Stiff Man Syndrome

The stiff man syndrome, also known as stiff person syndrome in America is a very rare disease related to the nerves. It is an auto-immune disorder that affects the central nervous system and leads to progressive condition of fluctuating muscle rigidity and spasms. This rigidity is mainly seen in the spinal region and lower limb area. The excess muscle tension, pressure affecting the shoulders, trunk, arms and legs cause muscle stiffness in stiff man syndrome.

The stiff man syndrome is triggered by external stimuli like a loud noise, emotional stress, etc. Many times a sudden surprise or minor physical contact, may also trigger the intermittent episodes of stiffness and spasms. Still, the exact cause remains unknown. It can affect both men and women. Moersch and Woltman were the first to describe the stiff man syndrome in 1956 at the Mayo Clinic. Studies have shown the simultaneous contraction of agonist and antagonist muscles made the patients fall down or walk like wooden men.

Synonyms
The stiff man syndrome is also known by the following names around the world:

• SMS
• Moersch-Woltmann Syndrome
• Muscular Rigidity - Progressive Spasm
• Stiff Person Syndrome

Symptoms
The symptoms of stiff man syndrome include:

• The fluctuating stiffness of the trunk and limb muscles that leads to stiffness and pain in the back.
• Exaggerated upright posture and stiff-legged walk.
• Severe muscle spasm of the arms and legs when the patient is startled, touched, upset, frightened and anxious.
• Deformed joints of the body with time and lordosis (curved lower back).
• Sudden muscle spasms makes the person fall down like a log.
• Shortness of breath as chest muscles are affected.
• Difficulty in smiling, swallowing, speaking as the cranial muscles may get affected.
• It is seen that about 10% of people with stiff man syndrome exhibit epilepsy symptoms.

Diagnosis
The stiff man syndrome is a very rare disorder and is often misdiagnosed as Parkinson's disease, multiple sclerosis, fibromyalgia or psychological disorder. Diagnosis includes physical examination that helps in identifying the symptoms. The symptoms should include rigidity and painful muscle spasms of the legs and lumbar spine. It should also affect the face, neck, abdomen and arms.

The next step of diagnosing is an antibody test. Presence of high GAD antibodies indicates stiff man syndrome. But, one should keep in mind that about 35% of individuals do not show presence of antibodies and associated cancers, although they suffer from stiff person syndrome. Electromyography is an important tool in diagnosing stiff man syndrome. Only a qualified doctor who is familiar with the findings of stiff man syndrome from the electromyography test should conduct the test.

Treatment
Treatment include administering high dosages of diazepam and anti-covulsants, gebaoentin and tiagabine. Intravenous immunoglobulin (IVIg) treatment has also shown success in reducing stiffness and decreasing the sensitivity to noise, touch and stress that leads to sudden muscle stiffness. Plasma exchange has also shown in reducing the symptoms in some patients. Corticosteroids may also help in reducing the muscle rigidity to some extent.

Prognosis
It is seen that the stiff man syndrome improves with treatment with IVIg, antianxiety drugs, anticonvulsants and pain relievers. But there is no cure for stiff man syndrome. It is very important to control the symptoms as the patients lack normal defensive reflexes. This may result in very serious and grave injuries, due to sudden fall.

If you or someone you know is suffering from sudden muscle contraction and rigidity, consult a doctor immediately. You should avoid driving and refrain from activities that require alertness and precaution. Never stop the intake of drugs prescribed by a medical practitioner abruptly without medical advice, as it may prove to be a life-threatening decision. Always wear a medical alert bracelet, that will help alert people around you to help you appropriately in case of emergency. Speak to your doctor and clear out all the doubts you may have regarding the treatment of stiff man syndrome.