Long-Term Bilateral Deep Brain Stimulation In Transitional Type Diffuse Lewy Body Disease: Clinical Response and Postmortem Correlation With Operative Targeting

 

Shearwood McClelland III, MD

Jean P. Vonsattel, MD

Rebeca E. Garcia, BAc

Maria D. Amaya, MD

Linda M. Winfield, RN, MPH

Seth L. Pullman, MD, FRCPC

Qiping Yu, PhD

Blair Ford, MD, FRCPC

Robert R. Goodman, MD, PhD

 

Departments of Neurological Surgery, Pathology and Neurology, Columbia University College of Physicians and Surgeons, New York, New York

 

Introduction:

Many clinical similarities exist between medically refractory Parkinson disease (PD) and diffuse Lewy body disease (DLBD), transitional type.  We present the first reported case of a patient with autopsy-confirmed transitional type DLBD who underwent bilateral deep brain stimulation (DBS) for clinically diagnosed PD.

 

Methods:

A 67-year-old medically refractory patient with persistent resting tremor, marginal response to levodopa, and onset of dementia years after motor symptomatology was clinically diagnosed with medically refractory PD and underwent chronic bilateral DBS directed to the subthalamic nucleus (STN), resulting in long-term improved motor performance until his death 40 months after electrode placement.  Postmortem studies of this patientís brain were obtained and correlated with his intraoperative microelectrode recording (MER) results, postoperative magnetic resonance (MR) imaging, and postoperative clinical outcome.

 

Results:

Brain autopsy demonstrated numerous cortical Lewy bodies and degenerative encephalopathy consistent with the diagnosis of transitional type DLBD rather than PD.  Autopsy revealed that chronic DBS elicited minor reactive changes confined to the immediate vicinity of the electrode tracks, and that both stimulators terminated in the STN region.  This anatomical finding was consistent with the patientís postoperative motor performance, MER results, and postoperative MR imaging.

 

Conclusion:

The improvement in this patientís postoperative motor performance indicates that there may be a role for DBS in the management of motor symptomatology consistent with transitional type DLBD.  To our knowledge, this is the first autopsy study of long-term DBS involving a patient with transitional type DLBD.

 

 

Objectives:

1. To understand the differences between PD and transitional type DLBD

2. To examine the impact of DBS on motor performance in transitional type DLBD

3. To understand the histopathological impact of long-term DBS on human brain tissue. 

 

Keywords:  

Deep Brain Stimulation, Diffuse Lewy Body Disease, Postmortem Analysis, Postoperative MRI