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Dystonia in Parkinson’s disease

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Abstract

Dystonia can occasionally be found in idiopathic Parkinson’s disease. It is very uncommon in untreated patients and is more frequently seen as a complication of its treatment. In this review, the various types of dystonia occurring in PD, the differential diagnosis with other parkinsonian syndromes associated with dystonia and treatments available are revised.

Dystonia unrelated to treatment can be typical (blepharospasm, torticollis), atypical (parkinsonian writer’s cramp, camptocormia, anismus), or occurring in early-onset Parkinson disease (the so-called kinesigenic foot dystonia, considered a hallmark of earlyonset Parkinson’s disease). Early and prominent dystonia in untreated patients with parkinsonism should raise the suspicion of other entities other than Parkinson’s disease, such as progressive supranuclear palsy, multiple system atrophy or corticobasal degeneration.

In patients on chronic dopaminergic treatment, peak-dose dystonia, diphasic dystonia and off-dystonia can be seen. The later constitutes the major dystonic feature of chronic levodopa therapy, and a wide variety of strategies are available to manage this complication. Among them, deep brain stimulation of the subthalamic nucleus has proved to be the most effective one.

Dystonic reactions (mainly involving oculomotor cranial nerves and limbs) in operated patients (especially carriers of deep brain stimulation (DBS) devices) are increasingly being reported, constituting a new type of dystonia in patients with Parkinson’s disease: dystonia linked to surgical treatment.

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References

  1. Albanese A, Valente EM, Romito LM, Bellacchio E, Elia AE, Dallapiccola B (2005) The PINK1 phenotype can be indistinguishable from idiopathic Parkinson disease. Neurology 64:1958–1960

    Article  PubMed  CAS  Google Scholar 

  2. Ashour R, Tintner R, Jankovic J (2005) Striatal deformities of the hand and foot in Parkinson’s disease. Lancet Neurol 4:423–431

    Article  PubMed  Google Scholar 

  3. Azher AN, Jankovic J (2005) Camptocormia. Pathogenesis, classification and response to therapy. Neurology 65:355–359

    Article  PubMed  Google Scholar 

  4. Barclay CL, Lang AE (1997) Dystonia in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 62:352–356

    PubMed  CAS  Google Scholar 

  5. Bejjani BP, Arnulf I, Demeret S, Damier P, Bonnet AM, Houeto JL, Agid Y (2000) Levodopa-induced dyskinesias in Parkinson’s disease: Is sensitization reversible? Ann Neurol 47:655–658

    Article  PubMed  CAS  Google Scholar 

  6. Boesch SM, Wenning GK, Ransmayr G, Poewe W (2002) Dystonia in multiple system atrophy. J Neurol Neurosurg Psychiatry 72:300–303

    Article  PubMed  CAS  Google Scholar 

  7. Bonifati V, Rizzu P, van Baren MJ et al. (2003) Mutations in the DJ-1 gene associated with autosomal recessive early-onset parkinsonism. Science 299:256–259

    Article  PubMed  CAS  Google Scholar 

  8. Bonifati V, Rohe CF, Breedveld GJ et al. (2005) Early-onset parkinsonism associated with PINK1 mutations: frequency, genotypes, and phenotypes. Neurology 65:87–95

    Article  PubMed  CAS  Google Scholar 

  9. Charcot JM. Lectures on the diseases of the nervous system, lecture V (1877) London: New Sydenham Society, pp 140–147

  10. Dale RC, Church AJ, Surtees RA, Lees AJ, Adcock JE, Harding B, Neville BG, Giovannoni G (2004) Encephalitis lethargica syndrome: 20 new cases and evidence of basal ganglia autoimmunity. Brain 127:21–33

    Article  PubMed  Google Scholar 

  11. Djaldetti R, Mosberg-Galili R, Sroka H, Merims D, Melamed E (1999) Camptocornia (bent spine) in patients with Parkinson’s disease—characterization and possible pathogenesis of an unusal phenomenon. Mov Disord 3:443–447

    Article  Google Scholar 

  12. van Duijn CM, Deker MCJ, Bonifati V, Galjaard RJ et al. (2001) PARK-7 a novel locus for autosomal recessive early-onset parkinsonism, on chromosome 1p36. Am J Hum Genet 69:629–623

    Article  PubMed  CAS  Google Scholar 

  13. Evidente VG, Advincula J, Esteban R, et al. (2002) Phenomenology of “Lubag” or X-linked dystonia-parkinsonism. Mov Disord 17:1271–1277

