Orthostatic tremor (OT) is a rare, enigmatic, and poorly understood movement disorder triggered exclusively by standing, typically with the clinical complaint of unsteadiness and pathognomonic neurophysiologic characteristic of 13–18 Hz bursting EMG pattern, i.e., partially fused muscle contraction.1 Abnormal muscle tone control with stiffness, hyperactive startle responses, and limb spasms characterize stiff-person disorders (SPD), i.e., classic stiff-person syndrome (SPS), variant SPS, and progressive encephalomyelitis with rigidity and myoclonus.2 Autoimmunity accounts for SPD as a rule,2,3 and for OT as an exception.4,5 Progression of OT into SPS has never been reported.
Orthostatic tremor heralding the onset of stiff-person syndrome
Antelmi, Elena;
2013-01-01
Abstract
Orthostatic tremor (OT) is a rare, enigmatic, and poorly understood movement disorder triggered exclusively by standing, typically with the clinical complaint of unsteadiness and pathognomonic neurophysiologic characteristic of 13–18 Hz bursting EMG pattern, i.e., partially fused muscle contraction.1 Abnormal muscle tone control with stiffness, hyperactive startle responses, and limb spasms characterize stiff-person disorders (SPD), i.e., classic stiff-person syndrome (SPS), variant SPS, and progressive encephalomyelitis with rigidity and myoclonus.2 Autoimmunity accounts for SPD as a rule,2,3 and for OT as an exception.4,5 Progression of OT into SPS has never been reported.
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