We describe a patient with bilateral orbital myositis, multiple crania
l neuropathies, a sensory polyneuropathy, serum and cerebrospinal flui
d paraproteins, and high-grade non-Hodgkin's lymphoma. Neurologic symp
toms began more than 1 year before diagnosis of the lymphoma. Results
of extraocular muscle biopsy showed extensive destruction of myofibers
and granulomatous features, with no evidence of direct tumor involvem
ent. The cranial neuropathies and orbital myositis improved with immun
osuppressive therapy, while the patient's tumor progressed. We believe
the orbital myositis and the multiple neurologic abnormalities were p
araneoplastic effects of the lymphoma. To our knowledge, this is the f
irst case of orbital myositis identified as a paraneoplastic syndrome.