About Paraneoplastic Neurological Disorders (PND) > Information for Patients > General Information on Treatment of Paraneoplastic Neurological Disorders (PND)

General Information on Treatment of Paraneoplastic Neurological Disorders (PND)

Except for a few PND of the peripheral nerves and neuromuscular junction there is no general agreement or set guidelines of how to treat many ofND. However, there are several principles in which most researchers agree:

  1. The tumor is the main trigger of all PND. Therefore, as a general principle the tumor should be promptly identified and treated.
  2. Many PND are immune mediated and therefore in many PND immunotherapy should be considered.
  3. The utility, timing, and type of immunotherapy will vary depending of the type of PND or whether the tumor is being treated at the same time.
    1. As far as the utility of immunotherapy is concerned: many PND develop rapidly and then stabilize. For a patient who has had stable symptoms for many months, immunotherapy should not be considered. In contrast, if a patient has progressive neurological symptoms and tests suggest active inflammation in the nervous system, then immunotherapy should be considered.
    2. As far as the timing of the immunotherapy is concerned: patients who are receiving chemotherapy for the cancer may develop serious complications if both chemotherapy and certain immunosuppressants are given at the same time. In this situation, the main priority is to treat the tumor. If immunotherapy is to be used, a treatment compatible (or with less side effects) when given concurrently with chemotherapy should be selected.
    3. As far as the type of immunotherapy is concerned: the selection should be based on the type of PND and for some cases the type of immunologic response. For example, PND mediated by T-lymphocytes (regardless of whether they are associated with antibodies or not) should be treated with immunotherapies aiming at the T-cell response rather than simply removing the antibodies. Many PND of the brain and cerebellum fit in this category, including paraneoplastic encephalitis with anti-Hu, anti-CV2/CRMP5, anti-Ri, or anti-Ma2 antibodies, and paraneoplastic cerebellar degeneration with anti-Yo antibodies.

      In contrast, PND that appear to be mediated by antibodies should be treated with immunotherapies that remove the antibodies and/or the cells that produce or regulate the antibody production. LEMS, myasthenia gravis, neuromyotonia, and several subtypes of encephalitis are examples of this group of disorders.
  4. The presentation of neurological symptoms that appear to be a PND in a patient in remission from a cancer treated within the prior 5 years usually implies that the cancer has started to grow back. In these patients a high index of suspicion for cancer recurrence should be considered and repeat cancer screening obtained.
  5. Similarly, a relapse or “sudden worsening” of neurological symptoms in a patient known to have PND, but whose cancer is thought to be in remission, should raise a high index of suspicion for cancer recurrence, and repeat cancer screening obtained.

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