Joint and skeletal deformities in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy
Ramsey Ashour BS
University of Texas Medical Branch, Galveston, Texas, USA
Search for more papers by this authorCorresponding Author
Joseph Jankovic MD
Department of Neurology, Baylor College of Medicine, Houston, Texas, USA
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, 6550 Fannin, Suite 1801, Houston, TX 77030Search for more papers by this authorRamsey Ashour BS
University of Texas Medical Branch, Galveston, Texas, USA
Search for more papers by this authorCorresponding Author
Joseph Jankovic MD
Department of Neurology, Baylor College of Medicine, Houston, Texas, USA
Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, 6550 Fannin, Suite 1801, Houston, TX 77030Search for more papers by this authorAbstract
The objective of this study is to characterize clinical features of joint and skeletal deformities in Parkinson's disease (PD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Clinical information including age, gender, presence of deformity, initial symptom side, neuropsychological and motor features, family history, and treatment with levodopa/dopamine agonists was collected on consecutive patients with PD, MSA, and PSP evaluated at the Movement Disorders Clinic at Baylor College of Medicine. In this series of 202 patients, 36.1% had deformities of the limbs, neck, or trunk, including 33.5% of PD, 68.4% of MSA, and 26.3% of PSP patients. “Striatal” hand and foot deformities were present in 13.4%, involuntary trunk flexion in 12.9%, anterocollis in 9.4%, and scoliosis in 8.4% of all patients. Patients with these joint and skeletal deformities had higher mean Unified Parkinson's Disease Rating Scale scores (57.4 vs. 46.6; P < 0.01) and were more often treated with levodopa (69.9% vs. 50.4%; P < 0.01) than patients without deformity, independent of disease duration. Patients with striatal deformity were younger than patients without deformity (mean 60.4 vs. 68.6 years; P < 0.01), and they tended to have an earlier age of onset of initial parkinsonian symptoms (mean 54.7 vs. 62.5 years; P < 0.01). Furthermore, the side of striatal deformity correlated with the side of initial parkinsonian symptoms in all patients (100%) with striatal hand and in 83.3% of patients with striatal foot. Joint and skeletal deformities are common and frequently under-recognized features of PD, MSA, and PSP that often cause marked functional disability independent of other motor symptoms. © 2006 Movement Disorder Society
Supporting Information
This article includes Supplementary Video, available online at http://www.interscience.wiley.com/jpages/0885-3185/suppmat
Filename | Description |
---|---|
jws-mds.21058.mpg13.1 MB | Segment 1. Right hand striatal deformity in a patient with Parkinson's disease. Segment 2. Left hand striatal deformity in a patients with Parkinson's disease. Segment 3. Patient with Parkinson's disease and marked anterocollis and left hand striatal deformity. Segment 4. Patient with Parkinson's disease and marked kyphoscoliosis. |
Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
REFERENCES
- 1 Gelb DJ, Oliver E, Gilman S. Diagnostic criteria for Parkinson's disease. Arch Neurol 1999; 56: 33–39.
- 2 Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci 1999; 163: 94–98.
- 3 Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP International Workshop. Neurology 1996; 47: 1–9.
- 4 Riley D, Lang AE, Blair RDG, Birnbaum A, Reid B. Frozen shoulder and other disturbances in Parkinson's disease. J Neurol Neurosurg Psychiatry 1989; 52: 63–66.
- 5 Dias R, Cutts S, Massoud S. Frozen shoulder. BMJ 2005; 331: 1453–1456.
- 6 Gudmundsson K, Arngrímsson R, Sigfússon N, Björnsson A, Jónsson T. Epidemiology of Dupuytren's disease: clinical, serological, and social assessment. The Reykjavik study. J Clin Epidemiol 2000; 53: 291–296.
- 7 Saar J, Grothaus P. Dupuytren's disease: an overview. Plast Reconstr Surg 2000; 106: 125–136.
- 8 Childs SG. Dupuytren's disease. Orthop Nurs 2005; 24: 160–163.
- 9 Moore JS. Flexor Tendon entrapment of the digits (trigger finger and trigger thumb). J Occup Environ Med 2000; 42: 526–545.
