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Diagnostic Criteria

  • Central feature (essential for a diagnosis of possible or probable DLB)
  • Dementia defined as progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function.
  • Prominent or persistent memory impairment may not necessarily occur in the early stages but is usually evident with progression.
  • Deficits on tests of attention, executive function, and visuospatial ability may be especially prominent.
  • Core features (two core features are sufficient for a diagnosis of probable DLB, one for possible DLB)
  • Fluctuating cognition with pronounced variations in attention and alertness
  • Recurrent visual hallucinations that are typically well formed and detailed
  • Spontaneous features of parkinsonism
  • Suggestive features (If one or more of these is present in the presence of one or more core features, a diagnosis of probable DLB can be made. In the absence of any core features, one or more suggestive features is sufficient for possible DLB. Probable DLB should not be diagnosed on the basis of suggestive features alone.)
  • REM sleep behavior disorder
  • Severe neuroleptic sensitivity
  • Low dopamine transporter uptake in basal ganglia demonstrated by SPECT or PET imaging
  • Supportive features (commonly present but not proven to have diagnostic specificity)
  • Repeated falls and syncope
  • Transient, unexplained loss of consciousness
  • Severe autonomic dysfunction, e.g., orthostatic hypotension, urinary incontinence
  • Hallucinations in other modalities
  • Systematized delusions
  • Depression
  • Relative preservation of medial temporal lobe structures on CT/MRI scan
  • Generalized low uptake on SPECT/PET perfusion scan with reduced occipital activity
  • Abnormal (low uptake) MIBG myocardial scintigraphy
  • Prominent slow wave activity on EEG with temporal lobe transient sharp waves
  • A diagnosis of DLB is less likely
  • In the presence of cerebrovascular disease evident as focal neurologic signs or on brain imaging
  • In the presence of any other physical illness or brain disorder sufficient to account in part or in total for the clinical picture
  • If parkinsonism only appears for the first time at a stage of severe dementia
  • Temporal sequence of symptoms
  • DLB should be diagnosed when dementia occurs before or concurrently with parkinsonism (if it is present). The term Parkinson disease dementia (PDD) should be used to describe dementia that occurs in the context of well-established Parkinson disease. In a practice setting the term that is most appropriate to the clinical situation should be used and generic terms such as LB disease are often helpful. In research studies in which distinction needs to be made between DLB and PDD, the existing 1-year rule between the onset of dementia and parkinsonism DLB continues to be recommended. Adoption of other time periods will simply confound data pooling or comparison between studies. In other research settings that may include clinicopathologic studies and clinical trials, both clinical phenotypes may be considered collectively under categories such as LB disease or alpha-synucleinopathy.

Management

Motor Parkinsonism

  • Levodopa

Neuropsychiatric Symptoms

Visual Hallucinations

  • Cholinesterase inhibitors
  • Atypical antipsychotics

Depression

  • SSRIs or SNRIs (tricyclic antidepressants and medications with anticholinergic properties should be avoided.)

REM Sleep Behavior Disorder

  • Clonazepam
  • Melatonin
  • Quetiapine

Apathy

  • Cholinesterase inhibitors

Cognitive Symptoms

  • Cholinesterase inhibitors

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