Paraneoplastic disorders in thymoma patients.
Thymic malignancy is often associated with paraneoplastic neurological diseases (PNDs) and recognition of these disorders is important for physicians who treat these patients. The most common thymoma-associated PNDs are myasthenia gravis (MG), acquired neuromyotonia (Isaacs' syndrome), encephalitis, Morvan's syndrome, and myositis. Diagnosis of these disorders is complex but often aided by testing for specific autoantibodies, including those to the acetylcholine receptor for MG and to contactin-associated protein-like 2, protein of the voltage-gated potassium channel complex, in patients with acquired neuromyotonia, Morvan's syndrome, or encephalitis. Patients who manifest these disorders should be screened for thymoma at diagnosis, and worsening of these PNDs may be associated with recurrent thymoma. These disorders can cause profound disability but usually respond to immunotherapy, and often improve with thymoma treatment. Close cooperation among a team of specialists is required to take proper care of these patients.
*Department of Neurology, Catholic University, Roma, Italy; and †Department of Neurology, The University of Pennsylvania, Philadelphia, PA.
Amelia
A
Evoli
Eric
E
Lancaster
D006801:Humans; D020361:Paraneoplastic Syndromes, Nervous System / Q000175:diagnosis* / Q000473:pathology*; D013945:Thymoma / Q000473:pathology*