Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Current Treatment Options in Neurology
Published in: Current Treatment Options in Neurology 10/2022

11-07-2022 | Vestibular Migraine | Neurologic Ophthalmology and Otology (R Shin and D Gold, Section Editors)

Central Positional Nystagmus

Authors: Ana Inês Martins, MD, André Jorge, MD, João Lemos, MD, PhD

Published in: Current Treatment Options in Neurology | Issue 10/2022

Login to get access

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.
Literature
1.
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
7.
Choi JY, Kim JS. Central positional nystagmus: characteristics and model-based explanations. Prog Brain Res. 2019;249:211–25.PubMedCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
11.
Nylen C. Statistical analysis of oto-neurological symptomatology in supra- and subtentorial tumours. Acta Otolaryngol. 1939;27:24–31.CrossRef
12.
Drachman DA, Diamond ER, Hart CW. Posturally - evoked vomiting; Association with posterior fossa lesions. Ann Otol Rhinol Laryngol. 1977;86:97–101.PubMedCrossRef
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.PubMed
14.
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.PubMedCrossRef
16.
Gregorius FK, Crandall PH, Baloh RW. Positional vertigo with cerebellar astrocytoma. Surg Neurol. 1976;6:283–6.PubMed
17.
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.PubMedCrossRef
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.PubMedCrossRef
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.CrossRef
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.CrossRef
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.PubMedCrossRef
23.
24.
Oh EH, Kim H, Choi SY, Choi KD, Choi JH. Paroxysmal central positional nystagmus responsive to clonazepam. J Neurol. 2022;269:1028–31.PubMedCrossRef
25.
Thomsen J, Zilstorff K, Johnsen NJ. Positional nystagmus of the persistent type. ORL. 1978;40:86–91.PubMedCrossRef
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.PubMedCrossRef
27.
Anagnostou E, Varaki K, Anastasopoulos D. A minute demyelinating lesion causing acute positional vertigo. J Neurol Sci. 2008;266:187–9.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
29.
30.
Gelfand EW. Intravenous immune globulin in autoimmune and inflammatory diseases. N Engl J Med. 2012;367:2015–25.PubMedCrossRef
31.
32.
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.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
35.
Kearsley JH, Johnson P, Halmagyi GM. Paraneoplastic cerebellar disease: remission with excision of the primary tumor. Arch Neurol. 1985;42:1208–10.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
37.
Tafur AJ, Baumann Kreuzinger LM, Quevedo F. 28-Year-old man with severe vertigo. Mayo Clin Proc. 2008;83:1070–3.PubMedCrossRef
38.
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.
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.PubMedPubMedCentralCrossRef
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.CrossRef
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
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.PubMedCrossRef
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.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
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.
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.PubMedCrossRef
53.
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.PubMedCrossRef
57.
58.
Dieterich M, Brandt T. Episodic vertigo related to migraine (90 cases): Vestibular migraine? J Neurol. 1999;246:883–92.PubMedCrossRef
59.
60.
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.PubMedCrossRef
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.PubMedCrossRef
63.
von Brevern M, Zeise D, Neuhauser H, Clarke AH, Lempert T. Acute migrainous vertigo: clinical and oculographic findings. Brain. 2004;128:365–74.CrossRef
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.PubMedCrossRef
65.
66.
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.PubMedCrossRef
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.PubMedCrossRef
69.
Von Brevern M, Radtke A, Clarke AH, Lempert T. Migrainous vertigo presenting as episodic positional vertigo. Neurology. 2004;62:469–72.CrossRef
70.
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.PubMedCrossRef
72.
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.PubMedCrossRef
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.PubMedPubMedCentralCrossRef
75.
Arbusow V, Strupp M, Brandt T. Amiodarone-induced severe prolonged headpositional vertigo and vomiting. Neurology. 1998;51:917.PubMedCrossRef
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.PubMedCrossRef
77.
De Schutter E, Adham ZO, Kattah JC. Central positional vertigo: a clinical-imaging study. Prog Brain Res. 2019;249:345–60.PubMedCrossRef
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.PubMedCrossRef
Metadata
Title
Central Positional Nystagmus
Authors
Ana Inês Martins, MD
André Jorge, MD
João Lemos, MD, PhD
Publication date
11-07-2022
Publisher
Springer US
Published in
Current Treatment Options in Neurology / Issue 10/2022
Print ISSN: 1092-8480
Electronic ISSN: 1534-3138
DOI
https://doi.org/10.1007/s11940-022-00731-6