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What is the history of Stiff Person Syndrome?

When was Stiff Person Syndrome discovered? What is the story of this discovery? Was it coincidence or not?

History of Stiff Person Syndrome

Stiff Person Syndrome (SPS) is a rare neurological disorder characterized by muscle stiffness and spasms. It was first described in the medical literature in the early 1950s by two neurologists, Moersch and Woltman. They reported a series of cases involving patients with progressive stiffness and muscle spasms, which they termed "stiff man syndrome." Over time, the term was modified to include both men and women, and it is now known as Stiff Person Syndrome.



The exact cause of SPS is still unknown, but it is believed to be an autoimmune disorder. In autoimmune diseases, the immune system mistakenly attacks the body's own tissues. In the case of SPS, the immune system targets the cells that produce a neurotransmitter called gamma-aminobutyric acid (GABA), which is responsible for inhibiting muscle contractions. The loss of GABA leads to uncontrolled muscle activity and the characteristic stiffness and spasms seen in SPS.



SPS is an extremely rare condition, with an estimated prevalence of 1 in 1 million individuals. It primarily affects adults, with symptoms typically appearing between the ages of 30 and 60. The onset of SPS is often gradual, with muscle stiffness and spasms initially occurring in the trunk and lower back. Over time, the symptoms can spread to involve other parts of the body, such as the limbs and face.



Diagnosing SPS can be challenging because its symptoms can mimic those of other neurological disorders. However, certain features can help differentiate SPS from other conditions. One of the hallmark signs of SPS is the presence of continuous muscle activity, even at rest. This is known as "axial stiffness" and can cause a person to have a rigid posture. Additionally, SPS is often associated with heightened sensitivity to sensory stimuli, such as noise, touch, or emotional stress.



Treatment for SPS aims to alleviate symptoms and improve quality of life. Medications that enhance GABA activity, such as benzodiazepines and baclofen, are commonly prescribed to reduce muscle stiffness and spasms. Physical therapy and muscle relaxants may also be beneficial in managing symptoms. In severe cases, intravenous immunoglobulin therapy or plasma exchange may be considered to modulate the immune response.



Living with SPS can be challenging, as the condition can significantly impact a person's mobility and daily activities. Supportive care, including assistive devices and modifications to the living environment, can help individuals with SPS maintain independence and improve their quality of life.



In conclusion, Stiff Person Syndrome is a rare neurological disorder characterized by muscle stiffness and spasms. It was first described in the 1950s and is believed to be an autoimmune condition targeting GABA-producing cells. Diagnosis can be challenging, but certain features distinguish SPS from other disorders. Treatment focuses on symptom management, and supportive care is essential for individuals living with SPS.


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3 answers
Thetinman.org is an excellent resource for all things related to SPS. The following is taken from the site.

At Mayo Clinic, physicians Dr. Frederick Moersch (1889-1975) and Dr. Henry Woltman (1889-1964) collected case studies of patients who presented with progressive, symmetrical rigidity of axial and proximal limb muscles.



In 1956, they presented a paper covering fourteen patients collected over thirty-two years. Ten patients were men and four were women. The average age of onset was forty-one years. All were progressive and responded poorly to treatments. Four had diabetes mellitus. Two had epilepsy: one with grand-mal seizures and one with petit-mal seizures. They concluded, because of the fluctuating nature of the symptoms and the association with diabetes, that a metabolic basis for the disease should be considered.



The disease was initially named Moersch-Woltman syndrome in their honor.

Posted Dec 25, 2021 by Pathdoc 2500
Translated from spanish Improve translation
There is a brutal form of presentation that forces you to make differential diagnosis with Tetanus.
There is an infantile form that affects a lower limb (stiff leg)
And the most common, which is that of a patient who begins to notice loss of elasticity in the sport or work activity. Pain and/or stiffness as he tries to overcome a effort. Spasms provoked by the slightest contact, sound and emotional experiences, The patient is blocked in the presence of a well-known friend or close friend, Trouble down the curb of a street and cross it. The patient tends to cross between the cars instead of the step enabled with traffic lights, to avoid being obsrvado by the drivers. The falls are mainly due to the inability to stay on a foot, The falls are accompanied by a noise glottic and in hyperextension. Are frequent falls with stitches in the chin.

Posted Sep 18, 2017 by Fernando Vela Vallejo 3250

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I am a 68 year old male, born in Scotland but live in Sydney Australia who has lived with back problems since 1973, mid 1986 while holidaying in Oregon I went white water rafting on the Rogue River on an inner tube, unfortunately I was thrown off and...
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I was misdiagnosed with Lupus in 2009 and finally got the correct diagnosis through GAD testing in Oct 2015. My neurologist, who specializes in SPS, Dr. Machado in Conn is the reason I am alive today along with my children and my husband and family. ...
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Symptoms started very gradually during/after first and only pregnancy in 2004. Full blown symptoms, including stiffness, drooping eyelids and soft palette, vertigo, dizziness, and altered gait by 2008. Diagnosed first with Myasthenia Gravis, and then...
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2009- infectious mononucleosis 2010 - diffuse toxic goiter 2011- endocrine ophthalmopathy 2013 - diabetes type 1 2013 - thyroidectomy 2014 - c-section 2015 - SPS
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> My name is Stacy Mayle and I'm 47 years old. I was diagnosed with Stiff Person Syndrome (SPS) 4 years ago, after developing symptoms at age 37. It took 10 years to diagnose since it is such a rare disorder. ...

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