High dose intravenous immune globulin in the treatment of hereditary recurrent brachial plexus neuropathy

We read with interest the article by Klein et al 1 providing pathological evidence that the neuropathic attacks in hereditary brachial plexus neuropathy (HBPN) are secondary to an inflammatory process. As a possible pathogenetic mechanism the authors suggest altered immune modulation. In the absence of controlled clinical trials they treated patients with intravenous methyl prednisolone. This treatment—in their experience—relieved the symptoms, particularly the pain, for a brief time, but as they tapered the corticosteroid dose the signs and symptoms reappeared. The authors concluded that in some cases of HBPN an inflammatory multifocal response arising from an immune dysfunction in the brachial plexus and upper limb nerves causes nerve abnormalities and axonal degeneration. Treatments based on immune modulation may therefore be useful in the management of HBPN. To further support the immunological pathogenesis we describe a 13 year old girl who since childhood had suffered from recurrent episodes of severe asymmetric pain and weakness of the shoulder and arm involving the same as well as the opposite side. Her father had similar attacks and genetic testing for hereditary neuropathy with liability to pressure palsy was negative. At the age of 4 years the girl experienced severe left shoulder pain that lasted for 20 days and was treated with corticosteroids. Three years later, a left deltoid muscle hypotrophy …