Table 1

Clinical features, course, and treatment in four cases of hereditary brachial plexus neuropathy

PatientAge (y) at 1st attack/ sexAge (y) at biopsyNo of episodesClinical features of current attackCSFHNPP gene testingCT/MRI plexusFamily members affectedTreatment of biopsied attackTreatment response
*Patient's father's history, examination, and nerve conduction/EMG studies (performed by us) consistent with recurrent brachial plexopathy.
BPN, brachial plexus neuropathy; CSF, cerebrospinal fluid; CT, computed tomography; d, day; HNPP, hereditary brachial plexus neuropathy; IV, intravenous; L, left; m, month; MP, methyl prednisolone; MRI, magnetic resonance imaging; N, normal; ND, not done; R, right; y, year;
119/F283Painful weakness bilaterally; BPN postpartumNNegNeg17IV MP tapered over 1.5 monthsDramatic pain reduction; worsened pain and weakness with steroid taper
210/M464Painful weakness L>R; BPNProtein↑ (60 mg/dl)NegND71 g IV MP ×3d; then 60 mg prednisone ×1m; then 50 mg ×1m; then tapered offDramatic pain reduction; worsened pain and possible weakness with steroid taper
318/F212Painful weakness bilaterally; BPN postpartumNNDND11Prednisone 60 mg/d tapered over 3 weeksUncertain
49M283Painful weakness R; BPNNNegNeg1*Prednisone 80 mg/d tapered over 4 weeksUncertain