Abstract
Clinically, central positional nystagmus (CPN) is often suspected when atypical forms of its peripheral counterpart, i.e., benign paroxysmal positional vertigo (BPPV), are observed, namely a linear horizontal nystagmus as in horizontal canal BPPV or a downwardly and torsionally beating nystagmus as in anterior canal BPPV. Pathophysiologically, CPN is caused by cerebellar and/or brainstem dysfunction. Recent work has provided further insights into the different clinical phenotypes and the underlying pathomechanisms. We performed a PubMed review focused on the findings on CPN using the key words “Central Positional Nystagmus”, “Central Positional Vertigo”, “Positional Nystagmus” OR “Positioning Nystagmus” OR “Positional Vertigo” OR “Positioning Vertigo” AND “Central” from January 2015 to August 2021. CPN may account for up to 12% of patients with positional nystagmus. Clinical data on CPN are mostly based on case reports or small retrospective case series. CPN is frequently associated with cerebellar and/or brainstem structural lesions, namely stroke, tumours or demyelination, or diffuse involvement of these structures due to degenerative or autoimmune/paraneoplastic diseases; it is also found in patients with vestibular migraine. CPN can be paroxysmal or persistent. The direction of the nystagmus is often downward in head-hanging or apogeotropic in lateral supine positions; combinations of both forms also occur. Clinically it is important to note that CPN is often associated with other central, often cerebellar ocular motor or other neurological signs; typically, it is not improved by the therapeutic liberatory manoeuvres for BPPV. These additional features are also important for the diagnosis, in particular if no structural lesions are found. Pathophysiologically, CPN is believed to reflect an abnormal integration of semicircular canal-related signals by the cerebellar nodulus, uvula and/or tonsil, ultimately providing an erroneous estimation of the head tilt and/or eye position coordinates. The natural course of CPN remains, so far, largely unknown. Symptomatic treatment of CPN consists of pharmacotherapy, e.g., with 4-aminopyridine, and causative treatment of the underlying disease if known. CPN is an important differential diagnosis to BPPV and a clinically relevant entity with heterogenous clinical presentations and various pathomechanisms and etiologies. In particular, studies on the natural course and treatment of CPN are needed.
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Abbreviations
- CPN:
-
Central positional nystagmus
- BPPV:
-
Benign paroxysmal positional vertigo
- SPV:
-
Slow phase velocity
- SCC:
-
Semicircular canal
- GEN:
-
Gaze-evoked nystagmus
- HSN:
-
Post-head shaking nystagmus
- OTR:
-
Ocular tilt reaction
- SVV:
-
Subjective visual vertical
- VM:
-
Vestibular migraine
- SCA:
-
Spinocerebellar ataxia
References
Eggers SDZ, Bisdorff A, Von Brevern M et al (2019) Classification of vestibular signs and examination techniques: Nystagmus and nystagmus-like movements: Consensus document of the Committee for the International Classification of Vestibular Disorders of the Bárány Society. J Vestib Res 29:57–87. https://doi.org/10.3233/VES-190658
Macdonald NK, Kaski D, Saman Y et al (2017) Central positional nystagmus: a systematic literature review. Front Neurol 8:141. https://doi.org/10.3389/fneur.2017.00141
Büki B, Mandalà M, Nuti D (2014) Typical and atypical benign paroxysmal positional vertigo: literature review and new theoretical considerations. J Vestib Res 24:415–423. https://doi.org/10.3233/VES-140535
Ling X, Zhao DH, Shen B et al (2020) Clinical characteristics of patients with benign paroxysmal positional vertigo diagnosed based on the diagnostic criteria of the Bárány Society. Front Neurol. https://doi.org/10.3389/fneur.2020.00602
Choi J-Y, Kim JH, Kim HJ et al (2015) Central paroxysmal positional nystagmus: characteristics and possible mechanisms. Neurology 84:2238–2246. https://doi.org/10.1212/WNL.0000000000001640
