The study of exercise tests in paroxysmal kinesigenic dyskinesia

Publication date: Available online 15 September 2018

Source: Clinical Neurophysiology

Author(s): Hai-Yan Zhou, Fei-Xia Zhan, Wo-Tu Tian, Chao Zhang, Yan Wang, Ze-Yu Zhu, Xiao-Li Liu, Yang-Qi Xu, Xing-Hua Luan, Xiao-Jun Huang, Sheng-Di Chen, Li Cao

Abstract

Objective

To unravel if there was muscular ion channel dysfunction in paroxysmal kinesigenic dyskinesia (PKD) patients using the exercises tests (ET).

Methods

Sixty PKD patients including 28 PRRT2 mutations carriers were enrolled in this study, as well as 19 hypokalaemic periodic paralysis (HypoPP) patients as the positive controls and 45 healthy subjects as the negative controls. ET including long exercise test (LET) and short exercise test (SET) was performed in the corresponding subjects.

Results

In the LET, both the overall PKD patients and HypoPP patients had greater CMAP amplitude and area increments during exercise than healthy controls. At most 25% of PKD patients were identified from the normality with greater amplitude increment than the area. On the contrary, 50% of HypoPP patients were differentiated with greater area increment than the amplitude. More percentage of PRRT2- patients than PRRT2+ patients had abnormal average amplitude increment. Unexpectedly, five PKD patients had abnormal maximum CMAP amplitude decrements after exercise in the LET, and one had abnormal maximum immediate amplitude decrement in the SET.

Conclusions

Distinct ET manifestations were found in PKD patients compared to normal controls and HypoPP patients.

Significance

Abnormal muscle membrane excitability might be involved in the mechanisms responsible for PKD.

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