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Surgical and postpartum hereditary brachial plexus attacks and prophylactic immunotherapy.


ABSTRACT: Surgery and childbirth can trigger attacks of hereditary brachial plexus neuropathy (HBPN), and inflammation was suggested as a component of the pathogenesis.HBPN patients who underwent surgery or parturition from January 1, 1996 to December 31, 2009 were studied.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.Corticosteroids may prevent parturition and surgical HBPN attacks in some patients. Diverse surgeries, anesthesia, and childbirth frequently trigger HBPN attacks.

SUBMITTER: Klein CJ 

PROVIDER: S-EPMC3528817 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature

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Surgical and postpartum hereditary brachial plexus attacks and prophylactic immunotherapy.

Klein Christopher J CJ   Barbara David W DW   Sprung Juraj J   Dyck Peter J PJ   Weingarten Toby N TN  

Muscle & nerve 20121005 1


<h4>Introduction</h4>Surgery and childbirth can trigger attacks of hereditary brachial plexus neuropathy (HBPN), and inflammation was suggested as a component of the pathogenesis.<h4>Methods</h4>HBPN patients who underwent surgery or parturition from January 1, 1996 to December 31, 2009 were studied.<h4>Results</h4>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  ...[more]

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