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PubMed2002

Olfactory function in idiopathic Parkinson's disease (IPD): results from cross-sectional studies in IPD patients and long-term follow-up of de-novo IPD patients.

Olfactory loss is a prominent symptom in idiopathic Parkinson's disease (IPD). Experiment 1 re-investigated the diagnostic value of psychophysical testing in the differentiation between idiopathic Parkinson disease (IPD) from non-IPD; 50 consecutive PS patients participated. In Experiment 2 five de-novo patients received 3 olfactory tests spread over a period of approximately one year. Nineteen IPD patients were anosmic, and 18 were hyposmic. All but one patient with MSA and PSP had mild/moderate hyposmia. Normosmia was found in CBD/misdiagnosed PS/psychogenic movement disorder. In Experiment 2, one of the de-novo patients was normosmic, 3 hyposmic, and 1 anosmic. Follow up investigations indicated decreased olfactory function in 3 patients while it improved in one. The normosmic patient retained olfactory abilities. This patient failed to respond to pharmacological treatment. In summary, olfactory tests differentiate IPD from non-IPD. Furthermore, tests of olfactory function may also be of interest in investigations related to treatment of PS.

Adult↗
PubMed2005

Electrogastrography.

Explore the source record for details and available documents.

Clinical Laboratory Techniques↗
PubMed2004

Clinical differentiation of parkinsonian syndromes: prognostic and therapeutic relevance.

Parkinson disease is the most common cause of parkinsonism, but other causes should always be excluded because they have a different prognosis, respond differently to medical treatment, and should not be managed by surgical means. However, diagnosis, even by experts, is challenging; one autopsy series showed an error rate of 24%. Distinction between various diagnostic possibilities depends on the history and examination findings. The use of certain medications, the rapid rate of disease progression, early onset of falling, the presence of certain dysautonomic symptoms, cognitive or behavioral changes, or a history of poor response to dopaminergic therapy may suggest an atypical form of parkinsonism. Postural hypotension, dementia, supranuclear ophthalmoparesis, or early postural instability should alert the examiner to consider an atypical cause of parkinsonism. Tests of autonomic function and brain imaging are often helpful in distinguishing these diseases.

Cerebral Cortex↗
PubMed2006

Nestin immunoreactivity of Purkinje cells in Creutzfeldt-Jakob disease.

Nestin, an intermediate filament protein, is mainly expressed in neural progenitor/stem cells in the central nervous system. Recently, we reported that nestin is expressed in Purkinje cells in patients with Creutzfeldt-Jakob disease (CJD). In this study, we examined a total of 19 CJD cerebella to analyze the intensity and pattern of nestin immunoreactivity of Purkinje cells in different pathological stages of degeneration in the cerebellar cortex. The results showed that the Purkinje cells were immunoreactive with nestin regardless of the severity of degenerative cerebellar cortex. Furthermore, we noted several different types of nestin immunoreactivity, indicated by diffuse and fine, coarse, and inclusion-like immunostainings within Purkinje cell bodies as well as dot-like staining outside of the cell bodies. In contrast, on examination of cerebella from non-CJD patients, 6 of 30 cases showed nestin immunoreactivity to a lesser extent. Thus, nestin-positive Purkinje cells are more common in CJD cerebella than in non-CJD cerebella. Although the mechanism of nestin expression in Purkinje cells is not yet understood, we suggest that such nestin-positive Purkinje cells are being reactivated to survive the cell death.

Adolescent↗
PubMed2004

The neuropsychiatry of Parkinson's disease and related disorders.

Parkinson's disease is associated with classical Parkinsonian features that respond to dopaminergic therapy. Neuropsychiatric sequelae include dementia, major depression, dysthymia, anxiety disorders, sleep disorders, and sexual disorders. Panic attacks are particularly common. With treatment, visual hallucinations, paranoid delusions, mania, or delirium may evolve. Psychosis is a key factor in nursing home placement, and depression is the most significant predictor of quality of life. Clozapine may be the safest treatment for psychotic features, but more research is needed to establish the efficacy of antidepressant treatments. Dementia with Lewy bodies, the second most common dementia in the elderly, may present in association with systematized delusions, depression, or RBD. Early evidence suggests the utility of rivastigmine, donepezil, low-dose olanzapine, and quetiapine in treating DLB. Parkinson-plus syndromes generally lack a good response to dopaminergic treatment and evidence additional features, including dysautonomia, cerebellar and pontine features, eye signs, and other movement disorders. MSA is associated with dysautonomia and RBD. SND (MSA-P) is associated with frontal cognitive impairments, but dementia, psychosis, and mood disorders have not been strikingly apparent unless additional pathological findings are present. In SDS (MSA-A), impotence is almost ubiquitous; urinary incontinence is frequent; depression is occasional, and sleep apnea should be treated to avoid sudden death during sleep. OPCA neuropsychiatric correlates await further definition. Progressive supranuclear palsy neuropsychiatric features include apathy, subcortical dementia, pathological emotionality, mild depression and anxiety, and lack of appreciable response to donepezil. CBD usually is recognized by early frontal dementia with ideomotor apraxia, often in the right upper extremity, attended later by poorly responsive unilateral Parkinsonism, with additional signs including cortical reflex myoclonus, limb dystonia, alien limb, oculomotor apraxia when asked to look horizontally, depression, personality changes, and, occasionally, Kluver-Bucy syndrome. The neuropsychiatry of FTDP-17 involves apraxia, executive impairment, personality changes, hyperorality, and occasional psychosis. Future research in these Parkinsonian disorders should target the characterization of neuropsychiatric sequelae and their treatment.

