Soft Signs in Essential Tremor Plus: A Prospective Study

Pandey, Sanjay; Bhattad, Sonali

doi:10.1002/mdc3.13350

Cited by 12 publications

(9 citation statements)

References 8 publications

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“…When looking at the types and frequency of soft signs associated with ET-plus, rest tremor was found to be largely the commonest (about 50% of cases), followed by questionable dystonia (about 11%) and undetermined slowing (about 9% of cases). These results are broadly in agreement with the majority of studies [ 17 , 21 , 22 ] but not with Pandey and Bhattad [ 19 ], who in their prospective assessment of ET-plus cases found rest tremor in only 2 out of 45 ET-plus cases (4.44%). The latter result might be due to the single-center recruitment as well as to the specific expertise of the raters (focused on dystonia) [ 24 ], limitations that are arguably minimized in the TITAN study because of its multi-center design, as mentioned above.…”

Section: Discussionsupporting

confidence: 91%

The Italian tremor Network (TITAN): rationale, design and preliminary findings

et al. 2022

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Introduction The recently released classification has revised the nosology of tremor, defining essential tremor (ET) as a syndrome and fueling an enlightened debate about some newly conceptualized entities such as ET-plus. As a result, precise information of demographics, clinical features, and about the natural history of these conditions are lacking. Methods The ITAlian tremor Network (TITAN) is a multicenter data collection platform, the aim of which is to prospectively assess, according to a standardized protocol, the phenomenology and natural history of tremor syndromes. Results In the first year of activity, 679 patients have been recruited. The frequency of tremor syndromes varied from 32% of ET and 41% of ET-plus to less than 3% of rare forms, including focal tremors (2.30%), task-specific tremors (1.38%), isolated rest tremor (0.61%), and orthostatic tremor (0.61%). Patients with ET-plus were older and had a higher age at onset than ET, but a shorter disease duration, which might suggest that ET-plus is not a disease stage of ET. Familial aggregation of tremor and movement disorders was present in up to 60% of ET cases and in about 40% of patients with tremor combined with dystonia. The body site of tremor onset was different between tremor syndromes, with head tremor being most commonly, but not uniquely, associated with dystonia. Conclusions The TITAN study is anticipated to provide clinically relevant prospective information about the clinical correlates of different tremor syndromes and their specific outcomes and might serve as a basis for future etiological, pathophysiological, and therapeutic research.

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Section: Discussionsupporting

confidence: 91%

The Italian tremor Network (TITAN): rationale, design and preliminary findings

et al. 2022

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“…So, the diagnosis of ET-plus is even more challenging with a high rate of discordance 15 , 16 . The proportion of questionable dystonia in ET-plus patients from the previous studies varied between 0 and 91%, depending on the research methodology and assessment tools 11 – 13 , 16 – 18 . A higher prevalence rate of cranial tremors and poorer responsiveness to medical treatment in ET-plus patients might result from questionable or subtle signs of undetected cranial-cervical dystonia.…”

Section: Discussionmentioning

confidence: 99%

The differences in clinical characteristics and natural history between essential tremor and essential tremor plus

Lolekha

Dharmasaroja

Uransilp

et al. 2022

Sci Rep

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The diverse clinical manifestation of essential tremor (ET) has led to the question whether the different phenotypes may affect the clinical outcome and progression. This study aimed to estimate the clinical characteristics and natural history of ET and ET-plus. A total of 221 patients with ET were included, 117 (52.9%) reclassified as ET and 104 (47.1%) as ET-plus. Patients with ET-plus were significantly older in age at onset (P < 0.001); had a higher frequency of cranial tremors (P < 0.001), neurological comorbidities (P < 0.001) and psychiatric comorbidities (P = 0.025); more tremor progression (P < 0.001); and poorer response to medical treatment (P < 0.001) compared to ET patients. Regression analysis revealed that late-onset tremor (OR 11.02, 95% CI 2.79–43.53), neurological comorbidities (OR 3.38, 95% CI 1.56–7.31), psychiatric comorbidities (OR 4.29, 95% CI 1.48–12.44), cranial tremors (OR 2.10, 95% CI 1.02–4.30), and poor response to medical treatment (OR 3.67, 95% CI 1.87–7.19) were associated with ET-plus diagnosis. ET and ET-plus differ in the age of onset, tremor distribution, comorbidities, treatment response rate, and progression. Identifying the ET phenotypes may increase the clinical value in therapeutic strategies and clinical research in the future.

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“…impaired tandem gait, and MCI, and thus could be categorized as ET-plus [29,[34][35][36]. At follow-up evaluation, we found that tremor severity in ET increased, with tremor spread in different body segments and a worsening of tremor during specific complex tasks (i.e., drawing spirals and several activities of daily living).…”

Section: Ta B L Ementioning

confidence: 89%

Longitudinal study of clinical and neurophysiological features in essential tremor

Angelini

Paparella

Biase

et al. 2022

Euro J of Neurology

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Background and purpose Essential tremor (ET) is a common and heterogeneous disorder characterized by postural/kinetic tremor of the upper limbs and other body segments and by non‐motor symptoms, including cognitive and psychiatric abnormalities. Only a limited number of longitudinal studies have comprehensively and simultaneously investigated motor and non‐motor symptom progression in ET. Possible soft signs that configure the ET‐plus diagnosis are also under‐investigated in follow‐up studies. We aimed to longitudinally investigate the progression of ET manifestations by means of clinical and neurophysiological evaluation. Methods Thirty‐seven ET patients underwent evaluation at baseline (T0) and at follow‐up (T1; mean interval ± SD = 39.89 ± 9.83 months). The assessment included the clinical and kinematic evaluation of tremor and voluntary movement execution, as well as the investigation of cognitive and psychiatric disorders. Results A higher percentage of patients showed tremor in multiple body segments and rest tremor at T1 as compared to T0 (all p‐values < 0.01). At T1, the kinematic analysis revealed reduced finger‐tapping movement amplitude and velocity as compared to T0 (both p‐values < 0.001). The prevalence of cognitive and psychiatric disorders did not change between T0 and T1. Female sex, absence of family history, and rest tremor at baseline were identified as predictive factors of worse disease progression. Conclusions ET progression is characterized by the spread of tremor in multiple body segments and by the emergence of soft signs. We also identified possible predictors of disease worsening. The results contribute to a better understanding of ET classification and pathophysiology.

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Soft Signs in Essential Tremor Plus: A Prospective Study

Cited by 12 publications

References 8 publications

The Italian tremor Network (TITAN): rationale, design and preliminary findings

The Italian tremor Network (TITAN): rationale, design and preliminary findings

The differences in clinical characteristics and natural history between essential tremor and essential tremor plus

Longitudinal study of clinical and neurophysiological features in essential tremor

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