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Systematic Clinical Approach for Diagnosing Upper Limb Tremor

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Systematic Clinical Approach for Diagnosing Upper Limb Tremor Movement disorders J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2019-322676 on 26 May 2020. Downloaded from REVIEW Systematic clinical approach for diagnosing upper limb tremor Jaron van de Wardt,1 A M Madelein van der Stouwe ,2,3 Michiel Dirkx,4 Jan Willem J Elting,2,3,5 Bart Post,4 Marina AJ Tijssen ,2,3 Rick C Helmich 4 1Department of Neurology, ABSTRact While this approach has not been tested yet, it is Canisius Wilhelmina Ziekenhuis, Tremor is the most common movement disorder based on the current literature and in line with the Nijmegen, The Netherlands 2 steps taken at our Expertise Centres for Move- Department of Neurology, worldwide, but diagnosis is challenging. In 2018, the University Medical Centre task force on tremor of the International Parkinson ment Disorders. The first three steps we propose Groningen (UMCG), Groningen, and Movement Disorder Society published a consensus will lead to identification of a clinical syndrome, The Netherlands corresponding to axis 1. The subsequent steps will 3 statement that proposes a tremor classification along Expertise Center Movement two independent axes: a clinical tremor syndrome and help in establishing an aetiology where possible Disorders Groningen, University Medical Center Groningen its underlying aetiology. In line with this statement, we (axis 2). Because of its prevalence and diagnostic (UMCG), Groningen, The here propose a stepwise diagnostic approach that leads complexity, we will focus on visible upper limb Netherlands to the correct clinical and aetiological classification of tremor, and omit discussion of related movement 4 Department of Neurology, upper limb tremor. We also describe the typical clinical disorders such as palatal tremor and orthostatic Donders Institute for Brain, tremor. Our aims are to provide organising princi- Cognition and Behaviour, signs of each clinical tremor syndrome. A key feature Radboud University Nijmegen of our algorithm is the distinction between isolated ples on how to interpret clinical symptoms, and to Medical Center, Nijmegen, The and combined tremor syndromes, in which tremor is provide guidelines for diagnostic testing, in which Netherlands we recommend what is indicated while steering 5 accompanied by bradykinesia, cerebellar signs, dystonia, Department of Clinical peripheral neuropathy or brainstem signs. This distinction clear from unnecessary investigations. We acknowl- Neurophysiology, University Medical Center Groningen subsequently informs the selection of appropriate edge that many of these recommendations are based (UMCG), Groningen, The diagnostic tests, such as neurophysiology, laboratory on expert opinion. This reflects the current state of Netherlands testing, structural and dopaminergic imaging and genetic the field, given that many clinical tremor syndromes copyright. testing. We highlight treatable metabolic causes of lack a gold standard against which clinical signs or Correspondence to tremor, as well as drugs and toxins that can provoke ancillary investigations can be tested. A systematic Dr A M Madelein van der tremor. The stepwise approach facilitates appropriate approach to phenotype patients with upper limb Stouwe, Neurology, University Medical Centre Groningen, diagnostic testing and avoids unnecessary investigations. tremor will, however, make it easier to test and Groningen 9700 RB, The We expect that the approach offered in this article will validate new biomarkers in future research. Our Netherlands; a. m. m. van. der. reduce diagnostic uncertainty and increase the diagnostic stepwise approach to tremor diagnosis is presented stouwe@ umcg. nl yield in patients with tremor. in figure 1. JvdW and AMMvdS are joint first authors. Step 1: is the movement disorder really tremor? http://jnnp.bmj.com/ Received 19 December 2019 INTRODUCTION The first step in diagnosing tremor is the identifi- Revised 19 March 2020 Although tremor is the most common movement cation of hyperkinetic movements as ‘tremulous’. Accepted 22 March 2020 disorder and therefore frequently encountered by Tremor is defined as an involuntary, rhythmic, neurologists,1 diagnosing tremor accurately can be oscillatory movement of a body part.2 The most challenging. The clinical presentation of a patient important alternative diagnosis is myoclonus, with tremor can range from a relatively isolated especially high frequency cortical myoclonus (e in symptom to a complex syndrome, incorporating familial cortical myoclonus), polyminimyoclonus multiple signs and symptoms. In 2018, the task (eg, in multiple system atrophy), peripheral myoc- on September 28, 2021 by guest. Protected force on tremor of the International Parkinson and lonus (which sometimes occurs in inflammatory Movement Disorder Society published a consensus neuropathies or radiculopathies) or asterixis (a nega- ► http:// dx. doi. org/ 10. 