Maurice F. Rabb » CNS Vasculitis Associated With APMPPE

This 25 year old man had been well until December, 1992 at which time he began to have chills and headaches. Initially this was considered to be a recurrence of the meningitis which the patient had had three years earlier in college. Within a few weeks bilateral uveitis developed with floaters and pain in the eyes. A biopsy of the conjunctiva was negative for sarcoid. Sedimentation rate was mildly elevated. An A.C.E. test was allegedly slightly elevated but subsequently normal. The patient was started on steroids (Medrol) and his status improved and he reported back to work within a few weeks. The Medrol was tapered and while on a low dose the headaches flared and a right hemiparesis developed along with difficulty with bladder and bowel control. The patient was hospitalized and a diagnosis of viral encephalomyelitis was made. Recovery occurred over a few weeks. Negative studies included a Lyme serology, HIV, lumbar puncture, bone scan, x-ray of the sacroiliac joints, head CT, head MRI, and chest x-ray. While again tapering the steroids, a recurrence of the floaters occurred in March along with headache and myalgias; high dose steroids were initiated again.

The patient returned home and an angiogram in April confirmed the presence of CNS vasculitis. He was started on Cytoxan, and the Medrol was increased. Initially he was started on 50 mg of Cytoxan which was increased to 100 without any signs of improvement. When the Cytoxan was increased to 200 mgm daily, his condition improved. His eye sight improved along with his general status.

The visual acuity at the present time on September 20, 1993, is 20/20 in each eye and he has begun lifting weights, is bicycling, and plans to return to work shortly. He is now on 8 mg of Medrol, alternating with 4 mg of Medrol on successive days.

This patient demonstrated CNS vasculitis associated with APMPPE. This disease can be fatal although some cases have had a self-limited clinical course but aggressive treatment with systemic steroids and cytotoxic therapy is recommended.