Stiff Person Syndrome (redirected from Moersch-Woltman syndrome)

Stiff Person 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

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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.