A 17-year-old woman with no morbid history presented with a two-week history of pulsatile bifrontal headache, with no postural pattern of progressive intensity, associated with nausea, vomiting and altered color perception.
Pathological examination revealed visual field alteration and papilledema.
Brain MRI was normal.
Fifteen days later she was hospitalized for continuous headache and progression of visual disturbances.
A history of repeated oral ulcers of years of evolution was obtained and genital ulcers were added in the last year.
Also arthralgias of hands and wrists.
Physical examination revealed oral ulcers and severe nodular acne.
Neurological examination showed no abnormalities, except for the presence of papilledema, with the rest of the normal examination.
MRI was repeated, including venous phase angioresonance and normal.
Lumbar puncture showed a cerebrospinal fluid (CSF) with 14 mg/dl protein, 64 mg/dl glucose, without leukocytes, with 20 fresh red blood cells, highlighting increased outflow pressure in 26 cm of water.
Laboratory tests showed hematocrit 41.9%, hemoglobin 14.4 g/dl, leukocyte count 7,800/mm3, 2,500 lymphocytes, normal platelet count and ESR 13 mm in one hour.
Renal function, liver tests and plasma electrolytes were normal and C-reactive protein 1.0 mg/dl. Serology: ANA, ANCA, Anti DNA, ENA profile, anticardiolipin, and Anti Beta 2 negative.
With these elements a pseudotumor cer with suspected BE was diagnosed and treatment with acetazolamide and colchicine was initiated.
His headache and visual disturbances increased torpidly.
A derivative valve was discarded due to the unfavorable anatomy of its cerebral ventricles.
Pulses of intravenous methylprednisolone (1,250 mg) and then prednisone 60 mg/d were indicated.
The patient was added to the headache treatment, due to mixed etiology, flunari malformation and cyclobenzaprine.
Headache subsided, but continued with visual disturbances, adding photopsias.
An electroencephalogram ruled out ictal activity.
A second evaluation showed anterior uveitis that helped to configure the diagnosis of BE.
For this reason topical ocular steroids and oral cyclosporine (vo) were added to the treatment.
1.
High management and management with multiple refractory oral and genital ulcers. Treatment with infliximab 5 mg/kg was initiated, with good response to ulcers and ocular involvement, but with headache that has been difficult to manage
