Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome

Article Abstract:

Stiff-man syndrome is a rare, serious, central nervous system disease whose cause is unknown. It is characterized by increasing rigidity and painful body spasms. Some symptomatic relief can be obtained by means of drugs that enhance transmission between nerves that use GABA (gamma-aminobutyric acid) as a neurotransmitter, a substance that enables electrical signals to pass between nerve cells, or neurons. Thus, a possible cause for stiff-man syndrome is a reduction in activity of GABA-ergic (GABA-containing) neurons, due to an autoimmune reaction in which a patient's own immune system destroys such cells. To test this possibility, autoantibodies to GABA-ergic neurons were measured in blood and cerebrospinal fluid (the fluid that cushions the brain and spinal cord) from 32 patients with stiff-man syndrome. Control subjects contributed samples of blood and included people with neurologic disorders, insulin-dependent diabetes (IDDM), and autoimmune diseases. Small biopsies of brain tissue were removed from the stiff-man patients during brain surgery to treat epilepsy or tumors, and used to confirm that GAD (glutamic acid decarboxylase, the enzyme that facilitates the formation of GABA) was, indeed, the substance against which antibodies had been made (autoantigen). Results showed that autoantibodies to GABA-ergic neurons were present in 20 patients, while no autoantibodies appeared in the other 13 patients. Other types of autoantibodies also appeared in samples that contained GABA-ergic ones. In the cases where autoantibodies appeared, GAD was the autoantigen. Other autoimmune diseases, such as IDDM, were present in these patients, as were antibodies to cells in the pancreas. In summary, the results suggest that stiff-man syndrome is an autoimmune disease, with GAD the main autoantigen. The presence of autoantibodies to GABA-ergic neurons could be diagnostic for this disorder. (Consumer Summary produced by Reliance Medical Information, Inc.)

Measurement, Autoimmune diseases, Central nervous system diseases, GABA

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Autoantibodies to a 128-kd synaptic protein in three women with the stiff-man syndrome and breast cancer

Article Abstract:

The development of autoantibodies against the 128-kd brain protein may indicate the need to evaluate women with stiff-man syndrome for breast cancer. Stiff-man syndrome is an uncommon disorder of the central nervous system that is characterized by progressive muscle stiffness and spasms. A study evaluated three women with stiff-man syndrome for the presence of autoantibodies against the 128-kd brain protein. Autoantibodies against the 128-kd brain protein were detected in all three women. Two of the women had been already been diagnosed with breast cancer, and one was diagnosed after detection of autoantibodies against the 128-kd brain protein. Autoantibodies against the 128-kd brain protein were not found in 73 patients with stiff-man syndrome who did not have breast cancer or in 30 cancer patients without stiff-man syndrome. Stiff-man syndrome is associated with the development of cancer in some patients.

Breast cancer

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The effect of air pollution on lung development from 10 to 18 years of age

Article Abstract:

A study of 1759 children, with an average age of ten years, was conducted to determine the adverse effect of exposure to air pollution on the growth of lung function during the period of rapid lung development. The results indicated that the levels of air pollution have chronic, adverse effects on lung development in children from the age of ten to eighteen years, which led to clinically significant deficits in attained forced expiratory volume in one second (FEV1) as children reached adulthood.

Health aspects, Risk factors, Lung diseases, Cardiopulmonary system, Respiratory system, Air pollution, Respiratory organs

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