Fancellu, Roberto and Corsini, Elena and Bernardi, Gaetano and Buzzo, Paolo and Ferrari, Maria Luisa and Lamantea, Eleonora and Garaventa, Alberto and Truini, Mauro and Salvarani, Sandro (2014) Paraneoplastic cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. Functional Neurology, 29 (4). pp. 277-280. ISSN 1971-3274
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Abstract
We describe a case of cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. A 28-year-old woman developed progressive ataxia with hyporeflexia at the age of 19. Brain MRI showed progressive cerebellar atrophy. Neurophysiological studies, screening of immune-mediated ataxias, oncological markers, vitamin E and genetic tests for spinocerebellar ataxia types 1,2,3, Friedreich ataxia and POLG1 were negative. Anti-Hu antibodies were positive in Western blot and indirect immunofluorescence (1:640). Total-body computed tomography revealed a mediastinum mass; the histological diagnosis was maturing ganglioneuroma. Immunohisto - chemistry showed a mild reaction between the tumor and the patient’s serum, and no reaction between the tumor and control serum. After surgery, serum anti-Hu titer decreased, while ataxic symptoms initially worsened and then stabilized. Ganglioneuroma is a benign tumor, usually derived from the maturation of a neuroblastoma. The benign histology and the presence of anti-Hu antibodies could be related to the positive oncological prognosis and to the slow clinical course mimicking a degenerative ataxia.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | anti-Hu antibodies, ganglioneuroma, immune-mediated ataxia, neuroblastoma, paraneoplastic syndrome |
| Subjects: | 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici) |
| Depositing User: | Marina Spanti |
| Date Deposited: | 15 Dec 2015 15:25 |
| Last Modified: | 15 Dec 2015 15:25 |
| URI: | http://eprints.bice.rm.cnr.it/id/eprint/11235 |
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