Dementia with Lewy bodies

Historical note and nomenclature
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By Ann Marie Hake MD

The first known description of abnormal deposits of protein, later termed Lewy bodies, dates back to 1912, when Fritz Heinrich Lewy (1885-1950) detailed these neuronal inclusion bodies that would later bear his name. His findings were first published in the Handbook of Neurology, compiled by Max Lewandosky in 1912 (Lewy 1912). The following year it was presented to the German Association of Psychiatrists and Neurologists in Breslau. In 1923, he published these findings in his own Tonus and Bewegund (Muscle Tone and Movement). In 1919, Konstantin Tretiakoff was the first to ascribe the name "corps de Lewy" or "Lewy bodies" to the inclusions (Tretiakoff 1919). In 1961, Okazaki and colleagues described patients with dementia who had Lewy bodies in the neocortex (Okazaki et al 1961), but it was not until the mid-1980s that immunocytochemical methods to better identify cortical Lewy bodies were developed (ubiquitin and later alpha-synuclein immunocytochemical assays) (Sweeney et al 1997; Holdorff 2002).

The nomenclature for the combination of dementia with neocortical Lewy bodies evolved in 1989 with a proposal made by Perry and colleagues, who called it “senile dementia of Lewy body type” (Perry et al 1989). In 1990, Hansen and colleagues proposed “Lewy body variant of Alzheimer’s disease” (Hansen et al 1990), and in 2000, Kosaka suggested it be named “diffuse Lewy body disease” (Kosaka 2000). In 1995, a consortium of experts convened the First International Workshop on what was termed “dementia with Lewy bodies” to establish consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB). Two subsequent workshops (in 1999 and 2005) by the consortium have been convened to refine these guidelines (McKeith et al 1996; 1999; 2005).

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited