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PubMed2003

[SPECT imaging using [123I]beta-CIT and [123I]IBF in extrapyramidal diseases].

Imaging of dopaminergic function is useful in the investigation of patients with Parkinson disease (iPD) and other extrapyramidal diseases. Using agents that bind to dopamine transporters ([123I]beta-CIT) and receptors ([123I]IBF SPECT), we investigated SPECT in 9 healthy volunteers and 24 patients for dopamine transporters as well as 15 patients for dopamine receptors. In beta-CIT SPECT studies, we examined 17 iPD patients (63.3 +/- 9.9 y/o), 3 multiple system atrophy (MSA) patients (OPCA type) (64.0 +/- 8.0 y/o), 2 vascular parkinsonism (VP) patients (71.0 +/- 0.0 y/o), 1 progressive supranuclear palsy (PSP) patient (69 y/o), 1 cortico-basal degeneration (CBD) patient (50 y/o) and nine healthy controls (39.1 +/- 9.3 y/o). For IBF SPECT studies 11 iPD patients (60.6 +/- 10.9 y/o), 3 MSA patients (2 OPCA type (50.5 +/- 3.5 y/o) and 1 SND type (65 y/o)) and 1 PSP patient (60 y/o) underwent SPECT scans after the injection of [123I]IBF. The specific to nonspecific striatal uptake ratio(St/Oc-1), ratio of putaminal uptake to caudatal uptake (Pu/Ca), and asymmetry indices (AI) were estimated. beta-CIT studies showed St/Oc-1 as follows; iPD: 2.66 +/- 1.09 (n = 17), VP: 5.73 and 7.39, MSA: 1.84 +/- 0.46 (n = 3), PSP: 2.34, CBD: 2.16. In all extrapyramidal diseases except VP, St/Oc-1 ratios were significantly lower than those in normal volunteers (6.46 +/- 1.08) (p < 0.01). Also in early-phase iPD patients (Yahr I-II), St/Oc-1 (3.16 +/- 1.49: n = 4) was significantly lower than those in normal volunteers (p < 0.01). In IBF studies, St/Oc-1 ratios were significantly higher in early-phase (Yahr I-II) iPD patients (1.82 +/- 0.25: n = 5) than those in late-phase (Yahr III-IV) iPD patients (1.38 +/- 0.32: n = 6) (p < 0.05). The Pu/Ca ratios in iPD patients (1.12 +/- 0.13) and MSA (OPCA type) patients (0.95 +/- 0.05) were higher than that in MSA (SND type) patient (0.78) and were lower than that in PSP patient (1.55). In conclusion, beta-CIT-SPECT is useful for the diagnosis of early-phase iPD patients and for differentiating VP from other extrapyramidal diseases. IBF-SPECT is useful for the diagnosis of the severity of iPD and has the possibility for ruling out MSA (SND type) or PSP from iPD. Both tracers are useful for investigating the pathophysiology of patients with iPD and other extrapyramidal diseases.

Aged↗

PubMed2003

[Contribution of oculomotor examination for the etiological diagnosis of parkinsonian syndromes].

Exploration of ocular motricity can be helpful for diagnosis in certain parkinsonian syndromes. Oculomotricity is perturbed in Parkinson's disease (PD) and in multiple system atrophy (MSA). The minimal anomalies, sometimes observed in these conditions, both clinically and on oculomotor recordings, cannot contribute significantly to diagnosis. In corticobasal degeneration (CBD), infraclinical oculomotor anomalies can be identified on oculomotor recordings and are relatively specific to atypical parkinsonian syndrome: particularly long latency of ocular saccades related to posterior parietal involvement. The most significant contribution is observed for progressive supanuclear palsy (PSP) where the oculomotor anomalies are evident at clinical examination and are even the cardinal signs of the condition: particularly paralysis of vertical ocular saccades (downbeat and upbeat) or downbeat saccades, ocular pursuit also being perturbed but not reflex movements (preservation of oculocephalic reflexes). This dissociation between palsy of saccade and pursuit movements and preservation of oculocephalic reflexes is the sign of the supranuclear origin of the oculomotor disorder in PSP. In early stage PSP before clinical expression, requiring oculomotor recordings to be recognized, it is relatively specific in the context of degenerative parkinsonian degeneration. PSP also involves other less specific anomalies such as a perturbation of the antisaccade movements (related to a frontal syndrome), anomalous ocular pursuit (becoming saccadic) and presence of square wave. A rigorous clinical examination of oculomotor function at a more advanced stage of PSP, or oculomotor recordings at an early stage of PSP or CBD, can thus contribute significantly to the diagnosis of both conditions.

