Skip to main content

Advertisement

SpringerLink
Log in

The Comprehensive Management of Cerebellar Ataxia in Adults

  • Movement Disorders (A Videnovich, Section Editor)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

In this review, we present the multidisciplinary approach to the management of the many neurological, medical, social, and emotional issues facing patients with cerebellar ataxia.

Recent findings

Our holistic approach to treatment, developed over the past 25 years in the Massachusetts General Hospital Ataxia Unit, is centered on the compassionate care of the patient and their family, empowering them through engagement, and including the families as partners in the healing process. We present the management of ataxia in adults, beginning with establishing an accurate diagnosis, followed by treatment of the multiple symptoms seen in cerebellar disorders, with a view to maximizing quality of life and effectively living with the consequences of ataxia. We discuss the importance of a multidisciplinary approach to the management of ataxia, including medical and non-medical management and the evidence base that supports these interventions. We address the pharmacological treatment of ataxia, tremor, and other associated movement disorders; ophthalmological symptoms; bowel, bladder, and sexual symptoms; orthostatic hypotension; psychiatric and cognitive symptoms; neuromodulation, including deep brain stimulation; rehabilitation including physical therapy, occupational therapy and speech and language pathology and, as necessary, involving urology, psychiatry, and pain medicine. We discuss the role of palliative care in late-stage disease.

Summary

The management of adults with ataxia is complex and a team-based approach is essential.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. 1998;121(4):561–79.

    PubMed  Google Scholar 

  2. Di Donato I, Bianchi S, Federico A. Ataxia with vitamin E deficiency: update of molecular diagnosis. Neurol Sci. 2010 Aug;31(4):511–5.

  3. Lough ME. Wernicke’s encephalopathy: expanding the diagnostic toolbox. Neuropsychol Rev. 2012;22(2):181–94.

    PubMed  Google Scholar 

  4. Musumeci O, Naini A, Slonim AE, Skavin N, Hadjigeorgiou GL, Krawiecki N, et al. Familial cerebellar ataxia with muscle coenzyme Q10 deficiency. Neurology. 2001;56(7):849–55.

    CAS  PubMed  Google Scholar 

  5. Pineda M, Montero R, Aracil A, et al. Coenzyme Q(10)-responsive ataxia: 2-year-treatment follow-up. Mov Disord. 2010;25(9):1262–8.

    PubMed  Google Scholar 

  6. Balreira A, Boczonadi V, Barca E, et al. ANO10 mutations cause ataxia and coenzyme Q10 deficiency. J Neurol. 2014;261(11):2192–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  7. •• Zesiewicz TA, Wilmot G, Kuo S-H, et al. Comprehensive systematic review summary: treatment of cerebellar motor dysfunction and ataxia. Neurology. 2018;90:464–71 Comprehensive systematic review from the AAN on treatments and their evidence base in the ataxias.

    PubMed  PubMed Central  Google Scholar 

  8. Ristori G, Romano S, Visconti A, et al. Riluzole in cerebellar ataxia: a randomized, double-blind, placebo-controlled pilot trial. Neurology. 2010;74:839–45.

    CAS  PubMed  Google Scholar 

  9. •• Romano S, Coarelli G, Marcotulli C, et al. Riluzole in patients with hereditary cerebellar ataxia: a randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2015;14:985–91 Seminal clinical trial demonstrating potential efficacy of riluzole in a mixed ataxia cohort.

    CAS  PubMed  Google Scholar 

  10. FDA Rilutek drug information. https://www.accessdata.fda.gov/drugsatfda_docs/label/2009/020599s011s012lbl.pdf. Accessed 2 Apr 2018.

  11. Trial of BHV-4157 (Troriluzole/Trigriluzole) in Adult Subjects With Spinocerebellar Ataxia. https://clinicaltrials.gov/ct2/show/NCT02960893. Accessed 2 Apr 2018.

  12. • Ilg W, Bastian AJ, Boesch S, Burciu RG, Celnik P, Claaßen J, et al. Consensus paper: management of degenerative cerebellar disorders. Cerebellum. 2014;13:248–68. An excellent review on evidence on medical treatment, rehabilitation and future potential therapies in the management of degenerative ataxias.

    CAS  PubMed  PubMed Central  Google Scholar 

  13. Strupp M, Kalla R, Claassen J, Adrion C, Mansmann U, Klopstock T, et al. A randomized trial of 4-aminopyridine in EA2 and related familial episodic ataxias. Neurology. 2011;77:269–75.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Wakerley BR, Uncini A. Yuki N; GBS classification group; GBS classification group. Guillain-Barré and miller fisher syndromes--new diagnostic classification. Nat Rev Neurol. 2014;10(9):537–44.

