Overview
Paraneoplastic syndromes are a group of uncommon disorders that develop in some individuals with cancer. These disorders can affect various organ systems, including the nervous system, endocrine system, skin, blood, and joints. Paraneoplastic syndromes of the nervous system occur when cancer-fighting agents of the immune system attack parts of the brain, spinal cord, peripheral nerves, or muscle. The injury to the nervous system can cause problems with muscle movement or coordination, sensory perception, memory or thinking skills, or sleep. Some of the types of paraneoplastic syndromes include cerebellar degeneration (cerebellar ataxia) and limbic encephalitis. These disorders are not caused by cancer cells directly disrupting nerve function, by the cancer spreading, or by other complications such as infections or treatment side effects. Instead, they occur alongside the cancer as a result of the activation of the immune system.
Understanding the disease
Symptoms
Symptoms of paraneoplastic syndromes of the nervous system can vary depending on the specific syndrome and the area of the nervous system affected. Some common symptoms include difficulty walking, maintaining balance, experiencing muscle coordination issues, loss of muscle tone or strength, loss of fine motor skills, difficulty swallowing, speech problems, memory loss, vision problems, sleep disturbances, seizures, hallucinations, and unusual involuntary movements. Examples of paraneoplastic syndromes of the nervous system include cerebellar degeneration, limbic encephalitis, and dysautonomia. If you have any signs or symptoms suggesting a paraneoplastic syndrome, it's important to see your doctor as soon as possible for an early diagnosis and appropriate care.
Risk Factors
Risk factors for developing paraneoplastic syndromes of the nervous system include having cancer, particularly cancers of the lung, ovary, breast, testis, or lymphatic system. These disorders can also occur in people with certain inherited syndromes that increase the risk of cancer, such as Multiple Endocrine Neoplasia, type 2 (MEN 2) and Von Hippel-Lindau disease. Radiation therapy for cancer and exposure to certain chemicals or viruses may also increase the risk of developing sarcoma, a type of cancer that can lead to paraneoplastic syndromes.
Development
Paraneoplastic syndromes of the nervous system develop as a result of the activation of the immune system, which attacks normal cells of the nervous system alongside cancer cells. The immune system's cancer-fighting abilities, such as antibodies and T cells, cause neurological disorders. There are various types of paraneoplastic syndromes that can affect different parts of the nervous system, including the brain, spinal cord, peripheral nerves, and muscles. The symptoms of these syndromes can be diverse, ranging from difficulty walking and maintaining balance to memory loss, vision problems, and sleep disturbances. It is essential to seek medical attention promptly if you have any signs or symptoms suggesting a paraneoplastic syndrome, as early diagnosis and appropriate care can be crucial for managing these disorders.
Assessment and Diagnosis
Triage
If you have signs or symptoms suggesting a paraneoplastic syndrome, you should see your doctor as soon as possible. Early diagnosis and appropriate care can be extremely important. Some of the signs and symptoms that might indicate a paraneoplastic syndrome include difficulty walking, loss of muscle coordination, weakness, slurred speech, memory loss, vision problems, sleep disturbances, seizures, hallucinations, and unusual involuntary movements.
Diagnosis
To diagnose paraneoplastic syndromes of the nervous system, a doctor will conduct a physical exam, order blood tests, and possibly request imaging tests or a spinal tap. They may also perform cancer screening tests based on the patient's age. Additional tests like urine tests and imaging scans may be used to diagnose carcinoid tumors. It's important to see a specialist in nervous system disorders or a cancer specialist for proper evaluation and treatment.
Management and Treatment
Complications
Paraneoplastic syndromes can result in severe damage to the nervous system that may not be reversible. Depending on the specific syndrome and its severity, potential complications can include:
- Permanent loss of muscle coordination or strength
- Permanent cognitive impairments, such as memory loss, difficulty speaking, or difficulty swallowing
- Chronic pain
- Difficulty maintaining balance, which can lead to falls and injuries
- Severe sleep disturbances, causing excessive daytime drowsiness or insomnia
- Seizures, which can be difficult to control and may lead to additional neurological complications
- Hallucinations, delusions, or other psychological disturbances
- Unusual involuntary movements, such as tremors or spasms While treatment may not always reverse the damage caused by paraneoplastic syndromes, it can help prevent further complications, improve symptoms, and enhance quality of life for those affected.
Treatment
Treatment for paraneoplastic syndromes involves addressing the underlying cancer and, in some cases, managing the immune response causing the neurological symptoms. The specific treatment approach depends on the type of paraneoplastic syndrome but may include the following options:
- Medications: Chemotherapy is typically used to combat cancer, while immunosuppressants, such as corticosteroids (prednisone), azathioprine (Imuran, Azasan), mycophenolate (CellCept), rituximab (Rituxan), and cyclophosphamide (Cytoxan), may be prescribed to suppress the immune system's attack on the nervous system.
- Diagnosis: To diagnose paraneoplastic syndrome of the nervous system, a physical exam, blood tests, spinal tap, and imaging tests may be conducted. Cancer screening tests may also be performed based on age and risk factors.
- Support groups: Connecting with others who have faced similar challenges can be beneficial. In-person or online support groups may provide helpful resources. Remember to work closely with your healthcare team and discuss any questions or concerns you may have.
Preparing for medical consultation
To prepare for an appointment for paraneoplastic syndromes, you should:
- Be aware of any pre-appointment restrictions or guidelines that your doctor may provide at the time of scheduling.
- Write down any symptoms you're experiencing, including those that may seem unrelated to paraneoplastic syndromes.
- Note down any key personal information, such as major stresses or recent life changes.
- Make a list of all medications, vitamins, or supplements you're currently taking.
- Consider bringing a family member or friend along for support and to help remember important details.
- Prepare a list of questions to ask your healthcare provider during the appointment. Some questions to consider asking include:
- What could be causing my paraneoplastic syndrome?
- What tests do I need to confirm the diagnosis?
- What are the treatment options available for paraneoplastic syndromes?
- What is the likely course and prognosis for my condition?
- How can I manage my symptoms and maintain my quality of life? Remember, being well-prepared for your appointment will help ensure that you have a productive and informative discussion with your healthcare provider.