## Abstract Mutations in __GTPโcyclohydrolase 1__ (__GCH1__) cause autosomal dominant dopaโresponsive dystonia (DRD), characterized by childhoodโonset foot dystonia that later generalizes. DRD patients frequently present with associated Parkinsonism. Conversely, earlyโonset Parkinson's disease (EOP
Early-onset Parkinson's disease
โ Scribed by Dr. P. Giovannini; I. Piccolo; S. Genitrini; P. Soliveri; F. Girotti; G. Geminiani; G. Scigliano; T. Caraceni
- Publisher
- John Wiley and Sons
- Year
- 1991
- Tongue
- English
- Weight
- 618 KB
- Volume
- 6
- Category
- Article
- ISSN
- 0885-3185
No coin nor oath required. For personal study only.
โฆ Synopsis
The study was conducted on 120 patients (76 men and 44 women) affected by idiopathic Parkinson's disease (IPD) responsive to L-dopa and observed for many years. Sixty had clinical onset between the ages of 20-40, representing 10.2% of our PD population; in the others the symptoms began after the 40th birthday. The two groups were matched for sex and length of illness. In all patients a diagnosis of IPD depended on history and clinical and neuroradiological findings. Clinical, pharmacological, evolutive, and epidemiological data were collected on all patients. Thirty-six patients from each group performed motor dexterity tests (reaction time to expected and unexpected stimuli) and cognitive tests (Wechsler Adult Intelligence Scale. Benton, Short tale, and Zazzo's speed and accuracy test). To assess the prevalence of dementia and the severity of psychiatric side effects of L-dopa administration, the 60 patients with early-onset PD were compared with 134 consecutive unselected PD patients. Five percent of early-onset PD patients had a family history of the disorder. Our study showed that early-onset PD does not differ fundamentally from the late-onset form except that the former is characterized by a more rapid establishment of the full-blown parkinsonian clinical picture and deterioration of the therapeutic efficacy of L-dopa, with an earlier appearance of side effects. The results of our neuropsychological investigations suggest that early-onset PD may be a "pure" form of extrapyramidal compromise with exclusively motor manifestations.
๐ SIMILAR VOLUMES
## Abstract It is controversial if early onset Parkinson's disease (EOPD) (onset at <41 years of age) is Parkinson's disease (PD) occuring at a younger age or a different disease. This controversy is due to some clinical and pathological differences between EOPD and PD. Within EOPD, there appear to
The role of heredity in early- versus late-onset Parkinson's disease (PD) is controversial. We estimated the degree of increased risk of PD in first-degree relatives of 221 PD probands with age of onset 50 years or younger and 266 PD probands with age of onset older than 50 years compared with the f
## Abstract Pathogenic __PINK1__ mutations have been described in PARK6โlinked Parkinson's disease (PD) patients of Asian origin. However, data on the frequency of __PINK1__ mutations in sporadic earlyโonset Parkinson's disease (EOPD) Asian patients are lacking. The objectives of this study were to
## Abstract ## Background: The definite etiology of neurodegenerative disorders such as Parkinson's disease (PD) is still unknown. Because of its role in the generation of reactive oxygen species and its association with neurodegeneration with brain iron accumulation, a possible involvement of cal