Stiff person syndrome is a rare neurological disorder. Even though it is a rare condition it causes significant morbidity and mortality. This article provides information about its symptoms, diagnosis, treatment, and prognosis.
The stiff person syndrome (SPS), also known as stiff man syndrome 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 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 this condition.
The SPS 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 of this condition remains unknown. It can affect both men and women. Moersch and Woltman were the first to describe this syndrome in 1956, at the Mayo Clinic. Studies have shown that 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 as:
- Moersch-Woltmann Syndrome
- Muscular Rigidity-Progressive Spasm
Symptoms
The symptoms of this condition include:
- The fluctuating stiffness of the trunk and limb muscles that lead 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, or anxious.
- Deformed joints of the body with time, and lordosis (curved lower back).
- Sudden muscle spasms which make the person fall down.
- Shortness of breath, as chest muscles are affected.
- Difficulty in smiling, swallowing, or speaking as the cranial muscles may get affected.
- It is seen that about 10% of the people with this condition 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 some 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 is an antibody test. Presence of high GAD antibodies indicate SPS. 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 this condition.
Treatment
Treatment includes administering high dosage of diazepam, anticovulsants, gabapentin, 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 good results 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 this condition. 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 experiences sudden muscle contraction and rigidity, consult a doctor immediately. You should avoid driving and refrain from activities that require alertness. Do not stop the drugs prescribed by a medical practitioner abruptly without prior medical advice. 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.
Disclaimer: This HealthHearty article is for informative purposes only, and should not be used as a replacement for expert medical advice.
