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Practical NeurologyStiff Person syndrome (SPS) is a rare autoimmune disorder of the central nervous system characterized by stiffness and spasms in the lumbar and proximal lower limb muscles. Nonmotor symptoms include phobias, anxiety, and depression. SPS exists on a spectrum ranging from a focal disease known as the stiff limb syndrome to progressive encephalomyelitis ...
Patrick W, Cullinane +3 more
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Parkinsonism & Related Disorders, 2009 Stiff person syndrome (SPS), stiff limb syndrome, jerking SPS and progressive encephalomyelitis with rigidity and myoclonus (PERM) are a family of rare, insidiously progressive diseases of the central nervous system. They all share the core clinical features of appendicular and axial rigidity caused by continuous involuntary motor unit activity, and ...
Martin E, Duddy, Mark R, Baker
core +6 more sources
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Neurologic Clinics, 2013
Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture.
Giuseppe Ciccotto
exaly +4 more sources
Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture.
Giuseppe Ciccotto
exaly +4 more sources
Scleritis in a Person with Stiff-Person Syndrome
Ocular Immunology and Inflammation, 2007To describe a case of anterior scleritis in a patient with stiff-person syndrome.Case report.A 55-year-old woman with stable stiff-person syndrome and off immunomodulatory therapy developed unilateral anterior scleritis, which resolved over three weeks with systemic nonsteroidal anti-inflammatory therapy.The authors are unaware of reports regarding the
Mehryar Taban, Careen Y Lowder
exaly +3 more sources
Immunobiology of Stiff-Person Syndrome
International Reviews of Immunology, 2008The two possibilities to explain the pathogenic basis of stiff-person syndrome (SPS) are intrathecal sensitization of GAD65-reactive CD4+T cells and synthesis of GAD65-specific autoantibodies within the CNS [Rakocevic et al., Arch. Neurol. 61: 902-904, 2004]; and peripheral antigen sensitization followed by CNS antigen recognition by autoantibodies ...
Raghavan Raju, Christiane S Hampe
exaly +3 more sources
Successful Treatment with Rituximab in a Patient with Stiff-Person Syndrome Complicated by Dysthyroid Ophthalmopathy [PDF]
Internal Medicine, 2010 We report a patient with stiff-person syndrome and insulin-dependent diabetes mellitus with anti-glutamic acid decarboxylase (GAD) antibody, who suddenly complained of diplopia due to dysthyroid ophthalmopathy.
Nagaaki Katoh, Shû-Ichi Ikeda
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Current Treatment Options in Neurology, 2003
Stiff-person syndrome (SPS) is a progressive neurologic disorder characterized by 1) stiffness that is prominent in axial muscles, with co-contraction of agonist and antagonist muscles; 2) sudden episodic spasms; and 3) absence of another disease that causes similar symptoms.
Olavo M., Vasconcelos +1 more
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Stiff-person syndrome (SPS) is a progressive neurologic disorder characterized by 1) stiffness that is prominent in axial muscles, with co-contraction of agonist and antagonist muscles; 2) sudden episodic spasms; and 3) absence of another disease that causes similar symptoms.
Olavo M., Vasconcelos +1 more
+6 more sources
Current Treatment Options in Neurology, 2007
Classic stiff-person syndrome (SPS) is a clinically diagnosed disease characterized by axial and often appendicular rigidity with lumbar hyperlordosis and painful spasms. Supportive data include increased glutamic acid decarboxylase autoantibody titers more than 20 nmol/L, a needle electromyography with continuous motor unit activity in at least one ...
Juliana, Lockman, Ted M, Burns
openaire +4 more sources
Classic stiff-person syndrome (SPS) is a clinically diagnosed disease characterized by axial and often appendicular rigidity with lumbar hyperlordosis and painful spasms. Supportive data include increased glutamic acid decarboxylase autoantibody titers more than 20 nmol/L, a needle electromyography with continuous motor unit activity in at least one ...
Juliana, Lockman, Ted M, Burns
openaire +4 more sources
Stiff-person syndrome: an atypical presentation and a review of the literature
Hospital Practice (1995), 2021 : Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.; In this ...
Hussam A Yacoub
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STIFF EYES IN STIFF-PERSON SYNDROME
Neurology, 2008The clinical findings of stiff-person syndrome (SPS) include trunk and limb muscle stiffness and paroxysmal cramps. Antibodies directed against glutamic acid decarboxylase (GAD) has been associated with SPS. We report a patient with SPS and anti-GAD antibodies (antiGAD-Abs) with a prominent supranuclear gaze palsy and bradykinesia mimicking progressive
B, Oskarsson +5 more
openaire +2 more sources