A 68-year-old man died after intermittent bouts of peripheral neuritis over a 12 year period which finally left him incapacitated and afflicted with grossly enlarged nerves. The protein in the cerebrospinal fluid was markedly increased without pleocytosis. At autopsy, all peripheral nerves and roots were found to be tremendously enlarged and showed a typical "onion bulb" neuropathy. With the electron microscope, lymphocytes and macrophages were seen to participate in the "onion bulbs." Teased preparations demonstrated demyelination, remyelination, shortened internodes, and tubes of proliferated Schwann cells. Thus, lesions typical of both the acute Guillain-Barré syndrome and a chronic "onion bulb" neuropathy were present.
Dolman CL, Allan BM. Relapsing Hypertrophic Neuritis. Arch Neurol. 1973;28(5):351–353. doi:10.1001/archneur.1973.00490230087013
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: