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Central Positional Nystagmus

  • Neurologic Ophthalmology and Otology (R Shin and D Gold, Section Editors)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of review

Central positional nystagmus (CPN) is attributed to disease affecting the central vestibulo-cerebellar pathways. It can be associated with prominent vertigo, oscillopsia, and dysautonomia. Its treatment highly depends on the etiology, and response is fairly heterogenous. This review presents a critical appraisal of CPN therapies.

Recent findings

Anecdotal reports have stated efficacy of 3,4-diaminopyridine, 4-aminopyridine, and clonazepam in downbeating CPN secondary to structural lesions. In tumors, CPN may improve after tumoral resection and radiotherapy. In multiple sclerosis, intravenous steroids may abate CPN during a relapse. In paraneoplastic CPN, remission has been occasionally observed after tumoral excision, but relapses may follow. In autoimmune ataxia, intravenous immunoglobulin and oral baclofen have been shown to improve upbeating CPN. In genetic ataxia, acetazolamide seems to be more effective in resolving ictal episodes than non-ictal CPN and ataxia. In vestibular migraine, prophylactic treatment seems to provide long-term improvement of attacks manifesting with CPN. Non-invasive vagus nerve stimulation has abolished CPN in a vestibular migraine attack. CPN secondary to toxics not responding to drug discontinuation might need further treatment such as midazolam or clonazepam.

Summary

Prospective randomized placebo-controlled treatment trials using objective baseline, short- and long-term assessments of CPN, and related symptoms are highly needed.

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References and Recommended Reading

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  1. • Lemos J, Strupp M. Central positional nystagmus: an update. J Neurol. 2021. https://doi.org/10.1007/S00415-021-10852-8. An updated review on central positional nystagmus.

  2. Choi J-Y, Glasauer S, Kim JH, Zee DS, Kim J-S. Characteristics and mechanism of apogeotropic central positional nystagmus. Brain. 2018;141:762–75.

    Article  PubMed  Google Scholar 

  3. Choi J-Y, Kim JH, Kim HJ, Glasauer S, Kim J-S. Central paroxysmal positional nystagmus: characteristics and possible mechanisms. Neurology. 2015;84:2238–46.

    Article  PubMed  Google Scholar 

  4. Glasauer S, Dieterich M, Brandt T. Central positional nystagmus simulated by a mathematical ocular motor model of otolith-dependent modification of Listing’s plane. J Neurophysiol. 2001;86:1546–54.

    Article  CAS  PubMed  Google Scholar 

  5. Choi SY, Jang JY, Oh EH, Choi JH, Park JY, Lee SH, Choi KD. Persistent geotropic positional nystagmus in unilateral cerebellar lesions. Neurology. 2018;91:e1053–7.

    Article  PubMed  Google Scholar 

  6. Büttner U, Helmchen C, Brandt T. Diagnostic criteria for central versus peripheral positioning nystagmus and vertigo: a review. Acta Otolaryngol. 1999;119:1–5.

    Article  PubMed  Google Scholar 

  7. Choi JY, Kim JS. Central positional nystagmus: characteristics and model-based explanations. Prog Brain Res. 2019;249:211–25.

    Article  PubMed  Google Scholar 

  8. Feil K, Strobl R, Schindler A, Krafczyk S, Goldschagg N, Frenzel C, Glaser M, Schöberl F, Zwergal A, Strupp M. What is behind cerebellar vertigo and dizziness? Cerebellum. 2019;18:320–32.

    Article  CAS  PubMed  Google Scholar 

  9. Young AS, Nham B, Bradshaw AP, Calic Z, Pogson JM, D’Souza M, Halmagyi GM, Welgampola MS. Clinical, oculographic, and vestibular test characteristics of vestibular migraine. Cephalalgia. 2021;033310242110060.

  10. Lee SU, Choi JY, Kim HJ, Park JJ, Zee DS, Kim JS. Impaired tilt suppression of post-rotatory nystagmus and cross-coupled head-shaking nystagmus in cerebellar lesions: image mapping study. Cerebellum. 2017;16:95–102.

    Article  PubMed  Google Scholar 

  11. Nylen C. Statistical analysis of oto-neurological symptomatology in supra- and subtentorial tumours. Acta Otolaryngol. 1939;27:24–31.

    Article  Google Scholar 

  12. Drachman DA, Diamond ER, Hart CW. Posturally - evoked vomiting; Association with posterior fossa lesions. Ann Otol Rhinol Laryngol. 1977;86:97–101.

    Article  CAS  PubMed  Google Scholar 

  13. Chan T, Logan P, Eustace P. Intermittent downbeat nystagmus secondary to vermian arachnoid cyst with associated obstructive hydrocephalus. J Clin Neuroophthalmol. 1991;11:293–6.

    CAS  PubMed  Google Scholar 

  14. Barber HO. Positional nystagmus Otolaryngol Neck Surg. 1984;92:649–55.

    Article  CAS  Google Scholar 

  15. Shoman N, Longridge N. Cerebellar vermis lesions and tumours of the fourth ventricle in patients with positional and positioning vertigo and nystagmus. J Laryngol Otol. 2007;121:166–9.

    Article  CAS  PubMed  Google Scholar 

  16. Gregorius FK, Crandall PH, Baloh RW. Positional vertigo with cerebellar astrocytoma. Surg Neurol. 1976;6:283–6.

    CAS  PubMed  Google Scholar 

  17. Lea J, Lechner C, Halmagyi GM, Welgampola MS. Not so benign positional vertigo: paroxysmal downbeat nystagmus from a superior cerebellar peduncle neoplasm. Otol Neurotol. 2014. https://doi.org/10.1097/MAO.0000000000000245.

    Article  PubMed  Google Scholar 

  18. Helmchen C, Gottschalk S, Sander T, Trillenberg P, Rambold H, Sprenger A. Beneficial effects of 3,4-diaminopyridine on positioning downbeat nystagmus in a circumscribed uvulo-nodular lesion. J Neurol. 2007;254:1126–8.

    Article  PubMed  Google Scholar 

  19. Kremmyda O, Zwergal A, La Fougère C, Brandt T, Jahn K, Strupp M. 4-Aminopyridine suppresses positional nystagmus caused by cerebellar vermis lesion. J Neurol. 2013;260:321–3.

    Article  CAS  PubMed  Google Scholar 

  20. Watson P, Barber HO, Deck J, Terbrugge K. Positional vertigo and nystagmus of central origin. Can J Neurol Sci / J Can des Sci Neurol. 1981;8:133–7.

    Article  CAS  Google Scholar 

  21. Etzion Y. Grossman Y (2001) Highly 4-aminopyridine sensitive delayed rectifier current modulates the excitability of guinea pig cerebellar Purkinje cells. Exp Brain Res. 2001;1394(139):419–25.

    Article  CAS  Google Scholar 

  22. Sakata E, Ohtsu K, Itoh Y. Positional nystagmus of benign paroxysmal type (BPPN) due to cerebellar vermis lesions. Pseudo-BPPN Acta Otolaryngol Suppl. 1991;481:254–7.

    Article  CAS  PubMed  Google Scholar 

  23. Lemos J, Martins AI, Duque C, Pimentel S, Nunes C, Gonçalves AF. Positional testing in acute vestibular syndrome: a transversal and longitudinal study. Otol Neurotol. 2018. https://doi.org/10.1097/MAO.0000000000002067.

    Article  Google Scholar 

  24. Oh EH, Kim H, Choi SY, Choi KD, Choi JH. Paroxysmal central positional nystagmus responsive to clonazepam. J Neurol. 2022;269:1028–31.

    Article  CAS  PubMed  Google Scholar 

  25. Thomsen J, Zilstorff K, Johnsen NJ. Positional nystagmus of the persistent type. ORL. 1978;40:86–91.

    Article  CAS  PubMed  Google Scholar 

  26. Frohman EM, Kramer PD, Dewey RB, Kramer L, Frohman TC. Benign paroxysmal positioning vertigo in multiple sclerosis: diagnosis, pathophysiology and therapeutic techniques. Mult Scler. 2003;9:250–5.

    Article  CAS  PubMed  Google Scholar 

  27. Anagnostou E, Varaki K, Anastasopoulos D. A minute demyelinating lesion causing acute positional vertigo. J Neurol Sci. 2008;266:187–9.

    Article  CAS  PubMed  Google Scholar 

  28. Anagnostou E, Mandellos D, Limbitaki G, Papadimitriou A, Anastasopoulos D. Positional nystagmus and vertigo due to a solitary brachium conjunctivum plaque. J Neurol Neurosurg Psychiatry. 2006;77:790–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Meier CA. Mechanisms of immunosuppression by glucocorticoids. Eur J Endocrinol. 1996;134:50.

    Article  CAS  PubMed  Google Scholar 

  30. Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2012;367:2015–25.

    Article  CAS  PubMed  Google Scholar 

  31. Ostermann PO, Westerberg CE. Paroxysmal attacks in multiple sclerosis. Brain. 1975;98:189–202.

    Article  CAS  PubMed  Google Scholar 

  32. Espir ML, Millac P. Treatment of paroxysmal disorders in multiple sclerosis with carbamazepine (Tegretol). J Neurol Neurosurg Psychiatry. 1970;33:528.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Ciampi E, Uribe-San-Martín R, Godoy-Santín J, Cruz JP, Cárcamo-Rodríguez C, Juri C. Secondary paroxysmal dyskinesia in multiple sclerosis: clinical-radiological features and treatment. Case report of seven patients. Mult Scler. 2017;23:1791–5.

    Article  PubMed  Google Scholar 

  34. Mitoma H, Manto M, Hadjivassiliou M. Immune-mediated cerebellar ataxias: clinical diagnosis and treatment based on immunological and physiological mechanisms. J Mov Disord. 2021;14:10–28.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Kearsley JH, Johnson P, Halmagyi GM. Paraneoplastic cerebellar disease: remission with excision of the primary tumor. Arch Neurol. 1985;42:1208–10.

    Article  CAS  PubMed  Google Scholar 

  36. Eggers SDZ, Pittock SJ, Shepard NT, Habermann TM, Neff BA, Klebig RR. Positional periodic alternating vertical nystagmus with PCA-Tr antibodies in Hodgkin lymphoma. Neurology. 2012;78:1800–2.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Tafur AJ, Baumann Kreuzinger LM, Quevedo F. 28-Year-old man with severe vertigo. Mayo Clin Proc. 2008;83:1070–3.

    Article  PubMed  Google Scholar 

  38. Wray SH, Dalmau J, Chen A, King S, Leigh RJ. Paraneoplastic disorders of eye movements. Ann N Y Acad Sci. 2011;1233:279–84.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Martins AI, Carvalho JN, Amorim AM, Geraldo A, Eggenberger E, Lemos J. Disabling central paroxysmal positioning upbeat nystagmus and vertigo associated with the presence of anti–glutamic acid decarboxylase antibodies. J Neuro-Ophthalmology. 2017;38:1.

    Google Scholar 

  40. Cohen B, Helwig D, Raphan T. Baclofen and velocity storage: a model of the effects of the drug on the vestibulo-ocular reflex in the rhesus monkey. J Physiol. 1987;393:703–25.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Jeong S-H, Nam J, Kwon MJ, Kim JK, Kim JS. Nystagmus and ataxia associated with antiganglioside antibodies. J Neuro-Ophthalmology. 2011;31:326–30.

    Article  Google Scholar 

  42. • Lee SU, Kim HJ, Choi JY, Kim JK, Kim JS. Acute vestibular syndrome associated with anti-GQ1b antibody. Neurology. 2019;93:e1085–e1092. An important series including patients with acute vestibular syndrome associated with anti-GQ1b antibody, further providing data on the occurrence of central positional nystagmus and treatment outcomes.

  43. Winters JL. Plasma exchange: concepts, mechanisms, and an overview of the American Society for Apheresis guidelines. Hematology. 2012;2012:7–12.

    Article  PubMed  Google Scholar 

  44. Ophoff RA, Terwindt GM, Vergouwe MN, et al. Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations in the Ca2+ channel gene CACNL1A4. Cell. 1996;87:543–52.

    Article  CAS  PubMed  Google Scholar 

  45. Yabe I, Sasaki H, Takeichi N, Takei A, Hamada T, Fukushima K, Tashiro K. Positional vertigo and macroscopic downbeat positioning nystagmus in spinocerebellar ataxia type 6 (SCA6). J Neurol. 2003;250:440–3.

    Article  PubMed  Google Scholar 

  46. Jen JC, Yue Q, Karrim J, Nelson SF, Baloh RW. Spinocerebellar ataxia type 6 with positional vertigo and acetazolamide responsive episodic ataxia. J Neurol Neurosurg Psychiatry. 1998;65:565–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Suzuki M, Fujiwara K, Tsubuku T, Yabe I, Sasaki H, Fukuda S. Time course of downbeat positioning nystagmus in familial hemiplegic migraine type 1 treated with acetazolamide. J Neurol Sci. 2016;368:206–8.

    Article  PubMed  Google Scholar 

  48. Yabe I, Kitagawa M, Suzuki Y, et al. Downbeat positioning nystagmus is a common clinical feature despite variable phenotypes in an FHM1 family. J Neurol. 2008;255:1541–4.