    PubMed  Google Scholar 

  15. Arińo H, Gresa-Arribas N, Blanco Y, Martínez-Hernández E, Sabater L, Petit-Pedrol M, et al. Cerebellar ataxia and glutamic acid decarboxylase antibodies. Immunologic profile and long-term effect of immunotherapy. JAMA Neurol. 2014;71:1009–16.

    PubMed  PubMed Central  Google Scholar 

  16. Matsunaga A, Ikawa M, Fujii A, Nakamoto Y, Kuriyama M, Yoneda M. Hashimoto’s encephalopathy as a treatable adultonset cerebellar ataxia mimicking spinocerebellar degeneration. Eur Neurol. 2013;69:14–20.

    PubMed  Google Scholar 

  17. Hadjivassiliou M, Grünewald RA, Chattopadhyay AK, Davies-Jones GA, Gibson A, Jarratt JA, et al. Clinical, radiological, neurophysiological and neuropathological characteristics of gluten ataxia. Lancet. 1998;352:1582–5.

    CAS  PubMed  Google Scholar 

  18. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol. 2008;7:327–40.

    PubMed  PubMed Central  Google Scholar 

  19. Mitoma H, Manto M, Hampe CS. Immune-mediated cerebellar ataxias: from bench to bedside. Cerebellum Ataxias. 2017;4:16. https://doi.org/10.1186/s40673-017-0073-7 eCollection 2017.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Schmahmann JD. Plasmapheresis improves outcome in postinfectious cerebellitis induced by Epstein-Barr virus. Neurology. 2004;62(8):1443.

    PubMed  Google Scholar 

  21. Vernino S. Paraneoplastic cerebellar degeneration. Handb Clin Neurol. 2012;103:215–23.

    PubMed  Google Scholar 

  22. • Mitoma H, Adhikari K, Aeschlimann D, Chattopadhyay P, Hadjivassiliou M, Hampe CS, et al. Consensus paper: neuroimmune mechanisms of cerebellar ataxias. Cerebellum. 2016;15(2):213–32 Excellent review on immune mediated ataxias.

    CAS  PubMed  Google Scholar 

  23. Cooper SA, Murray KL, Heath CA, Will RG, Knight RS. Sporadic Creutzfeldt-Jakob disease with cerebellar ataxia at onset in the UK. J Neurol Neurosurg Psychiatry. 2006;77(11):1273–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  24. •• van Gaalen J, Giunti P, van de Warrenburg BP. Movement disorders in spinocerebellar ataxias. Mov Disord. 2011;26(5):792–800 Review paper on the presence of other movement disorders in the ataxias.

    PubMed  Google Scholar 

  25. Schneider SA, Deuschl G. The treatment of tremor. Neurotherapeutics. 2014;11(1):128–38.

    CAS  PubMed  Google Scholar 

  26. Hall DA, O'keefe JA. Fragile x-associated tremor ataxia syndrome: the expanding clinical picture, pathophysiology, epidemiology, and update on treatment. Tremor Other Hyperkinet Mov (N Y). 2012;2.

  27. Striano P, Coppola A, Vacca G, et al. Levetiracetam for cerebellar tremor in multiple sclerosis: an open-label pilot tolerability and efficacy study. J Neurol. 2006;253:762–6.

    PubMed  Google Scholar 

  28. Niemann N, Jankovic J. Botulinum toxin for the treatment of hand tremor. Toxins (Basel). 2018;10(7).

  29. Nandagopal R, Moorthy SG. Dramatic levodopa responsiveness of dystonia in a sporadic case of spinocerebellar ataxia type 3. Postgrad Med J. 2004;80:363–5.

    CAS  PubMed  PubMed Central  Google Scholar 

  30. Kitahara M, Shimohata T, Tokunaga J, Nishizawa M. Cervical dystonia associated with spinocerebellar ataxia type 2 successfully treated with levodopa: a case report. Mov Disord. 2009;24:2163–4.

    PubMed  Google Scholar 

  31. Nunes MB, Martinez AR, Rezende TJ, et al. Dystonia in Machado-Joseph disease: clinical profile, therapy and anatomical basis. Parkinsonism Relat Disord. 2015;21(12):1441–7.

    PubMed  Google Scholar 

  32. Munchau A, Dressler D, Bhatia KP, Vogel P, Zuhlke C. Machado-Joseph disease presenting as severe generalised dystonia in a German patient. J Neurol. 1999;246:840–2.

