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Parkinsonian Syndromes (parkinsonian + syndrome)
Selected AbstractsDiagnostic and therapeutic value of apomorphine in Parkinsonian patientsINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 11 2004J.C. Sharma Summary Apomorphine is a dopamine agonist administered subcutaneously for the management of motor symptoms of Parkinson's disease (PD). Patients with Parkinsonian syndrome underwent an apomorphine challenge for therapeutic efficacy, a positive response being a reduction of >15% score on motor unified PD rating scale. Of the 42 patients, aged 37,81, disease duration 12 months to 20 years, 36 had a positive response. Six non-responders were later diagnosed as non-PD as compared with only two of the 36 responders. Tremor-predominant patients obtained higher motor response. Few patients demonstrated a delayed positive response. Seven (three idiopathic PD (iPD), four non-PD) suffered adverse reactions of nausea, vomiting or ill-sustained symptomatic fall in BP. Majority of the patients who continued with apomorphine therapy were able to reduce levodopa and achieved an improvement in dyskinesia and motor symptoms. Thirteen responding patients were managed by increasing dopamine agonists. Five patients, intolerant of oral dopamine agonists, were able to beneficially tolerate apomorphine. Age and disease duration did not influence tolerability or efficacy. The patients treated with apomorphine were able to significantly reduce the dose of levodopa, and there was a reduction in dyskinesia, hallucinations and fluctuations (all p < 0.05). In some patients, apomorphine prevented admission to institutions. We also describe the use of apomorphine in acutely ill patients unable to ingest oral medication. Apomorphine seems to have a diagnostic element for iPD. Its use leads to a reduction in dyskinesia, improvement in motor symptoms and prevention of institutional care. Apomorphine test also identifies patients likely to benefit with an increase in oral medication. Age and disease duration should not prevent the use of this valuable drug. Apomorphine also has a role in acutely ill PD patients. [source] Data protection in biomaterial banks for Parkinson's disease research: The model of GEPARD (Gene bank Parkinson's Disease Germany)MOVEMENT DISORDERS, Issue 5 2007Karla Eggert MD Abstract Parkinson's disease (PD) is the second most common neurodegenerative disease. Although 10 gene loci have been identified to cause a Parkinsonian syndrome, these loci account only for a minority of PD patients. Large, systematic research programs are required to collect, store, and analyze DNA samples and clinical information to support further discovery of additional genetic components of PD or other movement disorders. Such programs facilitate research into the relationship between genotype and phenotype. The German Competence Network on Parkinson's disease (CNP) initiated the Gene Bank Parkinson's Disease Germany (GEPARD), providing an administrative and scientific infrastructure for the storage of DNA and clinical data that are electronically accessible and protective of patient rights. In this article, we offer guidance on how to establish a framework for a clinical genetic data and DNA bank, and describe GEPARD as a model that may be useful to other local, national, and international research groups developing similar programs. © 2006 Movement Disorder Society [source] Anatomy, physiology, and pathophysiology of the pedunculopontine nucleus,,MOVEMENT DISORDERS, Issue 3 2009Ned Jenkinson PhD Abstract The pedunculopontine nucleus is composed of cholinergic and non-cholinergic neurones and is located in the caudal pontomesencephalic tegmentum. Evidence suggests that the nucleus plays a role in the production and control of movement. The nucleus has dense interconnections with the basal ganglia, as well as with other areas of the brain associated with motor control. Electrical stimulation of the pedunculopontine nucleus in the decerebrate cat or rat produces organized locomotor movements. Physiological studies show that the pedunculopontine nucleus modulates its activity in response to locomotion, as well as voluntary arm and eye movements. Degeneration of the pedunculopontine nucleus is seen in post-mortem brains in humans with Parkinson's