Anxiety↗
PubMed2004

Epidemiology and clinical features of sleep disorders in extrapyramidal disease.

Sleep disturbances are common in extrapyramidal diseases, including not only insomnia but excessive daytime sleepiness and parasomnias. In particular, complaints related to sleep are extremely common among patients affected by Parkinson's disease (PD). The underlying causes may include: patient age, associated illnesses, cognitive impairment, motor dysfunction caused by disease, neurochemical changes related to the disease, drugs, and secondary psychological responses to the disease. The exact prevalence of sleep disorders in PD is difficult to ascertain, due to the heterogeneity of patients as well as to the different criteria and methods used to diagnose and classify sleep disturbances. In this study, we will attempt to review the epidemiological data and to describe the various sleep disorders, which have been identified in extrapyramidal diseases, with particular reference to PD. There are no data available at present as to the role of gender in sleep disturbances. Finally, the benefit of sleep on extrapyramidal diseases will be addressed, taking into account that the above causes may modify the effects of sleep.

Aging↗
PubMed1999

Parkinson's disease is associated with oxidative damage to cytoplasmic DNA and RNA in substantia nigra neurons.

Oxidative damage, including modification of nucleic acids, may contribute to dopaminergic neurodegeneration in the substantia nigra (SN) of patients with Parkinson's disease (PD). To investigate the extent and distribution of nucleic acid oxidative damage in these vulnerable dopaminergic neurons, we immunohistochemically characterized a common product of nucleic acid oxidation, 8-hydroxyguanosine (8OHG). In PD patients, cytoplasmic 8OHG immunoreactivity was intense in neurons of the SN, and present to a lesser extent in neurons of the nucleus raphe dorsalis and oculomotor nucleus, and occasionally in glia. The proportion of 8OHG immunoreactive SN neurons was significantly greater in PD patients compared to age-matched controls. Midbrain sections from patients with multiple system atrophy-Parkinsonian type (MSA-P) and dementia with Lewy bodies (DLB) also were examined. These showed increased cytoplasmic 8OHG immunoreactivity in SN neurons in both MSA-P and DLB compared to controls; however, the proportion of positive neurons was significantly less than in PD patients. The regional distribution of 8OHG immunoreactive neurons within the SN corresponded to the distribution of neurodegeneration for these three diseases. Nuclear 8OHG immunoreactivity was not observed in any individual. The type of cytoplasmic nucleic acid responsible for 8OHG immunoreactivity was analyzed by preincubating midbrain sections from PD patients with RNase, DNase, or both enzymes. 8OHG immunoreactivity was substantially diminished by either RNase or DNase, and completely ablated by both enzymes. These results suggest that oxidative damage to cytoplasmic nucleic acid is selectively increased in midbrain, especially the SN, of PD patients and much less so in MSA-P and DLB patients. Moreover, oxidative damage to nucleic acid is largely restricted to cytoplasm with both RNA and mitochondrial DNA as targets.

Aged↗
PubMed1998

Differentiation of idiopathic Parkinson's disease from striatonigral degeneration and progressive supranuclear palsy using iodine-123 meta-iodobenzylguanidine myocardial scintigraphy.

