Clinical Features and Neurotologic Findings in Patients With Acute Unilateral Peripheral Vestibulopathy Associated With Antiganglioside Antibody

Abstract

Background and objectives: Anecdotal studies have reported the presence of antiganglioside antibodies in acute unilateral peripheral vestibulopathy (AUPV). This study aimed to determine the prevalence, clinical characteristics, and neurotologic findings of AUPV associated with antiganglioside antibodies.

Methods: Serum antigangliosides were measured in consecutive patients with AUPV according to the Bárány Society criteria during the acute and recovery phases in a referral-based university hospital in South Korea from September 2019 to January 2023. Clinical characteristics and neurotologic findings were compared between those with and without antiganglioside antibodies. The results of video-oculography, video head impulse and bithermal caloric tests, and other neurotologic evaluations including ocular and cervical vestibular-evoked myogenic potentials and subjective visual vertical were compared between the 2. MRIs dedicated to the inner ear were also conducted when considered necessary.

Results: One hundred five patients (mean age ± SD = 60 ± 13 years, 57 male) were included for analyses. During the acute phase, 12 patients (12/105, 11%) were tested positive for serum antiganglioside antibodies, including anti-GQ1b immunoglobulin (Ig) G (n = 5) or IgM (n = 4), anti-GM1 IgM (n = 3), and anti-GD1a IgG (n = 1, including 1 patient with a positive anti-GQ1b antibody). Patients with antiganglioside antibodies showed lesser intensity of spontaneous nystagmus (median [interquartile range] = 1.8 [1.2-2.1] vs 3.4 [1.5-9.5], p = 0.003) and a lesser degree of canal paresis (30 [17-47] vs 58 [34-79], p = 0.028) and gain asymmetry of the vestibulo-ocular reflex for the horizontal semicircular canal during head impulse tests (0.07 [-0.04 to 0.61] vs 0.36 [0.18-0.47], p = 0.032) than those without antibodies. Negative conversion of antibodies and vestibular recovery were observed in most patients (6/8, 75%). Among 30 patients with AUPV with 4-hour delayed 3D fluid-attenuated inversion recovery dedicated to the inner ear, gadolinium enhancement was observed in 18 (18/30, 60%), either in the vestibule (n = 9), semicircular canal (n = 6), or vestibular nerve (n = 5). The positivity rates based on specific antibodies could not be determined due to limited sample sizes.

Discussion: The association between antiganglioside antibodies and AUPV suggests an immune-mediated mechanism in acute vestibular failure and extends the clinical spectrum of antiganglioside antibody syndrome.

Figure 1. Neurotologic Findings Depending on the Presence of Antiganglioside Antibodies

(A) Spontaneous nystagmus was less prominent in patients with antibodies (median [interquartile range] = 1.8 [1.2–2.1] vs 3.4 [1.5–9.5], p = 0.003) compared with those without. In addition, patients with antibodies showed (B) a lesser degree of gain asymmetry (0.07 [−0.04 to 0.61] vs 0.36 [0.18–0.47], p = 0.032) and (C) canal paresis (30 [17–47] vs 58 [34–79], p = 0.028) than those without antibodies.

Figure 2. Comparison of the Head Impulse Gain of the VOR at Initial Presentation and During Follow-up

The initial VOR decrement was more profound in those without antibodies than in those with antibodies (median [IQR] = 0.61 [0.46–0.77] vs 0.80 [0.61–1.06], p = 0.048), whereas the VOR decrement was mostly normalized during the follow-up evaluation regardless of the presence of antiganglioside antibodies (median gain difference between initial and follow-up evaluation [IQR] = 0 [−0.08 to 0.03] vs 0.21 [0.06–0.35], F = 4.735, p = 0.034). IQR = interquartile range; VOR = vestibulo-ocular reflex.