Treatment Options for Paraneoplastic Neurological Disorders
Paraneoplastic Neurological Disorders (PNDs) can be extremely complex and difficult to treat. The most important part of treatment is to get a proper and rapid diagnosis of both the underlying cancer and the type of paraneoplastic disorder involved. This knowledge can help doctors provide more targeted and effective treatment that can ultimately improve both life expectancy and quality of life.
Typically, paraneoplastic patients are treated by an oncologist (a doctor specializing in cancer) and a neurologist (a doctor specializing in neurological disorders). A neuro-oncologist (a doctor trained in treating the neurological effects of cancer)may also be involved. Physical therapists, speech therapists and occupational therapists should also be involved when a patient is diagnosed with a PND. Their assistance can greatly improve quality of life and help slow down the progression of neurological deterioration.
Because paraneoplastic disorders are rare, many local doctors, even oncologists and neurologists, may never have seen a case and thus may not be up-to-date on the latest treatment options. It is therefore a good idea to get a consultation, or ask your doctor to consult with, a specialist who has experience treating these disorders.
When seeking a doctor for paraneoplastic treatment, it is wise to ask the following questions:
- Do you have recent experience with the management of paraneoplastic disorders?
- How many patients have you treated with paraneoplastic disorders? Which specific paraneoplastic disorders?
- Are you willing to consult with other specialists and researchers concerning my case?
- What is your treatment philosophy when it comes to paraneoplastic patients? How aggressive are you at treating underlying symptoms and considering different treatments?
Medical centers with extensive experience treating paraneoplastic disorders include but are not limited to:
Memorial Sloan Kettering Cancer Center, New York, NY (Contact: Dr. Jerome Posner)
Rockefeller University, New York, NY (Contact: Dr. Robert Darnell)
University of Texas Southwestern Medical Center, Neurology department (Contact: Dr. Steven Vernino)
Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA (Contact: Dr. Dalmau)
The Mayo Clinic, Rochester, Minnesota (and branches)
Department of Neurology, Johns Hopkins University, Baltimore, MD (Contact: Dr. Argye Beth Hillis)
Once the underlying cancer and the type of paraneoplastic disorder have been identified, treatment should have two coinciding aims: to eliminate the cancer AND to minimize and stabilize the neurological deterioration caused by the paraneoplastic disorder.
Treating the Underlying Cancer
Paraneoplastic disorders are most common in patients with lung, breast, ovarian, and lymphatic cancers, but other types of cancers can also be involved.
Because there have been some reported cases where the symptoms of paraneoplastic disorders have been abated after the cancer has been controlled, doctors usually suggest standard cancer treatments such as chemotherapy, tumor incision, and radiation as a first step in treating paraneoplastic patients. Some paraneoplastic patients may undergo many rounds, or even years, of cancer treatment. Our resource page lists many links and sources of information to help you understand the various cancer treatment options that may be offered to you.
It is very important that all paraneoplastic patients and their families ask about potential neurological side effects of any recommended cancer treatment, especially chemotherapy treatments. Whenever possible, you want to avoid the risk of additional neurological problems. Our resource page offers a few links that can help you to evaluate the neurological risks of various treatments.
Some patients have great difficulty identifying the location of their cancer. These patients are said to have "occult" or hidden cancers that are hard to detect even after many scans, blood tests and biopsies. Some doctors may choose to treat suspected occult cancers when paraneoplastic neurological symptoms and antibodies are present.
Treating the Paraneoplastic Disorder
Before any treatment can be attempted to stop the direct neurological affects of paraneoplastic disorders, it is important for doctors and their patients to properly identify the underlying antibodies and type of paraneoplastic disorder involved. Not all treatments are effective for all types of paraneoplastic disorders and their associated antibodies. In weighing suggested treatment options, patients should ask their doctors the name of the type of antibody, the form of paraneoplastic they have, and whether the recommended treatment has been shown to be effective for that particular combination. In rare instances, doctors may not be able to definitively identify the specific form of antibody or type of paraneoplastic, yet, because a paraneoplastic disorder is strongly suspected, they may recommend a trial of treatments typically used for paraneoplastic disorders.
Common paraneoplastic- associated antibodies include:
- Anti-Yo - Most commonly found in women with gynecological and breast cancers, related to Cerebellar Paraneoplastic Degeneration
- Anti-Hu - Expressed by a number of cancers, including small cell lung cancers, neuroblastomas, and other forms of cancer.
- Anti-Ri - Most comonly associatied with breast cancer, but can also be found in patients with lymphomas.
There are three main treatments used to directly treat the neurological effects of paraneoplastic disorders. The main goal of these treatments is to suppress the immune system. They are: Plasmapheresis, IvIg, and immunosuppresent medications. Sometimes a combination of treatments is used. None of these treatments are effective for all forms of PND.
Plasmapheresis can be thought of as a "blood cleansing" process. During plasmapheresis blood is passed through a device called a separator, which removes Plasma, the part of the blood containing the PND antibodies. The rest of the blood is returned to the patient.
Plasmapheresis is generally not painful, although some patients may find the process uncomfortable. A small thin tube is placed in one large vein, usually in the arm, and another tube is placed in the opposite hand or foot. Blood is removed through one tube, goes through the separator, and is returned to the body in the other tube. Only a small amount of blood is outside of the body at any one time. The process takes several hours and can be done on an outpatient basis. Usually paraneoplastic patients undergoing plasmapheresis will receive several treatments spaced out over several weeks.
