THE TIN MAN GUIDE TO STIFF-PERSON SYNDROME
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Corticosteroids are used as monotherapy or combined with other therapeutic agents with improvement of spasms and autoantibody titres. There has never been a good quality clinical trial to determine the overall role in SPS. However, there are case studies that showed improvement in SPS plus PERM with corticosteroids used in conjunction with plasmapheresis and IVIG.


Corticosteroids are a class of chemicals that includes steroid hormones naturally produced in the adrenal cortex. Analogues of these hormones are synthesized in laboratories. Corticosteroids are involved in a wide range of physiological processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior.


Glucocorticoids, such as cortisol, control carbohydrate, fat, and protein metabolism. They are anti-inflammatory by preventing phospholipid release, decreasing eosinophil action, and a number of other mechanisms.


Mineralocorticoids, such as aldosterone, control electrolyte and water levels, mainly by promoting sodium retention in the kidneys.


1. Hydrocortisone,  hydrocortisone acetate


This is a name for cortisol when used in oral administration, intravenous injection, or topical application. It can be used to treat people who lack adequate stores of naturally generated cortisol in their bodies. Hydrocortisone is used to treat many different conditions such as allergic disorders, skin conditions, ulcerative colitis, arthritis, lupus, psoriasis, or breathing disorders.  It is used as an immunosuppressive drug. It is given by injection in the treatment of severe allergic reactions, such as anaphylaxis and angioedema, in place of prednisolone in patients needing steroid treatment but unable take oral medication. It is used perioperatively in patients on long-term steroid treatment to prevent Addisonian crisis. It may also be injected into inflamed joints resulting from diseases such as gout. Fluticasone propionate is a corticosteroid used in nasal sprays and asthma inhalers.


Compared to hydrocortisone, prednisolone is about four times as strong and dexamethasone about forty times as strong in their anti-inflammatory effects. Drugs.com.


2. Cortisone acetate


Cortisone is one of the main hormones released by the adrenal gland in response to stress. It is a corticosteroid closely related to cortisol and is used to treat a variety of ailments. It can be administered intravenously, orally, intraarticularly (into a joint), or transcutaneously. Cortisone suppresses the immune system, thus reducing inflammation and attendant pain and swelling at the site of the injury. Risks exist, in particular in the long-term use of cortisone. Drugs.com.


3. Tixocortol pivalate, Pivalone


It is a form of hydrocortisone. Drugs. Com


4. Prednisolone


Prednisolone is a corticosteroid drug with predominant glucocorticoid and low mineralocorticoid activity, making it useful for the treatment of a wide range of inflammatory and auto-immune conditions such as: asthma, uveitis, pyoderma gangrenosum, rheumatoid arthritis, ulcerative colitis, pericarditis, temporal arteritis, Crohn's disease, Bell's palsy, multiple sclerosis, cluster headaches, vasculitis, acute lymphoblastic leukemia, autoimmune hepatitis, systemic lupus erythematosus, Kawasaki disease, and dermatomyositis. It is also used for treatment of sarcoidosis, though the mechanism is unknown. Drugs.com.


5. Methylprednisolone


Like most adrenocortical steroids, methylprednisolone is typically used for its anti-inflammatory effects. However, glucocorticoids have a wide range of effects, including changes to metabolism and immune responses. Common uses include arthritis therapy and short-term treatment of bronchial inflammation or acute bronchitis due to various respiratory diseases. It is used both in the treatment of acute periods and long-term management of autoimmune diseases, most notably systemic lupus erythematosus. It is also used as a treatment for multiple sclerosis and on penetrating spinal cord injuries. Drugs.com.


CORTICOSTEROIDS

6. Prednisone


Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases,  allergic disorders, skin conditions, ulcerative colitis, arthritis, lupus, psoriasis, and breathing disorders. Because it suppresses the immune system, it leaves patients more susceptible to infections. Drugs.com.



1. George TM, Burke JM, Sobotka PA, et al. Resolution of stiff-man syndrome with cortisol replacement in a patient with deficiencies of ACTH, growth hormone,  and prolactin. N Engl J Med 1984, 310:1511–1513.

Link to article


2. Kim JY, Chung EJ, Kim JH, Jung KY, Lee WY. Response to steroid treatment in anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia, stiff person syndrome and polyendocrinopathy. Mov Disord. 2006 Dec;21(12):2263-4. Link to article

Treatment options  GABAergic drugs   GABA A Agonists   GABA Modulators   GABA B Agonists   GABA P Agonists


GABA Transaminase   GABA RUI   GABA Analogues   GABA Supplement   Muscle Relaxers   Corticosteroids


Antiseizure   Opiate Analgesics   Experimental   IVIG   Plasmapheresis   Immunotherapy   Stem Cell Therapy  PT