disease and Parkinsonian syndromes. In animal models of Parkinson's disease, metabolic changes are seen in the pedunculopontine nucleus, and chemical inhibition or mechanical disruption of the nucleus can produce an akinetic state in animals and man. In this paper we review the literature in support of the suggestion that some of the symptoms of Parkinson's disease are caused by dysfunction of the pedunculopontine nucleus. In accordance with this view, direct stimulation of the nucleus can ameliorate some symptoms of the disease, as demonstrated in both experimental animals and man. © 2008 Movement Disorder Society [source] Iron metabolism in Parkinsonian syndromesMOVEMENT DISORDERS, Issue 9 2006Daniela Berg MD Abstract Growing evidence suggests an involvement of iron in the pathophysiology of neurodegenerative diseases. Several of the diseases are associated with parkinsonian syndromes, induced by degeneration of basal ganglia regions that contain the highest amount of iron within the brain. The group of neurodegenerative disorders associated with parkinsonian syndromes with increased brain iron content can be devided into two groups: (1) parkinsonian syndromes associated with brain iron accumulation, including Parkinson's disease, diffuse Lewy body disease, parkinsonian type of multiple system atrophy, progressive supranuclear palsy, corticobasal ganglionic degeneration, and Westphal variant of Huntington's disease; and (2) monogenetically caused disturbances of brain iron metabolism associated with parkinsonian syndromes, including aceruloplasminemia, hereditary ferritinopathies affecting the basal ganglia, and panthotenate kinase associated neurodegeneration type 2. Although it is still a matter of debate whether iron accumulation is a primary cause or secondary event in the first group, there is no doubt that iron-induced oxidative stress contributes to neurodegeneration. Parallels concerning pathophysiological as well as clinical aspects can be drawn between disorders of both groups. Results from animal models and reduction of iron overload combined with at least partial relief of symptoms by application of iron chelators in patients of the second group give hope that targeting the iron overload might be one possibility to slow down the neurodegenerative cascade also in the first group of inevitably progressive neurodegenerative disorders. © 2006 Movement Disorder Society [source] Parkinsonism in the course of HTLV-I-associated myelopathy,MOVEMENT DISORDERS, Issue 5 2005Marzia Puccioni-Sohler MD Abstract Parkinsonian syndromes may represent a complication of viral infection. Human T cell lymphotropic virus I (HTLV-I) is a cause of a chronic myelopathy in which encephalic involvement has been also found. We report on the case of a 60-year-old man with HTLV-I-associated myelopathy, complicated with bradykinesia, resting tremor, and cogwheel rigidity. These findings suggest that parkinsonian features may represent a neurological disorder associated with HTLV-I infection. © 2005 Movement Disorder Society [source] Glutamate and the glutamate receptor system: a target for drug actionINTERNATIONAL JOURNAL OF GERIATRIC PSYCHIATRY, Issue S1 2003Stefan Bleich Abstract Glutamate is the most important excitatory neurotransmitter in the central nervous system. In the process, glutamate fulfills numerous physiological functions, but also plays an important role in the pathophysiology of different neurological and psychiatric diseases, especially when an imbalance in glutamatergic neurotransmission occurs. Under certain conditions, glutamate has a toxic action resulting from an activation of specific glutamate receptors, which leads to acute or chronic death of nerve cells. Such mechanisms are currently under discussion in acute neuronal death within the context of hypoxia, ischaemia and traumas, as well as in chronic neurodegenerative or neurometabolic diseases, idiopathic parkinsonian syndrome, Alzheimer's dementia and Huntington's disease. It is hoped that glutamate antagonists will lead to novel therapies for these diseases, whereby the further development of glutamate antagonists for blocking disease-specific subtypes of glutamate receptors may be of major importance in the future. Copyright © 2003 John Wiley & Sons, Ltd. [source] Proposed neuroimaging criteria for the