Lewy body disease (LBD) is a spectrum of progressive neurodegenerative disorders characterized by the wide distribution of Lewy bodies and neurites in the central and peripheral nervous system (CNS, PNS). Clinical diagnoses include Parkinson’s disease (PD), dementia with Lewy bodies, or pure autonomic failure. All types of LBD are accompanied by non-motor symptoms (NMSs) including gastrointestinal dysfunctions such as constipation. Its relationship to Lewy body-related α-synucleinopathy (Lewy pathology) of the enteric nervous system (ENS) is attracting attention because it can precede the motor symptoms. To clarify the role of ENS Lewy pathology in disease progression, we performed a clinicopathological study using the Brain Bank for Aging Research in Japan. Five-hundred and eighteen cases were enrolled in the study. Lewy pathology of the CNS and PNS, including the lower esophagus as a representative of the ENS, was examined via autopsy findings. Results showed that one-third of older people (178 cases, 34%) exhibited Lewy pathology, of which 78 cases (43.8%) exhibited the pathology in the esophagus. In the esophageal wall, Auerbach’s plexus (41.6%) was most susceptible to the pathology, followed by the adventitia (33.1%) and Meissner’s plexus (14.6%). Lewy pathology of the esophagus was significantly associated with autonomic failures such as constipation (p < 0.0001) and among PNS regions, correlated the most with LBD progression (r = 0.95, p < 0.05). These findings suggest that the propagation of esophageal Lewy pathology is a predictive factor of LBD.

Background and Purpose: Cerebellar hypoperfusion in the contralateral hemisphere after stroke is well studied and termed crossed cerebellar diaschisis. However, studies of hypoperfusion in the ipsilateral thalamus have been few. The purpose of this study was to investigate the prevalence of hypoperfusion and

Objective-Previousreports about changes in cerebral blood flow (CBF) in transient global amnesia disclosed decreased flow in some parts of the brain. However, CBF analyses in most reports were qualitative but not quantitative. The purpose of this study was to determine changes in CBF in transient global amnesia. Methods-The CBF was measured and the vasoreactive response to acetazolamide was evaluated in six patients with transient global amnesia using technetium-99m hexamethylpropylene amine oxime singlephoton emission computed tomography (SPECT). The CBF was measured during an attack in two patients and soon after an attack in the other four. About one month later, CBF was re-evaluated in each patient. Results-Two patients examined during an attack and one patient examined five hours after an attack had increased blood flow in the occipital cortex and cerebellum. Three patients examined at six to 10 hours after an attack had decreased blood flow in the thalamus, cerebellum, or putamen. These abnormalities of blood flow almost disappeared in all patients one month after onset. The vasodilatory response to acetazolamide, which was evaluated initially using SPECT, was poor in areas of increased blood flow. By the second evaluation of CBF with acetazolamide, the vasodilatory response had returned to normal. Conclusions-In a patient with transient global amnesia, CBF increased in the vertebrobasilar territory during the attack and decreased afterwards. The vasodilatory response to acetazolamide may be impaired in the parts of the brain with increased blood flow. It is suggested that transient global amnesia is distinct from migraine but may share the same underlying mechanism.

To elucidate the role of the basal ganglia and deep white matter in the visual attention mechanism, a new visual attention task was carried out by 15 patients, 9 with left-side and 6 with right-side basal ganglia and/or deep white matter damage without visual field defects, and by 12 normal subjects. Their reaction times were recorded in response to a random visual stimulation by pushing a button with the hand ipsilateral to the side of the lesion. All the patients with damage to the right side of the brain had a longer reaction time in the left space than in the right or middle space. With conventional test, only one of them showed left unilateral spatial neglect. Seven of the 9 patients with a left lesion had a significantly longer reaction time in the right space than in the left. None had unilateral spatial neglect. Both the right and left brain-damaged groups showed longer reaction times in both spaces, compared to the normal groups. There was no significant difference in reaction time among the control subjects. These findings suggest that the basal ganglia and deep white matter in each hemisphere play some role in directing visual attentional factors into both spaces. Visual attentional disturbance was highly evident even with left-side brain damage, and this kind of disturbance is not usually revealed with the current tasks used for testing unilateral spatial neglect.

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