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Dementia with Lewy bodies (DLB) is characterized by visual hallucinations. Isolated auditory hallucinations as the presenting psychiatric symptom are uncommon, and misdiagnosis as a primary psychiatric disorder with consequent inappropriate antipsychotic exposure is a substantial risk. A case of probable DLB presenting with isolated auditory hallucinations as the initial manifestation, accompanied by RBD, parkinsonism, and characteristic neuroimaging findings, is described. Initial misdiagnosis as schizophrenia led to severe adverse reactions to antipsychotic agents. After multimodal evaluation, treatment with a cholinesterase inhibitor, combined with a monoamine oxidase-B inhibitor and a melatonin receptor agonist, improved motor and sleep symptoms, and the patient's condition gradually stabilized. Although isolated auditory hallucinations are not a typical presenting feature of DLB, they should be regarded as a warning signal for α-synucleinopathy when occurring against a background of neurodegeneration accompanied by RBD, autonomic dysfunction, or Parkinsonism. When clinical features satisfy the criteria for probable DLB, core clinical features are sufficient to establish the diagnosis; cholinesterase inhibitors should be the first-line treatment for neuropsychiatric symptoms in DLB.
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