Case Study: Headache, Confusion, and Seizures in Patient With SLE

 

RPLS Pathogenesis

The pathogenesis of RPLS remains unclear, but it appears to be related to disordered cerebral autoregulation and endothelial dysfunction.2 When the upper limit of cerebral autoregulation is exceeded, arterioles dilate and cerebral blood flow increases, with rises in systemic blood pressure.7 The resulting brain hyperperfusion may lead to breakdown of the blood-brain barrier.7

The primary involvement of posterior brain regions is not well understood.3

RPLS Treatment

Hypertension is present in most of those with RPLS, and controlled lowering of blood pressure often improves symptoms dramatically.2 In cases of malignant hypertension, the maximum initial fall in blood pressure should not exceed 25% of the baseline presenting value.8 Very aggressive and rapid blood pressure treatment goals may reduce the blood pressure below the autoregulatory range and can lead to ischemic events.7

The use of easily titratable parenteral agents such as nicardipine, labetalol, and nitroprusside are effective and safe in reducing the blood pressure to a desirable range.9

Phenytoin is used to treat most patients with RPLS and seizures, with the exception of those with eclampsia.10 Although long-term follow-up studies are limited, seizure recurrence or epilepsy appear to be rare.10

In RPLS associated with the use of cytotoxic agents, reduction in drug dosage or prompt removal of the cytotoxic drug is usually recommended and is often associated with clinical improvement.2

RPLS Prognosis

Most case series and case reports suggest that RPLS is usually benign and reversible.1 In many cases, RPLS seems to be fully reversible within a period of days to weeks following removal of the inciting factor and control of blood pressure.2 Radiologic improvement can lag behind clinical recovery, however.10 Recurrence of RPLS appears to be infrequent.12

References

1. Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc. 2010;85(5):427-432.

2. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334(8):494-500.

3. Staykov D, Schwab S. Posterior reversible encephalopathy syndrome. J Intensive Care Med. 2012;27(1):11-24.

4. Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol. 2002;23(6):1038-1048.

5. Leroux G, Sellam J, Costedoat-Chalumeau N, et al. Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature. Lupus. 2008;17(2):139-147.

6. Mak A, Chan BP, Yeh IB, et al. Neuropsychiatric lupus and reversible posterior leucoencephalopathy syndrome: a challenging clinical dilemma. Rheumatology (Oxford). 2008;47(3):256-262.

7. Paulson OB, Strandgaard S, Edvinsson L. Cerebral autoregulation. Cerebrovasc Brain Metab Rev. 1990;2(2):161-192.

8. Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet. 2000;356(9227):411-417.

9. Arnoldus EP, Van Laar T. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334(26):1745; author reply 1746.

10. Datar S, Singh T, Rabinstein AA, Fugate JE, Hocker S. Long-term risk of seizures and epilepsy in patients with posterior reversible encephalopathy syndrome. Epilepsia. 2015;56(4):564-568.

11. Schwartz RB, Jones KM, Kalina P, et al. Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases. AJR Am J Roentgenol. 1992;159(2):379-383.

12. Roth C, Ferbert A. Posterior reversible encephalopathy syndrome: long-term follow-up. J Neurol Neurosurg Psychiatry. 2010;81(7):773-777.

This article originally appeared on Rheumatology Advisor