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CRMP5 Paraneoplastic Syndrome

Multi-systemic neurological manifestations of autoimmune disease associated with anti-CRMP5/CV2 antibodies.

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 CRMP5 Paraneoplastic Syndrome?

CRMP5 (Collapsin Response Mediator Protein-5; also known as CV2) is the target of antibodies against an intracellular protein expressed in neurons and oligodendrocytes. Anti-CRMP5/CV2 antibodies indicate paraneoplastic neurological syndrome.

Underlying tumor is small-cell lung cancer (SCLC, 75%), thymoma (10-15%), and rarely other carcinomas. Clinical manifestations include cerebellar ataxia, sensorimotor neuropathy, chorea, optic neuritis, uveitis and limbic encephalitis.

Diagnosis is based on demonstration of anti-CRMP5/CV2 antibodies in serum, intensive tumor screening (thoracic CT, FDG-PET) and neurologic imaging. Treatment of the underlying tumor is the priority; immunotherapy (steroid, IVIG, plasmapheresis, rituximab) is added.

Symptoms

Cerebellar ataxia and gait disturbance
Sensorimotor peripheral neuropathy
Chorea and movement disorders
Optic neuritis (vision loss)
Uveitis and inflammatory eye findings
Cognitive impairment and seizures

Risk Factors

Smoking (SCLC association)
Underlying small-cell lung cancer
Thymoma
Advanced age (60-70 years)
Other autoimmune diseases
Concomitant paraneoplastic antibodies

When to See a Doctor?

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

  • Subacute progressive ataxia
  • Sensorimotor neuropathy findings
  • Vision loss and optic neuritis
  • Movement disorder (chorea)
  • Smoker with neurological symptoms
  • Cognitive impairment and seizures

Treatment Methods

01
Treatment of underlying tumor (priority)
02
Anti-CRMP5/CV2 antibody screen
03
Immunotherapy (IVIG, plasmapheresis, steroid)
04
Rituximab and cyclophosphamide (refractory cases)
05
Symptomatic: clonazepam (chorea), gabapentin
06
Long-term tumor follow-up and rehabilitation

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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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.