Basal Ganglia↗

PubMed2003

[Treatment of non-dopamine-dependent signs in parkinsonian syndromes: evaluation and results].

With dopaminergic systems, non dopaminergic neurotransmission probably plays a major role in parkinsonian syndromes (Multiple System Atrophy, Progressive Supranuclear Palsy, Pure Autonomic Failure, Cortical basal degeneration, Lewy Body Disease). A better understanding of the pathophysiology of these syndromes led to the development of molecules that interact with non dopaminergic systems. Thus, freezing, gait and balance disorders, dysautonomia and neuropsychiatric disorders are likely to benefit from specific treatments. However, due to methodological difficulties related to the evaluation of such molecules, controlled trials are rather rare and the results are often partial and sometimes unclear.

Anti-Dyskinesia Agents↗

PubMed2003

Transgenic models of alpha-synuclein pathology: past, present, and future.

Accumulation and toxic conversion to protofibrils of alpha-synuclein has been associated with neurological disorders such as Parkinson's disease (PD), Lewy body disease, multiple system atrophy, neurodegeneration with brain iron accumulation type 1, and Alzheimer's disease. In recent years, modeling these disorders in transgenic (tg) mice and flies has helped improve understanding of the pathogenesis of these diseases and has established the basis for the development of new experimental treatments. Overexpression of alpha-synuclein in tg mice in a region- and cell-specific manner results in degeneration of selective circuitries accompanied by motor deficits and inclusion formation similar to what is found in PD and related disorders. Furthermore, studies in singly and doubly tg mice have shown that toxic conversion and accumulation can be accelerated by alpha-synuclein mutations associated with familial parkinsonism, by amyloid beta peptide 1-42 (Abeta 1-42), and by oxidative stress. In contrast, molecular chaperones such as Hsp70 and close homologues such as alpha-synuclein have been shown to suppress toxicity. Similar studies are underway to evaluate the effects of other modifying genes that might play a role in alpha-synuclein ubiquitination. Among them considerable interest has been placed on the role of molecules associated with familial parkinsonism (Parkin, UCHL-1). Furthermore, studying the targeted overexpression of alpha-synuclein and other modifier genes in the nigrostriatal and limbic system by using regulatable promoters, lentiviral vectors, and siRNA will help improve understanding of the molecular mechanisms involved in selective neuronal vulnerability, and it will aid the development of new treatments.

Animals↗

PubMedDate not supplied

Apomorphine test in de novo Parkinson's disease.

We administered apomorphine, a powerful dopaminergic agonist, subcutaneously to 25 untreated patients with parkinsonian features and evaluated motor response with the aim of discriminating idiopathic Parkinson's disease (IPD) from multiple system atrophy and progressive supranuclear palsy. The response to apomorphine was strongly predictive of responsiveness to subsequent levodopa follow-up and of the final diagnosis, made on the basis of both clinical and instrumental evaluation. Our data confirm that the apomorphine test is helpful in the differential diagnosis of IPD.

Apomorphine↗

PubMed1992

Substantia nigra degeneration in motor neurone disease: a quantitative study.