    CAS  PubMed  Google Scholar 

  33. Sethi KD, Jankovic J. Dystonia in spinocerebellar ataxia type 6. Mov Disord. 2002;17:150–3.

    PubMed  Google Scholar 

  34. Wilder-Smith E, Tan EK, Law HY, Zhao Y, Ng I, Wong MC. Spinocerebellar ataxia type 3 presenting as an L-DOPA responsive dystonia phenotype in a Chinese family. J Neurol Sci. 2003;213:25–8.

    CAS  PubMed  Google Scholar 

  35. Jankovic J. Medical treatment of dystonia. Mov Disord. 2013;28(7):1001–12.

    CAS  PubMed  Google Scholar 

  36. Wu YR, Lee-Chen GJ, Lang AE, Chen CM, Lin HY, Chen ST. Dystonia as a presenting sign of spinocerebellar ataxia type 1. Mov Disord. 2004;19(5):586–7.

    PubMed  Google Scholar 

  37. Cardoso F, de Oliveira JT, Puccioni-Sohler M, Fernandes AR, de Mattos JP, Lopes-Cendes I. Eyelid dystonia in Machado-Joseph disease. Mov Disord. 2000;15(5):1028–30.

    CAS  PubMed  Google Scholar 

  38. Freeman W, Wszolek Z. Botulinum toxin type A for treatment of spasticity in spinocerebellar ataxia type 3 (Machado-Joseph disease). Mov Disord. 2005;20(5):644.

    PubMed  Google Scholar 

  39. Lu CS, Wu Chou YH, Yen TC, Tsai CH, Chen RS, Chang HC. Dopa-responsive parkinsonism phenotype of spinocerebellar ataxia type 2. Mov Disord. 2002;17:1046–51.

    PubMed  Google Scholar 

  40. Buhmann C, Bussopulos A, Oechsner M. Dopaminergic response in parkinsonian phenotype of Machado-Joseph disease. Mov Disord. 2003;18:219–21.

    PubMed  Google Scholar 

  41. Infante J, Berciano J, Volpini V, et al. Spinocerebellar ataxia type 2 with levodopa-responsive parkinsonism culminating in motor neuron disease. Mov Disord. 2004;19:848–52.

    PubMed  Google Scholar 

  42. Wilkins A, Brown JM, Barker RA. SCA2 presenting as levodopa-responsive parkinsonism in a young patient from the United Kingdom: a case report. Mov Disord. 2004;19:593–5.

    PubMed  Google Scholar 

  43. Gwinn-Hardy K, Chen JY, Liu HC, et al. Spinocerebellar ataxia type 2 with parkinsonism in ethnic Chinese. Neurology. 2000;55:800–5.

    CAS  PubMed  Google Scholar 

  44. • Flabeau O, Meissner WG, Tison F. Multiple system atrophy: current and future approaches to management. Ther Adv Neurol Disord. 2010;3(4):249–63 Excellent review paper on the management of MSA.

    PubMed  PubMed Central  Google Scholar 

  45. •• Artusi CA, Farooqi A, Romagnolo A, et al. Deep brain stimulation in uncommon tremor disorders: indications, targets, and programming. J Neurol. 2018. https://doi.org/10.1007/s00415-018-8823-x Excellent review of DBS in uncommon tremor disorders including the ataxias.

  46. Ferrara JM, Adam OR, Ondo WG. Treatment of fragile-X associated tremor/ataxia syndrome with deep brain stimulation. Mov Disord. 2009;24:149–51.

    PubMed  Google Scholar 

  47. Senova S, Jarraya B, Iwamuro H, et al. Unilateral thalamic stimulation safely improved fragile X-associated tremor ataxia: a case report. Mov Disord. 2012;27:797–9.

    PubMed  Google Scholar 

  48. Xie T, Goodman R, Browner N, et al. Treatment of fragile X-associated tremor/ataxia syndrome with unilateral deep brain stimulation. Mov Disord. 2012;27:799–800.

    PubMed  Google Scholar 

  49. Mehanna R, Itin I. Which approach is better: bilateral versus unilateral thalamic deep brain stimulation in patients with fragile X-associated tremor ataxia syndrome. Cerebellum. 2014;13:222–5.

    CAS  PubMed  Google Scholar 

  50. Weiss D, Mielke C, Wächter T, et al. Long-term outcome of deep brain stimulation in fragile X-associated tremor/ataxia syndrome. Parkinsonism Relat Disord. 2015;21:310–3.

    PubMed  Google Scholar 

  51. Tamás G, Kovács N, Varga NÁ, et al. Deep brain stimulation or thalamotomy in fragile X-associated tremor/ataxia syndrome? Case report. Neurol Neurochir Pol. 2016;50:303–8.

