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   Table of Contents      
CASE REPORT
Year : 2015  |  Volume : 1  |  Issue : 3  |  Page : 167-168

Life threatening posterior reversible encephalopathy syndrome in systemic lupus erythematosus


1 Department of Medicine, University College of Medical Sciences, New Delhi, India
2 Department of Rheumatology, CMC Vellore, Tamil Nadu, India

Date of Web Publication30-Sep-2015

Correspondence Address:
Nikhil Gupta
Department of Medicine, University College of Medical Sciences, New Delhi - 110 095
India
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Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.


DOI: 10.4103/2394-7438.166301

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  Abstract 


Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by headache, altered mental status, seizure and visual dysfunction, and transient motor deficit. The incidence of PRES in systemic lupus erythematosus (SLE) has not been clearly known. Risk factors for PRES in SLE include hypertension, renal failure, cytotoxic drugs, and active lupus. We describe a patient of SLE with nephritis on cyclophosphamide monthly pulse that developed PRES and, unfortunately, succumbed to her illness.

Keywords: Encephalopathy, lupus, posterior reversible encephalopathy syndrome, systemic lupus erythematosus


How to cite this article:
Sahu SK, Giri S, Gupta N. Life threatening posterior reversible encephalopathy syndrome in systemic lupus erythematosus . MAMC J Med Sci 2015;1:167-8

How to cite this URL:
Sahu SK, Giri S, Gupta N. Life threatening posterior reversible encephalopathy syndrome in systemic lupus erythematosus . MAMC J Med Sci [serial online] 2015 [cited 2019 Dec 5];1:167-8. Available from: http://www.mamcjms.in/text.asp?2015/1/3/167/166301




  Case Report Top


A 20-year-old female patient presented to us with swelling over the face and feet associated with erythematous rashes over the malar area for 15 days. She had decreased urine output 2 days prior to hospitalization. There was no other significant history. On examination, she was conscious, cooperative and oriented at the time of presentation. Her vitals were normal with a blood pressure of 120/70 mm Hg at the time of admission and 116/80 mm Hg at the time the patient developed seizures. She was pale and had an erythematous rash over malar areas extending on to the nasal bridge. The rest of the general physical examination was normal. Systemic examination was essentially normal. Her hemoglobin was 8 g/dl and thrombocytes count was 70,000/cumm. Blood urea was 100 mg/dl and serum creatinine of 3 mg/dl. Urine routine examination showed 3 + proteinuria and 20–30 red blood cells/high power field with 24 h urinary protein being 1,900 mg. Serum antinuclear antibody titer was 1:40 and antiphospholipid antibody syndrome work up was negative. However, anti-dsDNA was raised, and complement levels of C3 and C4 were low. Renal biopsy was consistent with WHO Class IV lupus nephritis.

She was treated with injection methylprednisolone 1 gm/day IV for three consecutive days along with injection cyclophosphamide 1 g monthly pulse therapy, followed by maintenance dose of oral prednisolone 1.5 mg/kg/day. With this treatment, her condition gradually improved clinically, but after 5 weeks (during the same hospital stay) she abruptly developed recurrent seizures and went into an altered mental state. Magnetic resonance imaging (MRI) of brain showed areas of hypointensities on T1-weighted images and hyperintensities on T2-weighted/fluid attenuation inversion recovery images, with no significant restricted diffusion, involving both grey and white matter in bilateral frontal and parietal regions [Figure 1] and in periventricular, corpus callosum, basal ganglia and occipital regions [Figure 2]. Other areas involved were centrum semiovale, pons, and both cerebellar hemisphere.
Figure 1: Magnetic resonance imaging of brain showing areas of hyperintensities on T2-weighted/fluid attenuation inversion recovery images with no significant restricted diffusion involving both grey and white matter in bilateral frontal and parietal region

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Figure 2: Magnetic resonance imaging of brain showing areas of hyperintensities on T2-weighted/fluid attenuation inversion recovery images with no significant restricted diffusion involving both grey and white matter in periventricular, corpus callosum, basal ganglia, and occipital regions

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MRI of the brain was consistent with features of PRES. Cerebrospinal fluid (CSF) examination was performed, which revealed five cells all lymphocytes, a protein of 25 mg and sugar of 50 mg. There were no oligoclonal bands or IgG in the CSF. CSF was also sterile and negative for viral serology. Methylprednisolone 1 g IV was repeated for 3 days and the second pulse of cyclophosphamide was given. The episode was so dramatic that in spite of all appropriate measures, she succumbed to her illness in 72 h of onset of the central nervous system symptoms.

