[Gait disorders and repeated falls in Steele-Richardson-Olszewski syndrome].
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Progressive supranuclear palsy (syndrome of Steele-Richardson-Olszewski) represents one of the neuro-degenerative diseases, difficult to distinguish from other forms of parkinsonism. Although uncommon, the syndrome should be included in the differential diagnosis of recurrent falls in the elderly, especially in cases of parkinsonism presenting with axial rigidity and associated with gaze paralysis and/or poor response to L-dopa-therapy. The diagnosis is mainly based on the clinical findings. At present, no effective therapy is known.Keywords:
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ABSTRACT Disturbances of gait manifest in almost all cases of Parkinson's disease (PD), often leading to loss of mobility and increased mortality. In this review a clinically oriented approach to gait disorders in different stages of PD is presented. In addition, interactions between motor behavior and mental processing will be discussed. Analyzing the clinical features of gait can be helpful to differentiate PD from atypical forms of parkinsonism. Bedside tests to distinguish parkinsonian gait disorders are reviewed. There is still an unmet need to effectively treat complex gait disturbances, which are frequently not responsive to dopamine replacement medication. We thus present current approaches for the management of dopa‐refractory gait disorders. © 2013 International Parkinson and Movement Disorder Society
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Abstract Disturbances of balance, gait and posture are a hallmark of parkinsonian syndromes. Recognition of these axial features can provide important and often early clues to the nature of the underlying disorder, and, therefore, help to disentangle Parkinson’s disease from vascular parkinsonism and various forms of atypical parkinsonism, including multiple system atrophy, progressive supranuclear palsy, and corticobasal syndrome. Careful assessment of axial features is also essential for initiating appropriate treatment strategies and for documenting the outcome of such interventions. In this article, we provide an overview of balance, gait and postural impairment in parkinsonian disorders, focusing on differential diagnostic aspects.
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Hughes et al., 2001;Dickson et al., 2009).Step 1 Diagnosis of Parkinsonian syndrome -Bradykinesia (slowness of initiation of voluntary movement with progressive reduction in speed and amplitude of repetitive actions) -and at least one of the following:-muscular rigidity -4-6 Hz rest tremor -postural instability not caused by primary visual, vestibular, cerebellar, or proprioceptive dysfunction Step 2 Exclusion criteria for idiopathic Parkinson's disease -Repeated strokes with stepwise progression of parkinsonian features -Repeated head injury -History of definite encephalitis -Oculogyric crises -Neuroleptic treatment at onset of symptoms -More than one affected relative -Sustained remission -Strictly unilateral features after 3 years -Supranuclear gaze palsy -Cerebellar signs -Early severe autonomic involvement -Early severe dementia with disturbances of memory, language, and praxis -Babinski sign -Presence of cerebral tumour or communicating hydrocephalus on CT scan -Negative response to large doses of levodopa (if malabsorption excluded) -MPTP exposure Step 3 Supportive prospective positive criteria for idiopathic Parkinson's disease (Three or more required for diagnosis of definite Parkinson's disease) -Unilateral onset -Rest tremor present -Progressive disorder -Persistent asymmetry affecting side of onset most -Excellent response (70-100%) to levodopa -Severe levodopa-induced chorea -Levodopa response for 5 years or more -Clinical course of 10 years or more Table 1.UK Parkinson's Disease Society Brain Bank (UKPDSBB) clinical diagnostic criteria for idiopathic Parkinson's disease (from (Hughes et al., 1992).Although numerous supplementary technical exams are available, which may increase diagnostic certainty, the initial diagnosis remains a clinical one and can be based purely on medical history and clinical examination.Motor symptoms in iPD are clinically most striking, but a number of less prominent non-motor symptoms may already be present at www.intechopen.
