stiff person syndrome

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stiff person syndrome

a rare disorder manifested clinically by the continuous isometric contraction of many of the somatic muscles; contractions are usually forceful and painful and most frequently involve the trunk musculature, although limb muscles may be involved. This is an autoimmune disease, with circulating antibodies against the GABA-synthesizing enzyme and glutamic acid decarboxylase, among other types of antibodies present.
Synonym(s): stiff man syndrome

stiff person syndrome

a rare disorder manifested clinically by the continuous isometric contraction of many of the somatic muscles; contractions are usually forceful and painful and most frequently involve the trunk musculature, although limb muscles may be involved. This is an autoimmune disease, with circulating antibodies against the GABA-synthesizing enzyme and glutamic acid decarboxylase, among other types of antibodies present.
Synonym(s): stiff man syndrome
A rare disabling GABAergic autoimmune motor dysfunction with a 2:1 male:female ratio
Aetiology Autoimmune disorder linked to production of anti-GAD65 antibodies against glutamic acid decarboxylase in 60% of patients and pancreatic islet cells; remaining 40% have other autoantibodies. It is unclear whether anti-GAD antibodies cause the loss of spinal inhibition. Some patients with cancer-related stiff-person syndrome have autoantibodies against a 128 kD synaptic protein
Diagnosis Simultaneous video-electroencephalographic surface EMG demonstrates continuous motor unit activity in affected muscles at rest, abnormal activity of small gamma motor neurons
Management Benzodiazepines, cortisol if adrenocortical dysfunction, plasma exchange, IVIG—IV immunoglobulins are well-tolerated and effective
Associated disorders Epilepsy, type 1 diabetes and other organ-specific autoimmune disorders—e.g., myasthenia gravis, thyroiditis, adrenalitis

stiff person syndrome

Stiff-man syndrome Neurology A rare GABAergic autoimmune motor dysfunction with a 2:1 ♂:♀ ratio Clinical Stiffness of axial and appendicular muscles with intermittent superimposed painful muscle spasms precipitated by emotional or physical stress, low back pain, hyperlordosis, motor and gait defects, diaphoresis, tachycardia Etiology Probably autoimmune, given presence of anti-GAD65 antibodies against glutamic acid decarboxylase in 60% of Pts and pancreatic islet cells; remaining 40% have other autoantibodies; some CA-associated SPS have autoantibodies against a 128 kD synaptic protein; associated with epilepsy, type 1 DM and other organ-specific autoimmune disorders–eg, myasthenia gravis, thyroiditis, adrenalitis Diagnosis Simultaneous video-electroencephalographic surface EMG demonstrates continuous motor unit activity in affected muscles at rest, abnormal activity of small gamma motor neurons Treatment Benzodiazepines, cortisol if adrenocortical dysfunction, plasma exchange, IVIG–well-tolerated, effective, expensive. See Anti-GAD65 antibodies.

stiff person syndrome

A rare nervous system disorder characterized by muscle pain, rigidity, spasm and severe stiffness. The condition is related to stress and is often precipitated by being startled. The disorder usually starts in one muscle group and then spreads progressively to other parts of the musculature. Often the whole of the trunk becomes involved and the spasms may become almost continuous so that the disability is severe. Sixty per cent of patients have autoantibodies to glutamic acid decarboxylase.
References in periodicals archive ?
Indeed, in 2016, the Fidelity Charitable Gift Fund, a spin-off of the global Fidelity asset management corporation, surpassed the United Way as the charity that raises the most funds in the US (Lindsay, Olsen-Phillips, and Stiffman 2016).
De Camilli, "Autoimmunity to glutamic acid decarboxylase (GAD) in stiffman syndrome and insulin-dependent diabetes mellitus," Trends in Neurosciences, vol.
Unfortunately, many African Americans are exposed to environments full of risk factors (e.g., racism, poverty) that can result in detrimental outcomes, such as low self-esteem or increased risk of depression (Repetto, Caldwell, & Zimmerman, 2004; Williams, Stiffman, & O'Neal, 1998).
Autoantibodies to glutamic acid decarboxylase in a patient with stiffman syndrome, epilepsy, and type I diabetes mellitus.
Stiffman, "Suicidal behavior in urban American Indian adolescents: a comparison with reservation youth in a southwestern state," Suicide and Life-Threatening Behavior, vol.
Louis and the University of Michigan who taught me much, including Curtis McMillen, Enola Proctor, Arlene Stiffman, and Nancy Morrow-Howell.
[39] Stiffman AR, Stelk W, Horwitz SM, Evans ME, Outlaw FH, Atkins M.
In another study, Native American youth identified cultural and tribal youth programs and the perceived sense of support that came from caring members in the community as significant factors in dealing with the adversity in their lives, which included alienation and discrimination faced in schools (Lafromboise, Hoyt, Oliver, & Whitbeck, 2006; Stiffman et al., 2007).
In order to capture any mental health services the student received outside the school, parents completed the parent version of the Service Assessment for Children and Adolescents (SACA) (Stiffman et al., 2000).
Given that delinquency and substance use generally precede sexual activity (Stiffman, Dore, Earls, & Cunningham, 1992), substance use and mental health problems could be viewed as components of a risk behavior syndrome that may predict sexual involvement and, by extension, sexual risk taking.
Due to a number of factors, including a history of oppression (Weaver & Brave Hart, 1999), Native Americans are disproportionately likely to wrestle with mental health challenges (Beals et al., 2005; Harris, Edlund, & Larson, 2005; Stiffman et al., 2006).