    Article  PubMed  Google Scholar 

  14. Fahn S (1988) Concepts and classification of dystonia. Adv Neurology 50:1–8

    CAS  Google Scholar 

  15. Fraix V, Pollak P, Van Blercom N et al. (2000) Effect of subthalamic nucleus stimulation on levodopa-induced dyskinesias in Parkinson’s disease. Neurology 55:1921–1923

    PubMed  CAS  Google Scholar 

  16. Gershanik OS, Leist A (1987) Juvenile onset Parkinson’s disease. Adv Neurol 45:213–216

    PubMed  CAS  Google Scholar 

  17. Gibb WR, Lees AJ (1988) A comparison of clinical and pathological features of young- and old-onset Parkinson’s disease. Neurology 38:1402–1406

    PubMed  CAS  Google Scholar 

  18. Golbe LI (1991) Young-onset Parkinson’s disease: A clinical review. Neurology 41:168–173

    PubMed  CAS  Google Scholar 

  19. Holler I, Dirnberger G, Pirker W et al. (2003) Camptocormia in idiopathic Parkinson’s disease: 123I beta CIT SPECT and clinical characteristics. Eur Neurol 50:118–120

    Article  PubMed  Google Scholar 

  20. Hozumi I, Piao YS, Inuzuka T, Matsuyama Z, Yamada Y, Hara A,Hirose T, Tsuchiya K, Takahashi H (2004) Marked asymmetry of putaminal pathology in an MSA-P patient with Pisa syndrome. Mov Disord 19:470–472

    Article  PubMed  CAS  Google Scholar 

  21. Hu MTM, Bland J, Clough C, Ellis CM, Chaudhuri KR (1999) Limb contractures in levodopa-responsive parkinsonism: a clinical and investigational study of seven new cases. J Neurol 246:671–676

    Article  PubMed  CAS  Google Scholar 

  22. Huang CC, Yen TC, Weng YH, Lu CS (2002) Normal dopamine transporter binding in dopa responsive dystonia. J Neurol 249:1016–1020

    Article  PubMed  CAS  Google Scholar 

  23. Jankovic J, Tintner R (2001) Dystonia and parkinsonsim. Parkinsonism Relat Disord 8:109–121

    Article  PubMed  CAS  Google Scholar 

  24. Juncos JL, Fabbrini G, Mouradian MM, Chase TN (1987) Controlled release levodopa-carbidopa (CR-5) in the management of parkinsonian motor fluctuations. Arch Neurol 44(10):1010–1012

    PubMed  CAS  Google Scholar 

  25. Katchen M, Duvoisin RC (1986) Parkinsonism following dystonia in three patients. Mov Disord 1:151–157

    Article  PubMed  CAS  Google Scholar 

  26. Khan NL, Graham E, Critchley P, et al. (2003) Parkin disease: a phenotypic study of a large case series. Brain 126:1279–1292

    Article  PubMed  Google Scholar 

  27. Kidron D, Melamed E (1987) Forms of dystonia in patients with Parkinson’s disease. Neurology 37:1009–1011

    PubMed  CAS  Google Scholar 

  28. Kitada T, Asakawa S, Hattori N et al. (1998) Mutations of the parkin gene cause autosomal recessive juvenile parkinsonism. Nature 392:605–608

    Article  PubMed  CAS  Google Scholar 

  29. Klawans H, Paleologos N (1986) Dystonia-Parkinson syndrome: differential effects of levodopa and dopamine agonists. Clin Neuropharmcol 9:298–302

    CAS  Google Scholar 

  30. Klostermann W, Vieregge P, Kompf D (1997) Apraxia of eyelid opening after bilateral stereotaxic subthalamotomy. J Neuroophthalmol 17(2):122–123

    PubMed  CAS  Google Scholar 

  31. Krack P, Batir A, Van Blercom N et al. (2003) Five-year follow-up of bilateral stimulation of the subthalamic nucleus in advanced Parkinson’s disease. N Engl J Med 349:1925–1934

    Article  PubMed  CAS  Google Scholar 

  32. Krack P, Marion MH (1994) “Apraxia of lid opening,” a focal eyelid dystonia: clinical study of 32 patients. Mov Disord 9:610–615

    Article  PubMed  CAS  Google Scholar 

  33. Krack P, Pollak P, Limousin P, Benazzouz A, Deuschl G, Benabid AL (1999) From off-period dystonia to peak-dose chorea. The clinical spectrum of varying subthalamic nucleus activity. Brain 122:1133–1146

    Article  PubMed  Google Scholar 

  34. Lamberti P, De Mari M, Zenzola A, Aniello MS, Defazio G (2002) Frequency of apraxia of eyelid opening in the general population and in patients with extrapyramidal disorders. Neurol Sci 23(Suppl 2):S81–S82

