Surgical and postpartum hereditary brachial plexus attacks and prophylactic immunotherapy

Area of Science:

• Neurology
• Immunology
• Genetics

Background:

• Hereditary brachial plexus neuropathy (HBPN) attacks can be triggered by surgery and childbirth.
• Inflammation is implicated in the pathogenesis of HBPN.

Purpose of the Study:

• To investigate the occurrence and characteristics of HBPN attacks following surgical procedures and parturition.
• To evaluate the efficacy of prophylactic immunosuppressive therapy in preventing such attacks.

Main Methods:

• Retrospective study of 25 HBPN patients who underwent 48 surgeries or parturitions between 1996 and 2009.
• Analysis of attack occurrence, timing (peri-procedural, postpartum), anesthesia types, and patient outcomes.
• Evaluation of prophylactic immunosuppressive therapy (corticosteroids ± immunoglobulin) in a subset of patients.

Main Results:

• 17 out of 25 patients (68%) experienced HBPN attacks, with 13 periprocedural and 7 postpartum.
• Prophylactic immunosuppressive therapy in 3 patients with a history of recurrent attacks prevented postoperative episodes.
• Attacks occurred in or distant to the surgical site, frequently impacting daily living with incomplete recovery.
• Five patients presented with perioperative attacks as their initial manifestation of HBPN.
• A SEPT9 mutation was identified in five patients.

Conclusions:

• Surgery and childbirth are frequent triggers for HBPN attacks.
• Corticosteroids may serve as a prophylactic measure against parturition and surgical HBPN attacks in susceptible individuals.