Summary

Though the history of Lewy body related diseases started in Germany, German neurology and psychiatry still ignore the existence of dementias with cortical Lewy bodies (LB). In order to estimate the proportion of senile dementia of the Lewy body type (SDLBT) in neuropathological postmortems in Germany, we screened all brains (n = 273) of adult patients, who died in 1991 and were examined by one neuropathologist at the University Medical Center in Mainz, Germany. A multi-stage approach to detecting LBs was used with final detection by ubiquitin immunohistochemistry.

Four cases were discovered (1.5% of all cases and 4.3% of demented or confused patients). Thirty cases from the series had a neuropathological diagnosis of Alzheimer's disease (AD). All four cases with cortical LBs showed additional neurofibrillary pathology. In other years, independent of this study, solitary cases attracted attention in which no additional neurofibrillary pathology was detected. The clinical spectrum and its nosological implications are discussed and a diagnostic separation between a condition with ‘pure’ cortical Lewy bodies and one representing a combination with Alzheimer pathology is suggested.

Introduction

In 1912 the history of Lewy body (LB)-related diseases started in Germany with Friederich H. Lewy's contribution to Lewandowsky's Handbuch der Neurologie (Lewy, 1912). In this chapter, concerning the pathology of paralysis agitans, Lewy gave the first description of ‘classical’ (‘brainstem-type’) and ‘serpiginous’ LBs in the neurons of the dorsal vagal nucleus. Lewy himself did not mention the substantia nigra in particular, even when he described the occurrence of LBs in other brainstem nuclei in his famous book Die Lehre vom Tonus und der Bewegung (Lewy, 1923).