Paraneoplastic Cerebellar Degeneration Differential Diagnoses

Updated: Jul 01, 2020
  • Author: Erika Lan, DO, MA; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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DDx

Diagnostic Considerations

When suspecting an immune-mediated etiology for the patient's cerebellar dysfunction, consideration of the targeted structures and the presence or absence of other associated conditions may help to narrow down the differential diagnoses. [48]

Diagnostic criteria

Graus et al put forth the following diagnostic criteria for paraneoplastic neurological syndromes (PNS). [33]

Definite PNS

1. A classical syndrome and cancer that develops within five years of the diagnosis of the neurological disorder.

2. A non-classical syndrome that resolves or significantly improves after cancer treatment without concomitant immunotherapy, provided that the syndrome is not susceptible to spontaneous remission.

3. A non-classical syndrome with onconeural antibodies (well characterized or not) and cancer that develops within five years of the diagnosis of the neurological disorder.

4. A neurological syndrome (classical or not) with well characterized onconeural antibodies (anti-Hu, Yo, CV2, Ri, Ma2, or amphiphysin), and no cancer.

Possible PNS

1. A classical syndrome, no onconeural antibodies, no cancer but at high risk to have an underlying tumor.

2. A neurological syndrome (classical or not) with partially characterized onconeural antibodies and no cancer.

3. A non-classical syndrome, no onconeural antibodies.

Differential Diagnoses