Category: Dystonia: Pathophysiology, Imaging
Objective: To describe a novel technology for detection of local field potentials in bilateral GPi-DBS to identify local field potential biomarker changes in dystonic camptocormia.
Background: Camptocormia is an abnormal involuntary non-fixed axial postural flexion deformity of the spine in the upright position that disappears with recumbence.[1,2] While there is no official consensus on defining camptocomia, most studies use a forward flexion angle of 45 degrees as diagnostic.[1,3] Camptocormia has been largely described in association with Parkinson’s Disease (PD) or primary dystonia; however there have been reports of its association with neuromuscular disorders, dystonia, inflammation, and lenticular lesions.[2–16] Deep brain stimulation (DBS) of bilateral globus pallidus internus (GPi) is known to be effective in dystonic camptocormia refractory to medical treatment.[17–20] However little is known about the electrophysiology behind camptocormia.
Method: We present the case of a 75 year-old man with 10 years of progressive camptocormia beginning as difficulty with running and flexion at the hips, to being bent over when upright and requiring a rolling walker (Burke-Fahn-Marsden dystonia rating scale (BFMDRS) score 7 (movement [trunk] = 3, disability [walk] = 4), with a forward angulation of ~55 degrees. He did not demonstrate any tremors, bradykinesia, hypophonia, rigidity, or other symptoms of PD. He was trialed on Artane, Sinemet, and botulinum toxin injections which did not yield satisfactory response. Given his refractory dystonic camptocormia, he underwent bilateral GPi-DBS. A new DBS technology capable of sensing local field potentials from the implanted leads was used to correlate the patient’s symptoms with the detected local field potentials (LFPs).
Results: Preliminary data shows that when the patient is symptomatic with forced postural extension while standing, there is an increase in LFP activity in the beta band ~15Hz compared to an asymptomatic state (lying down). This is akin to the beta band (13-35Hz) related to PD motor symptoms.[21,22] DBS stimulation with attenuation of this beta peak correlated with improvement in symptoms and assisted with DBS programming in real-time. His current BFMDRS score is 6, with forward angulation of ~30 degrees.
Conclusion: We postulate the 15Hz beta band to be a biomarker of camptocormia that has not previously been observed.
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To cite this abstract in AMA style:
M. Chua, C. Harrington, S. Stanslaski, M. Hayes, G. Cosgrove, T. Herrington. Exploring Local Field Potential changes in Dystonic Camptocormia. [abstract]. Mov Disord. 2021; 36 (suppl 1). https://www.mdsabstracts.org/abstract/exploring-local-field-potential-changes-in-dystonic-camptocormia/. Accessed November 10, 2024.« Back to MDS Virtual Congress 2021
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