Iodine-123 meta-iodobenzylguanidine ([123I]MIBG), an analogue of norepinephrine, is a tracer for functioning of sympathetic neurons. To investigate cardiac sympathetic function in PD, SND, and PSP, [123I]MIBG myocardial scintigraphy was performed in 25 patients with PD, 15 patients with SND, 14 patients with PSP, and 20 control subjects. In planar imaging studies, the heart-to-mediastinum average count ratio (H/M) was calculated for both early and delayed images. The mean value of H/M in patients with PD was significantly lower than those with SND, PSP, or no disease. Regardless of disease severity or intensity of anti-Parkinsonian pharmacotherapy, mean values for H/M were always low in patients with PD. The mean value of H/M in SND with orthostatic hypotension (OH) was lower than that in SND without OH. Although the mean value of H/M in PSP with amitriptyline treatment was significantly lower than that in PSP patients without amitriptyline treatment, there was no significant difference between the mean value of H/M in PSP patients without amitriptyline treatment and that in control. Thus, PD may have a abnormality of cardiac sympathetic function which has not been detected by previous cardiovascular autonomic studies. Moreover, particularly in early stages, [123I]MIBG myocardial scintigraphy may provide helpful diagnostic information in these akinetic-rigid syndromes.

3-Iodobenzylguanidine↗
PubMed2004

Alpha-synuclein: normal function and role in neurodegenerative diseases.

Synucleins are a family of small, highly charged proteins expressed predominantly in neurons. Since their discovery and characterization during the last decade, much has been learned about their structure, potential functions, interactions with other proteins, and roles in disease. One of these proteins, alpha-synuclein (alpha-syn), is the major building block of pathological inclusions that characterize many neurodegenerative disorders, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and neurodegeneration with brain iron accumulation type 1 (NBIA-1), which collectively are termed synucleinopathies. Furthermore, genetic and biological studies support a role for alpha-syn in the pathophysiology of these diseases. Therefore, research must be continued in order to better understand the functions of the synuclein proteins under normal physiological conditions as well as their role in diseases.

Amino Acid Sequence↗
PubMed1999

Argyrophilic glial inclusions in the midbrain of patients with Parkinson's disease and diffuse Lewy body disease are immunopositive for NACP/alpha-synuclein.

Argyrophilic glial inclusions occur in the midbrain of patients with Parkinson's disease (PD) and diffuse Lewy body disease (DLBD). These inclusions are immunohistochemically positive for NACP/alpha-synuclein but negative for tau protein. The results of the present study suggest that a primary degenerative process involves NACP/alpha-synuclein in PD and DLBD and that the process takes place not only in neurons but also in glial cells. Argyrophilic cytoplasmic inclusions, both glial and neuronal, in a variety of degenerative diseases may be grouped into two major categories; one related to aggregates of abnormally phosphorylated tau protein and the other to unusual accumulations of NACP/alpha-synuclein.

Aged↗
PubMed2000

Neurophysiological characterization of parkinsonian syndromes.

Electrophysiological examination can provide relevant information on functional abnormalities in patients with parkinsonism. The combined use of various electrodiagnostic techniques can contribute to the diagnosis of the illness, to its correct classification and differentiation from other diseases with a clinically similar presentation, and in particular the identification of the pathophysiological processes underlying some of the signs and symptoms characterizing the movement disorder. Tests which are useful in the differential diagnosis of various parkinsonian syndromes can now be performed in most electrodiagnostic laboratories. This article reviews some of the most relevant observations provided by neurophysiological studies on patients with parkinsonism, with a special focus on those that could be of more value in neurological clinical practice through their contribution to the characterization of the disease or to the recognition of underlying pathophysiological processes.

Autonomic Nervous System↗
PubMed2002

The mitochondrial common deletion in Parkinson's disease and related movement disorders.

The mitochondrial 4977-bp common deletion has been reported in some studies to occur exclusively or with increased frequency in the midbrain of patients with Parkinson's disease (PD). Other studies could not confirm these results; rather, it was suggested that the mitochondrial common deletion is associated with aging in the midbrain and not PD. One possible explanation for these conflicting results is the difficulty in quantifying mitochondrial DNA deletions or mutations in the whole midbrain or substantia nigra (SN) while only a subset of midbrain neurons degenerate in PD. In addition, none of the studies has addressed the cell types with the common deletion within the midbrain. In this study we used in situ hybridization to detect the common deletion in sections of midbrain from patients with PD, multiple system atrophy-parkinsonian type (MSA-P), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), age-matched controls, and individuals of different ages. The results demonstrated that the mitochondrial common deletion accumulated primarily in neurons but not glia in both the SN and other midbrain regions. There was no significant difference in the number or distribution of neurons with the common deletion or the average of the mean densities (AMD) of staining with the common deletion in nigral neurons among patients with PD, MSA-P, PSP, DLB, or age-matched controls. In addition, there was no difference in the number or distribution of neurons with the common deletion in nigral neurons between any age group, although there was a tendency for the common deletion to increase in the non-nigral neurons in older patients. These data indicate that accumulation of the 4977-bp common deletion in mitochondrial DNA in midbrain occurred primarily in neurons, and by this cytological approach, it was not associated with nigral neurodegeneration in the common movement disorders or aging.

Adolescent↗