A Symptoms and Signs Approach to the Patient With Neuromuscular Weakness

Nicholas J. Silvestri, MD, FAAN Muscle and Neuromuscular Junction Disorders p. 1580-1595 December 2022, Vol.28, No.6 doi: 10.1212/CON.0000000000001150
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PURPOSE OF REVIEW Muscle weakness is a common feature of many neuromuscular disorders. This article outlines a symptoms and signs approach to the patient presenting with neuromuscular weakness, highlighting key aspects of the clinical history and examination.

RECENT FINDINGS The past several years have seen a dramatic increase in the ability to test for many inherited and autoimmune neuromuscular disorders more reliably and accurately. Similarly, numerous targeted therapies have been recently approved to treat previously untreatable disorders. Therefore, timely and accurate diagnosis is essential so that patients can receive appropriate therapy, ultimately leading to better clinical outcomes.

SUMMARY Muscle weakness is a common symptom resulting from dysfunction that can occur at any level of the neuraxis and is a cardinal feature of many neuromuscular disorders. An accurate and meticulous history and a thorough neurologic examination are paramount in localizing the lesion in order to generate a differential diagnosis and guide appropriate ancillary testing. The patient’s age at symptom onset, any identified inciting factors, tempo of symptom progression, pattern of weakness, and associated symptoms and signs are all important diagnostic clues in the evaluation of a patient presenting with muscle weakness.

Address correspondence to Dr Nicholas Silvestri, University at Buffalo, Jacobs School of Medicine & Biomedical Sciences, 955 Main St, Buffalo, NY 14203, [email protected].

RELATIONSHIP DISCLOSURE: Dr Silvestri has received personal compensation in the range of $500 to $4999 for serving on a scientific advisory or data safety monitoring board or on a speakers bureau for Alexion Pharmaceuticals, argenx, Immunovant, Inc., Regeneron Pharmaceuticals, Sarepta Therapeutics, UCB, and Xeris Pharmaceuticals. Dr Silvestri has received publishing royalties from a publication relating to health care.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Silvestri reports no disclosure.

© 2022 American Academy of Neurology.