Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0752347 (Dementia with Lewy bodies)
 1,653 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Parkinson's disease (PD) is one of the major neurological diseases affecting elderly subjects. Presumed prevalence of PD is 80-100 per 100 thousands and it is estimated that there are approximately 100 thousands patients in Japan. From a statistic on age distribution of patients visiting doctors, it is estimated that patients with ages at 75-79(639/100,000) and 80-84(632/100,000) are highest in number. Juvenile parkinsonism is a syndrome with parkinsonian motor symptoms and dystonia developing before and after ten years of age. Reports on this syndrome have been accumulated in Japan and gene abnormality in a enzyme relating to dopamine metabolism was discovered in the hereditary progressive dystonia with marked diurnal fluctuation (Segawa). Among symptomatic parkinsonism, cerebrovascular parkinsonism is mainly due to multiple infarction in the basal ganglia or Binswanger's disease in Japan, and both conditions develops parkinsonism, pseudobulbar palsy and dementia progressively. Diffuse Lewy body disease and a unique type of progressive supranuclear palsy presenting a disorder designated "pure akinesia" were studied mainly in Japan. In drug therapies of PD, it has been argued why the maintenance dose of levodopa or dopamine agonists was generally low in Japan. In addition to constitution and drug metabolism, attitude of Japanese people to worry side effects more than merit with beneficial effects by a drug may have inevitably lead to low maintenance dose. Development of new drugs have been undertaken in parallel with US and Europe. Talipexole hydrochloride has been on market in Japan recently, but MAO-B inhibitors and COMT inhibitors are in final stages to wait evaluations by the governmental committee.
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PMID:[Clinical characteristics and trends in research of Parkinson's disease and parkinsonism in Japan]. 901 16

The cognitive (executive) ability of patients with Parkinson's-disease (PD) deteriorates gradually during the progression of the disease. Fluency of speech, word finding, working memory, ability to plan the future and flexibility decline. Cognitive disturbance was found to be proportional with the speech, posture, gait and balance problems and can not be influenced by L-dopa substitution. Apart the dorsal and ventral mesolimbic dopaminergic systems the coerulo-cortical noradrenergic, serotoninergic and cholinergic systems are also impaired in PD. Subcortical dementia in PD can also be explained by the functional disability of dorsolateral and anterior cingular circuits. Attention deficit can be explained by the dopamine depletion of cingular cortex. Cortical Lewy bodies, neurofibrillary tangles, neurit plaques and additional vascular pathology should also play a role in cognitive impairment of PD. In several systemic degenerative diseases associating with Parkinson's syndrome (PS) i.e. progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), multiple system atrophy (MSA) dementia can be detected with various severity, therefore the question arises concerning the correlation between cognitive disability and PS. Parkinson syndrome can also develop in frontotemporal dementias (FTD), Alzheimer's disease and cortical Lewy body disease (CLBD) but no correlation exists between motor disability and severity of dementia. In CLBD dementia can be the initial symptom in 18% of cases but PS can also preceeds the dementia. In PSP profound depletion of other monoaminergic neurotransmitter system was also reported. In FTDs associated with PS degeneration of substantia nigra, locus coeruleus and basal nucleus of Meynert has been reported with increased number of neurofibrillary tangles. In patients with vascular PS (VP) there is generally no tremor and rigidity, but pseudobulbar palsy, dementia, gate disturbance, incontinency appears; L-dopa treatment is generally ineffective. In VP no cellular loss can be found within the substantia nigra, but leukoaraiosis, lacunae in the white matter and basal ganglia are commonly demonstrated.
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PMID:[Parkinson syndrome and cognitive disorders]. 1220 Dec 29