    Article  PubMed  Google Scholar 

  49. Ling X, Kim HJ, Lee JH, Choi JY, Yang X, Kim JS. Positioning velocity matters in central paroxysmal positional vertigo: implication for the mechanism. Front Neurol. 2020;11: 591602.

    Article  PubMed  PubMed Central  Google Scholar 

  50. •• Muth C, Teufel J, Schöls L, Synofzik M, Franke C, Timmann D, Mansmann U, Strupp M. Fampridine and acetazolamide in EA2 and related familial EA. Neurol Clin Pract. 2021;11:e438–e446. A recent randomized placebo-controlled trial showing that both fampridine and acetazolamide significantly reduce the number of attacks in patients with episodic ataxia type 2.

  51. Na S, Kim T. Efficacy of levetiracetam in patients with episodic ataxia type 2 caused by CACNA1A mutation: three case reports. Neurol Sci. 2021. https://doi.org/10.1007/s10072-021-05368-y.

    Article  PubMed  Google Scholar 

  52. Yu-Wai-Man P, Gorman G, Bateman DE, Leigh RJ, Chinnery PF. Vertigo and vestibular abnormalities in spinocerebellar ataxia type 6. J Neurol. 2009;256:78–82.

    Article  PubMed  Google Scholar 

  53. Strupp M, Zwergal A, Brandt T. Episodic ataxia type 2. Neurotherapeutics. 2007;4:267–73.

    Article  CAS  PubMed  Google Scholar 

  54. Lee JY, Lee WW, Kim JS, Kim HY, Kim JK, Jeon BS. Perverted head-shaking and positional downbeat nystagmus in patients with multiple system atrophy. Mov Disord. 2009;24:1290–1295.

  55. • Byun YJ, Levy DA, Nguyen SA, Brennan E, Rizk HG. Treatment of vestibular migraine: a systematic review and meta-analysis. Laryngoscope. 2021;131:186–194. An important systematic review and meta-analysis showing that several classes of migraine prophylactic drugs are effective in vestibular migraine patients.

  56. • Wang F, Wang J, Cao Y, Xu Z. Serotonin-norepinephrine reuptake inhibitors for the prevention of migraine and vestibular migraine: a systematic review and meta-analysis. Reg Anesth Pain Med. 2020;45:323–30. An important systematic review and meta-analysis showing that serotonin-norepinephrine reuptake inhibitors are clinically safe and effective for vestibular migraine prophylaxis.

    Article  PubMed  Google Scholar 

  57. Bayer O, Adrion C, Al Tawil A, et al. Results and lessons learnt from a randomized controlled trial: prophylactic treatment of vestibular migraine with metoprolol (PROVEMIG). Trials. 2019. https://doi.org/10.1186/s13063-019-3903-5.

    Article  PubMed  PubMed Central  Google Scholar 

  58. Dieterich M, Brandt T. Episodic vertigo related to migraine (90 cases): Vestibular migraine? J Neurol. 1999;246:883–92.

    Article  CAS  PubMed  Google Scholar 

  59. Beh SC. Horizontal direction-changing positional nystagmus and vertigo: a case of vestibular migraine masquerading as horizontal canal BPPV. Headache. 2018. https://doi.org/10.1111/head.13356.

    Article  PubMed  Google Scholar 

  60. Roberts RA, Gans RE, Kastner AH. Differentiation of migrainous positional vertigo (MPV) from horizontal canal benign paroxysmal positional vertigo (HC-BPPV). Int J Audiol. 2006. https://doi.org/10.1080/14992020500429658.

    Article  PubMed  Google Scholar 

  61. Lechner C, Taylor RL, Todd C, MacDougall H, Yavor R, Halmagyi GM, Welgampola MS. Causes and characteristics of horizontal positional nystagmus. J Neurol. 2014;261:1009–17.

    Article  PubMed  Google Scholar 

  62. Bertholon P, Tringali S, Faye MB, Antoine JC, Martin C. Prospective study of positional nystagmus in 100 consecutive patients. Ann Otol Rhinol Laryngol. 2006;115:587–94.

    Article  PubMed  Google Scholar 

  63. von Brevern M, Zeise D, Neuhauser H, Clarke AH, Lempert T. Acute migrainous vertigo: clinical and oculographic findings. Brain. 2004;128:365–74.

    Article  Google Scholar 

  64. El-Badry MM, Samy H, Kabel AM, Rafat FM, Sanyelbhaa H. Clinical criteria of positional vertical nystagmus in vestibular migraine. Acta Otolaryngol. 2017;137:720–2.

