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Abstract
Objective To define a disorder characterized by recurrent spontaneous vertigo (RSV) of unknown etiology and interictal headshaking nystagmus (HSN).
Methods We characterized HSN in 35 patients with RSV-HSN compared to that recorded in randomly selected patients with compensated vestibular neuritis (VN), vestibular migraine (VM), and Ménière disease (MD).
Results The estimated time constant (TC) of the primary phase of HSN was 12 seconds (95% confidence interval [CI] 12–13) in patients with RSV-HSN, which was larger than those in patients with VN (5 seconds, 95% CI 4–5), VM (5 seconds, 95% CI 5–6), or MD (6 seconds, 95% CI 5–6). TCs of the horizontal vestibulo-ocular reflex were also larger during the rotatory chair test in patients with RSV-HSN. Among the 35 patients with RSV-HSN, 7 showed vigorous long-lasting HSN with a peak slow-phase velocity >50.0°/s. In 5 patients (5 of 7, 71%) with vigorous HSN, HSN could have been induced even with headshaking for only 2 to 5 seconds. Long-term prognosis was favorable, with a resolution or improvement of the symptoms in more than half of the patients during the median follow-up of 12 (range 2–58) years from symptom onset. None developed VM, MD, or cerebellar dysfunction during the follow-up.
Conclusion The clinical features and characteristics of HSN in our patients indicate a hyperactive and asymmetric velocity-storage mechanism that gives rise to intermittent attacks of spontaneous vertigo probably when marginal compensation of underlying pathology is disrupted by endogenous or exogenous factors.
Glossary
- BRV=
- benign recurrent vertigo;
- CI=
- confidence interval;
- HIT=
- head-impulse test;
- HSN=
- headshaking nystagmus;
- MD=
- Ménière disease;
- MR=
- magnetic resonance;
- MSSQ=
- motion sickness susceptibility questionnaire;
- RSV=
- recurrent spontaneous vertigo;
- SPV=
- slow-phase velocity;
- TC=
- time constant;
- VM=
- vestibular migraine;
- VN=
- vestibular neuritis;
- VOR=
- vestibulo-ocular reflex;
- VSM=
- velocity-storage mechanism
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
CME Course: NPub.org/cmelist
Editorial Page 1089
- Received December 3, 2017.
- Accepted in final form March 21, 2018.
- © 2018 American Academy of Neurology
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