"My Tongue is Dancing"

 

Patient History

A 47-year-old male presents to the physician complaining that "my tongue is dancing".  He has a 1-year history of difficulty swallowing solids and liquids as well as a decrease in the volume of his voice. Physical examination reveals weakness and atrophy of his leg muscles bilaterally and mildly slurred speach.  His sensory functions are intact.  Electromyography shows evidence of active and chronic denervation.

 

Q1: Which of the following is the most likely diagnosis?







Please answer the question.

 

Q2: Which of the following is the most likely histologic finding?                            







Please answer the question.

 

Q3: Which of the following medications may be helpful for this condition?                           






Please answer the question.

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Question 1 Answer

Question 1:  Amyotrophic lateral Sclerosis

Tongue atrophy and fasciculations are among the bulbar symptoms of amyotrophic lateral sclerosis. Here, tongue fasciculations are seen in a 47-year-old man with the disease. The bulbar symptoms of ALS include tongue atrophy and fasciculations, dysarthria, hypophonia, dysphagia, and sialorrhea.

Question 2 Answer

Question 2:  TDP-43

A hyperphosphorylated and ubiquitinated TAR DNA-binding protein-43 (TDP-43) is found in the neurons of ALS and Frontotemportal dementia.

Question 3 Answer

Question 3:  Riluzole

Riluzole was the first drug approved for ALS and may extend survival by 2-3 months.

 

Video source:  NEJM videos

Comments  

#1 Suzanne Havican 2016-08-28 18:10
Dear Dr. Barone,
Thank you for this great website and a fun way to sear some juicy knowledge into the hungry and thirsty USMLE brain appetite!! :D
Quote
Category: CNS

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