diagnosis of multiple system atrophy,,MOVEMENT DISORDERS, Issue 7 2009David J. Brooks MD, FRCP Abstract In this article, we review the state of the art knowledge concerning structural and functional imaging in multiple system atrophy (MSA). The relative value of imaging modalities in the differential diagnosis of MSA from other parkinsonian syndromes is debated. It is concluded that, although neuroimaging biomarkers provide valuable supportive data alongside clinical assessments, it is not possible to use them as surrogate markers. © 2009 Movement Disorder Society [source] Apparent diffusion coefficient of the superior cerebellar peduncle differentiates progressive supranuclear palsy from Parkinson's disease,MOVEMENT DISORDERS, Issue 16 2008Giuseppe Nicoletti MD Abstract The early diagnosis of progressive supranuclear palsy (PSP) may be challenging, because of clinical overlapping features with Parkinson's disease (PD) and other parkinsonian syndromes such as the Parkinsonian variant of multiple system atrophy (MSA-P). Conventional MRI can help in differentiating parkinsonian disorders but its diagnostic accuracy is still unsatisfactory. On the basis of the pathological demonstration of superior cerebellar peduncle (SCP) atrophy in patients with PSP, we assessed the SCP apparent diffusion coefficient (ADC) values in patients with PSP, PD, and MSA-P in order to evaluate its differential diagnostic value in vivo. Twenty-eight patients with PSP (14 with possible-PSP and 14 with probable-PSP), 15 PD, 15 MSA-P, and 16 healthy subjects were studied by using diffusion weighted imaging (DWI). ADC was calculated in regions of interest defined in the left and right SCP by two clinically blinded operators. Intrarater (r = 0.98, P < 0.001) and interrater reliability (r = 0.97; P < 0.001) for SCP measurements were high. Patients with PSP had higher SCP rADC values (median 0.98 × 10,3mm2/s) than patients with PD (median 0.79 × 10,3 mm2/s, P < 0.001), MSA-P (median 0.79 × 10,3 mm2/s, P < 0.001), and healthy controls (median 0.80 × 10,3 mm2/s, P < 0.001). DWI discriminated patients with PSP from PD and healthy subjects on the basis of SCP rADC individual values (100% sensitivity and specificity) and from patients with MSA-P (96.4% sensitivity and 93.3% specificity). The higher values of rADC in SCP of patients with PSP correspond with the in vivo microstructural feature of atrophy detected postmortem and provide an additional support for early discrimination between PSP and other neurodegenerative parkinsonisms. © 2008 Movement Disorder Society [source] Iron metabolism in Parkinsonian syndromesMOVEMENT DISORDERS, Issue 9 2006Daniela Berg MD Abstract Growing evidence suggests an involvement of iron in the pathophysiology of neurodegenerative diseases. Several of the diseases are associated with parkinsonian syndromes, induced by degeneration of basal ganglia regions that contain the highest amount of iron within the brain. The group of neurodegenerative disorders associated with parkinsonian syndromes with increased brain iron content can be devided into two groups: (1) parkinsonian syndromes associated with brain iron accumulation, including Parkinson's disease, diffuse Lewy body disease, parkinsonian type of multiple system atrophy, progressive supranuclear palsy, corticobasal ganglionic degeneration, and Westphal variant of Huntington's disease; and (2) monogenetically caused disturbances of brain iron metabolism associated with parkinsonian syndromes, including aceruloplasminemia, hereditary ferritinopathies affecting the basal ganglia, and panthotenate kinase associated neurodegeneration type 2. Although it is still a matter of debate whether iron accumulation is a primary cause or secondary event in the first group, there is no doubt that iron-induced oxidative stress contributes to neurodegeneration. Parallels concerning pathophysiological as well as clinical aspects can be drawn between disorders of both groups. Results from animal models and reduction of iron overload combined with at least partial relief of symptoms by application of iron chelators in patients of the second group give hope that targeting the iron overload might be one possibility to slow down the neurodegenerative cascade also in the first group of inevitably progressive neurodegenerative disorders. © 2006 