Posterior reversible encephalopathy as the first manifestation of Bickerstaff's brainstem encephalitis.
BACKGROUND
Posterior reversible encephalopathy syndrome (PRES) has been associated with Guillain-Barre syndrome in rare cases. Here we report a patient in whom PRES was the presenting manifestation of Bickerstaff's brainstem encephalitis.


METHODS
A 75-year-old woman presented with acute onset of hypertension, headache, blurred vision, and left eyelid drooping. Magnetic resonance imaging of the brain showed characteristic PRES lesions involving the parietal and occipital lobes bilaterally. On the 6th day after symptom onset, the patient developed complete ptosis and external ophthalmoplegia of both eyes, progressive ataxia, and bilateral lower limb weakness. Cerebrospinal fluid analyses revealed albuminocytological dissociation (protein: 66.6 mg/dL, WBC: 0/μl), and nerve conduction studies showed demyelinating sensorimotor polyneuropathy. The patient developed somnolence and a left extensor plantar response on the 8th day. A diagnosis of Bickerstaff's brainstem encephalitis was made. Treatment with plasmapheresis led to a rapid improvement of clinical symptoms. To date, only five similar cases have been reported, but this is the only case in which PRES developed prior to treatment.


CONCLUSIONS
PRES can be a comorbid condition with Bickerstaff's brainstem encephalitis, either preceding or following treatment; caution should be used in patients with either syndrome who exhibit atypical presentations.
Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, Taipei, 112, Taiwan.|Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan.
Pei-Ru
PR
Chen
Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, Taipei, 112, Taiwan. chensp1977@gmail.com.|Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan. chensp1977@gmail.com.|Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan. chensp1977@gmail.com.
Shih-Pin
http://orcid.org/0000-0003-3492-9902
SP
Chen
D000368:Aged; D001933:Brain Stem / Q000473:pathology; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009460:Neurologic Examination; D054038:Posterior Leukoencephalopathy Syndrome / Q000209:etiology*
Bickerstaff’s brainstem encephalitis; Guillain-Barre syndrome; Miller-Fisher syndrome; Posterior reversible encephalopathy syndrome