Tuesday, January 15, 2008

What is Paraneoplastic Syndrome?

Well that was our question when Haven's endocrinologist called and stated that she may also have this condition. Quickly I googled it to check it out and have somewhat of a description below. We're starting testing on this as soon as I can locate the tubes to do the testing. It's a special set of tubes we need and most places don't carry them. Not to mention we need 5 different tubes for this test. Three will be done at 11pm at night and two will be done at 8am in the morning. This will also check her levels of Cortisol - which they need to know for the surgery. Kris and I do not feel that this is the case as Haven does not express all of the symptoms below - but at least JH is continually researching her symptoms.

Below is a description.

A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body. These phenomena are mediated by humoral factors (by hormones or cytokines) excreted by tumor cells or by an immune response against the tumor.


What are Paraneoplastic Syndromes? Paraneoplastic syndromes are a group of rare degenerative disorders that are triggered by a person's immune system response to a neoplasm, or cancerous tumor. Neurologic paraneoplastic syndromes are believed to occur when cancer-fighting antibodies or white blood cells known as T cells mistakenly attack normal cells in the nervous system. These disorders typically affect middle-aged to older persons and are most common in persons with lung, ovarian, lymphatic, or breast cancer. Neurologic symptoms generally develop over a period of days to weeks and usually occur prior to tumor detection, which can complicate diagnosis. These symptoms may include difficulty in walking and/or swallowing, loss of muscle tone, loss of fine motor coordination, slurred speech, memory loss, vision problems, sleep disturbances, dementia, seizures, sensory loss in the limbs, and vertigo. Neurologic paraneoplastic syndromes include Lambert-Eaton myasthenic syndrome, stiff-person syndrome, encephalomyelitis (inflammation of the brain and spinal cord), myasthenia gravis, cerebellar degeneration, limbic and/or brainstem encephalitis, neuromyotonia, and opsoclonus (involving eye movement) and sensory neuropathy.

Is there any treatment?

The cancer is treated first, followed by efforts to decrease the autoimmune response - either through steroids such as cortisone or prednisone, high-dose intravenous immunoglobulin, or irradiation. Plasmapheresis, a process that cleanses antibodies from the blood, may ease symptoms in patients with paraneoplastic disorders that affect the peripheral nervous system. Speech and physical therapy may help patients regain some functions.

What is the prognosis?

There are no cures for paraneoplastic syndromes and resulting progressive neurological damage. Generally the presence of cancer determines the diagnosis.

What research is being done?

Research on neurologic paraneoplastic syndromes is aimed at enhancing scientific understanding of them and evaluating new therapeutic interventions. Researchers are seeking to learn what causes the autoimmune response in these disorders. Studies are directed toward developing assays that detect different types of antibodies. Scientists also hope to develop animal models for these diseases, which may be used to determine effective treatment strategies.

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