Choi J-Y, Glasauer S, Kim JH et al (2018) Characteristics and mechanism of apogeotropic central positional nystagmus. Brain 141:762–775. https://doi.org/10.1093/brain/awx381
Lechner C, Taylor RL, Todd C et al (2014) Causes and characteristics of horizontal positional nystagmus. J Neurol 261:1009–1017. https://doi.org/10.1007/s00415-013-7223-5
De Schutter E, Adham ZO, Kattah JC (2019) Central positional vertigo: a clinical-imaging study. Prog Brain Res 249:345–360. https://doi.org/10.1016/bs.pbr.2019.04.022
Choi SY, Jang JY, Oh EH et al (2018) Persistent geotropic positional nystagmus in unilateral cerebellar lesions. Neurology 91:e1053–e1057. https://doi.org/10.1212/WNL.0000000000006167
Laurens J, Angelaki DE (2011) The functional significance of velocity storage and its dependence on gravity. Exp Brain Res 210:407–422. https://doi.org/10.1007/s00221-011-2568-4
Glasauer S, Dieterich M, Brandt T (2001) Central positional nystagmus simulated by a mathematical ocular motor model of otolith-dependent modification of Listing’s plane. J Neurophysiol 86:1546–1554
Bertholon P, Bronstein AM, Davies RA et al (2002) Positional down beating nystagmus in 50 patients: Cerebellar disorders and possible anterior semicircular canalithiasis. J Neurol Neurosurg Psychiatry 72:366–372. https://doi.org/10.1136/jnnp.72.3.366
Bertholon P, Tringali S, Faye MB et al (2006) Prospective study of positional nystagmus in 100 consecutive patients. Ann Otol Rhinol Laryngol 115:587–594. https://doi.org/10.1177/000348940611500804
Cambi J, Astore S, Mandalà M et al (2013) Natural course of positional down-beating nystagmus of peripheral origin. J Neurol 260:1489–1496. https://doi.org/10.1007/s00415-012-6815-9
Feil K, Strobl R, Schindler A et al (2019) What is behind cerebellar vertigo and dizziness? Cerebellum 18:320–332. https://doi.org/10.1007/s12311-018-0992-8
Young AS, Nham B, Bradshaw AP et al (2021) Clinical, oculographic, and vestibular test characteristics of vestibular migraine. Cephalalgia. https://doi.org/10.1177/03331024211006042
Lemos J, Martins AI, Duque C et al (2018) Positional testing in acute vestibular syndrome: a transversal and longitudinal study. Otol Neurotol. https://doi.org/10.1097/MAO.0000000000002067
Lee SU, Choi JY, Kim HJ et al (2017) Impaired tilt suppression of post-rotatory nystagmus and cross-coupled head-shaking nystagmus in cerebellar lesions: image mapping study. Cerebellum 16:95–102. https://doi.org/10.1007/s12311-016-0772-2
Nylen C (1939) Statistical analysis of oto-neurological symptomatology in supra- and subtentorial tumours. Acta Otolaryngol 27:24–31. https://doi.org/10.3109/00016483909120560
Thomsen J, Zilstorff K, Johnsen NJ (1978) Positional nystagmus of the persistent type. ORL 40:86–91. https://doi.org/10.1159/000275390
Von Brevern M, Bertholon P, Brandt T et al (2015) Benign paroxysmal positional vertigo: diagnostic criteria. J Vestib Res 25:105–117. https://doi.org/10.3233/VES-150553
Ling X, Kim HJ, Lee JH et al (2020) Positioning velocity matters in central paroxysmal positional vertigo: implication for the mechanism. Front Neurol 11:591602. https://doi.org/10.3389/fneur.2020.591602
Choi JY, Kim JS (2019) Central positional nystagmus: characteristics and model-based explanations. Prog Brain Res 249:211–225. https://doi.org/10.1016/bs.pbr.2019.04.012
Büttner U, Brandt T, Helmchen C (1999) The direction of nystagmus is important for the diagnosis of central paroxysmal positioning nystagmus (cPPV). Neuro Ophthalmol 21:97–104. https://doi.org/10.1076/noph.21.2.97.3919
Kattah JC, Kolsky MP, Luessenhop AJ (1984) Positional vertigo and the cerebellar vermis. Neurology 34:527–529. https://doi.org/10.1212/wnl.34.4.527
Lee H-J, Kim ES, Kim M et al (2014) Isolated horizontal positional nystagmus from a posterior fossa lesion. Ann Neurol 76:905–910. https://doi.org/10.1002/ana.24292
Katsarkas A (1981) Vestibular and oculomotor disturbances in pathology of the fourth ventricle. Laryngoscope 91:71–77. https://doi.org/10.1288/00005537-198101000-00010
