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”11 . Fox MD, Cohen AB. “Bright tongue sign” in ALS. Neurology 2012;79:1520. (Figure), in this context suggestive of amyotrophic lateral sclerosis (ALS). ALS represents the most common form of neurodegenerative motor neuron disease22 . Oliveira AS, Pereira RD. Amyotrophic lateral sclerosis (ALS): three letters that change the people’s life. Forever. Arq Neuropsiquiatr 2009;67:750-782.. Although clinical and ENMG findings are highly suspicious, neuroimaging studies commonly present with typical features representing compromise of pyramidal tracts33 . 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., sometimes with signs of fatty replacement in the chronic denervated target musculature11 . Fox MD, Cohen AB. “Bright tongue sign” in ALS. Neurology 2012;79:1520..
References
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1Fox MD, Cohen AB. “Bright tongue sign” in ALS. Neurology 2012;79:1520.
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2Oliveira AS, Pereira RD. Amyotrophic lateral sclerosis (ALS): three letters that change the people’s life. Forever. Arq Neuropsiquiatr 2009;67:750-782.
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3Agosta 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
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Publication in this collection
July 2014
History
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Received
17 Mar 2014 -
Reviewed
17 Apr 2014 -
Accepted
07 May 2014