Comment

Amyotrophic Lateral Sclerosis (ALS): A progressive neurodegenerative disorder affecting both upper and lower motor neurons. Note the muscle wasting of the thenar eminence and palm in picture on left. The picture on the right demonstrates atrophy of intrinsic muscles of the hands (between the fingers), particularly evident in the anatomic snuff box (between the thumb and index fingers). With denervation and atrophy, the hand assumes a claw-like appearance. The wrist extensors are so weak that the hand can't be maintained in neutral position when held outward.

Usual age of onset for ALS is between 40-70. Symptoms include progressive motor weakness, breathy voice, shortness of breath, difficulty swallowing, weak cough. Primarily Bulbar symptoms are present in ~25% of patients, while extremity symptoms predominate in ~75%. Exam findings include a mixture of flaccid weakness (LMN) and spasticity (UMN), hyperreflexia, fasciculations of any muscles, muscle wasting. Sensory system function is preserved.

Image Credit

Charlie Goldberg, M.D.