Goto N, Hoshino T, Kaneko M, Ishikawa H (1983) Central positional vertigo: clinico-anatomic study. Neurol Med Chir (Tokyo) 23:534–540. https://doi.org/10.2176/nmc.23.534
Lee SU, Kim HJ, Lee ES et al (2021) Central positional nystagmus in inferior cerebellar peduncle lesions: a case series. J Neurol 1:3. https://doi.org/10.1007/s00415-021-10435-7
Anagnostou E, Varaki K, Anastasopoulos D (2008) A minute demyelinating lesion causing acute positional vertigo. J Neurol Sci 266:187–189. https://doi.org/10.1016/j.jns.2007.09.013
Habek M, Gabelić T, Pavliša G, Brinar VV (2011) Central positioning upbeat nystagmus and vertigo due to pontine stroke. J Clin Neurosci 18:977–978. https://doi.org/10.1016/j.jocn.2010.11.021
Imai T, Horii A, Takeda N et al (2010) A case of apogeotropic nystagmus with brainstem lesion: An implication for mechanism of central apogeotropic nystagmus. Auris Nasus Larynx 37:742–746. https://doi.org/10.1016/j.anl.2010.04.004
Lee SU, Park SH, Park JJ et al (2015) Dorsal medullary infarction: distinct syndrome of isolated central vestibulopathy. Stroke 46:3081–3087. https://doi.org/10.1161/STROKEAHA.115.010972
Polensek SH, Tusa RJ (2010) Nystagmus during attacks of vestibular migraine: an aid in diagnosis. Audiol Neurotol 15:241–246. https://doi.org/10.1159/000255440
El-Badry MM, Samy H, Kabel AM et al (2017) Clinical criteria of positional vertical nystagmus in vestibular migraine. Acta Otolaryngol 137:720–722. https://doi.org/10.1080/00016489.2017.1318220
ElSherif M, Reda MI, Saadallah H, Mourad M (2020) Eye movements and imaging in vestibular migraine. Acta Otorrinolaringol Esp 71:3–8. https://doi.org/10.1016/j.otorri.2018.10.001
Lee JY, Lee WW, Kim JS et al (2009) Perverted head-shaking and positional downbeat nystagmus in patients with multiple system atrophy. Mov Disord 24:1290–1295. https://doi.org/10.1002/mds.22559
Yu-Wai-Man P, Gorman G, Bateman DE et al (2009) Vertigo and vestibular abnormalities in spinocerebellar ataxia type 6. J Neurol 256:78–82. https://doi.org/10.1007/s00415-009-0068-2
Yabe I, Matsushima M, Yoshida K et al (2015) Rare frequency of downbeat positioning nystagmus in spinocerebellar ataxia type 31. J Neurol Sci 350:90–92. https://doi.org/10.1016/j.jns.2014.12.042
Wray SH, Dalmau J, Chen A et al (2011) Paraneoplastic disorders of eye movements. Ann N Y Acad Sci 1233:279–284. https://doi.org/10.1111/j.1749-6632.2011.06113.x
Eggers SDZ, Pittock SJ, Shepard NT et al (2012) Positional periodic alternating vertical nystagmus with PCA-Tr antibodies in Hodgkin lymphoma. Neurology 78:1800–1802. https://doi.org/10.1212/WNL.0b013e3182583085
Kattah JC, Zee DS (2020) Eye movements in demyelinating, autoimmune and metabolic disorders. Curr Opin Neurol 33:111–116. https://doi.org/10.1097/WCO.0000000000000772
Martins AI, Carvalho JN, Amorim AM et al (2017) Disabling central paroxysmal positioning upbeat nystagmus and vertigo associated with the presence of anti-glutamic acid decarboxylase antibodies. J Neuro Ophthalmol 38:1. https://doi.org/10.1097/WNO.0000000000000547
Lee SU, Kim HJ, Choi JY et al (2019) Acute vestibular syndrome associated with anti-GQ1b antibody. Neurology 93:e1085–e1092. https://doi.org/10.1212/WNL.0000000000008107
Kang BH, Il KJ, Lim YM, Kim KK (2018) Abnormal oculomotor functions in amyotrophic lateral sclerosis. J Clin Neurol 14:464–471. https://doi.org/10.3988/jcn.2018.14.4.464
Kim SH, Kim SS, Ha H, Lee SH (2016) X-linked adrenoleukodystrophy presenting with positional downbeat nystagmus. Neurology 86:2214–2215. https://doi.org/10.1212/WNL.0000000000002749
Kim YE, Kim JS, Yang HJ et al (2016) Perverted head-shaking and positional downbeat nystagmus in essential tremor. Cerebellum 15:152–158. https://doi.org/10.1007/s12311-015-0683-7
King S, Priesol AJ, Davidi SE et al (2019) Self-motion perception is sensitized in vestibular migraine: pathophysiologic and clinical implications. Sci Rep. https://doi.org/10.1038/s41598-019-50803-y
