Questionable Dystonia in Essential Tremor Plus: A Video‐Based Assessment of 19 Patients

Pandey, Sanjay; Bhattad, Sonali

doi:10.1002/mdc3.12838

Cited by 23 publications

(27 citation statements)

References 6 publications

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“…Using the Consensus Statement definition [ 1 ], a majority of our patients (72.1%, excluding ET-PD) were classified as ET plus. This is consistent with other recently published studies [ 9 10 18 ]. Similar to our prior report, involving 350 patients with ET [ 19 ], and other studies [ 20 ], the current series again demonstrated a bimodal age-at-onset distribution ( Figure 1 ).…”

Section: Discussionsupporting

confidence: 94%

“…The addition of "ET plus" as a diagnostic category in the Movement Disorder Society Consensus Statement [1] draws attention to these additional features. Since the publication, our and other studies [9][10][11]18] have found that most patients previously diagnosed as ET must be reclassified as ET plus. Furthermore, the additional, nontremor features suggest the possibility of etiopathogenic subtypes.…”

Section: Resultsmentioning

confidence: 99%

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Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic

Bellows

Jankovic

2021

Tremor and Other Hyperkinetic Movements

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Background: Patients with essential tremor were initially considered to have isolated tremor, but additional motor and non-motor features have been increasingly recognized. The term "essential tremor plus" was adopted by the Task Force on Tremor of the International Parkinson and Movement Disorder Society to describe essential tremor patients with additional neurologic signs.Objectives: To characterize essential tremor patients and their phenotypes in a movement disorders clinic population in the context of the new tremor classification.Methods: Demographic, clinical, historical, treatment, and diagnostic data were retrospectively collected on 300 patients diagnosed by movement disorder experts with essential tremor. Patients were classified as having essential tremor, essential tremor plus, or essential tremor-Parkinson's disease combination, and features between these groups were compared. Results:Of the 300 patients, 20.7% were classified as isolated essential tremor, 53.3% as essential tremor plus, and 26.0% as essential tremor-Parkinson's disease. There was no significant difference in the duration of tremor symptoms. Essential tremor plus patients were more likely to have dystonia, tandem gait abnormalities, head tremor and greater tremor severity. Essential tremor-Parkinson's disease patients were more likely to have RBD symptoms. There was no significant difference in cognitive impairment between essential tremor plus and essential tremor-Parkinson's disease patients.Conclusions: Additional motor and non-motor features, including parkinsonism, are common in patients with essential tremor. Further studies are needed to clarify essential tremor phenotypes and to provide insights into possible subtypes.Highlights: 300 patients with essential tremor from a movement disorders clinic were re-classified based on the Movement Disorder Society Consensus Statement on the Classification of Tremors. Additional motor and non-motor features, including parkinsonism, were common, and only 20.7% of patients remained classified as isolated essential tremor.

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Section: Discussionsupporting

confidence: 94%

Section: Resultsmentioning

confidence: 99%

Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic

Bellows

Jankovic

2021

Tremor and Other Hyperkinetic Movements

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“…However, the ET patients were followed up in our outpatient clinic for a relatively long period, thus minimizing the risk of misdiagnosis. In this regard, all patients had bilateral postural and kinetic tremor of the upper limbs, whilst none had subtle dystonia signs or clinically detectable parkinsonian features [25,26]. All patients had discontinued all therapies at least 48 h before the experimental evaluation, therefore reducing the possibility that medications affected our kinematic measurements.…”

Section: Discussionmentioning

confidence: 99%

Is there evidence of bradykinesia in essential tremor?

Bologna

Paparella

Colella

et al. 2020

Euro J of Neurology

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Background and purpose Essential tremor (ET) is a movement disorder primarily characterized by upper limb postural and kinetic tremor. Although still under‐investigated, bradykinesia may be part of the phenotypic spectrum of ET. The aim was to evaluate bradykinesia features in ET through clinical examination and kinematic analysis of repetitive finger movements. Data collected in ET patients were compared with those recorded in Parkinson’s disease patients and healthy controls. Methods Overall, 258 subjects participated in the study (90 ET patients, 84 Parkinson’s disease patients and 84 healthy controls). Repetitive finger tapping was kinematically recorded using a motion analysis system. Movement velocity, amplitude and decrement (sequence effect) were measured. The three groups were first compared by one‐way analysis of variance. A cluster analysis was also performed to better address the data variability observed in ET patients. Possible relationships between kinematic and clinical data were assessed in ET patients. Results Essential tremor patients were slower than healthy controls. Movement slowness in ET did not correlate with postural or kinetic tremor severity. It was also found that movement slowness in ET was not associated with a sequence effect, which instead is a common feature in Parkinson’s disease. Cluster analysis showed that a proportion of ET patients may have movement abnormalities similar to those observed in Parkinson’s disease. Conclusions Movement slowness without sequence effect is a common feature in ET patients. The present findings are relevant when interpreted in the context of the new tremor classification system and in the development of a more accurate bradykinesia definition.

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“…Patients with ET can during the course of their illness develop signs of dystonia and parkinsonism, and some may even develop a secondary PD (22,23). This is generally a later phenomenon.…”

Section: Discussionmentioning

confidence: 99%

Essential Tremor as a “Waste Basket” Diagnosis: Diagnosing Essential Tremor Remains a Challenge

2020

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Introduction: The diagnosis of essential tremor (ET) remains a clinical one, and diagnostic errors are common. We aimed to (1) determine precisely how frequently ET diagnoses are misapplied (i.e., what percentage of patients who have been assigned an "ET" diagnosis actually have another movement disorder), (2) determine which other movement disorders are most often misclassified as "ET," and (3) examine the clinical features that were most associated with diagnostic errors. Methods: One hundred four consecutive patients were included who met the following criteria: (1) initial outpatient evaluation by one of the authors (EDL) between January 2015 and December 2019 and (2) pre-evaluation diagnosis of ET. Data on an extensive number of clinical features were extracted from the electronic medical record. Results: Forty-seven (45.2%) patients received a post-evaluation diagnosis of ET, 29 (27.9%) of dystonia, and 28 (26.9%) of other diagnoses including Parkinson's disease (PD) [6 (5.8%)]. Factors associated with an alternative post-evaluation diagnosis other than ET were pre-evaluation diagnosis made by a non-neurologist, shorter tremor duration, irregular tremor, abnormal limb postures, among others. Discussion: Diagnosing ET remains a challenge, with the diagnosis being over-applied and being used as a "waste basket." More than one-half of the patients who were referred to our clinic with an intake diagnosis of ET were given an alternative post-evaluation diagnosis. While PD was reported to be the most frequently missed diagnosis in a past study, dystonia was most commonly missed in our study. Several clinical features can help to differentiate ET from other tremor disorders.

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Questionable Dystonia in Essential Tremor Plus: A Video‐Based Assessment of 19 Patients

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Cited by 23 publications

References 6 publications

Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic

Phenotypic Features of Isolated Essential Tremor, Essential Tremor Plus, and Essential Tremor-Parkinson’s Disease in a Movement Disorders Clinic

Is there evidence of bradykinesia in essential tremor?

Essential Tremor as a “Waste Basket” Diagnosis: Diagnosing Essential Tremor Remains a Challenge

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