Posterior reversible encephalopathy as the first manifestation of Bickerstaff’s brainstem encephalitis

Abstract

BackgroundPosterior 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.Case presentationA 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.ConclusionsPRES 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.

Posterior reversible encephalopathy syndrome, a clinically diverse and challenging disorder

“…Three of these cases developed PRES following IVIG. The remaining two, similar to our case, manifested PRES prior to developing MFS symptomatology and therapeutic intervention …”

“…Patient‐3 manifested PRES symptoms prior to developing clinical characteristics of MFS; furthermore, his condition improved following IVIG therapy. To the best of our knowledge, there are only five cases reporting comorbidity of PRES and MFS . Three of these cases developed PRES following IVIG.…”

“…Posterior reversible encephalopathy syndrome is rarely associated with Guillain‐Barre syndrome (GBS) and its different variants. The onset of PRES can either overlap with the clinical manifestations of GBS or, albeit rarely, precede it . PRES in GBS patients is more commonly associated with IVIG therapy than as a direct consequence of the immunopathophysiology of GBS .…”

Bickerstaff Brainstem Encephalitis Presenting With Negative Anti-GM1 and Anti-GQ1B Antibodies

“…Previous case reports highlight similar presentations characterised by a positive Babinski sign, ophthalmoplegia, and slurred speech [ 15 ]. Other documented cases provide valuable insights into the varied manifestations of BBE, showcasing instances of overlap with GBS, posterior reversible encephalopathy syndrome, and occurrences linked to chlamydia infection [ 16 , 17 ]. The pathophysiology of BBE is emphasised as autoimmune-mediated peripheral nerve damage triggered by antecedent infections, in various case reports [ 18 ].…”