Guillain Barré syndrome-related posterior reversible encephalopathy syndrome.

Abstract

Dear Sir, Reversible posterior encephalopathy syndrome (PRES), first described by Hinchey et al. in 1996, is a neurological condition characterized by headache, nausea/ vomiting, seizures, visual field disturbances, altered sensorium, decreased alertness, and focal neurological deficits. The main finding of PRES on magnetic resonance imaging (MRI) is usually bilateral, often symmetric, posterior white matter hyperintensity on T2 weighted images, consistent with vasogenic edema [1]. PRES can develop in association with a variety of conditions that may cause acute alteration of blood pressure such as preeclampsia/eclampsia, as well exposure to chemotherapeutic drugs. Concurrent occurrence of GuillainBarre syndrome (GBS) with PRES is a rare entity and only a few cases have been reported. GBS is considered a post-infectious syndrome triggered by different organisms or illnesses. The pathophysiology of the disease is most likely due to molecular mimicry. We report the unique case of PRES in a child with EpsteinBarr virus (EBV) infection-related GBS. A 5-year-old male was first referred to the hospital for several days of back pain and lower extremity weakness. He had a history of fever a few days prior to presentation. His exam revealed bilateral lower extremity weakness; however, his exam was limited by severe low back pain. He had diminished reflexes, a somewhat shuffling gait and lymphadenopathy. Laboratory findings revealed an elevated white cell count with the presence of atypical lymphocytes. This prompted testing for a possible EBV infection and a positive result was found for EBV viral capsid and antigen-IgG and IgM antibodies. MRI of the lumbar spine and pelvis was unremarkable. He returned to the hospital 8 days later with progressive difficulty standing and walking with evidence of dysarthia and dysphagia. A second MRI of the spine 1 week later revealed cervical lymphoadenopahy, elarged spleen, and abnormal contrast enhancement of the cauda equina roots and exiting nerve roots at the lumbar level. There was evidence of abnormal contrast enhancement of some of the exiting cervical and thoracic nerve roots bilaterally at multiple levels (Fig. 1a–b). Cerebrospinal fluid (CSF) analysis showed albumincytological dissociation with elevated CSF and 15 white cells. Nerve conduction studies revealed a severe demyelinating sensory motor polyneuropathy. The combination of clinical, laboratory, and radiological findings favored the diagnosis of GBS. The patient was admitted to the Pediatric Intensive Care Unit for close monitoring. During the second day of hospitalization, he was noted to have somwhat elevated blood pressure 170 s–180 s/100. He also developd some decreased responsiveness and respiratory difficulty which prompted endotracheal intubation. The MRI of the brain obtained on the same day showed vasogenic edema involving the parietooccipital and frontal lobes with abnormal contrast enhancement at the grey-white matter junction, suggestive of PRES (Fig. 1c). Intravenous immunoglobulin (IVIG) infusion was G. Zuccoli (*) Section of Neuroradiology, Children’s Hospital of Pittsburgh of the University of Pittsburgh Medical Center, 4401 Penn Avenue, Floor 2, Pittsburgh, PA 15224, USA e-mail: giulio.zuccoli@gmail.com