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Classic stiff-person syndrome is often found in the presence of other autoimmune diseases. This requires working with a team of doctors: your family physician, your neurologist (or neuromuscular specialist, rheumatologist, movement disorder specialist), and an endocrinologist. It may involve working with a pain management specialist or a psychologist.
DIABETES
There are four types of diabetes: Type 1 juvenile onset, Type 1.5 adult onset, Type 2 adult onset, and gestational diabetes.
Glucose is needed for energy and is provided by carbohydrates. In order for the cells to utilize and store glucose, they require insulin to act as the key to open the door to the cell.
In Juvenile Type 1 and adult Type 1.5 (LADA), autoantibodies (GAD-65) attack the insulin-producing beta cells in the Isles of Langerhans in the pancreas, therefore there are no more keys to unlock the cell doors. Without insulin, glucose builds in the bloodstream to toxic levels. This type requires replacement insulin via injection.
In type 2 diabetes, cells throughout the body develop rusty locks. The keys no longer open the cell doors sufficiently and glucose builds to dangerous levels in the bloodstream. Excessive intake of carbohydrates becomes excessive production of glucose which in turn requires ever increasing amounts of insulin. Eventually, the cells in the pancreas and/or receptors wear out from overexertion and cease producing enough insulin. This type of diabetes can be treated initially with changes in diet and exercise. Medication can help mediate the problem. If it progresses unchecked, the patient may require insulin injections.
For more information visit http://www.diabetes.org/.
THYROID DISEASE
The thyroid gland secretes hormones essential to all growth and metabolism. Thyroid disorders are found in 0.8-5% of the population and are 4 to 7 times more common in women.
Hashimoto's thyroiditis (chronic lymphocytic thyroiditis) is an autoimmune disease in which the thyroid gland is attacked by autoantibodies against thyroid peroxidase, thyroglobulin and TSH receptors, although a small percentage of patients may have none of these antibodies present. Hashimoto's thyroiditis is often misdiagnosed as depression, cyclothymia, PMS, chronic fatigue syndrome, fibromyalgia and, less frequently, as ED or an anxiety disorder.
For more information visit National Institutes of Health
Graves’ disease is an autoimmune disease in which thyroid stimulating immunoglobulins recognize and bind to the thyrotropin receptor (TSH receptor). It mimics the TSH to that receptor and activates the secretion of thyroxine (T4) and triiodothyronine (T3), and the actual TSH level will decrease in the blood plasma. The TSH levels fall because the hypothalamus-pituitary-thyroid negative feedback loop is working. The result is very high levels of circulating thyroid hormones and the negative feedback regulation will not work for the thyroid gland.
For more information visit National Institutes of Health
Endocrine myopathies cause an abnormal state of striated muscle which results in muscle weakness, pain, cramps, muscle tenderness, and spasms in various degrees.
For more information visit MedScape
ADRENAL DISEASE
Adrenal glands are small hormone-releasing organs located on top of each kidney. They are made up of an outer portion, called the cortex, and an inner portion, called the medulla. The cortex produces three hormones:
Glucocorticoid hormones (such as cortisol) maintain sugar (glucose) control, decrease (suppress) immune response, and help the body respond to stress. Mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium balance. Sex hormones, androgens (male) and estrogens (female), affect sexual development and sex drive.
In Cushing's syndrome, there's too much cortisol, while with Addison's disease, there is too little. Some people are born unable to make enough cortisol. Addison’s disease results from damage to the adrenal cortex. The damage causes the cortex to produce hormone levels that are too low.
For more information visit National Institutes of Health
Polyglandular autoimmune disease (type 1, 2, and 3) is comprised of three autoimmune conditions: diabetes, thyroid disease, and adrenal disease.
For more information visit National Institutes of Health
EPILEPSY
Epilepsies are a spectrum of brain disorders ranging from severe, life-threatening and disabling, to ones that are much more benign. The normal pattern of neuronal activity becomes disturbed, causing strange sensations, emotions, and behavior or sometimes convulsions, muscle spasms, and loss of consciousness. They have many possible causes and there are several types of seizures. Epilepsy may develop because of an abnormality in brain wiring, an imbalance of nerve signaling chemicals called neurotransmitters, changes in important features of brain cells called channels, or some combination of these and other factors.
For more information visit National Institutes of Health.
LUPUS
Lupus is an autoimmune disease that often starts between the ages of 15 and 44. Anti-nuclear antibodies play a role. Symptoms include painful or swollen joints (arthritis), unexplained fever, and extreme fatigue. A characteristic red skin rash may appear across the nose and cheeks. Rashes may also occur on the face and ears, upper arms, shoulders, chest, and hands and other areas exposed to the sun.
For more information visit National Institutes of Health
MYASTHENIA GRAVIS
Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body that increases during periods of activity and improves after periods of rest. Certain muscles such as those that control eye and eyelid movement, facial expression, chewing, talking, and swallowing are often, but not always, involved in the disorder. The muscles that control breathing and neck and limb movements may also be affected. Myasthenia gravis occurs when normal communication between the nerve and muscle is interrupted at the neuromuscular junction—the place where nerve cells connect with the muscles they control. Normally when impulses travel down the nerve, the nerve endings release a neurotransmitter substance called acetylcholine. Acetylcholine travels from the neuromuscular junction and binds to acetylcholine receptors which are activated and generate a muscle contraction. In myasthenia gravis, antibodies block, alter, or destroy the receptors for acetylcholine at the neuromuscular junction, which prevents the muscle contraction from occurring.
