This article reviews data on the internal organization, neuronal types
, and interconnections of limbic and motor components of the human bra
in, and the specific lesions which a few of them undergo during the co
urse of Parkinson's disease (neuronal loss associated with the develop
ment of Lewy bodies and Lewy neurites). The severe involvement of nigr
al neuromelanin-laden projection cells has received particular attenti
on during the past decades. This lesion interferes with normal functio
n of the striatum and probably contributes to many of the motor dysfun
ctions characteristically occurring in Parkinson's disease. The simila
rly severe involvement of several areas and nuclei outside of the subs
tantia nigra has often escaped notice. However, the pathology of Parki
nson's disease cannot be completely described unless changes in these
extranigral areas are taken into account. Interpretation of the charac
teristic lesional pattern is facilitated by combining schemata of both
the limbic and motor systems. This approach reveals a key role by the
amygdala and related structures in extranigral pathology. Severe lesi
ons occur in the central amygdaloid nucleus, in nuclei projecting to t
he cerebral cortex in a non-specific manner, and in nuclei regulating
endocrine and autonomic functions. It is suggested that extranigral le
sions contribute to the development of behavioral changes and autonomi
c dysfunction.