Stiff Person Syndrome – Symptoms, Treatment, Prognosis, Causes
What is Stiff Person Syndrome?
Stiff Person Syndrome is a rare neurological disorder that impacts the nervous system of the body. It is majorly characterized by an autoimmune disease and fluctuating muscle rigidity. The disorder generally impacts the spine and the extreme lower parts of the body. The disorder is often stimulated by the emotional stress. Most patient experience painful muscle spasms often simulated by stimuli. The stimuli can be noise, touch and emotional distress. The features of the disorder are abnormal postures, hunched and stiffed. The patient suffering from Stiff Person Syndrome may not walk properly or move. The traffic sound like horn can trigger the pain and spasms. Muscle rigidity is the major reason for pain and problem for Stiff Person Syndrome.
Symptoms of Stiff Person Syndrome
The most common symptom of the Stiff Person Syndrome is the initial axial muscle rigidity and pain. Apart from it, the exaggerated upright posture and back pain, discomfort and stiffness. This leads to stress of the patient.
The patient suffering from Stiff Person Syndrome show deprived sleep and also Rapid Eye Movement (REM) can be experienced. The continuous stiffness and pain generally do not let them sleep.
Physiological components can be visible for the patient at an early stage. At the later stage, proximal limb muscles get stiffed. The stiffness and pain become more prominent in the case of surprise, anger, fright and in other emotional stress. The contraction of abdominal muscles become even more prominent and reason for the problem.
In the later stage of Stiff Person Syndrome, the muscle stiffness may spread all over the body. The stiffness may also spread to facial bones. Deformities can also be the case in severe condition. It can lead to even skeleton fractures and muscle raptures. It can be even spontaneous rupture in Stiff Person Syndrome.
Slowly and steadily Stiff Person Syndrome sufferers become unable to move and walk properly.
Diagnosis of Stiff Person Syndrome
Diagnosis of Stiff Person Syndrome can be very complex and confusing as well. During the diagnosis, the syndrome often gets misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. However, with subsequent blood tests Stiff Person Syndrome can be diagnosed. The level of Glutamic Acid Decarboxylase (GAD) actually indicates the Stiff Person Syndrome. This is an antibody that works against an enzyme that involved the neurotransmitter in the brain. In the case of Stiff Person Syndrome it will be an elevated level GAD in the blood.
Apart from it Electromyography is an important tool to diagnose Stiff Person Syndrome. Low Frequency of Motor Unit Activity of agonist and antagonist muscles indicates the Stiff Person Syndrome in patients.
Physical examination can also be helpful for the diagnosis of Stiff Person Syndrome.
Treatment of Stiff Person Syndrome
There is no permanent cure of the Stiff Person Syndrome as of now. However, with the advancement of medical science the Stiff Person Syndrome can be reduced and suppressed. New study has established that the intravenous immunoglobulin treatment is really helpful which reduces the stiffness of the muscles. It also reduces the sensitivity of noise, touch and stress among the patient suffering from Stiff Person Syndrome.
Apart from it anti anxiety drugs, muscle relaxants, anti convulsants and pain relief drugs are used as part of the support programme for the Stiff Person Syndrome patients.
Prognosis of Stiff Person Syndrome
Even though the treatment for the Stiff Person Syndrome is available but that does no cure the syndrome. The patients suffering from the Stiff Person Syndrome generally live with the muscle pain and stiffness. It has been observed that throughout the life the patient experience frequent fall. It is described by the fact that Stiff Person Syndrome destroys the normal defensive reflexes of a person. The patient remained injury prone throughout the life. However, the symptoms of the disease can be controlled with medicines.
Life Expectancy of Stiff Person Syndrome
Life Expectancy of the patient suffering from the Stiff Person Syndrome is dependent on the criticality of the syndrome. It has been observed that the patient are generally injury prone, and that reduces their life span by considerable amount. However, the death remains unpredictable in case of Stiff Person Syndrome. If the Stiff Person Syndrome is diagnosed in babies then, it can be a difficult situation. For severe condition, babies generally meet unfortunate and untimely death within few months. For a grown adult, the normal life span of a person can be stated as 50 years. However, the average life span is often challenged and depends on the individual, condition of the individual and the course of treatment.
I was diagnosed with SPS in 2014 and can’t find a doctor that is even willing to treat this. What do I need to do?
Contact
Dr Scott Newsome
Johns Hopkins Hospital
Baltimore MD
I was diagnosed with sps, Iris, after 35 years. It was areliefactually.
You need to touch base with a doodneurologist.
Find a neuro that will treat you. Take info from your computer. Most of them are eager to learn about this condition. If not go to a teaching hospital.
Hi, My sister has this SPS. After going to many different doctors at no avail… she finally had an appointment with a neurologist who diagnosed her with the Stiff Man Syndrome. They can detect this through a blood test. Then they may want to also set you up for an EMG. You may want to have your doctor give you a referral to see a neurologist. Good luck.
My sister has had these problems with server Charlie Horses/Muscle spasms through out her upper torso for years now… In her ribs, and stomach.
After seeing many doctors over a span of years she was finally referred to a Neurologist who diagnosed her with Stiff Mans Syndrome. I feel so bad for her!!!! My suggestion to you is have your doctor give you a referral to a good Neurologist, which they can tell by a blood test if you in fact have this Syndrome.
If you’re in Utah, see Dr. Stacy Clardy, U of U Medical Center. Salt Lake City
Hi, contact the mater hospital in Brisbane Australia. We had a story on our news tonight. They have just cured a woman who had been unable to walk for 12 years. Now she can walk again. Good luck!
