Importance of juvenile parkinsonism in elucidating the pathogenesis of Parkinson’s disease
Juvenile parkinsonism provides a crucial clinical model as a prototype of dopamine deficiency syndrome caused by nigro-striatal dysfunction. It might eliminate the factors related to aging from clinical and pathobiochemical findings.
The case group of juvenile parkinsonism is situated between parkinson’s disease with onset after middle age and dopa-responsive dystonia with onset in childhood. Without study of juvenile parkinsonism, further development of nosological discussion of the dopamine deficiency syndrome will not be possible.
Interestingly, some cases have recently shown different pathological pictures in the substantia nigra from the traditional parkinsonian pathology of Lewy body pathology in autopsies. This evidence suggests that in juvenile parkinsonism, there is an underlying mechanism in the pathological process toward the lesion in the substantia nigra.
From these significant aspects, tracing of juvenile parkinsonism will contribute to finding the core of the pathophysiology and to determining the real pathogenesis of the dopamine deficiency syndrome.
In this paper, the clinical characteristics of epidemiology, symptomatology and prognosis, and pathological findings up to now, are presented. In addition, nosological discussion is developed on the basis of recent publications on juvenile parkinsonism.
KeywordsSubstantia Nigra Levodopa Treatment Juvenile Onset Lewy Body Pathology Familial Incidence
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