- 10 Akhtar S, Bradley MJ, Quinton DN, Burke FD. Management and referral for trigger finger/thumb. BMJ 2005; 331: 30–33.
- 11 Coughlin M, Dorris J, Polk E. Operative repair of the fixed hammertoe deformity. Foot Ankle Int 2000; 21: 94–104.
- 12 Dunn JE, Link CL, Felson DT, Crincoli MG, Keysor JJ, McKinlay JB. Prevalence of foot and ankle conditions in a multiethnic community sample of older adults. Am J Epidemiol 2004; 159: 491–498.
- 13 Dhukaram V, Hossain S, Sampath J, Barrie JL. Correction of hammer toe with an extended release of the metatarsophalangeal joint. J Bone Joint Surg [Br] 2002; 84: 986–990.
- 14 Ashour R, Tintner R, Jankovic J. Striatal deformities of the hand and foot in Parkinson's disease. Lancet Neurol 2005; 4: 423–431.
- 15 Rivest J, Quinn N, Marsden C. Dystonia in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. Neurology 1990; 40: 1571–1578.
- 16 Wenning GK, Tison F, Ben Shlomo Y, Daniel SE, Quinn NP. Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord 1997; 12: 133–147.
- 17 Quinn N. Multiple system atrophy: the nature of the beast. J Neurol Neurosurg Psychiatry 1989; (Suppl.): 78–89.
- 18 Rafal RD, Friedman JH. Limb dystonia in progressive supranuclear palsy. Neurology 1987; 37: 1546–1549.
- 19 Jankovic J, Friedman DI, Pirozzolo FJ, McCrary JA. Progressive supranuclear palsy: motor, neurobehavioural and neuro-ophthalmological findings. Adv Neurol 1990; 53: 293–304.
- 20 Barclay C, Lang A. Dystonia in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 1997; 62: 352–356.
- 21 Vanek Z, Jankovic J. Dystonia in corticobasal degeneration. Mov Disord 2001; 16: 252–257.
- 22 Jankovic J, Kirkpatrick J, Blomquist K, Langlais P, Bird E. Late-onset Hallervorden-Spatz disease presenting as familial parkinsonism. Neurology 1985; 35: 227–234.
- 23 Chen K, Chase T. Parkinsonism-dementia. In: PJ Vinken, GW Bruyn, HL Klawans, editors. Handbook of Clinical Neurology, Extrapyramidal Disorders. Amsterdam: Elsevier Science; 1986. p 167–183.
- 24 Charcot JM. Lectures on the Diseases of the Nervous System. Lecture V. London: The New Sydenham Society; 1877. p 140–147.
- 25
Purves-Stewart J.
Paralysis agitans; with an account of a new symptom.
Lancet
1898;
2:
1258–1260.
10.1016/S0140-6736(01)84377-4 Google Scholar
- 26 Reynolds F, Petropoulous G. Hand deformities in parkinsonism. J Chronic Dis 1965; 18: 593–595.
- 27 Kyriakides T, Langton HR. Hand contractures in Parkinson's disease. J Neurol Neurosurg Psychiatry 1988; 51: 1221–1223.
- 28 Kidron D, Melamed E. Forms of dystonia in patients with Parkinson's disease. Neurology 1987; 37: 1009–1011.
- 29 Nausieda P, Weiner W, Klawans H. Dystonic Foot response of parkinsonism. Arch Neurol 1980; 37: 132–136.
- 30 Poewe WH, Lees AJ. The pharmacology of foot dystonia in parkinsonism. Clin Neuropharmacol 1987; 10: 47–56.
- 31 Jankovic J, Tintner R. Dystonia and parkinsonism. Parkinsonism Relat Disord 2001; 8: 109–121.
- 32 Khan NL, Graham E, Critchley P, et al. Parkin disease: a phenotypic study of a large case series. Brain 2003; 126: 1279–1292.
- 33 Azher S, Jankovic J. Camptocormia: pathogenesis, classification and response to therapy. Neurology 2005; 65: 355–359.
