Thymoma associated with myasthenia and autonomic anti-Hu paraneoplastic neuropathy.
Along with myasthenia, other paraneoplastic neurological syndromes (PNS) may occur in thymoma. Anti-Hu antibodies and a clinical "anti-Hu syndrome" characterized by encephalitis and/or painful neuropathies have been reported in only three patients at the time of the diagnosis of thymoma. We describe a severe anti-Hu-related autonomic neuropathy with gastrointestinal paresis and intestinal pseudo-obstruction with malabsorption that occurred concomitantly with the worsening of myasthenic symptoms long after the initial diagnosis of thymoma in a young patient. The clinical anti-Hu syndrome preceded the radiological diagnosis of thymoma recurrence. Treatment with plasma exchange led to a transient improvement of neurological symptoms.
Simonelli
Matteo
M
Department of Medical Oncology and Hematology, Istituto Clinico Humanitas, Rozzano, Milan, Italy. matteo.simonelli@gmail.com
Banna
Giuseppe Luigi
GL
Santoro
Armando
A
D000328:Adult; D000912:Antibodies, Neoplasm / Q000097:blood*; D001342:Autonomic Nervous System Diseases / Q000150:complications* / Q000276:immunology / Q000628:therapy; D051959:ELAV Proteins / Q000276:immunology*; D041981:Gastrointestinal Tract / Q000503:physiopathology; D006801:Humans; D007415:Intestinal Obstruction / Q000209:etiology; D008286:Malabsorption Syndromes / Q000209:etiology; D008297:Male; D009157:Myasthenia Gravis / Q000150:complications* / Q000276:immunology / Q000628:therapy; D020364:Paraneoplastic Polyneuropathy / Q000150:complications* / Q000276:immunology / Q000628:therapy; D010291:Paresis / Q000150:complications / Q000209:etiology; D010951:Plasma Exchange; D013945:Thymoma / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D013953:Thymus Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000276:immunology
D000912:Antibodies, Neoplasm; D051959:ELAV Proteins