    PubMed  Google Scholar 

  52. dos Santos Ghilardi MG, Cury RG, dos Ângelos JS, et al. Long-term improvement of tremor and ataxia after bilateral DBS of VoP/zona incerta in FXTAS. Neurology. 2015;84:1904–6.

    PubMed  Google Scholar 

  53. Oyama G, Umemura A, Shimo Y, et al. Posterior subthalamic area deep brain stimulation for fragile X-associated tremor/ataxia syndrome. Neuromodulation. 2014;17:721–3.

    PubMed  Google Scholar 

  54. •• Oyama G, Thompson A, Foote KD, Limotai N, Abd-El-Barr M, Maling N, Malaty IA, Rodriguez RL, Subramony SH, Ashizawa T, Okun MS. Deep brain stimulation for tremor associated with underlying ataxia syndromes: a case series and discussion of issues. Tremor Other Hyperkinet Mov (N Y). 2014;4:228. https://doi.org/10.7916/D8542KQ5. eCollection 2014. Paper on DBS in ataxias and the comparison to essential tremor.

  55. Freund HJ, Barnikol UB, Nolte D, et al. Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor—a unusual clinical benefit. Mov Disord. 2007;22:732–5.

    PubMed  Google Scholar 

  56. Hashimoto T, Muralidharan A, Yoshida K, et al. Neuronal activity and outcomes from thalamic surgery for spinocerebellar ataxia. Ann Clin Transl Neurol. 2017;5(1):52–63.

    PubMed  PubMed Central  Google Scholar 

  57. Teixeira MJ, Cury RG, Galhardoni R, Barboza VR, Brunoni AR, Alho E, et al. Deep brain stimulation of the dentate nucleus improves cerebellar ataxia after cerebellar stroke. Neurology. 2015;85:2075e6.

    Google Scholar 

  58. Vidailhet M, Jutras MF, Grabli D, Roze E. Deep brain stimulation for dystonia. J Neurol Neurosurg Psychiatry. 2013;84(9):1029–42.

    PubMed  Google Scholar 

  59. Copeland BJ, Fenoy A, Ellmore TM, Liang Q, Ephron V, Schiess M. Deep brain stimulation of the internal globus pallidus for generalized dystonia associated with spinocerebellar ataxia type 1: a case report. Neuromodulation. 2014;17(4):389–92.

    PubMed  Google Scholar 

  60. Harries AM, Sandhu M, Spacey SD, Aly MM, Honey CR. Unilateral pallidal deep brain stimulation in a patient with dystonia secondary to episodic ataxia type 2. Stereotact Funct Neurosurg. 2013;91(4):233–5.

    PubMed  Google Scholar 

  61. França C, de Andrade DC, Teixeira MJ, et al. Effects of cerebellar neuromodulation in movement disorders: a systematic review. Brain Stimul. 2018;11(2):249–60.

    PubMed  Google Scholar 

  62. Shiga Y, Tsuda T, Itoyama Y, et al. Transcranial magnetic stimulation alleviates truncal ataxia in spinocerebellar degeneration. J Neurol Neurosurg Psychiatry. 2002;72:124–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  63. • Hagerman RJ, Hall DA, Coffey S, et al. Treatment of fragile X-associated tremor ataxia syndrome (FXTAS) and related neurological problems. Clin Interv Aging. 2008;3(2):251–62 Excellent review paper on the treatment of Fragile X Tremor Ataxia Syndrome.

    PubMed  PubMed Central  Google Scholar 

  64. Ilg W, Golla H, Thier P, Giese MA. Specific influences of cerebellar dysfunctions on gait. Brain. 2007;130(Pt 3):786–98.

    PubMed  Google Scholar 

  65. Ebersbach G, Sojer M, Valldeoriola F, et al. Comparative analysis of gait in Parkinson’s disease, cerebellar ataxia and subcortical arteriosclerotic encephalopathy. Brain. 1999;122(Pt 7):1349–55.

    PubMed  Google Scholar 

  66. Fonteyn EM, Schmitz-Hübsch T, Verstappen CC, Baliko L, Bloem BR, Boesch S, et al. Prospective analysis of falls in dominant ataxias. Eur Neurol. 2013;69(1):53–7.

    CAS  PubMed  Google Scholar 

  67. • Ilg W, Synofzik M, Brotz D, Burkard S, Giese MA, Schols L. Intensive coordinative training improves motor performance in degenerative cerebellar disease. Neurology. 2009;73:1823–30 Paper on outpatient PT in ataxia.

    CAS  PubMed  Google Scholar 

  68. Ilg W, Brotz D, Burkard S, Giese MA, Schols L, Synofzik M. Long-term effects of coordinative training in degenerative cerebellar disease. Mov Disord. 2010;25:2239–46.