    Article  CAS  PubMed  Google Scholar 

  65. Mahrous MM. Vestibular migraine and benign paroxysmal positional vertigo, close presentation dilemma. Acta Otolaryngol. 2020. https://doi.org/10.1080/00016489.2020.1770857.

    Article  PubMed  Google Scholar 

  66. Cutrer FM, Baloh RW. Migraine-associated dizziness. Headache J Head Face Pain. 1992. https://doi.org/10.1111/j.1526-4610.1992.hed3206300.x.

    Article  Google Scholar 

  67. Neuhauser H, Radtke A, Von Brevern M, Lempert T. Zolmitriptan for treatment of migrainous vertigo: a pilot randomized placebo-controlled trial. Neurology. 2003;60:882–3.

    Article  CAS  PubMed  Google Scholar 

  68. Young AS, Lechner C, Bradshaw AP, MacDougall HG, Black DA, Michael Halmagyi G, Welgampola MS. Capturing acute vertigo: a vestibular event monitor. Neurology. 2019;92:E2743–53.

    Article  PubMed  Google Scholar 

  69. Von Brevern M, Radtke A, Clarke AH, Lempert T. Migrainous vertigo presenting as episodic positional vertigo. Neurology. 2004;62:469–72.

    Article  Google Scholar 

  70. King S, Priesol AJ, Davidi SE, Merfeld D, Lewis R. Self-motion perception is sensitized in vestibular migraine: pathophysiologic and clinical implications. Sci Rep. 2019. https://doi.org/10.1038/s41598-019-50803-y.

  71. ● Beh SC. Nystagmus and vertigo in acute vestibular migraine attacks: response to non-invasive vagus nerve stimulation. Otol Neurotol. 2021;42:e233–6. A recent small series showing promising results with the use non-invasive vagus nerve stimulation in vestibular migraine attacks, including in one patient manifesting with central positional nystagmus.

    Article  PubMed  Google Scholar 

  72. Henssen DJHA, Derks B, van Doorn M, Verhoogt N, Cappellen V, van Walsum AM, Staats P, Vissers K. Vagus nerve stimulation for primary headache disorders: an anatomical review to explain a clinical phenomenon. Cephalalgia. 2019. https://doi.org/10.1177/0333102419833076.

    Article  PubMed  PubMed Central  Google Scholar 

  73. Choi JY, Park YM, Woo YS, Kim SU, Jung JM, Kwon DY. Perverted head-shaking and positional downbeat nystagmus in pregabalin intoxication. J Neurol Sci. 2014;337:243–4.

    Article  PubMed  Google Scholar 

  74. Oh S-Y, Kim JS, Lee YH, Lee AY, Kim J, Kim JM. Downbeat, positional, and perverted head-shaking nystagmus associated with lamotrigine toxicity. J Clin Neurol. 2006;2:283.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Arbusow V, Strupp M, Brandt T. Amiodarone-induced severe prolonged headpositional vertigo and vomiting. Neurology. 1998;51:917.

    Article  CAS  PubMed  Google Scholar 

  76. Scholtz AW, Steindl R, Burchardi N, Bognar-Steinberg I, Baumann W. Comparison of the therapeutic efficacy of a fixed low-dose combination of cinnarizine and dimenhydrinate with betahistine in vestibular neuritis: a randomized, double-blind, non-inferiority study. Clin Drug Investig. 2012;32:387–99.

    Article  CAS  PubMed  Google Scholar 

  77. De Schutter E, Adham ZO, Kattah JC. Central positional vertigo: a clinical-imaging study. Prog Brain Res. 2019;249:345–60.

    Article  PubMed  Google Scholar 

  78. Phillips JS, Newman J, Cox S. Towards providing an automated approach to differentiating the nystagmus of ménière’s disease, vestibular migraine, and benign paroxysmal positional vertigo. Otol Neurotol. 2021;42:890–6.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Neurology Department, Coimbra University Hospital Centre, Praceta Professor Mota Pinto, 3000-075, Coimbra, Portugal

    Ana Inês Martins MD, André Jorge MD & João Lemos MD, PhD

  2. Faculty of Medicine, Coimbra University, Coimbra, Portugal

    Ana Inês Martins MD & João Lemos MD, PhD

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Martins, A.I., Jorge, A. & Lemos, J. Central Positional Nystagmus. Curr Treat Options Neurol 24, 453–484 (2022). https://doi.org/10.1007/s11940-022-00731-6

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