There is increasing evidence of degeneration of nerve cells other than motor neurones in sporadic motor neurone disease (MND). Sporadic MND is occasionally associated with Parkinsonism. The aim of this study was to determine whether neuronal loss occurs in the substantia nigra of MND patients. Case records and pathological material from 14 patients with MND were reviewed. No patient had Parkinsonism documented. Sections of substantia nigra from the patients and 14 age/sex matched controls were analysed by neuronal and macrophage quantitation. The mean number of pigmented neurones at one standard level of substantia nigra was 863 in cases and 1094 in controls (p < 0.02); on average 21% (95% CI, 7-35%) fewer in the cases. The mean number of macrophages containing neuromelanin in cases was 106 and in controls 45 (p < 0.02). The results indicate that patients with MND have degeneration of the substantia nigra. Thus MND should be considered as a type of multiple system atrophy in which the motor neurone bears the brunt of the disease. The implication of this to the relationship between classical MND and MND with Parkinsonism-Dementia of the Western Pacific is discussed.

Adolescent↗

PubMed2003

[Blood pressure disorders during idiopathic Parkinson's disease].

SEVERAL EXPLANATIONS FOR THE HIGH PREVALENCE: In Parkinson's disease, the prevalence of symptomatic orthostatic hypotension (OH) concerns more than 20% of the patients. At least 2 factors explain this high prevalence. Dopamine-mimetic drugs may induce or enhance the occurrence of OH. Parkinson's disease is a cause of primary autonomic failure with involvement of the peripheral nervous system. CHARACTERISTIC HISTOLOGICAL AND PHARMACOLOGICAL ELEMENTS: Histology indicates that Lewy bodies exist in the sympathetic glands. Pharmacology reveals cardiac sympathetic denervation. These histological and pharmacological characteristics clearly differentiate autonomic failure of Parkinson's disease from multiple system atrophy (Shy-Drager's syndrome). If autonomic abnormalities appear to be present since the early stages of the disease, the early onset, within the first year, of symptomatic OH in the course of a parkinsonian syndrome can be considered as an exclusion criterion for idiopathic Parkinson's disease. REGARDING TREATMENT: No specific clinical trials have assessed the efficacy of antihypotensive drugs in the treatment of OH in Parkinson's disease. The management of this symptom requires education of the patient and the development of non-pharmacological measures. Drug therapy should be reserved for symptomatic patients who do not improve with the preceding methods. Among the drugs available, domperidone, although widely used, has no proven effect on OH like the cardiovascular analeptics. The alpha1-adrenergic agonists (midodrine) or mineralocorticosteroids (fludrocortisone) are the most frequently used drugs.

Adrenergic alpha-Agonists↗

PubMed2003

Primary degenerative cerebellar ataxias in ethnic Bengalees in West Bengal: some observations.

Seventy cases of primary degenerative cerebellar ataxias in ethnic Bengalees from southern West Bengal, India, were studied by the authors. Of these, 50 cases were of the familial type (hereditary ataxias) encountered in 23 families and the remaining 20 were of sporadic onset. 18 cases (from 11 families) were of "probable" autosomal recessive (AR) inheritance, 12 cases (8 families) had Friedreich's type ataxia (FA), 4 cases (2 families) had FA type ataxia with retained reflexes and in 2 cases (1 family) the exact phenotypic characterization could not be made. AR inheritance in these cases seemed most likely in view of the occurrence in a single generation with unaffected parents and history of consanguinity in many of the families studied. Genotypic confirmation of FA type ataxia and its variants could not be done in any case due to the non-availability of technology for studying the FA locus but some common dominant ataxia genotypes could be excluded. Thirty-two cases (from 12 families) with autosomal dominant ataxias (ADCA) were studied. Genotype analysis revealed 4 families with SCA2 genotype, 5 families with SCA3 and 3 families where genotypic characterization could not be made (phenotypically 2 were of ADCA I and 1 of ADCA II). No clear preponderance of one particular genotype of SCA over another could be demonstrated in our ethnic Bengalee patients. We also noted significant intra and inter-family variations in phenotypes within the same genotypic form as well as overlapping of clinical signs between different genotypes. Slow saccades and peripheral neuropathy were not seen consistently in our ethnic Bengalee subjects with SCA2 genotype. Similarly, extrapyramidal features, ophthalmoplegias and distal amyotrophy were seen in some but not all families with the SCA3 genotype. Phenotypic expression appeared to be an inconsistent marker of the SCA genotype in our patients. Of the 20 sporadic cases with cerebellar ataxia, genotype analysis revealed 2 cases with SCA1 and 1 with SCA2. Some of the sporadic ataxia cases had extracerebellar involvement and may warrant classification as Multiple System Atrophy. In all the 3 subjects with genotype characterization, phenotype correlation was lacking. The clinical pattern of hereditary ataxias in ethnic Bengalees seems to be somewhat different from that seen in Western India. The need for clinical and genetic studies of ataxias in different specific ethnic populations of India has been stressed.