    PubMed  Google Scholar 

  69. • Miyai I, Ito M, Hattori N, et al. Cerebellar ataxia rehabilitation trial in degenerative cerebellar diseases. Neurorehabil Neural Repair. 2012;265:515–22 Paper on an intensive inpatient treatment program for ataxia.

    Google Scholar 

  70. Gibson-Horn C. Balance-based torso-weighting in a patient with ataxia and multiple sclerosis: a case report. J Neurol Phys Ther. 2008;32(3):139–46.

    PubMed  Google Scholar 

  71. Silva RC, Saute JA, Silva AC, Coutinho AC, Saraiva-Pereira ML, Jardim LB. Occupational therapy in spinocerebellar ataxia type 3: an open-label trial. Braz J Med Biol Res. 2010;43(6):537–42.

    CAS  PubMed  Google Scholar 

  72. • Marsden J, Harris C. Cerebellar ataxia: pathophysiology and rehabilitation. Clin Rehabil. 2011;25(3):195–216 Review paper on rehabilitation in ataxia.

    PubMed  Google Scholar 

  73. •• Gillen G. Improving activities of daily living performance in an adult with ataxia. Am J Occup Ther. 2000;54(1):89–96 Excellent, practical review on OT for ataxia, which is very useful for occupational therapists treating ataxia patients.

    CAS  PubMed  Google Scholar 

  74. Broadhurst MJ, Stammers CW. Mechanical feeding aids for patients with ataxia: design considerations. J Biomed Eng. 1990;12(3):209–14.

    CAS  PubMed  Google Scholar 

  75. Morgan MH. Ataxia and weights. Physiotherapy. 1975;61(11):332–4.

    CAS  PubMed  Google Scholar 

  76. Aisen ML, Arnold A, Baiges I, Maxwell S, Rosen M. The effect of mechanical damping loads on disabling action tremor. Neurology. 1993;43:1346–50.

    CAS  PubMed  Google Scholar 

  77. Feys P, Romberg A, Ruutiainen J, et al. Assistive technology to improve PC interaction for people with intention tremor. J Rehabil Res Dev. 2001;38:235–43.

    CAS  PubMed  Google Scholar 

  78. Surdilovic T, Zhang YQ. Convenient intelligent cursor control web systems for Internet users with severe motor-impairments. Int J Med Inform. 2006;75:86–100.

    PubMed  Google Scholar 

  79. Lysaght R, Bodenhamer E. The use of relaxation training to enhance functional outcomes in adults with traumatic head injuries. Am J Occup Ther. 1990;44(9):797–802.

    CAS  PubMed  Google Scholar 

  80. • O'Connor MG, Kapust LR, Hollis AM. DriveWise: an interdisciplinary hospital-based driving assessment program. Gerontol Geriatr Educ. 2008;29(4):351–62 Review on a driving program in dementia and other degenerative disorders.

    PubMed  Google Scholar 

  81. Erler KS, Juengst SB, Smith DL, et al. Examining driving and participation 5 years after traumatic brain injury. OTJR (Thorofare N J). 2018;38(3):143–50.

    Google Scholar 

  82. • Kent RD, Kent JF, Duffy JR, Thomas JE, Weismer G, Stuntebeck, S: Ataxic dysarthria. J Speech Lang Hear Res. 2000;43(5):1275-1289. A good overview of ataxic dysarthria.

  83. Yorkston KM, Beukelman DR. Ataxic dysarthria: treatment sequences based on intelligibility and prosodic considerations. J Speech Hear Disord. 1981;46(4):398–404.

    CAS  PubMed  Google Scholar 

  84. Maas E, Robin DA, Austermann Hula SN, Freedman SE, Wulf G, Ballard KJ, et al. Principles of motor learning in treatment of motor speech disorders. Am J Speech Lang Pathol. 2008;17(3):277–98.

    PubMed  Google Scholar 

  85. Rosenbeck JC. Mind over Motor. ASHA Lead. 2017;22:44–9.

    Google Scholar 

  86. • Vogel AP, Folker J, Poole ML. Treatment for speech disorder in Friedreich ataxia and other hereditary ataxia syndromes. Cochrane Database Syst Rev. 2014;10:CD008953 Cochrane Review on treatment of speech disorder in the ataxias.

    Google Scholar 

  87. • Vogel AP, Keage MJ, Johansson K, Schalling E. Treatment for dysphagia (swallowing difficulties) in hereditary ataxia (review). Cochrane Database Syst Rev. 2015;11:CD010169 Cochrane Review on the treatment of dysphagia in the ataxias.

