Diffuse Lewy body disease: Disease, spectrum disorder or variety of Alzheimer disease

✍ Scribed by E. Jane Byrne

• Publisher: John Wiley and Sons
• Year: 1992
• Tongue: English
• Weight: 524 KB
• Volume: 7
• Category: Article
• ISSN: 0885-6230
• DOI: 10.1002/gps.930070403

✦ Synopsis

It is unusual, but not unique, for a non-infectious disease to rise from relative obscurity to prominence in as short a period as five years. In 1987 (Byrne et af., 1987) when we described our first two cases of' diffuse Lewy body disease (DLB) we identified 51 other cases in the literature to that date. In the last year there have been several postmortem studies of patients with dementia in which DLB is reported as being common (eg Perry et al., 1990; Burns et al., 1990; Clark et al., 1986), with reported frequencies in such series ranging from 12% to 20% of cases. The condition has now acquired three names (diffuse Lewy body disease-DLB; senile dementia of the Lewy body type-SDLT; Lewy body variant of Alzheimer's disease-LBV), which reflect the ongoing nosological debate.

LEWY BODIES

Lewy bodies (LB) are intracystoplasmic eosinophilic hyaline inclusion bodies which are associated with 'incipient or progressive neuronal degeneration' (Gibb, 1987). In the 'classical' form they are composed of a dense core of granular material surrounded by a halo of radiating filaments of between 7 and 20 ym, in diameter (Duffy and Tennyson, 1965). They vary in their shape and size in different locations in the brain (Gibb, 1987), having been found in substantia nigra, locus coeruleus, nucleus basalis, raphe nuclei, hypothalamus, oculomotor nucleus, dorsal vagal nucleus, sympathetic ganglia, and cerebral cortex (