Ryan Dewey's Story
What is Stiff-Person Syndrome?
Stiff-person syndrome (SPS) or stiff-man syndrome is a rare neurological disorder with features of an autoimmune disease. SPS is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms. Abnormal postures, often hunched over and stiffened, are characteristic of the disorder. People with SPS can be too disabled to walk or move, or they are afraid to leave the house because street noises, such as the sound of a horn, can trigger spasms and falls. SPS affects twice as many women as men. It is frequently associated with other autoimmune diseases such as diabetes, thyroiditis, vitiligo, and pernicious anemia. Scientists don’t yet understand what causes SPS, but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord. The disorder is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies in the blood. People with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain.
Is there any treatment?
People with SPS respond to high doses of diazepam and several anti-convulsants, gabapentin and tiagabine. A recent study funded by the NINDS demonstrated the effectiveness of intravenous immunoglobulin (IVIg) treatment in reducing stiffness and lowering sensitivity to noise, touch, and stress in people with SPS.
What is the prognosis?
Treatment with IVIg, anti-anxiety drugs, muscle relaxants, anti-convulsants, and pain relievers will improve the symptoms of SPS, but will not cure the disorder. Most individuals with SPS have frequent falls and because they lack the normal defensive reflexes; injuries can be severe. With appropriate treatment, the symptoms are usually well controlled.
What research is being done?
The National Institute of Neurological Disorders and Stroke (NINDS) conducts research related to SPS in its laboratories at the National Institutes of Health (NIH), and also supports additional research through grants to major medical institutions across the country. Current research is focused on understanding the cause of the disease and the role of the anti-GAD antibodies. A study using a new drug, Rituximab, is underway in patient trials at the NIH clinical center.
What is lymphoma?
Lymphoma is a cancer of the white blood cells, namely lymphocytes, that happen to constitute the lymphatic system. The two main types of lymphoma are Hodgkin lymphoma and non-Hodgkin lymphoma. Lymphoma is the most common blood cancer and the third most common cancer of childhood. Lymphoma occurs when lymphocytes, a type of white blood cell, grow abnormally. The body has two types of lymphocytes: B lymphocytes, or B-cells, and T lymphocytes, or T-cells. Although both cell types can develop into lymphomas, B-cell lymphomas are more common. Like normal lymphocytes, those that turn malignant can grow in many parts of the body, including the lymph nodes, spleen, bone marrow, blood or other organs.
What is non-Hodgkin lymphoma?
Of the more than 35 types of lymphoma, 30 are classified as non-Hodgkin lymphoma (NHL). Nearly all non-Hodgkin lymphoma cases occur in adults, with the average age of diagnosis in the 60’s. While scientists do not know the exact causes of non-Hodgkin lymphoma, they do know that it is not caused by injury or by coming into contact with someone else with the disease. Most people diagnosed with non-Hodgkin lymphoma have no known risk factors, though increasingly many scientists believe infections may play an important role in causing select types of non-Hodgkin lymphoma.
What is diffuse large B-cell lymphoma?
Diffuse large B-cell lymphoma is the most common of the non-Hodgkin lymphomas, accounting for up to 30 percent of newly diagnosed cases. Diffuse large B-cell lymphoma is an aggressive, or fast-growing lymphoma. It can arise in lymph nodes or outside of the lymphatic system, in the gastrointestinal tract, testes, thyroid, skin, breast, bone or brain. Often, the first sign of diffuse large B-cell lymphoma is a painless or even painful rapid swelling in the neck, armpit or groin caused by enlarged lymph nodes. Other symptoms include night sweats, unexplained fevers and weight loss.
How is diffuse large B-cell lymphoma diagnosed?
Doctors usually diagnose diffuse large B-cell lymphoma by taking a small sample (called a biopsy) of the tumor and looking at the cells under a microscope. They will also examine other organs, such as the spleen, liver and bone marrow. Additional tests, such as blood tests, x-rays, and scans may be used and can also help determine how far the cancer has spread, indicating its “stage.” In Stage I, lymphoma appears only in one group of lymph nodes in a particular body region, while in patients with Stage II, disease in more than one lymph node group is involved but limited to one side of the diaphragm (midline of chest and abdomen). In contrast, patients with Stage III diseasehave lymphoma on both sides of the diaphragm, while those with stage IV disease have involvement of other nonlymph node organs such as the liver or bone marrow. Most patients with diffuse large B-cell lymphoma are adults, although this lymphoma is sometimes seen in children.
What treatments are available?
Because diffuse large B-cell lymphoma advances very quickly, it requires immediate treatment. A combination of chemotherapy and the monoclonal antibody Rituxan can lead to a cure in a large number of people with this form of lymphoma. Even when a cure is not possible, treatment can often keep the disease away for many years. The most widely used chemotherapy treatment is a mixture of drugs abbreviated CHOP, a combination of medicines that includes cyclophosphamide, doxorubicin, vincristine and prednisone. In addition, doctors may treat diffuse large B-cell lymphoma with radiation often in combination with chemotherapy and Rituxan. Because the chemotherapy drug doxorubicin can damage the heart, doctors closely monitor heart function during treatment.
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