- 34 Djaldetti R, Mosberg-Galili R, Sroka H, Merims D, Melamed E. Camptocormia (bent spine) in patients with Parkinson's disease—characterization and possible pathogenesis of an unusual phenomenon. Mov Disord 1999; 14: 443–447.
- 35 Schabitz WR, Glatz K, Schuhan C, et al. Severe forward flexion of the trunk in Parkinson's disease: focal myopathy of the paraspinal muscles mimicking camptocormia. Mov Disord 2003; 18: 408–414.
- 36 Friedman JH. Episodic camptocormia in PD. Mov Disord 2001; 16: 1201.
- 37 Holler I, Dirnberger G, Pirker W, Auff E, Gerschlager W. Camptocormia in idiopathic Parkinson's disease: [(123) I] beta-CIT SPECT and clinical characteristics. Eur Neurol 2003; 50: 118–120.
- 38 Brodie BC. Pathological and Surgical Observations on the Diseases of the Joints. London: Longman; 1818. p 276–294.
- 39 Souques A, Rosanoff-Saloff. La camptocormie; incurvation du tronc, consecutive aux traumatismes du dos et des lombes; considerations morphologiques. Rev Neurol 1914; 28: 937–939.
- 40 Serratrice G, Pouget J, Pellissier JF. Bent spine syndrome. J Neurol Neurosurg Psychiatry 1996; 60: 51–54.
- 41 Laroche M, Delisle MB, Aziza R, Lagarrigue J, Mazieres B. Is camptocormia a primary muscular disease? Spine 1995; 20: 1011–1016.
- 42 Ekbom K, Lindholm L. New dystonic syndrome associated with butyrophenone therapy. Z Neurol 1972; 202: 94–103.
- 43 Suzuki T, Hori T, Baba A, et al. Effectiveness of anticholinergics and neuroleptic dose reduction on neuroleptic-induced pleurothotonus (the Pisa syndrome). J Clin Psychopharmacol 1999; 19: 277–280.
- 44 Kwak YT, Han IW, Baik J, Koo MS. Relation between cholinesterase inhibitor and Pisa syndrome. Lancet 2000; 355: 2222.
- 45 Vanacore N, Suzzareddu G, Maggini M, Casula A, Capelli P, Raschetti R. Pisa syndrome in a cohort of Alzheimer's disease patients. Acta Neurol Scand 2005; 111: 199–201.
- 46 Colosimo C. Pisa syndrome in a patient with multiple system atrophy. Mov Disord 1998; 13: 607–609.
- 47 Stubner S, Padberg F, Grohmann R, et al. Pisa syndrome (pleurothotonus): report of a multicenter drug safety surveillance project. J Clin Psychiatry 2000; 61: 569–574.
- 48 Villarejo A, Camacho A, Garcia-Ramos R, et al. Cholinergic-dopaminergic imbalance in Pisa syndrome. Clin Neuropharmacol 2003; 26: 119–121.
- 49 Bhattacharya KF, Giannakikou I, Munroe N, Chaudhuri KR. Primary anticholinergic-responsive Pisa syndrome. Mov Disord 2000; 15: 1285–1287.
- 50 Katz JS, Wolfe GI, Burns DK, Bryan WW, Fleckenstein JL, Barohn RJ. Isolated neck extensor myopathy: a common cause of dropped head syndrome. Neurology 1996; 46: 917–921.
- 51 Oerlemans WGH, de Visser M. Dropped head syndrome and bent spine syndrome: two separate clinical entities or different manifestations of axial myopathy? J Neurol Neurosurg Psychiatry 1998; 65: 258–259.
- 52 Smith DL, DeMario MC. Spasmodic torticollis: a case report and review of therapies. J Am Board Fam Pract 1996; 9: 435–441.
- 53
Quinn N.
Disproportionate antecollis in multiple system atrophy.
Lancet
1989;
i:
844.
10.1016/S0140-6736(89)92300-3 Google Scholar
- 54
Jorens PG,
Eycken MP,
Pariezel GA.
Antecollis in parkinsonism.
Lancet
1989;
i:
1320–1321.
10.1016/S0140-6736(89)92706-2 Google Scholar
- 55 Cohen O, Fahn S, Frucht S. Severe cervical dystonia in pathologically proven Parkinson's disease and dementia. Mov Disord 2003; 18: 1381–1382.
- 56 Daniel SE, de Bruin VMS, Lees AJ. The clinical and pathological spectrum of Steele-Richardson-Olszweski syndrome (progressive supranuclear palsy): a reappraisal. Brain 1995; 118: 759–770.
- 57 Lowe T, Edgar M, Margulies J, et al. Etiology of Idiopathic Scoliosis: current trends in research. J Bone Joint Surg [Am] 2000; 82: 1157–1168.
- 58 Roberts S, Menage J, Eisenstein SM. The cartilage end-plate and intervertebral disc in scoliosis: calcification and other sequelae. J Orthop Res 1990; 11: 747–757.
- 59 Echenne B, Barneon G, Pages M, et al. Skin elastic fiber pathology and idiopathic scoliosis. J Pediat Orthop 1988; 8: 522–528.
- 60 Slager UT, Hsu JD. Morphometry and pathology of the paraspinous muscles in idiopathic scoliosis. Dev Med Child Neurol 1986; 28: 749–756.
- 61 Duvoisin RC, Marsden CD. Note on the scoliosis of Parkinsonism. J Neurol Neurosurg Psychiatry 1975; 38: 787–793.
- 62 Indo T, Ando K. Studies on the scoliosis of parkinsonism. Clin Neurol 1980; 20: 40–46.
- 63 Grimes JD, Hassan MN, Trent G, Halle D, Armstrong GW. Clinical and radiographic features of scoliosis in Parkinson's disease. Adv Neurol 1987; 45: 353–355.
- 64 Robin G, Span Y, Steinberg R, Makin M, Menczel J. Scoliosis in the elderly. A follow-up study. Spine 1982; 7: 355–359.
- 65 Vanderpool D, James J, Wynne-Davies R. Scoliosis in the elderly. J Bone Joint Surg [Am] 1969; 51: 446–455.
- 66 Herrera-Marschiz M, Utsumi H, Ungerstedt U. Scoliosis in rats with experimentally-induced hemiparkinsonism: dependence upon striatal dopamine denervation. J Neurol Neurosurg Psychiatry 1990; 53: 39–43.
- 67 Lundblad M, Picconi B, Lindgren H, Cenci MA. A model of L-DOPA-induced dyskinesia in 6-hydroxydopamine lesioned mice: relation to motor and cellular parameters of nigrostriatal function. Neurobiol Dis 2004; 16: 110–123.
- 68 Di Petta G, Del Puente A, Scarpa R, Maglione S, Esposito A, Campanella G. Hand deformities in extrapyramidal disorders. Acta Neurol (Napoli) 1994; 16: 142–146.
- 69 Hu MTM, Bland J, Clough C, Ellis C, Chaudhuri KR. Limb contractures in levodopa-responsive parkinsonism: a clinical and investigational study of seven new cases. J Neurol 1999; 246: 671–676.
- 70 Cordivari C, Misra VP, Catania S, Lees AJ. Treatment of dystonic clenched fist with botulinum toxin. Mov Disord 2001; 16: 907–913.
- 71 Umapathi T, Chaudhry V, Cornblath D, Drachman D, Griffin J, Kuncl R. Head drop and camptocormia. J Neurol Neurosurg Psychiatry 2002; 73: 1–7.
- 72
Lomen-Hoerth C,
Simmons ML,
DeArmond SJ,
Layzer RB.
Adult-onset nemaline myopathy: another cause of dropped head.
Muscle Nerve
1999;
22:
1146–1150.
10.1002/(SICI)1097-4598(199908)22:8<1146::AID-MUS23>3.0.CO;2-U CASPubMedWeb of Science®Google Scholar
- 73 Padberg GW, Lunt PW, Koch M, Fardeau M. Diagnostic criteria for facioscapulohumeral muscular dystrophy. Neuromuscul Disord 1991; 1: 231–234.
- 74 Jankovic J. Levodopa-related motor fluctuations and dyskinesias: clinical manifestation and classification. Mov Disord 2005; 20(Suppl. 11): S11–S16.