Case Presentation:
A 41- year old female with history of hypertension was admitted to our hospital with complaints of intermittent diffuse abdominal pain, diarrhea, and generalized rash. Vital signs were normal. Physical examination was notable for diffuse maculopapular rash. Laboratory investigations showed pancytopenia and acute renal failure. Further investigations were positive for a speckled pattern ANA, anti-double stranded DNA, anti- SSA /SSB, and B2 glycoprotein, cardiolipin, phosphatidylserine IgM antibodies. Skin and renal biopsy confirmed Systemic lupus Erythematosus (SLE). She was started on IV Methylprednisolone with resolution of rash, pancytopenia and improvement in renal function. On day 3 of steroidal therapy, patient developed a thunderclap headache that peaked within seconds of onset. Headaches were associated with nausea, vomiting and photophobia. CT scan and MRI/MRA/MRV of brain were normal. On day 9 of steroidal therapy, patient developed an acute bilateral peripheral vision loss, and left sided hemipharesis. CT scan showed bilateral cerebellar infarcts with mass effect. CTA showed multifocal areas of moderate to severe stenosis in the bilateral MCA, ACA, PCA and vertebrobasilar arteries. MRI showed acute infarcts in the cerebellar hemisphere, right occipital lobe, right parietal lobe and left frontal lobe. Intra-arterial Verapamil was administered with resolution of cerebral vasospasm. CTA 7 days later showed improvement of arterial segmental stenosis. Given the clinical presentation and the location of the stenotic arteries, headaches were thought to be due to Reversible Cerebral Vasoconstriction Syndrome (RCVS). Patient was eventually discharged on oral Verapamil and a quick taper of methylprednisolone.
Discussion:
Differentiating headaches secondary to RCVS versus Cerebral Vasculitis in SLE patient is often challenging but a necessary step for proper management. In RCVS, headaches are sharp, thunderclap, with an acute onset and a rapid peak, within few seconds. Major complication of RCVS is stroke leading to morbidity and mortality. Diffused segmental constriction of cerebral arteries, mainly located in the circle of Willis, is seen on radiological imaging. These findings often resolve within 1-2 months. Calcium channel blockers remain the mainstay of treatment of RCVS. On the other hand, Cerebral Vasculitis presents as insidious and dull headaches with a step-wise progression pattern. On imaging, vasculitis usually presents as stenosis of the posterior arterial circulation. While cerebral vasculitis is managed with steroids, RCVS is usually made worse by steroids.
Conclusions:
Headache in patients with SLE is a common phenomenon with different and multiple underlying mechanisms. Distinguishing Reversible Cerbral Vasoconstriction Syndrome from Cerebral Vasculitis is necessary to ensure appropriate management to minimize further neurological complications.
