Sps is characterised by severe progressive muscle stiffness of the spine and lower extremities with superimposed muscle spasms triggered by external stimuli such as noise, touch and emotional distress. For symptoms like anxiety and depression, psychiatric. Stiff person syndrome sps varies from mild to severe, but if untreated it can be progressive and disabling. Stiff person syndrome is more likely seen in people with certain types of diseases including. As the disease progresses, stiffness of the leg muscles develops, and is often more pronounced on one side. The disorder is often stimulated by the emotional stress. The disorder generally impacts the spine and the extreme lower parts of the body. Autoimmune disorders including diabetes, thyroiditis, vitiligo and pernicious anemia.
Are there natural treatments that may improve the quality of life of people with stiff person syndrome. Quantitative clinical and autoimmune assessments in stiff person. The absence of underlying peripheral vascular disease with subsequent reperfusion therapy such as. This article provides information about its symptoms, diagnosis, treatment, and prognosis. The treatment of spsd centers on the triad of symptomatic treatment, immunotherapy, and tumor treatment where appropriate. Stiff person syndrome is a neurological disease and autoimmune disorder. Intravenous immunoglobulin ivig has also been used in the inpatient setting for the treatment of stiff person syndrome. There are many variants of sps, these include the classical sps, stiff leg syndrome sls, paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Posted mar 1, 2017 by michelle 1500 each sufferer will go through a process to find the correct medicationtreatments which suits the individual.
Pragmatic treatment of stiff person spectrum disorders. Introduction stiff man syndrome was first described in 1956 by moersch and woltman. Stiff person syndrome sps is one of the rarest autoimmune neurological disorders, which is mostly reported in women. Gammaaminobutyric acid is a neurotransmitter, a chemical that nerves in the brain use to send messages. This page is a variety of topics on livingcoping with a rare disorder like stiff person syndrome sps, or any chronic. Gad65 and gabarap antibodies exist in up to 70% of stiffperson patients. The british neurological surveillance unit identified 119 cases among the uk population over 5 years 20002005, implying a prevalence of 12 cases per million. Stiffperson syndrome sps is a rare neurological disorder with features of an autoimmune disease. Stiff person syndrome is a rare neurological disorder that impacts the nervous system of the body. Introduction stiff person syndrome sps is rare, although its true frequency has not been fully ascertained. Stiffness spasms later become continuous, precipitated by voluntary movements and preventing normal gait folli, 1998. More studies are needed to assist in guiding therapy for sps. However, its also regarded as an autoimmune disease. Autoimmune stiff person syndrome and related myelopathies.
Stiff person syndrome sps is a progressive syndrome characterized by recurrent episodes of severe muscle stiffness, rigidity, and painful spasms in the trunk and limbs. Stiff person syndrome sps is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord. Sps can be classified into classic sps, paraneoplastic sps, and sps variants. Although rare in general neurology practice, once observed it is unforgettable. Intravenous immunoglobulin ivig for the treatment of stiffman syndrome sms the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Stiffperson syndrome is a disabling central nervous system disorder with no satisfactory treatment that is characterized by muscle rigidity, episodic muscle spasms, high titers of antibodies.
Stiffperson syndrome sps is characterized by 1 stiffness of truncal and limb muscles due to continuous cocontracture of agonist and antagonist muscles resulting in hyperlordosis, difficulty bending or turning, and slow, widebased gait, 2 sudden spasms precipitated by unexpected noises and tactile or visual stimuli, and 3 overt anxiety with taskspecific phobias. This orphan is known as stiff person syndrome sps although actually an autoimmune disease, it is most often treated by a neurologist. Classic stiff person syndrome sps features stiffness, antiglutamic acid decarboxylase antigad antibodies, and other findings. The stiff person syndrome sps, also known as stiff man syndrome is a very rare disease related to the nerves. It is majorly characterized by an autoimmune disease and fluctuating muscle rigidity. The most common initial treatment for stiff person syndrome sps is benzodiazepines e. Intravenous immunoglobulin ivig for the treatment of. The paraneoplastic variant is associated with antiamphiphysin and antigephyrin antibodies. This case report is about the novel use of the anticd20 antibody, rituximab, in the treatment of a 41 year old woman with stiff person syndrome. However, in sps the titer of antigad antibodies does not always correlate with disease activity, and about 30 percent of patients are antibody. Successful treatment of stiff person syndrome with. Symptoms may include extreme muscle stiffness, rigidity and painful spasms in the trunk and limbs, severely impairing mobility.
Certain cancers including breast, lung, kidney, thyroid, colon and hodgkins lymphoma. Pdf treatment of stiff person syndrome with rituximab. Stiffperson syndrome sps is a rare acquired neurological disorder characterized by. Therapeutic plasma exchange in the treatment of stiff. It is characterised by fluctuating muscle rigidity and spasms. The cause of stiff person syndrome is unknown but an autoimmune mechanism is suggested because of presence of antibodies against glutamic acid. What are the best treatments for stiff person syndrome.
Benzodiazepines may work by enhancing the effects of gammaaminobutyric acid gaba in the brain. There are many other drugs used to treat stiff person syndrome since each of us is different and has different needs. Is stiff person syndrome benefited by physical therapy. An overview of stiff person syndrome health hearty. Stiff person syndrome is a rare neurological disorder.