    Article  PubMed  Google Scholar 

  35. Lees AJ, Hardie RJ, Stern GM (1984) Kinesigenic foot dystonia as a presenting feature of Parkinson’s disease. J Neurol Neurosurg Psychiatry 47:885

    PubMed  CAS  Google Scholar 

  36. Lees AJ, Stern GM (1980) Bromocriptine in treatment of levodopa-induced end-of-dose dystonia. Lancet 2:215–216

    Article  PubMed  CAS  Google Scholar 

  37. Lera G, Vaamonde J, Rodriguez M, Obeso JA (1993) Cabergoline in Parkinson’s disease: long-term follow-up. Neurology 43:2587–2590

    PubMed  CAS  Google Scholar 

  38. LeWitt PA, Burns RS, Newman RP (1986) Dystonia in untreated parkinsonism. Clin Neuropharmacol 9:293–297

    PubMed  CAS  Google Scholar 

  39. Limousin P, Krack P, Pollak P et al. (1998) Electrical stimulation of the subthalamic nucleus in advanced Parkinson’s disease. N Engl J Med 339:1105–1111

    Article  PubMed  CAS  Google Scholar 

  40. Lücking CB, Dürr A, Bonifati V et al. (2000) Association between early-onset Parkinson’s disease and mutations in the parkin-gene. New Engl J Med 342:1560–1567

    Article  PubMed  Google Scholar 

  41. Mathers SE, Kempster PA, Swash M, Lees AJ (1988) Constipation and paradoxical puborectalis contraction in anismus and Parkinson’s disease: a dystonic phenomenon? J Neurol Neurosurg Psychiatry 51:1503–1507

    PubMed  CAS  Google Scholar 

  42. Melamed E (1979) Eary-morning dystonia. A late side-effect of long-term levodopa therapy in Parkinson’s disease. Arch Neurol 36:308–310

    PubMed  CAS  Google Scholar 

  43. Merlo IM, Occhini A, Pacchetti C, Alfonsi E (2002) Not paralysis, but dystonia causes stridor in multiple system atrophy. Neurology 58:649–652

    PubMed  Google Scholar 

  44. Muñoz JE, Marti MJ, Marin C, Tolosa E (1997) Long-term treatment with intermitent intranasal or subcutaneous apormorphine in patients with levodopa-related motor fluctuations. Clin Neuropharmacol 20:245–252

    Article  Google Scholar 

  45. Nakanishi I, Hironishi M, Miwa H, Kondo T (2003) A patient with multiple system atrophy presenting prolonged levodopa-responsive parkinsonism and off-dystonia of the trunk. No To Shinkei 55:605–608

    PubMed  Google Scholar 

  46. Nyholm D, Aquilonius SM (2004) Levodopa infusion therapy in Parkinson disease: state of the art in 2004. Clin Neuropharmacol 27:245–256

    Article  PubMed  CAS  Google Scholar 

  47. Nyholm D, Nilsson Remahl AI, Dizdar N et al. (2005) Duodenal levodopa infusion monotherapy vs oral polypharmacy in advanced Parkinson disease. Neurology 64:216–223

    PubMed  CAS  Google Scholar 

  48. Obeso JA, Luquin MR, Martinez-Lage JM (1986) Lisuride infusion pump: a device for the treatment of motor fluctuations in Parkinson’s disease. Lancet 1:467–470

    Article  PubMed  CAS  Google Scholar 

  49. Oerlemans WGH, de Visser M (1998) Dropped head syndrome and bent spine syndrome: two separate clinical entities or different manifestations of axial myopathy. J Neurol Neurosurg Psychiatry 65:258–259

    Article  PubMed  CAS  Google Scholar 

  50. Oide T, Ohara S, Yazawa M et al. (2002) Progressive supranuclear palsy with asymmetric tau pathology presenting with unilateral limb dystonia. Acta Neuropathol (Berl) 104:209–214

    Article  PubMed  Google Scholar 

  51. O’Sullivan JD, Costa DC, Gacinovic S, Lees AJ (2001) SPECT imaging of the dopamine transporter in juvenile-onset dystonia. Neurology 56:266–267

    PubMed  CAS  Google Scholar 

  52. Panegyres PK, Hillman D, Dunne JW (2006) Laryngeal dystonia causing upper airway obstruction in progressive supranuclear palsy. J Clin Neurosci (in press; Epub ahead of print)

  53. Pastor P, Tolosa E (2003) Cabergoline in the treatment of Parkinson’s disease. Neurología 18(4):202–209

    PubMed  CAS  Google Scholar 

  54. Poewe WH, Lees AJ, Stern GM (1988) Dystonia in Parkinson’s disease: clinical and pharmacological features. Ann Neurol 23:73–78