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There have been continual changes in medical diagnosis and treatment throughout the generations. Present medical practice emphasises accuracy and accountability, and an evidence base for diagnosis and therapeutic intervention. The differential diagnosis of tremor disorders and parkinsonism serves as a good example of evolving concepts and treatment approaches, and is the subject of the present thesis. It is necessary first to review present knowledge about the accuracy of diagnosis and why this is important (Chapter 1), then to review how imaging techniques have contributed to the knowledge base (Chapter 2) and then report current understanding of the dopamine transporter (Chapter 3) - as it is at this site that the new isotope -123I-N-fluoropropyl-2beta-carbomethoxy-3beta-(4-iodophenyl) nortropane (123I-FP-CIT) is known to work. It is the aim of the present thesis to examine an advanced phase of clinical application of the technique of SPECT imaging with 123I-FP-CIT to the differential diagnosis of selected movement disorders as follows: In study 1 (Chapter 4) the design and result of study of 220 subjects with idiopathic Parkinson's disease (iPD), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), essential tremor (ET) and healthy volunteers are presented. A new technique of assessing 123I-FP-CIT SPECT by visual inspection was developed which proved to be useful in differentiating essential tremor from other parkinsonian syndromes. Semi-quantitative analysis showed limited usefulness of 123I-FP-CIT SPECT in differentiating iPD from atypical parkinsonism. This study provides evidence against significant association between iPD and ET. Study 2 (Chapter 5) addressed the issue of the correlation of severity, staging and duration of parkinsonian features with 123I-FP-CIT striatal uptake in 41 patients with iPD. A positive correlation of striatal uptake was identified for these variables and for bradykinesia, but not for tremor suggesting that tremor could have an origin outwith the dopamine transporter system. The clinical lateralisation of parkinsonian signs was studied in relation to striatal uptake with the finding that clinical asymmetry in iPD was more marked than the degree of the asymmetry in 123I-FP-CIT uptake. The patterns of 123I-FP-CIT uptake in iPD, 8 MSA, 3 PSP and 6 healthy volunteers were reported, confirming that the posterior putamen is the most affected part in iPD and showing that there are limitations in attempting to differentiate parkinsonism subtypes using this technique. In study 3 (Chapter 6) 10 patients with tremor and/or clinical features suggesting possible parkinsonism (but not fulfilling diagnostic criteria for either iPD or ET) were compared to 9 unilateral patients fulfilling diagnostic criteria for iPD and 6 healthy volunteers. Reduced 123I-FP-CIT striatal uptake was found bilateral in 7 tremor patient and all 9 of the unilateral iPD patients, confirming the presymptomatic phase of the illness not only in early parkinsonism cases but even in monosymptomatic cases who have yet to develop definitive clinical features. Of the 10 tremor cases, 3 showed striatal uptake values above the range established for iPD patients fulfilling clinical criteria, even although the duration of disease in the tremor patients was similar to that in definite iPD patients. Three of the 10 tremor patients showed normal striatal uptake. This indicated the usefulness of this technique in demonstrating the nigrostriatal change at a very early stage of disease, helping to exclude or confirm the diagnosis of parkinsonism. In study 4 (Chapter 7) the relationship between cerebrovascular disease and parkinsonism was studied. 123I-FP-CIT striatal uptake in 12 patients with vascular parkinsonism was compared to 6 healthy volunteers. Also an index case of isolated tremor related to ischaemic lesion was reported. A reduction in uptake was shown in all vascular parkinsonism patients except one. Two distinct patterns of dopaminergic loss, both of which differ from idiopathic Parkinson's disease were identified, indicating that vascular lesions may disrupt the dopaminergic system to produce parkinsonian features. In summary, a simple visual inspection of 123I-FP-CIT SPECT scans helps in differentiating parkinsonian syndromes from essential tremor and detecting changes in the dopaminergic system in patients with unilateral parkinsonism, even in patients not fulfilling diagnostic criteria for iPD. However there are limitations in using this technique in differentiating iPD from MSA or PSP. A reduction in striatal uptake was shown in vascular parkinsonism patients supporting a dopaminergic source of symptoms in this group. The correlation of disease staging, severity and duration with striatal 123I-FP-CIT uptake indicates the potential usefulness of SPECT in monitoring disease progression and the effects of putative neuroprotective therapy. In conclusion, this new technique represents a significant advance in medical diagnosis of movement disorders..
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Aim:To explore the characteristics of a group of movement disorders which showed primary symptom of freezing gait.Methods: To study prospectively clinical materials of 8 patients who showed primary symptom of freezing gait.Results: Of eight patients followed up for 1 to 3.5 years,two were diagnosed as progressive supranuclear palsy(PSP),one diagnosed as dementia diffuse Lewy body(DLB),one was parkinsonism,one was normal pressure hydrocephalus(NHP),three were primary progressive freezing gait (PPFG).Conclusion: Freezing gait is a sign with diverse causes,which may be the early predominant symptom of some movement disorders.
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Parkinson's disease (PD) is a common neurodegenerative disorder. In 2015, The Movement Disorder Society Clinical Diagnostic Criteria for PD was published. In the criteria, the absolute exclusion criteria and red flags were designed to minimize diagnostic error, in particular to differentiate from neurodegenerative or secondary parkinsonism. Here, we reviewed neurodegenerative disorders that we should differentiate from PD. The common differential diagnoses, such as multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, dementia with Lewy bodies, and essential tremor are important but sometimes difficult to differentiate. We also described the features of rare but important differential diagnoses: neuronal intranuclear inclusion disease, Perry syndrome, Fragile X tremor/ataxia syndrome, Huntington's disease, dopa-responsive dystonia, Wilson disease, and neurodegeneration wit,1 brain iron accumulation.
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Summary The characteristics of acute deterioration in truncal movements, posture and gait were studied in patients admitted with acute non-neurological disease. Reversible gait abnormalities with characteristics of a 'lower-half parkinsonism' were associated with a disorder of axial movement, disability (as assessed by an activities of daily living score), and low mental test score. There was no association of gait dysfunction with tests of upper limb apraxia. An 'at risk' sub-group of elderly patients was identified who during an acute illness lose their walking independence, of whom some also have great difficulty performing axial movements. Furthermore, any assessment of locomotor and axial movement in elderly patients must consider that any impairment may be the result of an acute medical illness.
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