Submitted by Rebecca O'Bryan, MD
Edited by Clark Pinyan, MD
Carpal Tunnel Syndrome: A Potential Early, Red-Flag Sign of Amyloidosis.
Joseph P. Donnelly MD; Mazen Hanna MD; Brett W.Sperry MD; William H.Seitz Jr MD
J Hand Surg Am.
This article reports on the clinical significance of certain presentations of carpal tunnel syndrome as they relate to the potential earlier diagnosis of amyloidosis in patients with systemic disease. Due to the importance of diagnosing systemic amyloidosis (either light chain or transthyretin), a high index of suspicion is vital in identification and early treatment of these patients.
Presentation of bilateral carpal tunnel syndrome and multiple releases are identified as “red flag” symptoms in men over 50 and women over 60. Biopsy in conjunction with carpal tunnel surgery can reveal amyloid precursor proteins and amyloid deposition both in the connective tissue and within the median nerve itself. With ATTR patients, endoneurial amyloid deposits in the median nerve may directly lead to median neuropathy (as opposed to entrapment as the etiology). Carpal tunnel surgery frequently precedes diagnosis of amyloidosis by many years.
Other concomitant diagnoses may bolster clinical suspicion for systemic amyloidosis (spinal stenosis, biceps tendon rupture, afib/flutter, pacemaker, CHF, family history of ATTR). An algorithm is presented to help provide guidance of value of biopsy in the context of carpal tunnel release.
Much of the article provides background to the less exposed hand surgeon regarding pathophysiology of the amyloidosis patient, but there is a good review of literature providing background as to the value of a high index of suspicion in a certain population with regards to bilateral and repeat patients with carpal tunnel syndrome. The algorithm is again presented in the context of guidance for the surgical specialist, but this history would be available to the EDX or neuromuscular physician as well, and could be used as a screening tool in the context of providing recommendations within the surgical referral, as well as educational guidance in the context of the neuromuscular specialist to the surgical teams with which we interface and overlap.
This article is an excellent reminder to the EDX physician that a patient presenting with bilateral CTS, especially in the context of previous history of release, a more detailed screening history may be valuable in recommending to the surgical team that a biopsy be sent to potentially identify amyloidosis patients early in the process. This may allow for an earlier intervention in the disease progression, possibly significantly improving prognosis and quality of life in this patient population.