    Google Scholar 

  88. Petracca M, Guidubaldi A, Ricciardi L, Ialongo T, Del Grande A, Mulas D, et al. Botulinum Toxin A and B in sialorrhea: Long-term data and literature overview. Toxicon. 2015;107(Pt A):129–40.

    CAS  PubMed  Google Scholar 

  89. • Leigh JR, Zee DS (1999) The Neurology of Eye Movements, 3rd edn. Oxford University Press, New York, p 223–228, 238. Excellent resource on the neurology of eye movements.

  90. Leigh JR, Zee DS. The neurology of eye movements. 3rd ed. New York: Oxford University Press; 1999. p. 104–8. 310-327, 367-375, 406-416

  91. Leigh JR, Zee DS. The neurology of eye movements. 3rd ed. New York: Oxford University Press; 1999. p. 535–42.

    Google Scholar 

  92. Volkmann FC, Schick AML, Riggs LA. Time course of visual inhibition during voluntary saccades. J Opt Soc Am. 1968;58(4):562–9.

    CAS  PubMed  Google Scholar 

  93. Pierrot-Deseilligny C, Milea D. Vertical nystagmus: clinical facts and hypotheses. Brain. 2005;128:1237–46.

    CAS  PubMed  Google Scholar 

  94. Glasauer S, Kalla R. Buttner U, et al. 4-aminopyridine restores visual ocular motor function in upbeat nystagmus. J Neurol Neurosurg Psychiatry. 2005;76:451–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  95. Kalla R, Glasauer S. Schautzer F, et al. 4-aminopyridine improves downbeat nystagmus, smooth pursuit, and VOR gain. Neurology. 2004;62:1228–9.

    CAS  PubMed  Google Scholar 

  96. Strupp M, Schuler O, Krafczyk S, et al. Treatment of downbeat nystagmus with 3,4-diaminopyridine – a placebo-controlled study. Neurology. 2003;61(2):165–70.

    CAS  PubMed  Google Scholar 

  97. Currie JN, Matsuo V. The use of clonazepam in the treatment of nystagmus-induced oscillopsia. Ophthalmology. 1986;93(7):924.

    CAS  PubMed  Google Scholar 

  98. Dieterich M, Straube A, Brandt T, et al. The effects of baclofen and cholinergic drugs on upbeat and downbeat nystagmus. J Neurol Neurosurg Psychiatry. 1991;54:627–32.

    CAS  PubMed  PubMed Central  Google Scholar 

  99. Waespe W, Cohen B, Raphan T. Dynamic modification of the vestibulo-ocular reflex by the nodulus and uvula. Science. 1985;228:199–202.

    CAS  PubMed  Google Scholar 

  100. Jeong HS, Oh JY, Kim JS, et al. Periodic alternating nystagmus in isolated nodular infarction. Neurology. 2007;68:956–7.

    PubMed  Google Scholar 

  101. Halmagyi GM, Rudge P, Gresty MA, et al. Treatment of periodic alternating nystagmus. Ann Neurol. 1980;8:609–11.

    CAS  PubMed  Google Scholar 

  102. Koeppen AH, Barron KD, Dentinger MP. Olivary hypertrophy: histochemical demonstration of hydrolytic enzymes. Neurology. 1980;30(5):471.

    CAS  PubMed  Google Scholar 

  103. Shaikh AG, Thurtell MJ, Optican LM, et al. Pharmacological tests of hypotheses for acquired pendular nystagmus. Ann N Y Acad Sci. 2011;1233(1):320–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  104. • Zhang L, Cao B, Ou R, et al. Non-motor symptoms and the quality of life in multiple system atrophy with different subtypes. Parkinsonism Relat Disord. 2017;35:63–8 Good resource on non-motor symptoms in MSA.

    CAS  PubMed  Google Scholar 

  105. Musegante AF, Almeida PN, Monteiro RT, Barroso U Jr. Urinary symptoms and urodynamics findings in patients with Friedreich’s ataxia. Int Braz J Urol. 2013;39(6):867–74.

    PubMed  Google Scholar 

  106. • Lad M, Parkinson MH, Rai M. Urinary, bowel and sexual symptoms in a cohort of patients with Friedreich’s ataxia. Orphanet J Rare Dis. 2017;12(1):158 Excellent paper on non-motor symptoms in Friedreich’s ataxia.

    PubMed  PubMed Central  Google Scholar 

  107. Lin DJ, Hermann KL, Schmahmann JD. The diagnosis and natural history of multiple system atrophy, Cerebellar Type. Cerebellum. 2016;15(6):663–79.

