Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the Consortium on DLB International Workshop

Abstract

Dementia with Lewy bodies (DLB) was considered to be an uncommon cause of dementia until improved neuropathological staining methods for ubiquitin were developed in the late 1980's. Subsequent recognition that 10-15% of dementia cases in older people were associated with Lewy body pathology led to the publication in 1996 of Consensus clinical and pathological diagnostic criteria for the disorder. These have greatly raised global awareness of DLB and helped to generate a body of knowledge which informs modern clinical management of this pharmacologically sensitive group of patients. They have also enabled important issues surrounding the relationships of DLB with Alzheimer's disease and Parkinson's disease to be addressed and partially resolved. A recent re-evaluation of the Consensus criteria has confirmed many aspects of the original recommendations, supplementing these with suggestions for improved pathological characterisation, clinical detection and management. Virtu-ally unrecognised 20 years ago, DLB could within this decade be one of the best characterised and potentially treatable neurodegenerative disorders of late life.

Clinical and pathological features of an Alzheimer's disease patient with the MAPT ΔK280 mutation

“…Lewy bodies were demonstrated by alpha synuclein immunohistochemistry in the brainstem, midbrain and limbic system (not shown). The extent of Lewy body disease was consistent with McKeith limbic category dementia with Lewy bodies (McKeith et al 1996), or Parkinson disease related Braak Stage IV (Braak et al 2003). There were also changes consistent with vascular dementia with moderate to severe atherosclerosis in the vessels at the base of the brain, which resulted in 50% stenosis of the left posterior cerebral artery and 75% stenosis of the left vertebral artery.…”

Phospholipase D1 regulates autophagic flux and clearance of α-synuclein aggregates

“…43 The diagnosis of DLB was based in the initial clinical presentation with dementia followed by parkinsonism and the presence of a-synuclein and ubiquitin-positive LBs in cortical and subcortical regions. 44,45 The diagnosis of PD was based on the initial presentation with parkinsonism and presence of a-synuclein and ubiquitin-positive LBs in subcortical regions.…”

Arrestins and two receptor kinases are upregulated in Parkinson's disease with dementia

“…Differential diagnosis of PD with dementia and dementia with Lewy bodies remains complicated, because at later stages clinical and neuropathological features of the two disorders are similar [93,102]. Dementia with Lewy bodies diagnosed based on published consensus criteria [70]. In particular, any case with PD preceding dementia for less than a year (the one year rule) was diagnosed as dementia with Lewy bodies and only the cases with PD preceding dementia for at least one year were classified as PD with dementia.…”

“…Of 21 PD cases, 8 (38%) had no additional clinical diagnosis, whereas 13 (62%) were additionally diagnosed with dementia. All PD cases, including those with dementia, met criteria for definite PD [70] (Table 1). Five out of 13 cases in the group with dementia and one out of 8 cases of nondemented PD patients were also assigned intermediate probability of AD according to NIA-Reagan Institute criteria (Braak score III-IV; CERAD score B) [1], whereas remaining cases showed low load of Alzheimer's pathology (Table 1).…”