Büttner U, Helmchen C, Brandt T (1999) Diagnostic criteria for central versus peripheral positioning nystagmus and vertigo: A review. Acta Otolaryngol 119:1–5. https://doi.org/10.1080/00016489950181855
Asprella-Libonati G, Pecci R (2019) Apogeotropic variant of posterior canal benign paroxysmal positional vertigo. B-ENT, pp 119–125
Mandalàà M, Salerni L, Nuti D (2019) Benign positional paroxysmal vertigo treatment: a practical update. Curr Treat Options Neurol. https://doi.org/10.1007/s11940-019-0606-x
Kim HJ, Park JH, Kim JS (2020) Update on benign paroxysmal positional vertigo. J Neurol 1:1. https://doi.org/10.1007/s00415-020-10314-7
Zuma e Maia F, Ramos BF, Cal R et al (2020) Management of Lateral Semicircular Canal Benign Paroxysmal Positional Vertigo. Front Neurol 11:1040. https://doi.org/10.3389/fneur.2020.01040
Kim JM, Lee SH, Cho SH et al (2021) Cerebellar infarction presenting with isolated positional vertigo: differentiating factors for benign paroxysmal positional vertigo. Neurol Sci 42:1045–1052. https://doi.org/10.1007/s10072-020-04617-w
Jorge A, Martins AI, Gouveia A, Lemos J (2020) The use of video-head impulse test in different head positions in vertical nystagmus and ataxia associated with probable thiamine deficiency. Cerebellum 19:611–615. https://doi.org/10.1007/s12311-020-01140-8
Choi SY, Park SH, Kim HJ, Kim JS (2014) Paraneoplastic downbeat nystagmus associated with cerebellar hypermetabolism especially in the nodulus. J Neurol Sci 343:187–191. https://doi.org/10.1016/j.jns.2014.05.030
Dawn Nelson M, Mann L, Nicholson C, Lehman M (2019) Static positional nystagmus in the healthy vestibular system. J Am Acad Audiol 30:883–895. https://doi.org/10.3766/jaaa.18024
Martens C, Goplen FK, Nordfalk KF et al (2016) Prevalence and characteristics of positional nystagmus in normal subjects. Otolaryngol Neck Surg 154:861–867. https://doi.org/10.1177/0194599816629640
Jeffery H, Hopkins M, Anderson R et al (2017) The interpretation of static positional nystagmus in a balance clinic. Int J Audiol 56:958–966. https://doi.org/10.1080/14992027.2017.1357841
Young AS, Rosengren SM, D’Souza M et al (2020) Nystagmus characteristics of healthy controls. J Vestib Res 30:1–8. https://doi.org/10.3233/ves-200022
Strupp M, Brandt T, Dieterich M (2021) Vertigo and dizziness: Common complaints, 3rd edn. Springer Nature, London
Kremmyda O, Zwergal A, La Fougère C et al (2013) 4-Aminopyridine suppresses positional nystagmus caused by cerebellar vermis lesion. J Neurol 260:321–323
Tatu L, Moulin T, Bogousslavsky J, Duvernoy H (1996) Arterial territories of human brain: Brainstem and cerebellum. Neurology 47:1125–1135. https://doi.org/10.1212/WNL.47.5.1125
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JL: Conceptualization (equal); methodology (equal); writing—original draft (lead); writing—review and editing (equal). MS: Conceptualization (equal); methodology (equal); supervision (lead); writing—review and editing (equal). JL and MS had the idea for the article, performed the literature search and data analysis, and critically revised the work. JL wrote the first draft.
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J. Lemos reports no conflicts of interest; M. Strupp is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology and Section Editor of F1000. He has received speaker’s honoraria from Abbott, Auris Medical, Biogen, Eisai, Grünenthal, GSK, Henning Pharma, Interacoustics, J&J, MSD, Otometrics, Pierre-Fabre, TEVA, UCB, and Viatris. He is a shareholder and investor in IntraBio. He distributes “M-glasses” and the “Positional vertigo App”. He acts as a consultant for Abbott, AurisMedical, Heel, IntraBio and Sensorion.
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Lemos, J., Strupp, M. Central positional nystagmus: an update. J Neurol 269, 1851–1860 (2022). https://doi.org/10.1007/s00415-021-10852-8
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DOI: https://doi.org/10.1007/s00415-021-10852-8