Discussion

CSF examination is not needed in PRES. However, in the setting of SLE in which other causes of the neurological disease have to be ruled out, the CSF analysis serves as an important differential diagnostic tool.[1],[2]

PRES has been reported in association with multiple conditions which include preeclampsia, eclampsia, sepsis, bone marrow, and solid organ transplant, and with the use of cytotoxic and immunosuppressive drugs such as cyclophosphamide, autoimmune diseases (SLE, systemic sclerosis, and polyarteritis nodosa) and electrolyte disturbances as hypercalcemia.[3]

PRES may present with an acute or subacute manifestations such as headache, mental status impairment ranging from mild somnolence to confusion, agitation, and even coma.[3] The patient may present with seizures usually generalized tonic-clonic. Visual disturbances such as hemianopia, visual hallucinations, and cortical blindness may also occur.[3] Hypertension occurs in 66–85% of patients with PRES.[4]

PRES must be diagnosed early, and causative factors should be identified. Symptomatic treatment should be given immediately, and the causative factors corrected. PRES is usually a benign condition; however, it may lead to brain infarctions or hemorrhages with permanent neurological sequelae or even death.[5]

Death in PRES is a rare event. Death has been reported in up to 15% of patients.[6] Poor prognostic markers include extensive - lesions, an extension of edema, brain herniation, cytotoxic edema on diffusion-weighted imaging and presence of hemorrhages.[7]

Some authors have suggested a better name may be "potentially reversible encephalopathy syndrome" instead of "PRES".[8] It is true that PRES is mostly reversible if promptly recognized and adequately treated for most patients. However, it is not a rule, and some patients may have an adverse outcome, in spite of a prompt correct therapy.[9]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Burnett MM, Hess CP, Roberts JP, Bass NM, Douglas VC, Josephson SA. Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors. Clin Neurol Neurosurg 2010;112:886-91.  Back to cited text no. 1
    
2.
Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: Fundamental imaging and clinical features. Am J Neuroradiol 2008;29:1036-42.  Back to cited text no. 2
    
3.
Pedraza R, Marik PE, Varon J. Posterior reversible encephalopathy syndrome: A review. Crit Care Shock 2009;12:135-43.  Back to cited text no. 3
    
4.
Barber CE, Leclerc R, Gladman DD, Urowitz MB, Fortin PR. Posterior reversible encephalopathy syndrome: An emerging disease manifestation in systemic lupus erythematosus. Semin Arthritis Rheum 2011;41:353-63.  Back to cited text no. 4
    
5.
Shaharir SS, Remli R, Marwan AA, Said MS, Kong NC. Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: Pooled analysis of the literature reviews and report of six new cases. Lupus 2013;22:492-6.  Back to cited text no. 5
    
6.
Mak A, Chan BP, Yeh IB, Ho RC, Boey ML, Feng PH, et al. Neuropsychiatric lupus and reversible posterior leucoencephalopathy syndrome: A challenging clinical dilemma. Rheumatology (Oxford) 2008;47:256-62.  Back to cited text no. 6
    
7.
Saeed MU, Dacuycuy MA, Kennedy DJ. Posterior reversible encephalopathy syndrome in HIV patients: Case report and review of the literature. AIDS 2007;21:781-2.  Back to cited text no. 7
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8.
Narbone MC, Musolino R, Granata F, Mazzù I, Abbate M, Ferlazzo E. PRES: Posterior or potentially reversible encephalopathy syndrome? Neurol Sci 2006;27:187-9.  Back to cited text no. 8
    
9.
Antunes NL, Small TN, George D, Boulad F, Lis E. Posterior leukoencephalopathy syndrome may not be reversible. Pediatr Neurol 1999;20:241-3.  Back to cited text no. 9
    


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