ABSTRACT
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-123I-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.
Subject(s)
Cerebellar Diseases/drug therapy , Cerebellum/diagnostic imaging , Cerebrovascular Circulation/physiology , Cognition/physiology , Donepezil/therapeutic use , Cerebellar Diseases/diagnosis , Cerebellar Diseases/physiopathology , Cerebellum/blood supply , Cholinesterase Inhibitors/therapeutic use , Humans , Male , Middle Aged , Tomography, Emission-Computed, Single-PhotonABSTRACT
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.
