Anti-Ri-associated paraneoplastic cerebellar degeneration. Report of a case and revision of the literature.
Paraneoplastic cerebellar degeneration associated with anti-Ri antibodies mainly presents with opsoclonus-myoclonus-ataxia. We report here the case of a patient with anti-Ri-antibody paraneoplastic syndrome, who presented four years after treatment for small-cell lung cancer (SCLC) with oscillopsia and gait disorder. On neurological examination vertical nystagmus, ataxic gait and postural tremor of all four limbs was detected. He died one year after the onset of the symptoms because of a acute exacerbation of his severe chronic obstructive pulmonary disease. No SCLC relapse or new cancer has been detected during the one-year follow-up period.To our knowledge, our patient is the first case of anti-Ri associated disorder with oscillopsia and vertical nystagmus as the initially prominent clinical features. The findings of this case study support the variability of anti-Ri-antibody-associated paraneoplastic syndrome. Further studies must be directed to better characterize the mechanisms underlying this syndrome. Finally, paraneoplastic neurological syndromes should be kept in mind also when a neoplastic disease is not demonstrated.
Neurological Institute University of Pisa. mmancuso@inwind.it
Michelangelo
M
Mancuso
Daniele
D
Orsucci
Andrea
A
Bacci
Elena
E
Caldarazzo Ienco
Gabriele
G
Siciliano
D000888:Antibodies, Anti-Idiotypic / Q000134:cerebrospinal fluid*; D000951:Antigens, Neoplasm / Q000276:immunology*; D020233:Gait Disorders, Neurologic / Q000150:complications; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology*; D000090682:Neuro-Oncological Ventral Antigen; D009759:Nystagmus, Pathologic / Q000150:complications; D020362:Paraneoplastic Cerebellar Degeneration / Q000134:cerebrospinal fluid* / Q000209:etiology / Q000276:immunology*; D049268:Positron-Emission Tomography; D016601:RNA-Binding Proteins / Q000276:immunology*; D055752:Small Cell Lung Carcinoma / Q000150:complications; D014057:Tomography, X-Ray Computed
D000888:Antibodies, Anti-Idiotypic; D000951:Antigens, Neoplasm; D009419:Nerve Tissue Proteins; D000090682:Neuro-Oncological Ventral Antigen; D016601:RNA-Binding Proteins