Corpus Striatum ; Dopaminergic Neurons ; Humans ; Mesencephalon ; Parkinson Disease/drug therapy* ; Pars Compacta ; Substantia Nigra

No abstract available.
Animals ; Neurons* ; Oxidopamine* ; Rats* ; Substantia Nigra*

No abstract available.
Animals ; Immunohistochemistry* ; Neurons* ; Rats* ; Substantia Nigra*

Parkinson disease (PD) is a neurodegenerative disease characterized by the selective loss of dopaminergic neurons from the substantia nigra pars compacta leading to the impairment of motor functions. Recent genetic studies have uncovered several genes involved in inherited forms of the disease. These gene products are likely to be implicated in the biochemical pathways underlying the etiology of sporadic PD. Our review discusses the pathogenetic mechanisms of the mutated genes.
Dopaminergic Neurons ; Genetics ; Neurodegenerative Diseases ; Parkinson Disease* ; Substantia Nigra

BACKGROUND: Quantitative susceptibility mapping (QSM) has been used to measure iron accumulation in the deep nuclei of patients with Parkinson's disease (PD). This study examined the relationship between non-motor symptoms (NMSs) and iron accumulation in the deep nuclei of patients with PD. METHODS: The QSM data were acquired from 3-Tesla magnetic resonance imaging (MRI) in 29 patients with early PD and 19 normal controls. The Korean version of the NMS scale (K-NMSS) was used for evaluation of NMSs in patients. The patients were divided into high NMS and low NMS groups. The region-of-interest analyses were performed in the following deep nuclei: red nucleus, substantia nigra pars compacta, substantia nigra pars reticulata, dentate nucleus, globus pallidus, putamen, and head of the caudate nucleus. RESULTS: Thirteen patients had high NMS scores (total K-NMSS score, mean = 32.1), and 16 had low NMS scores (10.6). The QSM values in the deep were not different among the patients with high NMS scores, low NMS scores, and controls. The QSM values were not correlated linearly with K-NMSS total score after adjusting the age at acquisition of brain MRI. CONCLUSION: The study demonstrated that the NMS burdens are not associated with iron accumulation in the deep nuclei of patients with PD. These results suggest that future neuroimaging studies on the pathology of NMSs in PD should use more specific and detailed clinical tools and recruit PD patients with severe NMSs.
Basal Ganglia ; Brain ; Caudate Nucleus ; Cerebellar Nuclei ; Globus Pallidus ; Head ; Humans ; Iron* ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease* ; Pars Compacta ; Pars Reticulata ; Pathology ; Putamen ; Red Nucleus

BACKGROUND: The term 'young-onset Parkinson's disease (YOPD)' refers to patients who have developed parkison-ian symptoms or signs between the ages of 21 and 40, and the term 'old onset Parkinson's disease (OOPD)' refers to those with onset after the age of 65. Patients with YOPD may show clinical features different from those with OOPD. METHODS: We compared the clinical features and courses of Parkinson's disease between 27 patients with YOPD and 31 patients with OOPD. RESULTS: YOPD more frequently affected male patients. Patients with YOPD more frequently had family members also affected by Parkinson's disease. Rest tremor was the most frequent initial symptom in both YOPD and OOPD. Other initial symptoms included akinesia-rigidity and dystonia, but occurred more frequently in patients with YOPD than OOPD. Patients with YOPD developed levodopa induced motor complications more frequent-ly but had much less hallucinations and delusions. The disease progression of YOPD was slower than the progression of OOPD. CONCLUSIONS: Patients with YOPD may develop clinical features and courses different from those of OOPD because they have neuronal degeneration relatively confined to the substantia nigra with changes in the central pharma-cokinetics and pharmacodynamics.
Delusions ; Disease Progression ; Dystonia ; Hallucinations ; Humans ; Levodopa ; Male ; Neurons ; Parkinson Disease* ; Substantia Nigra ; Tremor

