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Table of Contents    
NEUROIMAGE
Year : 2022  |  Volume : 70  |  Issue : 2  |  Page : 824-825

Bright Tongue Sign in Amyotrophic Lateral Sclerosis


1 Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
2 Department of General Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Submission26-Jul-2020
Date of Decision02-Aug-2020
Date of Acceptance14-Aug-2020
Date of Web Publication3-May-2022

Correspondence Address:
Dr. Sarbesh Tiwari
Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur - 342 008, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.344609

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How to cite this article:
Saxena S, Tiwari S, Khera PS, Midha NK. Bright Tongue Sign in Amyotrophic Lateral Sclerosis. Neurol India 2022;70:824-5

How to cite this URL:
Saxena S, Tiwari S, Khera PS, Midha NK. Bright Tongue Sign in Amyotrophic Lateral Sclerosis. Neurol India [serial online] 2022 [cited 2022 Jul 3];70:824-5. Available from: https://www.neurologyindia.com/text.asp?2022/70/2/824/344609




A 55-year-old woman presented with bilateral muscle cramps, difficulty in walking upstairs, and inability to maintain the posture while sitting without support progressing over a span of 2 years. She also complained of difficulty in fine movements of hands and rhythmic sensation over the tongue for 3 months. She also reported difficulty swallowing food and slurring of speech for the last 1 month. There was no history of any sensory symptoms and visual disturbance. On clinical examination, she was found to have diffuse atrophy of bilateral upper and lower limb muscles, facial muscle weakness, dysarthria with tongue weakness, and fasciculations. Nerve conduction study revealed distal symmetrical motor axonal polyneuropathy in the upper limb and lower limb. Electromyography (EMG) was consistent with active on chronic denervation in upper-lower limb nerves and cranial nerves. Based on clinical examination, symptomatology, and EMG findings diagnosis of Amyotrophic lateral sclerosis was made with an ALS functional rating score (revised) of 22/48.

MRI brain revealed marked T1 hyperintensity involving the tongue with suppression of signal on the fat saturated T1 images suggesting fatty replacement of tongue musculature secondary to chronic denervation. There was significant reduction in the bulk of the tongue musculature with rectangular rather than the usual elliptical shape of tongue [Figure 1].[1],[2] MRI imaging of brain did not reveal any other remarkable findings. The MRI-based T1 tongue intensity appears to be a marker of bulbar involvement in ALS. Imaging differentials with bright tongue on MRI include ALS, Pompe's disease[3] and Kennedy disease.[4]
Figure 1: The sagittal non fat-saturated T1 image (a) shows atrophy and hyperintense signal within the tongue (white arrow) secondary to fatty replacement giving a “bright tongue” appearance. The axial T2 image (b) confirms the hyperintensity within the intrinsic muscles of the tongue. Note the suppression of hyperintense signal on the T2 fat saturated images (c). The image (d) is T1 sagittal image of an age matched normal individual showing the normal bulk and signal of the tongue

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  References Top

1.
Fox MD, Cohen AB. “Bright tongue sign” in ALS. Neurology 2012;79:1520.  Back to cited text no. 1
    
2.
Konagaya M, Konagaya Y, Konishi T, Mano Y. MRI findings of the tongue in neurodegenerative diseases with bulbar sign. Rinsho Shinkeigaku 1990;30:665-7.  Back to cited text no. 2
    
3.
Karam C. Bright tongue sign in Pompe disease. Neurology 2016;86:401.  Back to cited text no. 3
    
4.
Lin X, Wu S. Bright tongue sign in Kennedy disease. QJM 2019;113:45-6.  Back to cited text no. 4
    


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