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Case Report

Posterior reversible encephalopathy syndrome due

to seronegative systemic lupus erythematosus

Sawan Verma, Irfan Yousuf, Mushtaq Ahmad Wani, Ravouf Asimi, Sheikh Saleem, Mudasir Mushtaq,
Irfan Shah, Skeikh Nawaz, Riyaz Ahmad Daga
Department of Neurology, Sher-e-Kashmir Institute of Medical Sciences, Soura, Srinagar 190011, Jammu and Kashmir, India.

ABSTRA CT

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a unique computed tomography or magnetic
resonance imaging (MRI) appearance. Recognized in the setting of a number of complex conditions (preeclampsia/eclampsia,
allogeneic bone marrow transplantation, organ transplantation, autoimmune disease and high‑dose chemotherapy) in the imaging,
clinical and laboratory features of this toxic state are becoming better elucidated. We are presenting a case of PRES due to
seronegative systemic lupus erythematosus, with MRI findings of diffuse vasogenic edema.
Key words: Posterior reversible encephalopathy syndrome, seronegative systemic lupus erythematosus, vasogenic edema

INTRODUCTION Usually, it is a reversible phenomenon, as indicated
by the name, but if not recognized early and treated
Posterior reversible encephalopathy syndrome (PRES) appropriately, permanent brain injury may ensue. [3]
is a clinicoradiological entity that was well-described
by Hinchey et al. in 1996. This condition has been CASE REPORT
[1]
designated by a variety of names (reversible posterior
leukoencephalopathy syndrome, reversible posterior A 55-year-old female, known case of hypertension,
cerebral edema syndrome, and reversible occipital hypothyroidism, old treated pulmonary tuberculosis
parietal encephalopathy). PRES is now the accepted (12 years back) admitted with the chief complaints of
term but has been challenged recently based on the risk generalized swelling, recurrent vomiting and weakness
of neurological impairment and up to 15% mortality of all 4 limbs for 3 months and altered sensorium
rate. The most common clinical symptoms and signs for 1-week duration. Patient was initially evaluated
[2]
are headache, altered alertness and behavior ranging outside and following studies elicited no abnormalities:
from drowsiness to stupor, seizures, vomiting, mental complete blood count, kidney function tests, liver
abnormalities including confusion and diminished function tests, blood glucose, and electrolytes.
spontaneity and speech, and abnormalities of visual Abdominal ultrasonogram detects bilateral pleural
perception. Hypertension is the most commonly effusion (right > left side) and free fluid in pelvis.
[1]
identified cause of PRES, followed by medications, Nerve conduction studies of all 4 limbs revealed mild
eclampsia and other systemic factors. The asymmetrical large fiber, motor axonal neuropathy in
pathophysiology of hypertension related to PRES is due lower limbs > upper limbs, whereas electromyography
to a failure of cerebrovascular autoregulation, which revealed acute denervation in proximal and distal
in turn results in vasogenic edema. Nonhypertensive muscles of both lower limbs. Magnetic resonance
PRES may be due to an autoimmune or immune imaging (MRI) of spinal cord showed spondylotic
response to various stimuli. changes with mild disc dehydration and posterior
disc bulge at L4-L5 level. Vasculitic profile, including
Access this article online antinuclear antibody (ANA) three was negative. She was
Quick Response Code: managed with intravenous (i.v.) fluids, levothyroxine,
Website: antihypertensive, and i.v. methylprednisolone
www.nnjournal.net
(1 g daily for 5 days). She improved clinically to some
DOI: extent and was discharged. Soon within 2 days after
10.4103/2347-8659.139720 discharge, she became confused and agitated and was
brought to our department as a case of encephalopathy.

Corresponding Author: Dr. Sawan Verma, Department of Neurology, Sher‑e‑Kashmir Institute of Medical Sciences, Soura,
Srinagar 190011, Jammu and Kashmir, India. E‑mail: verma.sawan@gmail.com

Neuroimmunol Neuroinflammation | Volume 1 | Issue 2 | September 2014 89