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We report on an autopsy case of corticobasal degeneration (CBD) with Lewy bodies in only the sympathetic ganglia. A 79-year-old man showed walking disturbance as an initial symptom, and developed dementia and bradykinesia within the next 2 years. Neurological examination revealed parkinsonism-like akinesia and rigidity in the trunk and neck without resting tremor. Brain magnetic resonance imaging showed frontal lobe atrophy predominantly on the right side. Cardiac uptake of meta-iodobenzylguanidine (MIBG) was reduced (H/M ratio: 1.14). A diagnosis of dementia with Lewy bodies (DLB) was made, but L-dopa treatment was not effective. Seven years later he died of pneumonia. On pathological examination, the frontal cortex and white matter were degenerated, predominantly on the right side. Gallyas-Braak silver staining and AT-8 immunostaining revealed neurofibrillary tangles, pretangles, argyrophilic threads, and astrocytic plaques in the cerebral cortex and basal ganglia, confirming the diagnosis of CBD. Lewy bodies, which were not seen in the central nervous system, were seen only in the sympathetic ganglia, and a severe loss of nerve fibers was apparent in the sympathetic nerve endings in the heart. MIBG is currently used to differentiate DLB from other parkinsonisms, such as CBD, multiple system atrophy, and progressive supranuclear palsy, because reduced cardiac uptake of MIBG represents a pathological change in the sympathetic nerve endings in the heart. However, the distribution of Lewy bodies cannot be determined from this finding. Thus, MIBG should not be used alone to confirm a diagnosis of DLB; other neurodegenerative diseases with incidental Lewy body disease, as in the present case, must be also considered.

rinshō shinkeigaku/rinsho shinkeigaku

Decreased myocardial uptake of meta-iodobenzylguanidine in an autopsy-confirmed case of corticobasal degeneration with Lewy bodies restricted to the sympathetic ganglia