Adolescent↗

PubMed2003

Recent developments in the neuropathological diagnosis of Parkinson's disease and parkinsonism.

The diagnosis of Parkinson's disease (PD) is based mainly on clinical criteria. Large clinicopathological studies reveal however a different diagnosis in up to 25% of the cases (Hughes et al., 1992). Recent advances in molecular biology have shown that some proteins, especially tau and alpha-synuclein, play an essential role in the pathogenesis of parkinsonian and dementing disorders. Such diseases are now classified as tauopathies and synucleinopathies. Progressive supranuclear palsy and corticobasal degeneration are the major tauopathies. To the synucleinopathies belong PD, Lewy body dementia and multiple system atrophy. In pathological conditions abnormal proteins will aggregate in neurons and glial cells and form inclusion bodies. Lewy bodies are the hallmark of Parkinson's disease and Lewy body dementia. Identification of these inclusions and other specific lesions in parkinsonian disorders is facilitated by the routine application on formalin fixed brain of immunohistochemistry for alpha-synuclein, tau and ubiquitin. The purpose of this paper is to briefly review and illustrate the value of these new techniques in the postmortem diagnosis of parkinsonian disorders. Neuropathological examination of the brain is however time consuming and immunohistochemistry represents additional costs. As the selection of brain samples for microscopical examination and antibodies for immunohistochemistry depends on the underlying pathology, some clinical information should be provided to the pathologist such as the clinical diagnosis and when indicated the results of brain imaging studies. A close co-operation between the neurologist and neuropathologist is thus essential to select the most appropriate brains for complete neuropathological investigation.

Brain↗

PubMed2003

[Sleep disorders during synucleopathies and taupathies].

Dissociate states of rapid eye movement sleep (REM) are much more frequent in patients with synucleopathy than in patients with taupathy (except that REM sleep behavior disorder may affect one third of patients with supranuclear palsy), as are sleep onset REM periods, observed in patients with Parkinson's disease, multiple systemic atrophy and Lewy body disease. Recent evidences suggest that they may result from non dopaminergic lesions, including cholinergic neurons in the locus subcoeruleus and orexinergic neurons in the lateral hypothalamus.

Acetylcholine↗

PubMed1992

Treatment of autonomic failure.

The group of primary autonomic failure (AF) syndromes comprises pure AF (PAF), in which there are no central neurological signs, and multiple system atrophy (MSA) in which AF is accompanied by the overlapping degenerative disorders, striatonigral degeneration and olivopontocerebellar atrophy. Patients with these syndromes deteriorate progressively but, based on an understanding of the precise sites of the lesions, there are several ways of alleviating their deterioration, in particular the most disabling symptom, postural hypotension. The principles can be used in the treatment of AF in other general medical disorders, such as diabetes.

Autonomic Nervous System↗

PubMedDate not supplied

[Study of the pre and post-synaptic dopaminergic system by DaTSCAN/IBZM SPECT in the differential diagnosis of parkinsonism in 75 patients].

