Maintenance therapy with subcutaneous immunoglobulin in a patient with immune‐mediated neuropathic postural tachycardia syndrome

Figures

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  Fig. 1. COMPASS-31 final domain scores (orthostatic, vasomotor, secretomotor, gastrointestinal, bladder, pupillomotor) and the sum score before and after therapy. There was an improvement in orthostatic, vasomotor and secretomotor symptoms and in the sum score.

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  Fig. 2. Whereas heart rate parameters in tilt table examination and daytime sleepiness (ESS = Epworth sleepiness Scale) did not worsen over time, orthostatic symptoms (sum score of points of the Winker Scale) improved by therapy. Orthostatic intolerance improved from 3 points before therapy (orthostatic symptoms occur in most occasions and orthostatic stress regularly produces symptoms, impairment of daily activities, standing time about 1 min) to 2 points (orthostatic symptoms occur frequently, at least once a week and orthostatic stress usually produces symptoms, standing time about 5 minutes). HF=Heart rate.

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  Fig. 3. Quantitative sensory testing before and after treatment: the z-profile in QST at the left side shows a central sensitization before treatment (gain of function in PPT and MPS, 2 paradoxical heat sensations), which improves under therapy. Hyperalgesia (gain of function in MPT) and hypesthesia (loss of function in MDT and CDT) developed over time. CDT = cold detection threshold; WDT = warm detection threshold; TSL = thermal sensory limen; CPT = cold pain threshold; HPT = heat pain threshold; MDT = mechanical detection threshold; MPT = mechanical pain threshold; MPS = mechanical pain sensitivity; WUR = wind-up ratio; VDT = vibration detection threshold; PPT = pressure pain threshold; DMA = dynamic mechanical allodynia; PHS = paradoxical heat sensation.