Objective: Feasibility treatment in X-linked dystonia Parkinsonism with MR guided focused ultrasound.

Background: XDP is a sex-linked disease initially presented with distal dystonia and rapidly progressed to generalized symptoms. Deep brain stimulation could help relieve symptoms but the economic status and health care system for these people are also an issue. Recently, the magnetic resonance (MR)-guided focused ultrasound technology (MRgFUS) offers feasible results in treating essential tremor and tremor dominant Parkinson’s disease with optimized accuracy. We describe here the first experience of the MRgFUS treatment of XDP in Chang Bin Show Chwan Memorial hospital.

Method: This 42-year old male patient suffered from hand dystonia for 5 years and rapid progressed to generalize dystonia. XDP was diagnosed by Dr. Jamora with clinical survey, XDP gene and MRI findings. The scales of UPDRS, EQ-5D-3L, Unified Dyskinesia Rating Scale and Video-clips were collected. He was treated with unilaterally MRgFUS ablation over right pallido-thalamic tract (PTT). Final temperatures at target were between 56°-60oC. Primary relief symptoms were evaluated with subjective statement and clinical neurologic examinations. The MR examinations were performed before, during and immediately after the operation.

Results: This is the first treatment using unilateral PTT tractotomy with MRgFUS in the world. The target was defined real time with patient’s subjective statements during low power sonication. As a consequence, the treatment protocol was adapted by applying repetition of the final temperatures up to 56-600C. MRI showed a thermal lesion on PTT in T2-weighted (T2W) images after sonication. There were no neurologic deficits after treatment.

Conclusion: Our experience demonstrated the feasibility, safety, and accuracy of the MRgFUS tractotomy in treating XDP. In the treatment of movement disorders, the MRgFUS may be an alternative choice.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/first-experience-with-mr-guided-focused-ultrasound-in-the-treatment-of-x-linked-dystonia-parkinsonism-xdp/