Objective: To investigate whether there are clinical features that would allow us to segregate between paraneoplastic cerebellar degeneration (PCD) from other adult onset cerebellar ataxia.

Background: The initial presentation of adult onset cerebellar ataxia remains a diagnostic challenge due to its multiple etiologies, of which PCD is one rare cause not to be missed. The presence of paraneoplastic antibodies and an underlying cancer in patients with cerebellar ataxia may allow the diagnosis of PCD to be made. And the prevalence of different causes of ataxia differs in the Asian and Western populations.

Methods: Retrospective case note analysis was performed in patients presenting to the National Neuroscience Institute between 2007 and 2014 with ataxia who had anti-neuronal antibodies tested.

Results: (1) 81 patients (48 males and 33 females) were included in the study. 4 patients (4.9%) met the diagnosis of PCD, 29 (35.8%) patients with sporadic degenerative ataxias, 14 patients (17.3%) had ataxia that was unexplained. (2) In our series, there were 2 patients for which the initial clinical suspicion of PCD was strong due to a positive malignancy history who were subsequently reclassified as MSA-C after a period of observation. When these patients were compared with the 4 definite cases of PCD in our series: we found that they had a longer presentation time (12 months vs 0.75 months), longer time to reach the use of a walking aid (29 months vs 7.0 months). 3) Compared with patients of sporadic degenerative ataxia, our results showed that patients with PCD have more truncal ataxia (75% vs 3.4%) and less cerebellar dysarthria (25% vs 44.8%), gait ataxia (25% vs 75.9%), and no nystagmus, cognitive impairment and motor symptoms at onset. In patients with PCD, the magnetic resonance imaging (MRI) brain scan at presentation was normal, whereas there were frequently abnormal in patients with other sporadic degenerative ataxias (atrophy in 55.2%, unremarkable change in 17.2%).

Conclusions: In adult onset cases of cerebellar ataxia, only a small subset of patients were diagnosed with PCD, with sporadic degenerative causes being more common. Factors such as an earlier presentation to medical services, normal MRI scans, truncal ataxia and the absence of other features such as nystagmus, and cognitive impairment tends to point towards a possible diagnosis of PCD.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/early-clinical-features-of-paraneoplastic-cerebellar-degeneration-diagnostic-outcomes-of-adult-onset-cerebellar-ataxia/