INTRODUCTION: Essential tremor (ET) may be misdiagnosed as idiopathic Parkinson's disease (PD). In neurodegenerative diseases, structural imaging, such as CT or MRI, is of limited value for differentiating parkinsonian syndromes since structural changes are often only evident by the time the disease is far advanced. Most cases of symptomatic parkinsonism are vascular parkinsonism, but PD may coexist. The differential diagnosis between Alzheimer's disease (AD) and dementia with Lewy bodies (LBD) is often difficult. OBJECTIVE: To define the utility of functional neuroimaging test to establish differential diagnosis between PD and ET, drug induced parkinsonism, multiple system atrophy and vascular parkinsonism, and between AD and LBD, when clinical presentation, evolution or treatment response are atypical. PATIENTS AND METHODS: A group of 75 patients with parkinsonism was examined by clinical assessment and DaTSCAN (123I-FP-CTI, dopamine transporter protein marker) and/or IBZM SPECT (D2 receptor marker). The patients were recruited from our outpatient clinic. RESULTS: Correlation between initial clinical diagnosis and functional imaging studies (DaTSCAN and/or IBZM SPECT) in our patients did not reach that described (more than 90 %) for these techniques in previously published studies. Conclusions. According to sensitivity and sensibility reported in previous imaging studies of the pre and/or postsynaptic dopaminergic system using DaTSCAN and/ or IBZM, SPECT may be a new tool in the diagnosis of parkinsonian patients with difficult clinical diagnosis.

Adult↗

PubMed2004

[Evaluation of autonomic dysfunction by novel methods].

The autonomic nervous system innervates every organ in the body. Since autonomic disturbances affect patient survival, an understanding and recognition of these disturbances are important. We adopted several new methods to evaluate autonomic function accurately. 123I-metaiodobenzylguanidine scintigraphy can assess the cardiac autonomic function even in the presence of cardiac arrhythmia. Laser-Doppler flowmetry, ultrasonographic study in the vessels and near-infrared spectrophotoscopy techniques serve as useful markers for screening the dysfunction of vasomotor neurons and blood circulation. Electrogastrography and the circadian rhythms of protein C secretion could be markers of the visceromotor nerves in the abdomen. Electrogastrography is a particularly useful tool for reflecting on functional changes in gastrointestinal motility. The evaluation of anemia could be a marker of autonomic dysfunction in the kidney and bone marrow in patients with familial amyloidotic polyneuropathy, pandysautonomia, and multiple system atrophy. Normocytic and normochromic anemia correlated with the severity of autonomic dysfunction were shown in these patients. We also evaluated the dysfunction of the neuroendocrine system and sudomotor neuron using our new autonomic function tests. The glucose-tolerance test could become one of the most useful clinical tools for detecting autonomic dysfunction in the endocrine system. Microhydrography and thermography could be useful tools for diagnosing the lesion site of dyshidrosis. Moreover, it is clinically important to check the systemic circulation and autonomic function in patients treated with sildenafil citrate and organ transplantation to save their lives. Our new autonomic function tests, such as laser-Doppler flowmetry and 123I-metaiodobenzylguanidine scintigraphy, are crucial tools in supplying the best symptomatic treatment for such patients.

3-Iodobenzylguanidine↗

PubMed2004

[Essential points to differentiate various diseases causing parkinsonism].

This review article deals with the cardinal features to differentiate various conditions which present with parkinsonism other than Parkinson's disease. Special attention is paid to the distinctive clinical features, laboratory data and neuroimaging findings of frequent diseases as well as important ones including multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, Lewy body disease, drug-induced parkinsonism, vascular pseudo-parkinsonism, normal pressure hydrocephalus and manganese intoxication due to parenteral nutrition. MRI is useful to diagnose MSA, vascular pseudo-parkinsonism and manganese intoxication. Benzamide derivatives including sulpiride and metoclopramide are the main causes of drug-induced parkinsonism in recent years in Japan.

Humans↗

PubMed1992

Neuropathology of Parkinson's disease and related syndromes.

A progressive parkinsonian disorder predicts pathology in the substantia nigra and possibly elsewhere in the basal ganglia. Parkinson's disease is a manifestation of Lewy body disease, which is characterized by the association between Lewy bodies and cell degeneration in specific neuronal populations. Striatonigral degeneration is part of multiple system atrophy and is characterized by striatal and nigral degeneration without neuronal inclusion bodies, but glial inclusions have been described. Steele-Richardson-Olszewski disease is characterized by the globose neurofibrillary tangle and predominant brain stem pathology. Corticobasal degeneration shows similar midbrain pathology and a round, filamentous inclusion in the substantia nigra, not unlike the globose tangle, but there is also focal frontoparietal cortical atrophy. The combination of the distribution of degeneration and nerve cell morphology identify apparently distinct disorders, but most of the neuronal inclusions are not disease specific.