Just diagnosed. 45 year old female. Walk with cane but still fall. Some form of tremors everyday. Also have Hasimotos and CVID. Been on ivig for a year and ativan for a month but doesn’t help…just makes me a little foggier. You mentioned lifespan of 50 years old. So that means I have about 5 more years?
I was diagnosed with sps, Iris, after 35 years. It was areliefactually.
You need to touch base with a good neurologist.
I was diagnosed aged 43, after three years of SPS. I take diazepam, Gabapentin, Prednisone and have occasional plasma exchange (? efficacy after first few sessions). I am now 53, still working PT in a sedentary job but I walked my dog (slowly) for two hours today and I am working tomorrow at 9am.
I walk with a stick, I cannot cross roads or open spaces and occasionally experience a level of social anxiety which affects my walking. I am lucky: brilliant neurologist, brilliant neurology dept. at famous teaching hospital and four legged physiotherapist who has made me keep going.
I work with my consultant, and we make decisions based on his knowledge and my feelings/opinions. SPS is not necessarily the quick life sentence it’s made out to be.
I suffered from neck pain about 5 years ago. it was pretty intense. The orthopaedic specialist insisted it needs an operation. When I woke up, I was in severe pain. The doctor could not understand what was happening, and I lived in hell for a full year before another doctor introduced me to a neurologist. I go for octogam when I can afford it. I am fifty years old. I have spasms all over my body, even in the smaller muscles. I can move my head about 25% to each side. my knees ache, and I get confused a lot of the time. I get spasms in my chest, and a lot of flem during the night. There are a few things on my bucket list. should I do them now?I honestly want to know from you should I start executing that bucket list? I still try and work, but it is like I suffer from Alzheimer.
Your opinion PLEASE
I suffered from neck pain about 5 years ago. it was pretty intense. The orthopaedic specialist insisted it needs an operation. When I woke up, I was in severe pain. The doctor could not understand what was happening, and I lived in hell for a full year before another doctor introduced me to a neurologist. I go for octogam when I can afford it. I am fifty years old. I have spasms all over my body, even in the smaller muscles. I can move my head about 25% to each side. my knees ache, and I get confused a lot of the time. I get spasms in my chest, and a lot of flem during the night. There are a few things on my bucket list. should I do them now?I honestly want to know from you should I start executing that bucket list? I still try and work, but it is like I suffer from Alzheimer.
Your opinion PLEASE
I have CVID too along with SPS. I just had a stem cell transplant for the SPS. still in recovery, so too soon to tell.
I was diagnosed with SPS a little over 2 years ago i am now 58 years old. It took 12 years of going to several different neurologists and UCLA and UCSF to finally find a doctor that was able to find out what was wrong with me! I started IVIG treatments in May and now go monthly for 5 days at a time. I am still working full-time. I am taking backlofin , anti-seizure medicine, metformin for Type diabetes and am still functoning. It is not easy but with the medicine and IVIG I am able to work. Just trying to work as long as possible to be able to afford the treatments.
Hello, i es diagnosed with SPS a year ago , after being mis diagnosed for several years , also multiple testing that all came back negative finally my pain management doctor referred me to a neurologist that diagnosed me within a short period of time , my triggers are stress and fatigue I become over tired , he is treating me with Valium, Baclofen and tramadol seems to be working for the most part. I’m in Connecticut and medical marijuana is also prescribed for my sleep deprivation and myoclonus seizures however when I’m over tired rest and sleep is the only thing that works .
I HAVE WHOM I FEAR MAY BE READY TO JUST GIVE UP DUE TO LACK OF EDUCATION OF FRIENDS AND FAMILY AND IS CONSTANTLY ANGRY AND READY TO KILL OR FIGHT ARE THERE ANY SUPPORT GROUPS FOR PEOPLE IN THIS RARE DISEASE BATTLE ?
My son Michael at the age of 14 was diagnosed with Leukodystrophy by John Hopkins. We were sent home to Tampa Fla with no treatment.After see so many different doctors he was started on IVIG. Couple years later we were told it was MS not Leukodystrophy. Then a year later was told it was Stiff Person Syndrome and they stop the IVIG and now we are doing blood plasma that gives him some strength back that last about 4 weeks. Also started a new medicine Ocrevus infusion. Not sure how this is helping he only has had the infusion. Michael is not able to walk or even stand on his own. He spends his days in bed because if he sits up to longs he gets nauseous.He also is taking diazepam and has a baclofen pump. I hope and pray that one day we will get the news that they have the cure.
I would like to make a correction. There are cases where a person can be GAD negative. I am GAD positive, however. But my lab work showed a low positive and the neurologist who diagnosed me wanted to be certain so I was diagnosed by a spinal tap in which my level of GAD antibodies were much higher. A physician will normally tell you that a Spinal tap will always be much more accurate because the brain and spinal cord is bathed in spinal fluid.
For anyone who has this disease, you need not only a neurologist, but a neurologist who has experience with this disease. There is nothing wrong with politely asking them if they have had experience treating this disease. Because it is so rare that can be the greatest challenge.
It is also important to begin treatment as soon as possible. IVIG is commonly used to slow the progression down, while Diazepam is often used to treat the often increasingly worsening stiffening and spasms which can lead to a lack of ability to move and is often very painful and disabling.
The idea that this disease is emotional in anyway is a fallacy. While it is true that stimuli such as noise and light can worsen the symptoms, it is a physical illness in every sense of the word.