Brainstem reversible leukoencephalopathy syndrome

Síndrome da leucoencefalopatia reversível de tronco cerebral

A 39-year-old man presented with progressive headache, nauseas, blurriness and cortical blindness. Blood pressure: 230x140 mmHg. Glasgow score: 15. Fundoscopy revealed hypertensive retinopathy and papille- dema. Neurological examination was normal. Brain magnetic resonance imaging revealed area of swelling and high signal on FLAIR-weighted images on the brainstem and cerebral white-matter abnormalities ( Figure 1 ). Hypertension management improved his clinical and radiological findings ( Figure 2 ).

Figure 1
. Initial brain magnetic resonance imaging revealed area of swelling and high signal on the brainstem (mainly in pons and medulla oblongata) and cerebral white-matter abnormalities, which are compatible with the brainstem variant of posterior reversible leukoencephalopathy syndrome (A, FLAIR-weighted images; B, diffusion-weighted images; C, T1-weighted images after contrast infusion; D, apparent diffusion coefficient).

Figure 2
. After 6 months of the hypertension management, follow-up brain magnetic resonance imaging showed regression of vasogenic edema that was graded as complete in brainstem (A, FLAIR-weighted images; B, diffusion-weighted images).

Posterior reversible leukoencephalopathy syndrome (PRES), with classical reversible cerebral vasogenic edema, occurred predominantly in the posterior distribution (occipital and parietal lobes) on brain imaging ^{1 - 3} . The brainstem involvement is an atypical feature which may be encountered in midbrain (13%), pons (20%) and medulla oblongata (5%) ^{1 - 3} . This PRES variant should be differentiated from brainstem infarction, pontine glioma, infective encephalitis, central pontine myelinolysis and others demyelinating disorders because PRES is potentially full reversible after treatment ^{1 - 3} .

References

• ¹
  Kitaguchi H, Tomimoto H, Miki Y, et al. A brainstem variant of reversible posterior leukoencephalopathy syndrome. Neuroradiology 2005;47:652-656.
• ²
  Liman TG, Bohner G, Heuschmann PU, Endres M, Siebert E. The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study. J Neurol 2012;259:155-164.
• ³
  Stevens CJ, Heran MK. The many faces of posterior reversible encephalopathy syndrome. Br J Radiol 2012;85:1566-1575.

Publication Dates

• Publication in this collection
  Jan 2014

History

• Received
  05 Feb 2013
• Reviewed
  11 July 2013
• Accepted
  18 July 2013