Basal Ganglia Diseases↗

PubMed2005

[Sleep apnea syndrome].

Obstructive sleep apnea syndrome (OSAS) is strongly associated with cardiovascular disease which means these patients could suffer sudden death from these cardiovascular diseases, but it is a rare case that common OSAS itself causes sudden death directly. On the other hand, we never wrongly diagnose serious sleep-related breathing disorders (SBDs) such as sleep hypoventilation syndrome, OSAS patients with cardiovascular disease (i.e. ischemic heart disease, cardiac arrhythmia and cardiac failure) and bilateral vocal cord paralysis caused by multiple system atrophy as common OSAS. This section describes how to distinguish these SBDs from common OSAS.

Death, Sudden↗

PubMed2005

Is Parkinson's disease a homogeneous disorder--what is the burden of Parkinson's disease in India.

Movement disorders are common neurological illnesses among the elderly. These include essential tremor, Perkinsonian disorders and chorea of different aetiologies. Parkinsonian disorders can be divided into two major groups of disorders--classical idiopathic Parkinson's disease and Parkinson plus syndrome. The most common and important cause of Parkinsonism is idiopathic Parkinson's disease. Idiopathic Parkinson's disease is most confidently clinically diagnosed if we follow the United Kingdom Parkinson's Disease Society Brain Bank Diagnostic Criteria for Parkinson's disease. The most common degnerative diseases, which minic idiopathic Parkinson's disease are collectively called Parkinson plus syndrome. The most important diseases comprising Parkinson plus syndrome are: progressive supranuclear palsy, multiple system atrophy, cortical-basal ganglionic degneration, diffuse Lewy body disease and Parkinson-dementia-ALS complex. In India the prevalence of Parkinson's disease varied markedly from one study to another. The prevalence rate is high among the urban Parsi community of Mumbai. Incidence and prevalence of Parkinson's disease increase with increasing age. Some risk factors for Parkinson's disease have been narrated briefly. As the number of cases of Parkinsonism is likely to increase along with increasing population, the general practitioners or consultant physicans should have to play a greater role referring the cases to attend neurologists or movement disorder clinic early.

Aged↗

PubMed2005

Parkinson's disease. Diagnosis and the initiation of therapy.

Parkinson's disease (PD) is the most common cause of parkinsonism. Parkinsonism is characterized by resting tremor, bradykinesia, rigidity and gait impairment. There is no specific diagnostic test for PD and it is important for clinicians to understand the clinical signs which help to distinguish PD from parkinsonism. It is equally important to be aware of the clinical signs which can be an indication that the diagnosis is not PD. These so-called Parkinson-plus syndromes include progressive supranuclear palsy (PSP), multiple systems atrophy (MSA), corticobasal degeneration (CBD), vascular parkinsonism (VP) and parkinsonism with dementia (Lewy body dementia, LBD). The differential diagnosis of parkinsonism will be discussed. Initiating pharmacologic therapy for PD must take into consideration the degree of dysfunction the patient is experiencing, the question of neuroprotection, the degree of motor response required, and the potential complications of long-term treatment. Neuropro-tective trials of coenzyme Q10 (CoQ), vitamin C, vitamin E, monoamine oxidase B inhibitors (MAO-I) and dopamine agonists do not support the use of any of these drugs for a neuroprotective effect. There is recent supportive evidence that levodopa may have a neuroprotective effect. Either dopamine agonists or levodopa may be initiated. Dopamine agonists are associated with fewer motor fluctuations and dyskinesias, while levodopa is associated with better motor performance. Initiation of therapy should be tailored to individual patients with the emphasis on symptom control, with the hope that new approaches to treatment of PD (including neuroprotection) will be forthcoming.

Antioxidants↗