Movement Disorder Society [source] Role of dopamine transporter SPECT for the practitioner and the general neurologistMOVEMENT DISORDERS, Issue S7 2003Wolfgang H. Oertel Abstract The accurate clinical diagnosis of parkinsonism may be impeded by atypical presentations and confounding comorbidity. The presence of parkinsonism is misdiagnosed in up to a quarter of cases in general practice. Movement disorder specialists misdiagnose parkinsonian syndromes using histopathological findings as the "gold standard" in up to 10% of cases. Dopamine transporter SPECT represents a simple and fast method to confirm nigrostriatal degeneration in a given patient. This study provides several case reports to illustrate when dopamine transporter SPECT might be carried out and discusses whether dopamine transporter SPECT should be used in primary health care practice or by general neurologists in uncertain cases. Ideally, all possible cases of parkinsonism should be referred to a neurologist experienced in the field of movement disorders. If this could be achieved then the role of dopamine transporter SPECT in the general practitioner's or local neurologist's practice would be extremely limited. Future studies must clarify whether it is cost effective to generously perform dopamine transporter SPECTs to minimize the time until parkinsonism can be diagnosed. © 2003 Movement Disorder Society [source] Olfaction in neurodegenerative disorderMOVEMENT DISORDERS, Issue 4 2003Christopher Hawkes MD Abstract There has been an increase of interest in olfactory dysfunction since it was realised that anosmia was a common feature of idiopathic Parkinson's disease (PD) and Alzheimer-type dementia (AD). It is an intriguing possibility that the first sign of a disorder hitherto regarded as one of movement or cognition may be that of disturbed smell sense. In this review of PD, parkinsonian syndromes, essential tremor, AD, motor neurone disease (MND) and Huntington's chorea (HC) the following observations are made: 1) olfactory dysfunction is frequent and often severe in PD and AD; 2) normal smell identification in PD is rare and should prompt review of diagnosis unless the patient is female with tremor-dominant disease; 3) anosmia in suspected progressive supranuclear palsy and corticobasal degeneration is atypical and should likewise provoke diagnostic review; 4) hyposmia is an early feature of PD and AD and may precede motor and cognitive signs respectively; 5) subjects with anosmia and one ApoE-4 allele have an approximate 5-fold increased risk of later AD; 6) impaired smell sense is seen in some patients at 50% risk of parkinsonism; 7) smell testing in HC and MND where abnormality may be found, is not likely to be of clinical value; and 8) biopsy of olfactory nasal neurons shows non-specific changes in PD and AD and at present will ot aid diagnosis. © 2003 Movement Disorder Society [source] Anal sphincter EMG in the diagnosis of parkinsonian syndromesACTA NEUROLOGICA SCANDINAVICA, Issue 3 2010K. Winge Winge K, Jennum P, Lokkegaard A, Werdelin L. Anal sphincter EMG in the diagnosis of parkinsonian syndromes. Acta Neurol Scand: 2010: 121: 198,203. © 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Background,,, The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective. Methods,,, In this study, we prospectively investigated six patients with Parkinson's Disease (IPD), 14 patients with multiple system atrophy (MSA) and eight with progressive supranuclear palsy (PSP) using EMG of the EAS, motor-evoked potential (MEP) to the EAS and EMG of m. gastrocnemius and nerve conduction velocity measured at the sural nerve. Patients were followed up for 2 years to secure correct diagnosis. Results,,, The mean duration of motor unit potentials (MUPs) recorded from the EAS was significantly longer in patients with MSA and PSP compared with MUPs recorded from patients with PD (P < 0.005 for both). There were no signs of diffuse loss of motor neurons or peripheral neuropathy. MEP revealed signs of supranuclear affection in patients with MSA, whereas in patients with PSP the mechanism is a focal loss of motor neurons in Onuf's nucleus. Conclusion,,, Abnormal EMG of the EAS is strongly suggestive of atypical parkinsonism and the pathophysiology may be different in patients with MSA and PSP. [source] | ||||||||||