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Central variant reversible encephalopathy syndrome

© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION A 28yearold female patient who did not have any systemic disease before presented with complaints of sudden onset of abnormal jerky movements of all limbs along with tongue bite lasting for about 10 min. She had history of caesarean section 1 week back. Neurological examination revealed no focal neurological deficit. Her routine blood investigations and cerebrospinal fluid analysis were normal. MRI of brain showed areas of altered signal intensity (areas of T2 and FLAIR hyperintensities) involving bilateral basal ganglia, along cortical and subcortical regions predominantly along posterior aspect involving bilateral parietooccipital lobes and left frontal lobe representing vasogenic oedema. There was also involvement of left cerebellar region and vermis (figure 1). These MRI changes were suggestive of vasogenic oedema secondary to cerebrovascular autoregulatory dysfunction which was consistent with the diagnosis of postpartum central variant of reversible encephalopathy syndrome (PRES). 2 The patient was treated with intravenous levetiracetam, intravenous phenytoin and supportive care, she responded well to treatment and did not have any further seizures or neurological issues. Repeat MRI brain on followup after 8 weeks revealed near complete resolution of the lesions (figure 2). The central variant of PRES has been described in past as ‘hypertensive brainstem oedema encephalopathy’, ‘hypertensive brainstem encephalopathy’, ‘reversible pontine oedema’, ‘reversible brainstem hypertensive encephalopathy’ or ‘brainstem hyperintensity in arterial hypertension’. These labels are confusing because PRES doesn’t need hypertension as a cause and it is not limited solely to brainstem involvement. Hence it is proposed that terms like ‘central variant PRES’ or ‘central reversible encephalopathy syndrome’ are more appropriate, particularly because central structures other than the brainstem can also be involved in this variant. Central variant PRES was reported for the first time in 2010 the setting of systemic lupus erythematosus. There are no established risk factors which predispose patients to atypical variant of PRES. As per available literature, the differential diagnosis for current case included inflammatory demyelination, osmotic myelinolysis syndrome, infiltrative glioma, lupusassociated central nervous system vasculitis, reversible cerebral vasoconstriction syndrome and rhomboencephalitis. The pathophysiological process in central variant PRES is suggested to be the endothelial cell dysfunction occurring within smaller, perforating vessels supplying the brainstem and basal ganglia.