The many faces of demyelinating diseases: acute disseminated encephalomyelitis and Guillain-Barré syndrome in the same patient

As várias faces das doenças desmielinizantes: encefalomielite aguda disseminada e síndrome de Guillain-Barré no mesmo paciente

Acute disseminated encephalomyelitis and Guillain-Barré syndrome represent distinct demyelinating diseases that share an autoimmune pathogenesis. A history of viral infection or vaccination are essential for the diagnosis¹,²,³.

A 26-month-old boy presented with fever 10 days after vaccination (inactivated polio vaccine and tetravalent), progressive drowsiness, lower limb strength/sensory loss and urinary retention. The cerebrospinal fluid showed mild pleocytosis and an elevated total protein concentration; it was negative for infections. The initial MRI study was compatible with acute disseminated encephalomyelitis, with no spine abnormalities (Figure 1).

Figure 1
Brain MRI revealed white matter hyperintensities lesions in T2/fluid- attenuated inversion recovery (FLAIR) (A, B) in the cerebral hemispheres and cerebellum, without restriction to water molecules diffusivity or gadolinium enhancement, suggestive of acute disseminated encephalomyelitis . Sagittal T1 SPIR post-gadolinium (C), without cauda equina root enhancement.

Ten days later, after therapy with corticosteroids, he experienced acute paraplegia. Follow-up brain and spine MRI scans demonstrated partial regression of brain lesions and nerve root thickening with intense enhancement extending along the cauda equina, compatible with Guillain-Barré syndrome. (Figure 2).

Figure 2
Follow-up MRI one week after steroids, showed partial recovery of the brain lesions; axial (A) and sagittal T1 SPIR post-gadolinium (B), enhancement of the cauda equina roots (white arrow), compatible with Guillain-Barré Syndrome.

References

• ¹
  Deshmukh IS, Bang AB, Jain MA, Vilhekar KY. Concurrent acute disseminated encephalomyelitis and Guillain-Barré syndrome in a child. J Pediatr Neurosci 2015;10(1):61-3. https://doi.org/10.4103/1817-1745.154357
  » https://doi.org/10.4103/1817-1745.154357
• ²
  Okumura A, Ushida H, Maruyama K, Itomi K, Ishiguro Y, Takahashi M et al. Guillain-Barré syndrome associated with central nervous system lesions. Arch Dis Child. 2002;86(4):304-6. https://doi.org/10.1136/adc.86.4.304
  » https://doi.org/10.1136/adc.86.4.304
• ³
  Mohamed RR, Jan MM. Co-morbid Guillain-Barré syndrome and acute disseminated encephalomyelitis. Neurosciences (Riyadh). 2013;18(2):166-8.

Publication Dates

• Publication in this collection
  May 2017

History

• Received
  31 Aug 2016
• Reviewed
  29 Dec 2016
• Accepted
  03 Feb 2017