Objective: investigate the clinical features, imaging findings and immunological characteristics of Paraneoplastic cerebellar ataxia (PCA) in our cohort.

Background: Paraneoplastic cerebellar ataxia (PCA) is a rare neurological disorder characterized by autoimmune-mediated damage to the cerebellum in association with an underlying malignancy. Despite its rarity, PCA poses significant diagnostic and therapeutic challenges.

Method: We retrospectively reviewed the medical records of patients diagnosed with PCA at the Institute of Neurology Mongi Ben Hmida over a span of 9 years. Our analysis included demographic data, clinical presentations, laboratory findings, imaging studies, types of associated cancers, and the presence of onconeural antibodies and tumor markers.

Results: We conducted 10 patients with a mean age of 55 years [35-77] and a sex-ratio of M/F= 7/3. Most patients presented with isolated ataxia (4 patients). Apart from ataxia, patients developed cognitive impairment (2 patients), peripheral neuropathy (2 patients), nystagmus (1 patient) and myoclonus (1 patient). Tumor markers were negative in all patients while 4 patients had positive onconeural antibodies [anti-titin antibody (n=1), anti-amphiphysin antibody (n=1), anti-CV2 antibody (n=1), anti-recoverin antibody (n=1)]. Hyperproteinorachia was detected in 2 patients. Brain MRI showed cerebellar atrophy in 7 patients. Various types of cancer were identified in half of cases.

Conclusion: our study provides insights into the clinical, radiological and immunological characteristics of PCA. Early recognition and prompt initiation of appropriate treatment are crucial for optimizing patient outcomes in this challenging condition. Further prospective studies are warranted to validate our findings and explore optimal management strategies for PCA.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/paraneoplastic-cerebellar-ataxia-a-rare-cause-of-late-onset-ataxia/