Patients with adult‐onset dystonic tremor resembling parkinsonian tremor have scans without evidence of dopaminergic deficit (SWEDDs)
Identifieur interne : 001F04 ( Istex/Corpus ); précédent : 001F03; suivant : 001F05Patients with adult‐onset dystonic tremor resembling parkinsonian tremor have scans without evidence of dopaminergic deficit (SWEDDs)
Auteurs : Susanne A. Schneider ; Mark J. Edwards ; Pablo Mir ; Carla Cordivari ; Juzar Hooker ; John Dickson ; Niall Quinn ; Kailash P. BhatiaSource :
- Movement Disorders [ 0885-3185 ] ; 2007-11-15.
English descriptors
- KwdEn :
Abstract
We present the clinical details and dopamine transporter SPECT scan results of 10 patients with arm tremor, including a rest component and reduced arm swing on the affected side, in whom the possibility of PD had been raised. All patients had signs of dystonia or components of their arm tremor that were compatible with dystonic tremor, and none had true akinesia with fatiguing or decrement, even after a mean follow‐up period of 5.8 years. All patients had normal dopamine transporter SPECT scans. Clinicians should be aware that primary adult‐onset dystonia can present with an asymmetric resting arm tremor, with impaired arm swing and sometimes also facial hypomimia or a jaw tremor, but without evidence of true akinesia. Given the important consequences of misdiagnosing such patients as PD, in cases with diagnostic uncertainty functional imaging should be considered. Among patients suspected of PD, dystonic tremor may be one cause of SWEDDs (Scans Without Evidence of Dopaminergic Deficit). © 2007 Movement Disorder Society
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DOI: 10.1002/mds.21685
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<correspondenceTo>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, University College of London, Queen Square, London WC1N 3BG, United Kingdom</correspondenceTo>
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<titleGroup><title type="main" xml:lang="en">Patients with adult‐onset dystonic tremor resembling parkinsonian tremor have scans without evidence of dopaminergic deficit (SWEDDs)</title>
<title type="short" xml:lang="en">Adult‐onset Dystonic Tremor</title>
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<supportingInformation><p> This article includes supplementary video clips, available online at <url href="http://www.interscience.wiley.com/jpages/0885-3185/suppmat"> http://www.interscience.wiley.com/jpages/0885‐3185/suppmat </url>
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<caption>Segment 1. Patient 1: There is some facial hypomimia, a pronation‐supination rest tremor of the right hand, and a vertical jaw tremor. On flexing the outstretched arms, there is dystonic elevation of the right upper arm and shoulder and her head tilts to the right. There is irregularity, but no fatiguing or decrement, of repetitive movements. There is decreased right arm swing when walking. On follow‐up, there is postural tremor and clear dystonia of the right arm when keepings the arm outstretched with the elbows flexed.Segment 2. Patient 2: A rest tremor of the right hand is observed involving the thumb, which is extended. With arms outstretched, there is an elevation of the right shoulder and mild postural tremor and dystonic posturing of both hands. There is no fatiguing or decrement of repetitive movements.Segment 3. Patient 4: There is a pill‐rolling rest tremor involving the thumb and the index finger, with thumb extension, on the right and a milder rest tremor of the left arm. She has a jaw tremor and a side‐to‐side head tremor. There is dystonia of the patient's outstretched arms, more on the right. There is no fatiguing or decrement on repetitive movements. When walking, there is slightly reduced arm swing bilaterally and a tremor of her right hand. When talking, there is laryngeal dystonia, some upper facial tremor, and more florid dystonia of both hands.Segment 4. Patient 7: This segment shows rest tremor of the right hand with thumb extension, which is one of the characteristic features in many of these cases (see Table 2).Segment 5. Patient 10: There is bilateral, right more than left, and tremor at rest. On writing, she has dystonic posturing of the left index finger and exacerbation of the tremor. The flurries of tremor evident on certain tasks are again a characteristic feature of note in some cases (see Table 2).</caption>
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<p>We present the clinical details and dopamine transporter SPECT scan results of 10 patients with arm tremor, including a rest component and reduced arm swing on the affected side, in whom the possibility of PD had been raised. All patients had signs of dystonia or components of their arm tremor that were compatible with dystonic tremor, and none had true akinesia with fatiguing or decrement, even after a mean follow‐up period of 5.8 years. All patients had normal dopamine transporter SPECT scans. Clinicians should be aware that primary adult‐onset dystonia can present with an asymmetric resting arm tremor, with impaired arm swing and sometimes also facial hypomimia or a jaw tremor, but without evidence of true akinesia. Given the important consequences of misdiagnosing such patients as PD, in cases with diagnostic uncertainty functional imaging should be considered. Among patients suspected of PD, dystonic tremor may be one cause of SWEDDs (Scans Without Evidence of Dopaminergic Deficit). © 2007 Movement Disorder Society</p>
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<abstract lang="en">We present the clinical details and dopamine transporter SPECT scan results of 10 patients with arm tremor, including a rest component and reduced arm swing on the affected side, in whom the possibility of PD had been raised. All patients had signs of dystonia or components of their arm tremor that were compatible with dystonic tremor, and none had true akinesia with fatiguing or decrement, even after a mean follow‐up period of 5.8 years. All patients had normal dopamine transporter SPECT scans. Clinicians should be aware that primary adult‐onset dystonia can present with an asymmetric resting arm tremor, with impaired arm swing and sometimes also facial hypomimia or a jaw tremor, but without evidence of true akinesia. Given the important consequences of misdiagnosing such patients as PD, in cases with diagnostic uncertainty functional imaging should be considered. Among patients suspected of PD, dystonic tremor may be one cause of SWEDDs (Scans Without Evidence of Dopaminergic Deficit). © 2007 Movement Disorder Society</abstract>
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<note type="content"> This article includes supplementary video clips, available online at http://www.interscience.wiley.com/jpages/0885‐3185/suppmatSupporting Info Item: Segment 1. Patient 1: There is some facial hypomimia, a pronation‐supination rest tremor of the right hand, and a vertical jaw tremor. On flexing the outstretched arms, there is dystonic elevation of the right upper arm and shoulder and her head tilts to the right. There is irregularity, but no fatiguing or decrement, of repetitive movements. There is decreased right arm swing when walking. On follow‐up, there is postural tremor and clear dystonia of the right arm when keepings the arm outstretched with the elbows flexed.Segment 2. Patient 2: A rest tremor of the right hand is observed involving the thumb, which is extended. With arms outstretched, there is an elevation of the right shoulder and mild postural tremor and dystonic posturing of both hands. There is no fatiguing or decrement of repetitive movements.Segment 3. Patient 4: There is a pill‐rolling rest tremor involving the thumb and the index finger, with thumb extension, on the right and a milder rest tremor of the left arm. She has a jaw tremor and a side‐to‐side head tremor. There is dystonia of the patient's outstretched arms, more on the right. There is no fatiguing or decrement on repetitive movements. When walking, there is slightly reduced arm swing bilaterally and a tremor of her right hand. When talking, there is laryngeal dystonia, some upper facial tremor, and more florid dystonia of both hands.Segment 4. Patient 7: This segment shows rest tremor of the right hand with thumb extension, which is one of the characteristic features in many of these cases (see Table 2).Segment 5. Patient 10: There is bilateral, right more than left, and tremor at rest. On writing, she has dystonic posturing of the left index finger and exacerbation of the tremor. The flurries of tremor evident on certain tasks are again a characteristic feature of note in some cases (see Table 2). - </note>
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