Paraneoplastic Cerebellar Degeneration Follow-up

  • Author: Abbas Mehdi, MD; Chief Editor: Stephen A Berman, MD, PhD, MBA   more...
 
Updated: Feb 3, 2012
 

Further Outpatient Care

  • Patients usually require long-term rehabilitation and/or hospice care in severe situations. Treatment is unsatisfactory, and chance of long-term survival is poor in reported cases.
  • In patients in whom no tumor can be detected after a comprehensive workup, routinely perform outpatient measurement of antibody levels with tumor markers and other tests related to diagnosis of possible malignancies.
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Prognosis

Prognosis greatly depends on early detection of the underlying neoplasm and its stage at the time of detection.

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Contributor Information and Disclosures
Author

Abbas Mehdi, MD  Director, MDA Center of Central California; Consulting Staff, Department of Neurology, California Neurological Center, Inc

Abbas Mehdi, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

David Y Ko, MD  Associate Professor of Clinical Neurology, Associate Director, USC Adult Epilepsy Program, Keck School of Medicine of the University of Southern California

David Y Ko, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, and American Headache Society

Disclosure: GSK Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching; Lundbeck Consulting fee Consulting; Westward Consulting fee Consulting

Specialty Editor Board

Frederick M Vincent Sr, MD  Clinical Professor, Department of Neurology and Ophthalmology, Michigan State University Colleges of Human and Osteopathic Medicine

Frederick M Vincent Sr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American College of Forensic Examiners, American College of Legal Medicine, American College of Physicians, and Michigan State Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge C Kattah, MD  Head, Associate Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Stephen A Berman, MD, PhD, MBA  Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

References

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The workup of paraneoplastic cerebellar degeneration.
MRI of a 29-year-old female with ARCA1. Sagittal T1 shows marked diffuse cerebellar atrophy with no atrophy of the cerebral cortex, midbrain, pons, or medulla. Image from National Institutes of Health.
Table. Antibodies Associated With Paraneoplastic Cerebellar Degeneration* (Adapted from Dalmau et al[22] )
Antibodies Predominantly Associated With PCDPredominant SyndromeAssociated Cancer
Anti-Yo (PCA-1) antibodiesPCDOvarian



Breast cancers



Anti-Tr antibodiesPCDHodgkin's lymphoma
Anti-mGluR1 antibodies**PCDHodgkin's lymphoma
Anti-Zic4 antibodies†PCDSmall-cell lung cancer
Sometimes Associated With PCD
Anti-VGCC antibodiesEaton-Lambert syndrome, PCDSmall-cell lung cancer



Lymphoma



Anti-Hu (ANNA-1) antibodiesEncephalomyelitis, PCD, sensory neuronopathySmall-cell lung cancer



Other cancers



Anti-Ri (ANNA-2) antibodiesPCD, brain-stem encephalitis, paraneoplastic opsoclonus-myoclonusBreast cancer



Gynecologic cancer



Small-cell lung cancer



Anti-CV2/CRMPS antibodiesEncephalomyelitis, PCD, chorea, peripheral neuropathy, uveitisSmall-cell lung cancer



Thymoma



Other cancers



Anti-Ma protein antibodies‡Limbic, hypothalamic, brain-stem encephalitis (infrequently PCD)Testicular cancer



Lung cancer



Other cancers



Anti-amphiphysin antibodiesStiff-person syndrome, encephalomyelitis, PCDBreast cancer



Small-cell lung cancer



*There is no uniform nomenclature for some of these antibodies; variant names appear in parentheses. mGluR1: metabotropic glutamate receptor 1, Zic4: zing finger of the cerebellum 4, and VCGG: voltage-gated calcium channel.



**Anti-mGluR1 antibodies have been identified in only 2 patients.



† Anti-Zic4 antibodies are predominantly associated with PCD only when no other paraneoplastic antibodies are detectable.



‡Ma proteins include Ma1 and Ma2. Patients with brain-stem and cerebellar dysfunction usually have antibodies against both MA1 and Ma2.



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