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Paraneoplastic Encephalomyelitis Medication

  • Author: David S Liebeskind, MD; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
 
Updated: Apr 07, 2014
 

Medication Summary

Although no effective treatment is available, immunosuppressive therapies are frequently used.[20] Immunosuppressive medications include corticosteroids, cyclophosphamide, and intravenous immunoglobulin (IVIG). Recent trials have included rituximab as a treatment for this condition.[21] Anticonvulsants are used for seizure prophylaxis.

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Corticosteroids

Class Summary

These agents modify autoimmune-mediated inflammation.

Methylprednisolone (Solu-Medrol, Medrol, Adlone, Depo-Medrol)

 

Has anti-inflammatory properties. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

Initial PO daily dosage variable, with subsequent dose modification based on clinical response. Constant monitoring may be necessary to adjust for changes in clinical status and environmental stressors. After long-term therapy, taper drug gradually.

Prednisone (Deltasone, Meticorten, Orasone)

 

Has anti-inflammatory properties. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Initial PO daily dosage variable, with subsequent dose modification based on clinical response. Constant monitoring may be necessary to adjust for changes in clinical status and environmental stressors. After long-term therapy, taper drug gradually.

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Immunomodulators

Class Summary

They cause immunosuppressive reduction in inflammation-mediated neurologic injury.

Cyclophosphamide (Cytoxan, Neosar)

 

Has immunosuppressive properties. Chemically related to nitrogen mustards. As alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

PO/IV daily dosage recommendations have not been formulated for treatment of PEM. Modify dose based on clinical response or degree of leukopenia.

Intravenous immunoglobulin (IVIG; Gamimune, Gammagard, Sandoglobulin, Gammar-P)

 

Neutralizes circulating antibodies through anti-idiotypic antibodies. Down-regulates proinflammatory cytokines, including IFN-gamma. Blocks Fc receptors on macrophages. Suppresses inducer T and B cells and augments suppressor T cells. Blocks complement cascade. May increase CSF IgG (10%).

IV dosage recommendations have not been formulated for treatment of PEM.

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Anticonvulsants

Class Summary

These agents are used for treatment and prophylaxis of seizures.

Fosphenytoin (Cerebyx)

 

Diphosphate ester salt of phenytoin acts as water-soluble prodrug of phenytoin. Following administration, plasma esterases convert fosphenytoin to phosphate, formaldehyde, and phenytoin. Phenytoin in turn stabilizes neuronal membranes and decreases seizure activity.

To avoid need to perform molecular weight-based adjustments when converting between fosphenytoin and phenytoin sodium doses, express dose as phenytoin sodium equivalents (PE). Although can be administered IV and IM, IV route is route of choice and should be used in emergency situations.

Concomitant administration of an IV benzodiazepine usually necessary to control status epilepticus. Full antiepileptic effect of phenytoin, whether given as fosphenytoin or parenteral phenytoin, is not immediate.

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Contributor Information and Disclosures
Author

David S Liebeskind, MD Professor of Neurology, Program Director, Vascular Neurology Residency Program, University of California, Los Angeles, David Geffen School of Medicine; Neurology Director, Stroke Imaging Program, Co-Medical Director, Cerebral Blood Flow Laboratory, Associate Neurology Director, UCLA Stroke Center

David S Liebeskind, MD is a member of the following medical societies: American Academy of Neurology, Stroke Council of the American Heart Association, American Heart Association, American Medical Association, American Society of Neuroimaging, American Society of Neuroradiology, National Stroke Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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 Institute, American College of Legal Medicine, American College of Physicians

Disclosure: Nothing to disclose.

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Paraneoplastic encephalomyelitis.
Paraneoplastic encephalomyelitis.
Mesial temporal hyperintensity demonstrated on T2-weighted (left) and fluid-attenuated inversion recovery (FLAIR, right) MRI.
 
 
 
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