1136/ jnnp- 2020- 323189 statement in which a tremor classification along tive myoclonus that looks like a flapping tremor of two independent axes is proposed2: determination the hands when the wrists are extended).4 5 More- of a clinical syndrome based on clinical features over, the rhythmic, low-frequency jerks in ongoing (axis 1), and determination of an aetiology (axis focal epilepsy (epilepsia partialis continua) can © Author(s) (or their 2). For example, a bilateral upper limb action be misinterpreted as unilateral jerky rest tremor.4 employer(s)) 2020. Re- use tremor without other neurological signs is classi- Generally, a ‘jerky’ aspect ought to raise suspicion permitted under CC BY. Published by BMJ. fied as essential tremor (ET) on axis 1. The same of myoclonus as the movement disorder in ques- patient may have a mutation in a gene that often tion, rather than tremor. However, the distinction To cite: Wardt Jv, van der causes dystonia; this is classified as the aetiology on between high frequency myoclonus and tremor on Stouwe AMM, Dirkx M, et al. axis 2. However, the axis 1 diagnosis of ET is not the basis of ‘rhythmicity’ is complicated by the fact J Neurol Neurosurg Psychiatry 3 Epub ahead of print: [please changed until dystonia develops (if it ever does). In that tremor is almost never perfectly rhythmic. In include Day Month Year]. the current paper, we propose a practical approach fact, a jerky or irregular aspect is part of the char- doi:10.1136/jnnp-2019- in line with the consensus statement, to aid prac- acteristic phenotype of some tremor syndromes, 322676 tising clinicians in diagnosing patients with tremor. such as dystonic tremor or Holmes tremor.6 When Wardt Jv, et al. J Neurol Neurosurg Psychiatry 2020;0:1–9. doi:10.1136/jnnp-2019-322676 1 Movement disorders J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2019-322676 on 26 May 2020. Downloaded from a contralateral finger tapping task), distractibility (cessation of tremor on distraction by a mental or contralateral movement task) and temporary arrest of tremor during a ballistic pointing test of the other hand (‘pointing test’). Inability to perform a simple cognitive task can also be a sign of a functional movement disorder, as well as a sudden onset of the tremor.8 Sometimes marked fluctuations of the frequency of a functional tremor (between different contexts or spontaneously) can be noticed by visual inspection alone, otherwise polymyography can be used (step 3). Finally, the examiner may influence the co-contraction pattern by moving the patient’s hand passively and note whether or not the tremor fluctuates in parallel to the muscle tone.9 This can be done during clinical assessment or during polymyography (allowing a quantification of muscle contraction and tremor amplitude). An important pitfall is that functional movement disorders may occur in patients who also have an organic move- ment disorder. Second, we suggest looking for so-called ‘prominent additional signs’. This distinction is important, because tremor can occur in isolation (isolated tremor syndromes) or together with other neurological signs (combined tremor syndromes). A distinction is made between ‘prominent additional signs’ and so- called ‘signs of uncertain significance’.2 The first are clear neurological signs, used to define a combined tremor syndrome, such as bradyki- nesia, cerebellar signs, dystonic posturing, signs of peripheral neuropathy and brainstem signs. Examples of ‘signs of uncertain significance’ include a mildly unsteady tandem gait, questionably abnormal posturing of a body part, mild cognitive impairment, questionable bradykinesia or rigidity and other mild neurological signs that do not suffice to make an additional syndrome classifi- copyright. cation.2 As can be seen in the algorithm, the weight placed by the clinician on an additional symptom (such as dystonic posturing of a limb) determines whether a tremor is called dystonic tremor or ET ‘plus’. This can be difficult, as outlined under ‘Isolated tremor syndromes’ section. Combined tremor syndromes In the right column of the algorithm, the combined tremor syndromes are depicted. In classifying a combined tremor http://jnnp.bmj.com/ Figure 1 Eight- step diagnostic approach to upper limb tremor. DT, syndrome, there are five prominent additional signs to look for. dystonic tremor; EPT, enhanced physiological tremor; ET, essential tremor. Bradykinesia points to a parkinsonism- associated tremor syndrome, such as Parkinson’s disease (PD) or atypical parkin- in doubt, polymyography can be used to differentiate between sonism (progressive supranuclear palsy (PSP), multiple system myoclonus
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