Objective: We present Lewy body disease (LBD) showing unique neuropathological findings which are widely recognized Lewy bodies (LBs) in the cerebral cortexes including the occipital lob and very weak neuropathological reactions in the CA2/3 region.

Background: The pathological features of Dementia with Lewy Bodies(DLB) are recognized LBs in the substantia nigra (SN), the locus coeruleus (LC), the limbic system such as cingulate gyrus and amygdala, and the cerebral cortexes with several Alzheimer’s pathology. And also showed pathological changes in the hippocampal CA2/3 region.

Method: CASE: A man who died at the age of 59. X-10 years, he suffered from a resting tremor of his left hand. He then visited the a hospital. Within a few year bradykinesia appeared. X-8 years, he visited University Hospital because of his akinesia. He was shown to have akinesia, rigidity, postural instability. Following the diagnosis of Parkinson’s disease, L-dopa treatment was started, and the effect was confirmed. A 123I MIBG H/M showed early 1.92 late 1.29. Among the symptoms he experienced were visual hallucinations, and he was moved to a dementia clinic. X-2 years, due to deterioration in his motor function, he was admitted our hospital. His motor performance and mental status improved, so he was discharged to his home. X-1 year, he was admitted by respiratory dysfunction. The DAT SBR showed right 1.00 left 1.22. X years, death was confirmed.

Results: NEUROPATHOLOGICAL FINDINGS: Macroscopic findings; Frontal lobe showed moderate atrophy. The cross sections showed substantial depigmentation in the SN and the LC. Microscopic findings; Severe neuronal cell loss was observed in the SN and LC, together with the presence of LBs. α-synuclein positive structures were also observed in the cerebral cortexes including the occipital cortex. Scattered senile plaques were observed in several cortexes but the neurofibrillary tangles are rare. Tau positive structures were observed in the entorhinal cortex. The findings of pathological changes in CA2/3 were very weak.

Conclusion: We recognized LBs in this LBD case at the occipital lob which appears in rarely most of DLB cases and the pathological change in the hippocampal CA2/3 region is hardly observed that is one of characteristic feature of DLB. We are convinced that this case will help clarify the pathology of LBs formation.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-lewy-body-disease-of-widely-confirmed-lewy-bodies-in-the-cerebral-cortexes-including-the-occipital-lobe-without-pathological-changes-in-the-hippocampal-ca2-3-region/