    CAS  PubMed  PubMed Central  Google Scholar 

  108. Bassotti G, Usai-Satta P, Bellini M. Linaclotide for the treatment of chronic constipation. Expert Opin Pharmacother. 2018 Jul;9:1–6. https://doi.org/10.1080/14656566.2018.1494728.

  109. Hussain IF, Brady CM, Swinn MJ, Mathias CJ, Fowler CJ. Treatment of erectile dysfunction with sildenafil citrate (Viagra) in parkinsonism due to Parkinson’s disease or multiple system atrophy with observations on orthostatic hypotension. J Neurol Neurosurg Psychiatry. 2001;71:371–4.

    CAS  PubMed  PubMed Central  Google Scholar 

  110. Freeman R, Wieling W, Axelrod FB, Benditt DG, Benarroch E, Biaggioni I. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome. Clin Auton Res. 2011;21(2):69–72.

    PubMed  Google Scholar 

  111. Palma JA, Kaufmann H. Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies. Mov Disord. 2018;33(3):372–90.

    PubMed  PubMed Central  Google Scholar 

  112. Singer W, Sandroni P, Opfer-Gehrking TL, Suarez GA, Klein CM, Hines S, et al. Pyridostigmine treatment trial in neurogenic orthostatic hypotension. Arch Neurol. 2006;63(4):513–8.

    PubMed  Google Scholar 

  113. Newton JL, Frith J. The efficacy of nonpharmacologic intervention for orthostatic hypotension associated with aging. Neurology. 2018. https://doi.org/10.1212/WNL.0000000000005994.

  114. • Lo RY, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez C, et al. Depression and clinical progression in spinocerebellar ataxias. Parkinsonism Relat Disord. 2016;22:87–92 Paper detailing depression in the SCAs.

    PubMed  Google Scholar 

  115. Zhang LY, Cao B, Zou YT, Wei QQ, Ou RW, Zhao B, et al. Depression and anxiety in multiple system atrophy. Acta Neurol Scand. 2018;137(1):33–7.

    PubMed  Google Scholar 

  116. Almeida L, Ahmed B, Walz R, De Jesus S, Patterson A, Martinez-Ramirez D, et al. Depressive symptoms are frequent in atypical parkinsonian disorders. Mov Disord Clin Pract. 2017;4(2):191–7.

    PubMed  Google Scholar 

  117. Ströhle A, Schmidt DK, Schultz F, Fricke N, Staden T, Hellweg R, et al. Drug and exercise treatment of Alzheimer disease and mild cognitive impairment: a systematic review and meta-analysis of effects on cognition in randomized controlled trials. Am J Geriatr Psychiatry. 2015;23(12):1234–49.

    PubMed  Google Scholar 

  118. • Li F, Harmer P, Fitzgerald K, et al. Tai chi and postural stability in patients with Parkinson’s disease. N Engl J Med. 2012;366(6):511–9. Important paper on the benefits of exercise in Parkinson’s disease, with relevance to other degenerative movement disorders.

    CAS  PubMed  PubMed Central  Google Scholar 

  119. Schenkman M, Moore CG, Kohrt WM, et al. Effect of high-intensity treadmill exercise on motor symptoms in patients with De novo Parkinson disease: a phase 2 randomized clinical trial. JAMA Neurol. 2018;75(2):219–26.

    PubMed  Google Scholar 

  120. Zhang L, Wang F, Shi Y, Wang J, Xie Q. [Plum-blossom needle tapping at Jiaji (EX-B 2) combined with rehabilitation for ataxia of cerebellar apoplexy: a randomized controlled trial]. Zhongguo Zhen Jiu. 2016;36(2):131–4.

    PubMed  Google Scholar 

  121. •• Wiblin L, Lee M, Burn D. Palliative care and its emerging role in multiple system atrophy and progressive supranuclear palsy. Parkinsonism Relat Disord. 2017;34:7–14 Excellent paper on palliative care in atypical parkinsonism.

    PubMed  Google Scholar 

  122. Quill TE, Abernethy AP. Generalist plus specialist palliative care- creating a more sustainable model. N Engl J Med. 2013;368:1173–5.

    CAS  PubMed  Google Scholar 

  123. Bernacki RE, Block SD, American College of Physicians High Value Care Task Force. Communication about serious illness care goals: a review and synthesis of best practices. JAMA Intern Med. 2014;174(12):1994–2003.

    PubMed  Google Scholar 

  124. Wright AA, Zhang B, Ray A, et al. Associations between end-of-life discussions, patient mental health, medical care near death, and caregiver bereavement adjustment. JAMA. 2008;300(14):1665–73.

