stiff person syndrome

(redirected from Moersch-Woltman 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

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 ?
On the mechanism of sudden death in Moersch-Woltman syndrome.
It is called Moersch-Woltman syndrome, "stiff-man syndrome," or SMS.