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.

stiff person syndrome image

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.

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9 thoughts on “Stiff Person Syndrome – Symptoms, Treatment, Prognosis, Causes

  • 13/11/2016 at 7:10 PM

    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?

    • 24/11/2016 at 9:54 AM

      Dr Scott Newsome
      Johns Hopkins Hospital
      Baltimore MD

    • 16/12/2016 at 3:16 PM

      I was diagnosed with sps, Iris, after 35 years. It was areliefactually.

      You need to touch base with a doodneurologist.

  • 24/11/2016 at 4:30 PM

    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!

  • 08/12/2016 at 2:52 AM

    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?

    • 16/12/2016 at 3:19 PM

      I was diagnosed with sps, Iris, after 35 years. It was areliefactually.

      You need to touch base with a good neurologist.

    • 22/04/2017 at 2:21 AM

      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.

  • 27/02/2017 at 3:58 AM

    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.

  • 06/04/2017 at 2:03 AM

    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 .


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