Objective: To describe a patient who has PD and a history of PFD implanted with DBS.

Background: PD is the second most common neurogenerative disorder with 90,000 diagnosed annually in the USA¹. PD has a constellation of motor and non-motor symptoms. Motor symptoms include bradykinesia, rest tremor, rigidity and postural instability².

PFD is a rare, non-inherited skeletal disorder characterized with the replacement of normal bone tissue with fibrous tissue³. This can lead to weakened bones, deformities and increased risk of fractures³. Despite his condition, the benefits of DBS outweighed the risks.

We describe a PD patient who had predominant symptoms of left-hand tremor, bradykinesia, rigidity, gait abnormalities. Multiple medications in his regimen were rasagiline, levodopa (immediate and controlled release), pramipexole and entacapone. Medication was lasting three hours or less.

Method: The patient was clinically evaluated for on/off testing. He had significant improvement with his motor symptoms with levodopa therapy with his ON compared to his OFF-medication evaluation. MRI brain, neuropsychological testing was completed. At the regional movement disorders team meeting, his diagnosis of the PFD of the skull was reviewed. Per neurosurgery, specific risk factors of operating on someone with fibrous dysplasia of the skull and undergoing DBS could occur. E.g., stereotactic frame pins which fixate the frame to the skull during surgery could pose as an issue due to the soft nature of the fibrous dysplasia bone. Theoretical risk involves that the frame could shift even by sub millimeter and thus cause significant problems with accuracy of targeting. Fibrous dysplasia may progress over time and encroach on the DBS cranial hardware, increasing the risk of hardware erosion. Further risks were difficult to describe since this was a rare case without literature evidence of risks.

Results: DBS surgery with bilateral leads was placed in the subthalamic nuclei. No complications occurred peri or post-operatively. Post-programming, he had a significant improvement with motor symptoms. He currently maintains a minimal dose of levodopa and pramipexole. DBS improved his PD motor symptoms.   ​​​​​​​

Conclusion: DBS candidates with PD and a history of PFD can be evaluated, implanted and programmed.

References: 1) Willis, A. W., Roberts, E., Beck, J. C., Fiske, B., Ross, W., Savica, R., Van Den Eeden, S. K., Tanner, C. M., & Marras, C. (2022). Incidence of Parkinson disease in North America. npj Parkinson’s Disease, 8(1), 170.

2) Postuma, R. B., Berg, D., Stern, M., et al. (2015). MDS clinical diagnostic criteria for Parkinson’s disease. Movement Disorders, 30(12), 1591-1601.

3) Lichtenstein, L. (1938). Polyostotic fibrous dysplasia. Archives of Surgery, 36(5), 874-898.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-dbs-placement-in-a-patient-with-parkinsons-disease-pd-and-polyostotic-fibrous-dysplasia-pfd/