Recently the reports of the autologous grafting of adrenal medullary tissue into the brain of parkinsonian patient have given the wide attention to the neurosurgeons as well as other clinicians, because the current therapeutic modalities are either imperfect or palliative. Although neural grafting of adrenal medullary tissue of fetal brain which can supply the dopamine seems to be a ideal form of treatment theoretically, many problems must be overcome for this approach to be a routine procedure. Authors made the rat parkinsonian model by destroying the substantia nigra and nigrostriatal fiber selectively with 6-OHDA. And abnormal behaviors and growth patterns were observed and studied using rotometry, T-maze and metabolic cage. With the results, some parameters which would be useful in further experiments could be established.
Animals ; Brain ; Dopamine ; Humans ; Oxidopamine ; Parkinson Disease ; Rats* ; Substantia Nigra ; Transplants

Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests various symptoms and signs of extrapyramidal and pyramidal involvement. Authors report a 28-year-old female patient with suspected Hallervorden-Spatz disease in the aspects of clinical and MRI findings suggesting metal deposition in the globus pallidus, substantia nigra, and red nucleus on both side.
Adult ; Female ; Globus Pallidus ; Humans ; Magnetic Resonance Imaging ; Pantothenate Kinase-Associated Neurodegeneration* ; Red Nucleus ; Substantia Nigra

BACKGROUND AND PURPOSE: Previous T2 relaxometry studies have provided evidence for regional brain iron deficiency in patients with restless legs syndrome (RLS). Measurement of the iron content in several brain regions, and in particular the substantia nigra (SN), in early- and late-onset RLS patients using T2 relaxometry have yielded inconsistent results. In this study the regional iron content was assessed in patients with early- and late-onset RLS using magnetic resonance imaging (MRI), and compared the results with those in controls. METHODS: Thirty-seven patients with idiopathic RLS (20 with early onset and 17 with late onset) and 40 control subjects were studied using a 3.0-tesla MRI with a gradient-echo sampling of free induction decay and echo pulse sequence. The regions of interest in the brain were measured independently by two trained analysts using software known as medical image processing, analysis, and visualization. The results were compared and a correlation analysis was conducted to investigate which brain areas were related to RLS clinical variables. RESULTS: The iron index in the SN was significantly lower in patients with late-onset RLS than in controls (p=0.034), while in patients with early-onset RLS there was no significant difference. There was no significant correlation between the SN iron index of the late-onset RLS group and clinical variables such as disease severity. CONCLUSIONS: Late-onset RLS is associated with decreased iron content in the SN. This finding supports the hypothesis that regional brain iron deficiency plays a role in the pathophysiology of late-onset RLS.
Brain* ; Humans ; Iron ; Magnetic Resonance Imaging* ; Red Nucleus ; Restless Legs Syndrome* ; Substantia Nigra

Although secondary delayed neuronal death has been considered as a therapeutic target to minimize brain damage induced by several injuries, delayed neuronal death does not occur always. In this study, we investigated possible mechanisms that prevent delayed neuronal death in the ATP-injected substantia nigra (SN) and cortex, where delayed neuronal death does not occur. In both the SN and cortex, ATP rapidly induced death of the neurons and astrocytes in the injection core area within 3 h, and the astrocytes in the penumbra region became hypertropic and rapidly surrounded the damaged areas. It was observed that the neurons survived for up to 1-3 months in the area where the astrocytes became hypertropic. The damaged areas of astrocytes gradually reduced at 3 days, 7 days, and 1-3 months. Astrocyte proliferation was detectable at 3-7 days, and vimentin was expressed in astrocytes that surrounded and/or protruded into the damaged sites. The NeuN-positive cells also reappeared in the injury sites where astrocytes reappeared. Taken together, these results suggest that astroycte survival and/or gliosis in the injured brain may be critical for neuronal survival and may prevent delayed neuronal death in the injured brain.
Adenosine Triphosphate ; Astrocytes ; Brain Injuries ; Brain* ; Gliosis ; Neurons* ; Substantia Nigra ; Vimentin