Lewy body disease is defined as Lewy body-related neuronal degeneration involving the nigrostriatal system, limbic-neocortical system, and peripheral autonomic nervous system (PANS). We investigated whether the adrenal gland, which is evolutionarily related to sympathetic ganglia and is routinely examined in general autopsy, could be used to assess pathology of the PANS in Lewy body disease. Brains, spinal cords, and adrenal glands from 783 consecutive autopsy cases from a general geriatric hospital were examined immunohistochemically with antiphosphorylated alpha-synuclein antibodies and routine staining. Parkinson disease (PD) with dementia and dementia with Lewy bodies (DLB) were defined using 1996 Consensus Guidelines for DLB and the secondary Lewy body-related alpha-synucleinopathy or amygdala variants using previously established criteria. Lewy body-related alpha-synucleinopathy was found in 207 (26.4%) of 783 cases, with 1 case solely in the adrenal gland. In all 18 PD cases with or without dementia and in 33 of 38 DLB cases, the adrenal gland was involved, but it was spared in all cases of amygdala variants. Our results indicate that the adrenal gland can provide useful information for evaluation of the PANS in Lewy body disease.
Cryptococcus neoformans (C. neoformans) var. gattii infection usually occurs in tropical and subtropical areas, and rarely in the northern hemisphere. Wereport the first Japanese with cryptococcal meningoencephalitis caused by C. neoformans var. gattii infection that occurred during a trip to Australia. This agent was identified in a cerebellar biopsy specimen by immunohistochemical technique with serotype-specific anti-sera. Because the meningitis caused by it did not respond well to conventional therapy, we used an aggressive therapeutic regimen to successfully treat the patient. Evenin areas where C. neoformans var. gattii does not exist, this infection should be considered possible as a travel-related infection.
Cerebellar damage can cause not only disturbance in motor control but also higher brain dysfunction known as cerebellar cognitive affective syndrome (CCAS). Although CCAS has a high prevalence, the precise mechanism and effective medications are unknown. We herein report a CCAS patient whose symptoms were ameliorated with the cholinesterase inhibitor donepezil. N-isopropyl-p-
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I-iodoamphetamine-single-photon emission computed tomography showed improvement in hypoperfusion in the contralateral frontal and parieto-temporal lobes. Some projections with cholinergic transmission might form a functional connectivity between the cerebellum and contralateral association cortices, and cholinergic dysfunction is involved in CCAS pathophysiology. Donepezil might be worth considering for some CCAS patients.
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