    Article  PubMed  CAS  Google Scholar 

  55. Poewe WH, Lees AJ (1987) The pharmacology of foot dystonia in parkinsonism. Clin Neuropharmacol 10:47–56

    Article  PubMed  CAS  Google Scholar 

  56. Purves-Stewart J (1898) Paralysis agitans, with an account of a new symptom. Lancet 2:1258–1260

    Article  Google Scholar 

  57. Quinn NP (1993) Parkinsonism and dystonia, pseudoparkinsonism and pseudodystonia. Adv Neurol 60:540–543

    PubMed  CAS  Google Scholar 

  58. Quinn N, Critchley P, Marsden CD (1987) Young Onset Parkinson’s disease. Mov Disord 2:73–91

    Article  PubMed  CAS  Google Scholar 

  59. Quinn N, Marsden CD (1986) Lithium for painful dystonia in Parkinson’s disease. Lancet 1:1377

    Article  PubMed  CAS  Google Scholar 

  60. Rafal RD, Friedman JH (1987) Limb dystonia in progressive supranuclear palsy. Neurology 37:1546–1549

    PubMed  CAS  Google Scholar 

  61. Richardson JC, Steele J, Olszewski J (1963) Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of “heterogenous system degeneration”. Trans Am Neurol Assoc 88:25–29

    PubMed  CAS  Google Scholar 

  62. Rivest J, Quinn N, Marsden CD (1990) Dystonia in Parkinson’s disease,mutiple system atrophy and progressive supranuclear palsy. Neurology 40:1571–1578

    PubMed  CAS  Google Scholar 

  63. Rodriguez-Oroz MC, Obeso JA, Lang AE (2005) Bilateral deep brain stimulation in Parkinson’s disease: a multicentre study with 4 years follow-up. Brain 128:2240–22

    Article  PubMed  CAS  Google Scholar 

  64. Sauleau P, Raoul S, Lallement F et al. (2005) Motor and non motor effects during intraoperative subthalamic stimulation for Parkinson’s disease. J Neurol 252:457–464

    Article  PubMed  Google Scholar 

  65. Slawek J, Derejko M, Lass P (2003) Camptocornia as a forn of dystonia in Parkinson’s disease. Eur J Neurol 10:107–110

    Article  PubMed  CAS  Google Scholar 

  66. Slawek J, Derejko M, Lass P, Dubaniewicz M (2005) Camptocormia or Pisa syndrome in multiple system atrophy. Clin Neurol Neurosurg (in press; Epub ahead of print)

  67. Soonawala N, Bathia KP, Yeung JH, Quinn NP, Marsden CD (1999) Idiopathic blepharospasm does not lead to a parkinsonian syndrome: results of a questionnaire-based follow-up study. J Neurol 246:283–286

    Article  PubMed  CAS  Google Scholar 

  68. Tan EK, Chan LL, Wong MC (2003) Levodopa-induced oromandibular dystonia in progressive supranuclear palsy. Clin Neurol Neurosurg 105:132–134

    Article  PubMed  CAS  Google Scholar 

  69. Tassin J, Durr A, Bonnet AM et al. (2000) Levodopa-responsive dystonia. GTP cyclohydrolase I or parkin mutations? Brain 123:1112–1121

    Article  PubMed  Google Scholar 

  70. Valente EM, Abou-Sleiman PM, Caputo V et al. (2004) Hereditary early-onset Parkinson’s disease is caused by mutations in PINK1. Science 304:1158–1160

    Article  PubMed  CAS  Google Scholar 

  71. Vanek ZF, Jankovic J (2000) Dystonia in corticobasal degeneration. Adv Neurol 82:61–67

    PubMed  CAS  Google Scholar 

  72. Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP (1997) Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord 12:133–147

    Article  PubMed  CAS  Google Scholar 

  73. Zadikoff C, Lang AE (2005) Apraxia in movement disorders. Brain 128:1480–1497

    Article  PubMed  Google Scholar 

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  1. Neurology Service, Institut de Neurociències, Hospital Clínic i Universitari de Barcelona, c./ Villarroel 170, 08036, Barcelona, Catalonia, Spain

    Eduardo Tolosa M.D., F.R.C.P. & Yaroslau Compta M.D.

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  1. Eduardo Tolosa M.D., F.R.C.P.
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Tolosa, E., Compta, Y. Dystonia in Parkinson’s disease. J Neurol 253 (Suppl 7), vii7–vii13 (2006). https://doi.org/10.1007/s00415-006-7003-6

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