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Research paper
Tremor in primary adult-onset dystonia: prevalence and associated clinical features
  1. Giovanni Defazio1,
  2. Angelo Fabio Gigante1,
  3. Giovanni Abbruzzese2,
  4. Anna Rita Bentivoglio3,
  5. Carlo Colosimo4,
  6. Marcello Esposito5,
  7. Giovanni Fabbrini4,
  8. Arianna Guidubaldi3,
  9. Paolo Girlanda6,
  10. Rocco Liguori7,
  11. Lucio Marinelli2,
  12. Francesca Morgante6,
  13. Lucio Santoro5,
  14. Michele Tinazzi8,
  15. Paolo Livrea1,
  16. Alfredo Berardelli4
  1. 1Department of Neurosciences and Sensory Organs, School of Motor Sciences, ‘Aldo Moro’ University of Bari, Bari, Italy
  2. 2Department of Neurosciences, Ophthalmology and Genetics, University of Genoa, Italy
  3. 3Department of Neurosciences, Catholic University, Rome, Italy
  4. 4Department of Neurology and Psychiatry, Sapienza University of Rome and IRCCS Neuromed Institute, Roma, Italy
  5. 5Department of Neurological Science, Federico II University of Naples, Naples, Italy
  6. 6Department of Neurosciences, Psychiatry, and Anesthesiological Science, University of Messina, Messina, Italy
  7. 7Department of Neurosciences, University of Bologna, Bologna, Italy
  8. 8Department of Neurological, Neuropsychological, Morphological and Motor Sciences, University of Verona, Verona, Italy
  1. Correspondence to Dr Giovanni Defazio, Department of Neuroscience and Sensory Organs, ‘Aldo Moro’ University of Bari, Piazza Giulio Cesare, Bari 2-70124, Italy; gdefazio{at}


Objective To investigate the frequency and the main clinical features of tremor in primary adult-onset dystonia (PAOD).

Methods This cross-sectional study was conducted on 429 patients with PAOD from eight Italian movement disorder centres.

Results Of the 429 dystonic patients, 72 (16.7%) had tremor. Although sex and age at dystonia onset were similar in dystonic patients who had tremor and those who did not, patients who had tremor were affected more often by focal cervical dystonia and less often by focal blepharospasm. Dystonia had a greater tendency to spread in patients with tremor. According to the Movement Disorder Society Consensus Statement, tremor was classified as dystonic tremor (DT) in 43 patients and tremor associated with dystonia (TAWD) in 23 patients. Six patients had both types of tremor. Taking into account potential confounding by age at onset and body distribution of the corresponding dystonia type, all the clinical features in patients with DT and in those with TAWD were comparable except the tendency of dystonia to spread, which was greater in patients with DT.

Conclusions Tremor is a relatively common feature occurring in about 17% of patients with primary late-onset dystonia. The association between tremor and dystonia spread suggests that this form of tremor may be a dystonic manifestation. Similarities in phenotypic features of DT and TAWD predominated over differences, suggesting that the two forms of tremor may be manifestations of the same disease. Differences in gender and body distribution of tremor between patients with dystonia and tremor and those of patients with essential tremor also suggest that tremor in dystonia and essential tremor are different entities.

  • Dystonia
  • Tremor

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