal nerve).\nThere was a previous history of right-sided focal motor seizures, and a previous brain surgery 10 years ago for resection of a low grade tumour of the left frontal lobe (there was no pathological or immunohistochemistry report of that surgery available).\nOn examination the patient had complete third nerve palsy, sixth nerve palsy and loss of pinprick and thermic sensations in the territories of the Ophtalmic (V1) and Maxillary (V2) divisions of the trigeminal nerve on the left side. Other parts of the neurological examination, including cognition, motricity, reflexes and coordination were normal. There was no papilledema.\nA clinical diagnosis of cavernous sinus syndrome was made, and the patient was started on prednisone 60 mg/day, with significant improvement of headache intensity, resolution of ptosis and improvement in adduction, elevation and partial improvement in abduction of the left eye. An additional movie file shows this in more detail [see Additional file ].\nBrain magnetic resonance imaging (MRI) revealed an asymmetry of the cavernous sinuses, with the left cavernous sinus being larger than the right (Fig. ). There were also four meningeal lesions with contrast enhancement and without calcification in the upper convexity adjacent to the left and right frontal lobes, in the left cerebellopontine angle and left posterior temporal region (Fig. ).\nA biopsy of the meningeal lesion in the frontal convexity was performed, and resulted in a meningeal fragment with histiocytic proliferation (Fig. ). Immunohystochemical profile was negative for CD1a and ALK-1 and was positive for S100, CD68 (KP1 clone) and EMA (E29 clone), compatible with Rosai-Dorfman disease. Whole body computerized tomography (CT) scans did not reveal any other masses or lymphadenomegalies, characterizing an isolated CNS Rosai-Dorfman disease.\nThe patient was treated with intravenous methylprednisolone 1 g/kg daily for five days, with significant improvement of the cavernous sinus syndrome. Maintenance treatment consisted of prednisone 10 mg/day after tapering. He is asymptomatic and has remained seizure-free for two years. Follow-up brain MRI has showed a decrease in size of meningeal lesions and of cavernous sinus asymmetry (Fig. ).
A 43-year-old woman with a history of recurrent headaches and associated vision disturbances for the past 4 years presented to our emergency department with a 4-week history of progressive left-eye pain that radiated to the back of her head, swelling around the left eye, double vision, and a unilateral left-sided headache. There were no factors that worsened or improved her symptoms. She first developed this 4 years ago while living in another state. At that time, she was admitted to a hospital, given intravenous (IV) then oral steroids which helped suppress the pain and swelling, but she did not recall a specific diagnosis. Since that time, she has had three separate flare-ups, each resolving after steroid treatment, with the current episode being the most recent and severe. On physical exam she had left periorbital swelling and edema, 20/25 vision in her right eye and 20/30 in her left eye, and extra ocular muscles were intact, but she reported left-eye pain when at extreme medial and lateral gaze. She did not have temporal lobe tenderness on examination. There was normal muscle tone, range of motion, and sensation in all four extremities bilaterally. Laboratory results at admission were as follows: erythrocyte sedimentation rate (ESR) of 30 mm/h and normal complete blood count, renal function, hepatic function, and transaminases. Computed tomography (CT) of the head without contrast, CT of the orbits with contrast (), CT angiogram of the head, and an electrocardiogram (EKG) were performed. All were normal aside from the CT angiogram of the head which suggested a varix versus aneurysm involving the anterior cerebral arteries in the region of the inferior sagittal sinus (). Ophthalmology was consulted, excluded acute angle closure glaucoma, retinal disorders, diagnosed her with recurrent idiopathic orbital inflammation syndrome and possibly Tolosa-Hunt syndrome, and recommended treatment with methylprednisolone 80 mg IV daily for 3 days. Within 24 h she noted a 50% reduction in pain and had complete resolution of her symptoms within 48 h. Magnetic resonance imaging (MRI) of the brain with and without contrast conducted the next day was normal and did not confirm the presence of a vascular anomaly suggested by the CT scan. At this point she was discharged on 60 mg oral prednisone daily with a taper over the course of 2 weeks. One week after discharge she was seen at her ophthalmologist’s office and remained asymptomatic.