Posterior Reversible Encephalopathy Syndrome due to High Dose Corticosteroids for an MS Relapse

Abstract

Increased blood pressure is a known adverse effect associated with corticosteroids but little is published regarding the risk with the high doses used in multiple sclerosis (MS). A 53-year-old female with known relapsing remitting MS presented with a new brainstem relapse. Standard of care treatment for an acute MS relapse, 1250 mg of oral prednisone for 5 days, was initiated. She developed an occipital headache and dizziness and felt generally unwell. These symptoms persisted after treatment was complete. On presentation to medical attention, her blood pressure was 199/110 mmHg, although she had no history of hypertension. MRI changes were consistent with posterior reversible encephalopathy syndrome (PRES), demonstrating abnormal T2 signal in both thalami, the posterior occipital and posterior parietal white matter with mild sulcal effacement. As her pressure normalized with medication, her symptoms resolved and the MRI changes improved. No secondary cause of hypertension was found. This is the first reported case of PRES secondary to high dose corticosteroid use for an MS relapse without a history of hypertension and with no other secondary cause of hypertension identified. This rare complication should be considered in MS patients presenting with a headache or other neurological symptoms during treatment for a relapse.

Corticosteroid therapy and severity of vasogenic edema in posterior reversible encephalopathy syndrome

“…Our finding of frequent steroid exposure prior to PRES is consistent with numerous case reports demonstrating a temporal association between steroid exposure and PRES [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48]. In our cohort and in the literature, steroid therapy is often co-administered with other potential culprit drugs or administered in the context of nephritis, which causes hypertension.…”

“…This is consistent with reports of PRES occurring after isolated exposure to steroids in non-autoimmune disorders, such as in asthma and brain metastasis [20, 24]. Similarly, PRES has been reported in patients receiving steroid therapy for autoimmune conditions that do not cause nephritis, such as neuromyelitis optica and multiple sclerosis [30, 34]. Among patients with lupus, PRES has been described after high-dose steroids in the absence of nephritis [5].…”

“…However, steroids are also used in the treatment of vasogenic edema, such as in patients with intracranial mass lesions [9]. Case reports have implicated steroids as both the precipitant [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48] and treatment [1, 6, 8, 12, 13, 18, 25, 28, 30, 33, 36, 37] of PRES. Thus, the role of steroids in PRES remains unclear.…”

Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study

“…It has been suggested that CSA may contribute to PRES in HLH, 37,38 but PRES has also been associated with other drugs, including corticosteroids. 39,40 Notably, encephalopathy is common in the natural course of HLH, and CSA has been reported to be beneficial in rheuma-associated HLH/macrophage activation syndrome. 41 In our data, we found no evidence of a marked increase of CSA-induced PRES.…”

Posterior reversible leukoencephalopathy syndrome (PRES) in intensive care unit – Case series

“…High-dose steroids, azathioprine, cyclophosphamide, asparaginase, and methotrexate have all been individually associated with PRES. [ 12 13 14 ] Some of our patients were on one or more of these medications when diagnosed with PRES. Since primary disease control is equally important, expert help is required to modulate the medications so as to control both, the disease and symptoms of PRES.…”