    CAS  PubMed  PubMed Central  Google Scholar 

  125. Davison SN, Simpson C. Hope and advance care planning in patients with end stage renal disease: qualitative interview study. BMJ. 2006;333(7574).

  126. Detering KM, Hancock AD, Reade MC, Silvester W. The impact of advance care planning on end of life care in elderly patients: randomised controlled trial. BMJ. 2010;340:c1345.

    PubMed  PubMed Central  Google Scholar 

  127. Dayal AM, Jenkins ME, Jog MS, Kimpinski K, MacDonald P, Gofton TE. Palliative care discussions in multiple system atrophy: a retrospective review. Can J Neurol Sci. 2017;44(3):276–82.

    PubMed  Google Scholar 

  128. Miyashita M, Narita Y, Sakamoto A, Kawada N, Akiyama M, Kayama M, et al. Care burden and depression in caregivers caring for patients with intractable neurologic disease at home in Japan. J Neurol Sci. 2009;276(1–2):148–52.

    PubMed  Google Scholar 

  129. Schulz R, Beach SR. Caregiving as a risk factor for mortality: the caregiver health effects study. JAMA. 1999;282(23):2215–9.

    CAS  PubMed  Google Scholar 

  130. Miyasaki JM. Treatment of advanced Parkinson disease and related disorders. Continuum. 2016;22(4 Movement Disorders):1104–16.

    PubMed  Google Scholar 

  131. Beach TG, Adler CH, Sue LI, Serrano G, Shill HA, Walker DG, et al. Arizona study of aging and neurodegenerative disorders and brain and body donation program. Neuropathology. 2015;35(4):354–89.

    PubMed  PubMed Central  Google Scholar 

Download references

Acknowledgements

Supported in part by the National Ataxia Foundation, Ataxia Telangiectasia Children’s Project, and the MINDlink Foundation.

Author information

Authors and Affiliations

  1. Ataxia Unit, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    Christopher D. Stephen MB ChB, MRCP (UK), Kate T. Brizzi MD, Marc A. Bouffard MD & Jeremy D. Schmahmann MD

  2. Movement Disorders Division, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Christopher D. Stephen MB ChB, MRCP (UK)

  3. Laboratory for Neuroanatomy and Cerebellar Neurobiology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Christopher D. Stephen MB ChB, MRCP (UK) & Jeremy D. Schmahmann MD

  4. Division of Palliative Care, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Kate T. Brizzi MD

  5. Division of Advanced General and Autoimmune Neurology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Marc A. Bouffard MD

  6. Department of Urology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Pablo Gomery MD

  7. Speech Language Pathology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Stacey L. Sullivan MS, CCC-SLP

  8. Physical Therapy, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Julie Mello DPT, NCS

  9. Occupational Therapy, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Julie MacLean OTR/L

  10. Cognitive Behavioral Neurology Unit, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann MD

Authors
  1. Christopher D. Stephen MB ChB, MRCP (UK)
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kate T. Brizzi MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Marc A. Bouffard MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Pablo Gomery MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Stacey L. Sullivan MS, CCC-SLP
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Julie Mello DPT, NCS
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Julie MacLean OTR/L
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Jeremy D. Schmahmann MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Christopher D. Stephen MB ChB, MRCP (UK).

Ethics declarations

Conflict of Interest

Kate Brizzi, Marc Bouffard, Pablo Gomery, Stacey Sullivan, Julie Mello, and Julie MacLean declare no potential conflicts of interest. Jeremy Schmahmann reports personal fees from Bayer, personal fees from Biogen, personal fees from Biohaven, personal fees from Cadent, personal fees from Pfizer, outside the submitted work; in addition, Dr. Schmahmann has a Brief Ataxia Rating Scale licensed to Schmahmann and the General Hospital Corporation, a Cerebellar Neuropsychiatric Rating Scale pending to Schmahmann and the General Hospital Corporation, and a Cerebellar Cognitive Affective syndrome/Schmahmann Scale pending to Schmahmann and the General Hospital Corporation, and Royalties from Elsevier, MacKeith, Oxford, Springer; Grants from National Ataxia Foundation, Ataxia-Telangiectasia Children’s Project, MINDlink Foundation. Christopher D. Stephen has been paid for work as an investigator as part of a multi-center US trial of troriluzole in the ataxias.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Movement Disorders

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Stephen, C.D., Brizzi, K.T., Bouffard, M.A. et al. The Comprehensive Management of Cerebellar Ataxia in Adults. Curr Treat Options Neurol 21, 9 (2019). https://doi.org/10.1007/s11940-019-0549-2

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11940-019-0549-2

Keywords

Search

Navigation