Bright tongue sign: a diagnostic marker for amyotrophic lateral sclerosis

Sinal da língua brilhante: um marcador diagnóstico para esclerose lateral amiotrófica

A 53-year-old woman complained about a 2-year-history of progressive tetraparesia with global brisk reflexes, global amyotrophy and fasciculations. As her clinical picture suggested a motor neuron disease, she performed electroneuromyography (ENMG) unvealing anterior horn compromise and brain MRI study with the so-called “bright tongue sign”¹ (Figure), in this context suggestive of amyotrophic lateral sclerosis (ALS). ALS represents the most common form of neurodegenerative motor neuron disease². Although clinical and ENMG findings are highly suspicious, neuroimaging studies commonly present with typical features representing compromise of pyramidal tracts³, sometimes with signs of fatty replacement in the chronic denervated target musculature¹.

Figure
. Sagittal T1-weighted brain MRI discloses abnormal diffuse hyperintensity of the tongue musculature (A), correspondent to severe atrophic tongue (B). Normal tongue MRI feature is showed (C).

References

• ¹
  Fox MD, Cohen AB. “Bright tongue sign” in ALS. Neurology 2012;79:1520.
• ²
  Oliveira AS, Pereira RD. Amyotrophic lateral sclerosis (ALS): three letters that change the people’s life. Forever. Arq Neuropsiquiatr 2009;67:750-782.
• ³
  Agosta F, Chiò A, Cosottini M, et al. The present and future of neuroimaging in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol 2010;31:1769-1777.

Publication Dates

• Publication in this collection
  July 2014

History

• Received
  17 Mar 2014
• Reviewed
  17 Apr 2014
• Accepted
  07 May 2014