Risks associated with plasmapheresis include a drop in blood pressure, bleeding, and allegic reactions to the solutions used to replace the plasma. In some patients, excessive suppression of the immune system can temporarily occur.
Plasmapheresis has been shown to have mixed results in paraneoplastic disorders, usually resulting in only a temporary improvement unless the underlying cause of antibody production in the body (the cancer) has been found and alleviated.
Intravenoous immunoglobulin (IVIG) therapy
IVIG therapy uses immunoglobulins, a type of protein that is found in human blood which helps to fight off harmful bacteria, viruses, and other germs. IVIG products are derived from the plasma of large numbers of individuals. All donors are tested for contagious diseases including HIV, and hepatitis.
During an IVIG treatment, the immunoglobulins are given intervaneously. An IV catheter is placed int hepatient's hand or lower arm and the IVIG solution is dripped into the vein. Side effects can include headache, flu-like fatigue, fever, chilld and nausea.
Like plasmapheresis, results from IVIG can be mixed for paraneoplastic patients. Doctors usually advise patients that it can take several weeks to see results such as improvement in neurological function or the slowing of neurological deterioration. Many paraneoplastic patients will need follow up IVIG treatments.
Some research has suggested that IVIG may not be effective for some types of paraneoplastic disorders. Dr. Franz Blaes, a German researcher into paraneoplastic disorders, has noted, for instance, that anti-Hu antibody-associated syndromes are unlikely to respond to IVIG.
Many paraneoplastic specialists recommend a combination of immunosuppressent drugs. Immunosuppresent drugs are designed to suppress the body's production of the antibodies associated with paraneoplastic disorders.
Commonly used immunosuppressent drugs include cyclophosphamide, azathioprine, and rituxan. They are usually given orally.
Some specialists use these drugs in combination with plasmapheresis or IVIG. One study conducted by Dr. Steven Vernino at the Mayo Clinic found that out of 19 patients treated with a combination of oral cyclophosphamide and plasmapheresis, 53% experienced a positive response. Dr. Vernino noted that better outcome was achieved the earlier treatment began after diagnosis.
Other treatments to help paraneoplastic patients
Paraneoplastic patients usually have a wide range of symptoms related to both the paraneoplastic disease and their cancer. Doctors may prescribe a wide variety of drugs and therapies to address these issues. For example, if a patient has poor appetite, an appetite stimulant medication such as Megace can often improve appetite. Some doctors prescribe steriods like predisone and anti-seizure medications such as Topamax to help with various symptoms. Anti-naseau medications may also be used to combat the side effects of chemotherapy and the effects of vertigo in PND patients.
Since depression is very common among PND patients, anti-depressent drugs may also be prescribed.
In addition to drugs and treatments, the PND patients who seem to do best, both in the short term and in the long term, are those that receive on-going physical therapy, occupational therapy, and speech therapy. Unfortunately, physical therapy may be stopped after a relatively short period of time because no apparent "improvement" has been seen. Doctors and physical therapists should understand that in PND patients stabilization, not improvement, may be the goal for physical therapy. Continuing PT on a regular basis can help patients maintain their balance and muscular control enough to make a large difference in their quality of life.
It is a good idea for all newly diagnosed patients to have at least a consultation with an occupational therapist and a speech therapist. The occupational therapist can help the patient and caregivers find ways to use adaptive techniques and technologies that keep the patient safer and more independent.
An initial consultation with a speech therapist soon after diagnosis can be useful for establishing a baseline for future speech difficulties as the symptoms of paraneoplastic disorderss progress. Many paraneoplastic patients eventually have trouble speaking clearly, leading to enormous frustration. A physical therapist can help the patient improve breathing and speech techniques, as well as recommend various assisted communications devices that even the most severely disabled can use to communicate. Both an occupational therapist and speech therapist can also work together to solve and minimize swallowing difficulties.
There are a number of researchers and medical facilities investigating both the underlying causes of paraneoplastic neurological disorders and treatment options. As new studies come to the attention of the IPA, we post them to our email support group. To subscribe go to paraneoplastic support group and follow the directions for subscribing to the email list.
As of October, 2005, Dr. Robert Darnell at Rockefeller University in New York City is recruiting patients for a study of the effects of tacromilus, an immune suppressant used in transplant patients. For details of this study, as well as information about participation, go to this link: Rockefeller University Study
Many patients and families ask whether homeopathic and alternative treatments may help paraneoplastic patients.
It is wise to always use caution when considering alternative treatments and homeopathic options. Paraneoplastic patients already have a host of neurological and immunological issues, and there is always a danger that non-FDA approved preparations and/or alternative treatments can exacerbate these symptoms. Therefore, before trying any homeopathic remedy or alternate treatment ALWAYS consult with a medical doctor who is knowledgeable about paraneoplastic disoders about any risks that may be associated with these products or treatments.
Additionally, be particularly careful about information found on the Internet that has not been supported by research, or claims made by non-medical professionals regarding "cures" or "treatments" for paraneoplastic disorders, cancer and the neurological and other symptoms associated with paraneoplastic and cancer.
Paraneoplastic disorders are extremely complex. The best source of medical information and treatment options is a specialist familiar with all aspects of paraneoplastic disorders, or a doctor willing to consult with such a specialist.