Synucleinopathies, with and without dementia, encompass a wide range of diseases including Parkinson's disease, multiple system atrophy, rapid eye movement (REM) sleep behavior disorder, and dementia with Lewy bodies (DLB). DLB is a neurodegenerative disorder resulting in slowly progressive and unrelenting dementia until death.
Diagnostic Criteria for Dementia with Lewy Bodies (DLB)
Central feature
- Progressive dementia: deficits in attention and executive function are typical. Prominent memory impairment may not be evident in the early stages.
Core features:
- Fluctuating cognition with pronounced variations in attention and alertness.
- Recurrent complex visual hallucinations
- Spontaneous features of Parkinsonism.
Suggestive features:
- REM sleep behaviour disorder (RBD), which can appear years before the onset of dementia and Parkinsonism.
- Severe sensitivity to neuroleptics occurs in up to 50% of DLB patients who take them.
- Low dopamine transporter uptake in the brain's basal ganglia as seen on SPECT and PET imaging scans.
Supportive features:
- Repeated falls and syncope (fainting).
- Transient, unexplained loss of consciousness.
- Autonomic dysfunction.
- Hallucinations of other modalities.
- Visuospatial abnormalities like depth perception, object orientation, directional sense and illusions
- Other psychiatric disturbances like systematized delusions, aggression and depression.
A probable DLB diagnosis requires either:
- Dementia plus two or more core features, or
- Dementia plus one core feature and one or more suggestive features.
A possible DLB diagnosis requires:
- Dementia plus one core feature, or
- Dementia plus one or more suggestive features.
References:
- McKeith IG, Dickson DW, Lowe J, Emre M, O'Brien JT, Feldman H, Cummings J, Duda JE, Lippa C, Perry EK, Aarsland D, Arai H, Ballard CG, Boeve B, Burn DJ, Costa D, Del Ser T, Dubois B, Galasko D, Gauthier S, Goetz CG, Gomez-Tortosa E, Halliday G, Hansen LA, Hardy J, Iwatsubo T, Kalaria RN, Kaufer D, Kenny RA, Korczyn A, Kosaka K, Lee VM, Lees A, Litvan I, Londos E, Lopez OL, Minoshima S, Mizuno Y, Molina JA, Mukaetova-Ladinska EB, Pasquier F, Perry RH, Schulz JB, Trojanowski JQ, Yamada M; Consortium on DLB. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology. 2005 Dec 27;65(12):1863-72. [Medline]
- Weisman D, McKeith I. Dementia with Lewy bodies. Semin Neurol. 2007 Feb;27(1):42-7.[Medline]
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