Objective: Emphasis of CSVD in PD through clinical-pathological correlation study.

Background: PD affects 1.7%, while CSVD affects about 45% [1], of the population aged over 60. Deep brain stimulation (DBS) can ameliorate motor symptoms of PD, while CSVD associate with deterioration of motor and cognitive function in PD [2,3]. There is no neuropathological report of PD patient treated with DBS coexisting with CSVD yet.

Method: Brain only autopsy was conducted at 9.5 hours after the patient’s death and his clinical presentations were reviewed. The brain was dissected in half and fixed for four weeks. After fixation, the brain tissue was cut coronally at 3mm intervals and regions of interest were sampled. The tissue blocks were dehydrated, paraffin embedded, and cut into 5µm sections for HE and alpha-synuclein immunostaining.

Results: Clinically, the patient had hypertension and diabetes for about ten years. He had clinical diagnosis of PD at age 51. He underwent DBS treatment at age 61 following unsatisfactory Levodopa management. At age 66, he developed confusion and cognitive fluctuation. He died from sudden death at age 69.
Pathologically, macroscopic examination showed the electrode tracts passing through the middle frontal gyrus, obliquely through the underlying white matter, caudate nucleus, internal capsule into the globus pallidus interna (GPi). The texture of the electrode tracts surrounding tissue was soft underlying with significant neuronal loss and moderate gliosis. The number of neuromelanin-laden neurons was significantly reduced, and some of the remaining neurons contained eosinophil and alpha-synuclein positive Lewy bodies. alpha-Synuclein deposition also wide spreads in multipe brain regions, consistent with Braak PD Stage 5 [4]. In addition, small blood vessel changes were magnificent, manifested with significant smooth muscle cells proliferation, thickened and rigid vessel walls, narrowed vessel lumen, and enlarged perivascular space, which was more severe in the basal ganglia.

Conclusion: The unexpected pathological findings of CSVD in this patient suggest that CSVD often clinically undercovered by PD. Although the presence of CSVD in PD is relatively rare[5], it may lead to rapid progression of PD [2,3,6].Our case suggests that it is necessary to screen for CSVD comorbidity in PD clinics particularly in those with hypertension or diabetes while with treatment of DBS.

References: [1] Hilal S, et al. Prevalence, risk factors and consequences of cerebral small vessel diseases: data from three Asian countries. J Neurol Neurosurg Psychiatry 2017;0:1–6 [2] Mollenhauer B, et al. Baseline predictors for progression 4 years after Parkinson’s disease diagnosis in the De Novo Parkinson cohort (DeNoPA). Mov Disor 2019; 34:67-77 [3] Park YW, et al. Magnetic resonance imaging – visible perivascular spaces in basal ganglia predict cognitive decline in Parkinson’s disease. Mov Disor 2019; 34:1672-1679 [4] Braak H, et al. Staging of brain pathology related to sporadic Parkinson’s disease. Neurobiology of Aging. 2003; 24:197-211 [5] Schwartz RS, et al. Small-vessel disease in patients with Parkinson’s disease: a clinicopathological study. Mov Disor 2012; 27:1506-1512 [6] Rundek T, et al. Perivascular spaces in basal ganglia – an innocent bystander in Parkinson’s disease? Mov Disor 2019; 34:1585-1587

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