INTRODUCTION: Surgery and childbirth can trigger attacks of hereditary brachial plexus neuropathy (HBPN), and inflammation was suggested as a component of the pathogenesis. METHODS: HBPN patients who underwent surgery or parturition from January 1, 1996 to December 31, 2009 were studied. RESULTS: Twenty-five HBPN patients underwent 48 surgeries or parturitions. Seventeen patients (68%) had attacks, including 13 periprocedural and 7 postpartum by varied anesthesia types. Three patients who had 8 earlier combined attacks (after thyroidectomy, laminectomy, and Caesarean section) were given prophylactic immunosuppressive therapy (corticosteroids ± immunoglobulin). None suffered postoperative attacks, which is uncharacteristic of their prior experience. Five had perioperative attacks as their first HBPN manifestation. Median follow-up was 11 months (3-48 months). Attacks occurred in the operated limb (n = 6) or distant (n = 7) to surgical sites. All attacks interfered with daily living, with frequent incomplete recovery. Five patients had a SEPT9 mutation. CONCLUSIONS: Corticosteroids may prevent parturition and surgical HBPN attacks in some patients. Diverse surgeries, anesthesia, and childbirth frequently trigger HBPN attacks.
INTRODUCTION: Surgery and childbirth can trigger attacks of hereditary brachial plexus neuropathy (HBPN), and inflammation was suggested as a component of the pathogenesis. METHODS: HBPN patients who underwent surgery or parturition from January 1, 1996 to December 31, 2009 were studied. RESULTS: Twenty-five HBPN patients underwent 48 surgeries or parturitions. Seventeen patients (68%) had attacks, including 13 periprocedural and 7 postpartum by varied anesthesia types. Three patients who had 8 earlier combined attacks (after thyroidectomy, laminectomy, and Caesarean section) were given prophylactic immunosuppressive therapy (corticosteroids ± immunoglobulin). None suffered postoperative attacks, which is uncharacteristic of their prior experience. Five had perioperative attacks as their first HBPN manifestation. Median follow-up was 11 months (3-48 months). Attacks occurred in the operated limb (n = 6) or distant (n = 7) to surgical sites. All attacks interfered with daily living, with frequent incomplete recovery. Five patients had a SEPT9 mutation. CONCLUSIONS: Corticosteroids may prevent parturition and surgical HBPN attacks in some patients. Diverse surgeries, anesthesia, and childbirth frequently trigger HBPN attacks.
Authors: Gregor Kuhlenbäumer; Mark C Hannibal; Eva Nelis; Anja Schirmacher; Nathalie Verpoorten; Jan Meuleman; Giles D J Watts; Els De Vriendt; Peter Young; Florian Stögbauer; Hartmut Halfter; Joy Irobi; Dirk Goossens; Jurgen Del-Favero; Benjamin G Betz; Hyun Hor; Gert Kurlemann; Thomas D Bird; Eila Airaksinen; Tarja Mononen; Adolfo Pou Serradell; José M Prats; Christine Van Broeckhoven; Peter De Jonghe; Vincent Timmerman; E Bernd Ringelstein; Phillip F Chance Journal: Nat Genet Date: 2005-09-25 Impact factor: 38.330
Authors: C J Klein; P J B Dyck; S M Friedenberg; T M Burns; A J Windebank; P J Dyck Journal: J Neurol Neurosurg Psychiatry Date: 2002-07 Impact factor: 10.154
Authors: P J Dyck; W R Sherman; L M Hallcher; F J Service; P C O'Brien; L A Grina; P J Palumbo; C J Swanson Journal: Ann Neurol Date: 1980-12 Impact factor: 10.422