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.
Highlights
Posterior reversible encephalopathy syndrome (PRES) has been associated with Guillain-Barre syndrome in rare cases
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
Our patient presented a clinical and neuroradiological pattern characteristic of PRES preceding a constellation of neurological symptoms, including complete ptosis, external ophthalmoplegia, progressive ataxia, weakness of the lower limbs bilaterally, altered consciousness, and a left extensor plantar reflex, which were consistent with Bickerstaff ’s brainstem encephalitis with overlapping Guillain-Barre syndrome (GBS) [9]
Summary
Our patient presented a clinical and neuroradiological pattern characteristic of PRES preceding a constellation of neurological symptoms, including complete ptosis, external ophthalmoplegia, progressive ataxia, weakness of the lower limbs bilaterally, altered consciousness, and a left extensor plantar reflex, which were consistent with Bickerstaff ’s brainstem encephalitis with overlapping GBS [9]. Three previous cases have shown the co-occurrence of MFS or MFS/ BBE-overlap syndrome and non-hypertensive PRES; in these cases, IVIG treatment was considered to be the culprit of PRES [4, 5, 12]. One recent case demonstrated extensive vasogenic edema in BBE without any intervention; whether the vasogenic edema of the deep white matter, brainstem, and cerebellum was attributable to PRES is uncertain [13]. The etiology of SIADH in our patient was uncertain, but we considered it to be comorbidity attributed to her Bickerstaff’s brainstem encephalitis. The accurate rate of co-occurrence of these two syndromes remains to be explored in large-scale prospective studies, a comorbid PRES should be considered in patients with initial signs of GBS or its spectral disorders, especially in those with a blood pressure surge or visual field defect
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