Autopsy findings in a case of dementia with Lewy bodies with marked autonomic failure and repetitive cardiopulmonary arrest]

We report herein a patient with dementia with Lewy bodies (DLB) who presented with severe autonomic failure, dementia and parkinsonism. At onset, the 70-year-old man exhibited dementia and gait disturbance. Over the next 3 years, he developed symptoms of autonomic dysfunction, such as sleep apnea, orthostatic hypotension and bladder and bowel dysfunction. Cranial magnetic resonance imaging revealed moderate frontotemporal atrophy. Single photon emission computed tomography images depicted bilateral hypoperfusion in the parietal lobes. Interestingly, recurrent episodes of cardiopulmonary arrest together with unconsciousness occurred during the 2-year period before the patient died at the age of 74. At autopsy, axial slices of the brainstem showed depigmentation of the substantia nigra and locus ceruleus. Lewy bodies were present in areas of the brainstem such as the substantia nigra, locus ceruleus, solitary nucleus, raphe nucleus and dorsal vagal nucleus and in the intermediolateral column of the spinal cord, sympathetic ganglia, parahippocampal gyrus and cerebral cortex. Neuronal loss was observed in the intermediolateral column, but neurons in the sympathetic ganglia were well preserved. On the basis of the clinical history and pathological findings, we diagnosed this as a case of DLB. There are a few reported cases of DLB associated with various manifestations of autonomic failure. In our patient, autonomic failure including cardiopulmonary arrest may have resulted from widespread impairment of the autonomic nervous system.