Deep Brain stimulation of the posterior subthalamic area: a novel target for Fragile X-Associated Tremor/ataxia Syndrome (FXATS)
EANS Academy. Low J. 10/04/21; 339168; EP08003
Dr. Jacob Chen Ming Low
Contributions
Contributions
Abstract
Background: Fragile X-associated tremor/ataxia syndrome (FXATS) is a rare neurodegenerative disorder presenting with progressive generalised tremor and limb ataxia. Although normally managed medically, thalamic deep brain stimulation (DBS) has been used to control tremor with up to 73.4% improvement. Recent studies suggest that the posterior subthalamic area (PSA) and especially the dentatorubrothalamic tract (DRTT) are better targets for tremor control.
Methods: We report a case of FXATS treated with PSA-DBS where the DRTT was specifically targeted.
Results: An FXATS patient presented with a 10-year history of worsening generalised tremor but relatively mild and stable ataxia. He could not feed or clean himself due to tremor and it proved refractory to maximal medical therapy. He underwent left PSA-DBS where the DRTT was targeted using tractography. The patient experienced a profound reduction in right-sided tremor following surgery. At the 6-month assessment, the patient did not have any head or right leg tremor. He could write and feed himself with his right arm. The overall tremor score decreased from 88 to 33 whereas the tremor scores for the treated side fell from 26 to 5 (80.1% improvement). Interestingly his mini-BEST and ataxia rating scores improved following surgery due to the reduction in leg tremor.
Conclusion: Our case demonstrates that unilateral PSA-DBS can be used safely and effectively in selected FXATS patients. The DRTT may play a central role in most forms of tremor. Finally, there is a suggestion that the DRTT may exhibit somatotopy.
Methods: We report a case of FXATS treated with PSA-DBS where the DRTT was specifically targeted.
Results: An FXATS patient presented with a 10-year history of worsening generalised tremor but relatively mild and stable ataxia. He could not feed or clean himself due to tremor and it proved refractory to maximal medical therapy. He underwent left PSA-DBS where the DRTT was targeted using tractography. The patient experienced a profound reduction in right-sided tremor following surgery. At the 6-month assessment, the patient did not have any head or right leg tremor. He could write and feed himself with his right arm. The overall tremor score decreased from 88 to 33 whereas the tremor scores for the treated side fell from 26 to 5 (80.1% improvement). Interestingly his mini-BEST and ataxia rating scores improved following surgery due to the reduction in leg tremor.
Conclusion: Our case demonstrates that unilateral PSA-DBS can be used safely and effectively in selected FXATS patients. The DRTT may play a central role in most forms of tremor. Finally, there is a suggestion that the DRTT may exhibit somatotopy.
Background: Fragile X-associated tremor/ataxia syndrome (FXATS) is a rare neurodegenerative disorder presenting with progressive generalised tremor and limb ataxia. Although normally managed medically, thalamic deep brain stimulation (DBS) has been used to control tremor with up to 73.4% improvement. Recent studies suggest that the posterior subthalamic area (PSA) and especially the dentatorubrothalamic tract (DRTT) are better targets for tremor control.
Methods: We report a case of FXATS treated with PSA-DBS where the DRTT was specifically targeted.
Results: An FXATS patient presented with a 10-year history of worsening generalised tremor but relatively mild and stable ataxia. He could not feed or clean himself due to tremor and it proved refractory to maximal medical therapy. He underwent left PSA-DBS where the DRTT was targeted using tractography. The patient experienced a profound reduction in right-sided tremor following surgery. At the 6-month assessment, the patient did not have any head or right leg tremor. He could write and feed himself with his right arm. The overall tremor score decreased from 88 to 33 whereas the tremor scores for the treated side fell from 26 to 5 (80.1% improvement). Interestingly his mini-BEST and ataxia rating scores improved following surgery due to the reduction in leg tremor.
Conclusion: Our case demonstrates that unilateral PSA-DBS can be used safely and effectively in selected FXATS patients. The DRTT may play a central role in most forms of tremor. Finally, there is a suggestion that the DRTT may exhibit somatotopy.
Methods: We report a case of FXATS treated with PSA-DBS where the DRTT was specifically targeted.
Results: An FXATS patient presented with a 10-year history of worsening generalised tremor but relatively mild and stable ataxia. He could not feed or clean himself due to tremor and it proved refractory to maximal medical therapy. He underwent left PSA-DBS where the DRTT was targeted using tractography. The patient experienced a profound reduction in right-sided tremor following surgery. At the 6-month assessment, the patient did not have any head or right leg tremor. He could write and feed himself with his right arm. The overall tremor score decreased from 88 to 33 whereas the tremor scores for the treated side fell from 26 to 5 (80.1% improvement). Interestingly his mini-BEST and ataxia rating scores improved following surgery due to the reduction in leg tremor.
Conclusion: Our case demonstrates that unilateral PSA-DBS can be used safely and effectively in selected FXATS patients. The DRTT may play a central role in most forms of tremor. Finally, there is a suggestion that the DRTT may exhibit somatotopy.
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