For more information visit National Institutes of Health
PERNICIOUS ANEMIA
Pernicious anemia is a decrease in red blood cells that occurs when the intestines cannot properly absorb vitamin B12. The body needs vitamin B12 to make red blood cells. You get this vitamin from eating foods such as meat, poultry, shellfish, eggs, and dairy products. A special protein, called intrinsic factor (IF), helps your intestines absorb vitamin B12. This protein is released by cells in the stomach. When the stomach does not make enough intrinsic factor, the intestine cannot properly absorb vitamin B12. Common causes of pernicious anemia include: weakened stomach lining (atrophic gastritis), and an autoimmune condition in which the body's immune system attacks the actual intrinsic factor protein or the cells in the lining of your stomach that make it.
For more information visit National Institutes of Health
VITILIGO
Vitiligo is a pigmentation disorder in which melanocytes (the cells that make pigment) in the skin are destroyed. As a result, white patches appear on the skin in different parts of the body. Similar patches also appear on both the mucous membranes (tissues that line the inside of the mouth and nose) and the retina (inner layer of the eyeball). The hair that grows on areas affected by vitiligo sometimes turns white. The depigmentation is caused by autoantibodies. People's bodies produce proteins called cytokines that, in vitiligo, alter their pigment-producing cells and cause these cells to die. Another theory is that melanocytes destroy themselves.
For more information visit National Institutes of Health
1. Spada PT, Spada JB. Stiff-man syndrome: a rare disorder of the central nervous system. J Neurosci Nurs. 1994 Dec;26(6):364-6. Review. Link to article.
SPS is occasionally associated with epilepsy and endocrine disorders, including insulin-dependent diabetes mellitus.
2. Dalaks, MC. Stiff Person Syndrome: Advances in Pathogenesis and Therapeutic Interventions, Curr Treat Options Neurol 2009;11:102–10. Link to article.
A 47-year-old female with muscular rigidity, new onset diabetes, and hypothyroidism.
3. Werk EE Jr, Sholiton LJ. The "stiff-man" syndrome and hyperthyroidism. Am J Med 196;31:647 Link to article.
4. Chia SY, Chua R, Lo YL, Wong MC, Chan LL, Tan EK. Acute ataxia, Graves' disease, and stiff person syndrome. Mov Disord. 2007 Oct 15;22(13):1969-71. Link to article.
5. Orija IB, Gupta M, Zimmerman RS. Graves' disease and stiff-person (stiff-man) syndrome: case report and literature review. Endocr Pract. 2005 Jul-Aug;11(4):259-64. Link to article
6. Goëb V, Dubreuil F, Cabre P, Jean-Baptiste G, Arfi S. Lupus revealing itself after a stiff-person syndrome. Lupus. 2004;13(3):215. Link to article.
7. Munhoz RP, Fameli H, Teive HA. Stiff person syndrome as the initial manifestation of systemic lupus erythematosus. Mov Disord. 2010 Mar 15;25(4):516-7. doi: 10.1002/mds.22942. Link to article.
8. Aso Y, Sato A, Narimatsu M, Takiguchi Y, Yamaguchi Y, Inukai T, Takemura Y. Stiff-man syndrome associated with antecedent myasthenia gravis and organ-specific autoimmunopathy. Intern Med. 1997 Apr;36(4):308-11.
9. Nicholas AP, Chatterjee A, Arnold MM, Claussen GC, Zorn GL Jr, Oh SJ. Stiff-persons' syndrome associated with thymoma and subsequent myasthenia gravis. Muscle Nerve. 1997 Apr;20(4):493-8. Review. Link to article.
10. Piccolo G, Martino G, Moglia A, et al. Autoimmune myasthenia gravies with thymoma following the spontaneous remission of stiff-man syndrome. Ital J. Neurol Sci 1990;11;177. Link to article.
A patient who developed generalized autoimmune myasthenia gravis six years after the spontaneous remission of a stiff-man syndrome is described. He also suffered from chronic active hepatitis and had radiological evidence of a thymoma. He did not have diabetes mellitus.
11. Saravanan PK, Paul J, Sayeed ZA. Stiff person syndrome and myasthenia gravis. Neurol India. 2002 Mar;50(1):98-100.
12. Ergün I, Akbostanci MC, Canbakan B, et al. Minimal change nephrotic syndrome with stiff-person syndrome: is there a link? Am J Kidney Dis. 2005 Jul;46(1):e11-4. Link to article.
CO-EXISTING DISEASES
Getting a Diagnosis Co-existing Diseases Differential Diagnoses Finding a Doctor Types of Doctors
Patient Rights Specialty Hospitals & Clinics Prepare for an Appointment Patient Forms
Disclaimer: The material presented in this site is intended for public educational purposes only. The author is not offering medical or legal advice. Accuracy of information is attempted but not guaranteed. Before undertaking any diet, or health improvement program, you should consult your physician. The author is in no way liable or responsible for any bodily harm, physical, mental or emotional state of any patient reacting to any of the content on this site. Thetinman.org has not examined, reviewed or tested any product or service mentioned herein. We are not being paid to advertise or promote any product or service mentioned herein. The links are offered strictly as examples of resources available. The site assumes no responsibility or liability of any kind related to the content of external sites or the usage of any product or service referenced. Links to external sites were live at the time of creation of the link. Thetinman.org does not create content for or manage external sites. The information can be changed or removed by the external site’s administrators at any time and they are responsible for the veracity of their information. Links are provided to support our data and supply additional resources. Please report broken links to administrator@thetinman.org. Thetinman.org is not a charitable foundation. It neither accepts nor distributes donations or funds of any kind.