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Degeneration of the frontal lobe
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Early memory loss
Personality changes
Rapid eye movement (REM) sleep behavior disorder
Stepwise cognitive decline
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This patient presents with fluctuating cognition and well-formed visual hallucinations with later onset of parkinsonism symptoms (rigidity, bradykinesia, shuffling gait), suggestive of dementia with Lewy bodies (DLB). One of the core clinical features of DLB is REM sleep behavior disorder. DLB is marked by the presence of at least 2 of the following core features: fluctuating cognition, recurrent visual hallucinations, REM sleep behavior disorder, and parkinsonism. REM sleep behavior disorder is the pathologic loss of REM sleep atonia, leading to dream enactment during sleep. The differential diagnosis includes Parkinson disease dementia, in which parkinsonism is present for more than 1 year prior to the onset of dementia. The diagnostic workup includes cognitive assessment (e.g., Mini-Mental State Examination) along with magnetic resonance imaging and laboratory studies to rule out treatable disorders (e.g., vitamin B12 deficiency). Patients with DLB are exquisitely sensitive to antipsychotic medications, which can exacerbate their parkinsonism and/or cause confusion and autonomic dysfunction, so antipsychotics should be avoided when possible. McKeith et al. reported the consensus of the DLB consortium on the diagnosis and management of DLB. They discussed potential diagnostic biomarkers and pointed out the lack of randomized controlled trial data on management strategies. They recommended that minor modifications to the diagnostic criteria of DLB be made, including taking into account substantia nigra neuronal loss. Incorrect Answers: Answers 1 and 3: Degeneration of the frontal lobe and personality changes refer to frontotemporal dementia, which is characterized by prominent deficits in executive function with marked personality changes. Fluctuating cognition, hallucinations, and parkinsonism would be atypical in frontotemporal dementia. Answer 2: Early memory loss characterizes Alzheimer dementia, which presents first and most prominently with memory loss. In contrast, DLB presents initially with impairments in attention and executive function; memory is affected later on. Answer 5: Stepwise cognitive decline describes vascular dementia, which is caused by successive vascular insults. Fluctuating cognition in which there is interval recovery is more characteristic of DLB. Bullet Summary: Dementia with Lewy bodies is characterized by the core clinical features of fluctuating cognition, recurrent visual hallucinations, REM sleep behavior disorder, and parkinsonism.
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