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Paraneoplastic Cerebellar Degeneration (Neurology)

A subacute progressive cerebellar ataxia syndrome associated with malignancy.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Paraneoplastic Cerebellar Degeneration (Neurology)?

Paraneoplastic cerebellar degeneration is a subacute progressive ataxia syndrome that develops in association with an underlying malignancy. Different antibodies are associated: anti-Yo (gynecologic, breast), anti-Tr/DNER (Hodgkin lymphoma), anti-Hu (SCLC), anti-Ri, anti-mGluR1.

The pathologic process is loss of cerebellar Purkinje cells. The clinical course progresses subacutely (weeks-months) and severe ataxia, dysarthria, vertigo, nystagmus and diplopia develop. Anti-Yo predominantly affects women and is associated with gynecologic-breast cancer.

Diagnosis is supported by paraneoplastic antibody panel, brain MRI (cerebellar atrophy), CSF analysis, intensive tumor screening (CT, FDG-PET, mammography). Treatment of the underlying tumor is the priority; immunotherapy is added (steroid, IVIG, plasmapheresis, rituximab, cyclophosphamide). Prognosis is generally poor.

Symptoms

Subacute progressive cerebellar ataxia
Severe gait disturbance and instability
Dysarthria and scanning speech
Vertigo and nausea
Nystagmus and diplopia
Coexisting tumor symptoms

Risk Factors

Underlying gynecologic cancer (anti-Yo)
Breast cancer (anti-Yo)
Hodgkin lymphoma (anti-Tr/DNER)
Small-cell lung cancer (anti-Hu)
Female sex (anti-Yo predominance)
Middle and advanced age

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Subacute progressive ataxia
  • Severe gait disturbance
  • Dysarthria and slurred speech
  • Persistent vertigo and nausea
  • Underlying cancer history or screening result
  • Diplopia and nystagmus

Treatment Methods

01
Treatment of underlying tumor (priority)
02
Paraneoplastic antibody panel
03
First-line: IVIG, methylprednisolone, plasmapheresis
04
Second-line: rituximab, cyclophosphamide
05
Tumor screening (CT, FDG-PET, mammography)
06
Symptomatic and rehabilitation support

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.