This means we have access to the latest knowledge in stiff person syndrome and can apply that insight to each patient. The british neurological surveillance unit identified 119 cases among the uk population over 5 years 20002005, implying a. Stiff person syndrome sps is a rare disorder, characterised by fluctuating rigidity and stiffness of the axial and proximal lower limb muscles, with superimposed painful spasms and continuous motor unit activity on electromyography. Natural cure for stiffperson syndrome and alternative. Its underlying pathogenesis is probably autoimmune, as in most cases antibodies against glutamic acid decarboxylase gad are observed. This study was designed to determine the benefits of pt in cases with sps through analysis of case reports, thereby to raise awareness among physical therapist. Chronic pain, impaired mobility, and lumbar hyperlordosis are common symptoms. Stiff person syndrome sps is a rare autoimmune condition characterized by stiffness and rigidity in the lower limb muscles. Stiffperson syndrome sps, formerly called stiffman syndrome is an uncommon disorder characterized by progressive muscle stiffness, rigidity, and spasm involving the axial muscles, resulting in severely impaired ambulation. Stiff person syndrome is an extremely rare neurological condition which affects the nervous system of the body. Stiff person syndrome an overview sciencedirect topics. Stiff person syndrome symptoms, treatment, prognosis, causes. It is interesting to be aware that there is no randomized clinical trials rct research article except a few casestudy reports which have been reported about the physical therapy pt intervention for stiff person syndrome sps. Major achievements that contributed to stiff person syndrome sps treatment and research.
Intrathecal baclofen therapy, plasmapheresis, intravenous immunoglobulin, physical therapy and occupational therapy are other treatment options. What is stiff person syndromecausessymptomstreatment. Spasms can be prolonged and extremely forceful, with the ability to generate enough force to fracture. Antibodies against glutamic acid decarboxylase provide an excellent diagnostic marker, but their role in disease pathogenesis is uncertain. Stiff person syndrome is a rare disorder characterized by rigidity of axial or limb muscles and episodes of cocontraction of agonist and antagonist muscles during spasms as a result of involuntary motor unit discharges at rest.
The disease had progressed despite a favourable initial response to conventional treatment with. The usagov link left goes directly to the site section about sps and is preloaded with a search for anything stiff person syndrome. Antigad antibodies are also detected in some neurological syndromes such as ataxia in which stiffness is inconsistently present. To raise public stiff person syndrome awareness about the daily. Sps is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set off muscle spasms. Listing a study does not mean it has been evaluated by the u. New york new york physician directory stiffperson syndrome is a rare neurological disorder that is often associated with autoimmune conditions such as diabetes, thyroiditis, vitiligo, and pernicious anemia.
Multiple similar case descriptions have since followed. Sps is characterized by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch, and emotional distress, which can set. Stiff person syndrome living forward home facebook. Because sps often is misdiagnosed as a psychiatric illness and psychiatric comorbidities are common in patients with this disorder, 1 awareness and recognition of this unique condition is essential. An individual with stiff person syndrome will experience muscles spasms with severe pain stimulated normally by noise, touch, and most commonly emotional stress.
The management involves continuous monitoring of symptoms of the disease, its autoimmune associations, and potential treatment side effects fig. Stiffperson syndrome fact sheet page 2 research findings the cause of stiffperson syndrome is unknown. Here you can see if there is any natural remedy andor treatment that can help people with stiff person syndrome. Although progress has been made in understanding and treating sps, the disease remains underdiagnosed, delaying treatment.
Department of neurology, royal victoria infirmary queen victoria road, newcastle upon tyne ne1 4lp, uk. Stiff person syndrome, sps chronic neurologic disorder 1. It is characterized by rigidity andor spasticity of the skeletal muscles, tremors, and anxiety. Treatment for refractory symptoms of paraneoplastic stiff person syndrome to the editor stiff person syndrome sps is a disorder characterized by. She was admitted to hospital as an emergency with prolonged and painful extensor spasms affecting the neck and back, arms, and legs. Stiff person syndrome sps is a rare neurological disorder characterized by progressive rigidity of axial and limb muscles associated with painful spasms. Stiffperson syndrome journal of movement disorders. Stiff person syndrome sps is a rare neurological disease with features of an autoimmune disease. Know the causes, symptoms, treatment and prognosis of stiff person syndrome. The condition is frequently associated with other diseases, including malignancies. Stiffperson syndrome sps is a rare disorder characterized by continuous motor unit activity at rest and painful spasms in response to sound, touch, or electric stimulation. The length of the series is variable and dependent upon patient response.
The stiffness primarily affects the truncal muscles and is superimposed by spasms, resulting in postural deformities. Paraneoplastic stiffman syndrome also known as moerschwoltmann syndrome is a rare progressive neurological disorder characterized by progressive rigidity and stiffness of the axial musculature, associated with painful spasms, primarily in the lower limbs. Stiff person syndrome is a rare movement disorder that causes muscles of the trunk, legs and other body muscles to become stiff. Backgroundlittle information is available about the incidence of stiffman syndrome sms the classic form or its variants or about longterm treatment respon. This us government site brings together sps and spsrelated links across the internet.
Stiffperson syndrome sps, also known as stiffman syndrome sms, is a rare neurologic disorder of unclear cause characterized by progressive rigidity and stiffness. The general neurologist may see only one or two cases during his or her. Symptoms of stiffperson syndrome include heightened sensitivity to stimuli and. This neurological disease thought to be caused by an autoimmune response can leave patients unable to. Stiff person syndrome sps is a rare and disabling neurological disorder of autoimmune origin, characterized. The age that symptoms begin can vary, but most people start experiencing symptoms between ages 30 and 60.
In the first 30 pages, there are 600 randomly arranged links, not all of which are sps relatedconnected. Medical cannabis as an effective treatment for refractory. Even though it is a rare condition it causes significant morbidity and mortality. Stiff person syndrome associated with compartment syndrome. Patients with stiff person syndrome will benefit from our multifaceted approach to treatment, with insights from experts in autoimmune disorders, neurology, neurovisual problems, movement disorders and chronic pain management.
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