1. Anti-yo-antibody mediated paraneoplastic cerebellar degeneration - a case series.
- Author
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Auwal, Abdusshakur, Noushad, Muhammad, and Bhattacharjee, Shakya
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AUTOANTIBODIES , *PARANEOPLASTIC syndromes , *DYSARTHRIA , *METASTASIS , *CONFERENCES & conventions , *MAGNETIC resonance imaging , *INTRAVENOUS immunoglobulins , *NYSTAGMUS , *CEREBELLUM diseases , *CASE studies , *POSITRON emission tomography , *MASTECTOMY , *COMPUTED tomography , *BREAST tumors , *ATAXIA , *SYMPTOMS - Abstract
Background Paraneoplastic cerebellar degeneration (PCD) is a clinical syndrome in which cerebellar dysfunction is associated with identifiable or occult cancer but without the direct involvement of the nervous system by cancer.1,2 Typically, the disorder evolves sub-acutely, causes severe immune-mediated pan cerebellar dysfunction, and is associated with extensive purkinje cell loss, sometimes accompanied by meningeal and deep cerebellar nuclear inflammatory infiltrates.1 It has been linked to elevated anti-yo antibody serum titre in affected patients. It is most commonly associated with breast and other gynaecological cancers and often presents in patients prior to their cancer diagnosis. Hence, an aggressive search for an underlying malignancy would confirm the diagnosis and provide a basis for treatment, which is what this case series intends to highlight. Materials and method We present three cases of patients (aged 50-70 years) presenting with cerebellar features; truncal and/or limb ataxia, dysarthria and nystagmus. All of them have a rise in anti-yo antibody titre, as well as a positive oligoclonal band in the CSF, raising a strong suspicion of PCD.3,4 Their preliminary CT TAP and MRI brain were unyielding. However, the high index of suspicion prompted a positron emission tomographic (PET) scan, which identified the origin of the malignancy as breast in the first two cases and fallopian tube in the third case (metastatic deposits in the pelvic side wall were seen here on the PET scan necessitating an exploratory laparotomy). All the cancers were confirmed on histology. Results All three patients received intravenous immunoglobulin (IVIg) as part of their treatment. Of the two breast cancer cases, one was a grade 3 non-invasive DCIS, hence mastectomy was offered in addition. Unfortunately, none of the patients improved despite treatment at the time this information was collected, although there was no long-term follow up. Conclusion The case series demonstrated the need for a high index of suspicion of underlying malignancy in patients presenting with features of PCD and the need to pursue it aggressively. This would not only help to confirm the diagnosis but would aid in management. However, further studies are needed to explore alternative treatment options. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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