Gabapentin Relieves Vertigo of Periodic Vestibulocerebellar Ataxia: 3 Cases and Possible Mechanism

Abstract

Objective The aim of this study was to report relief of optokinetic‐triggered vertigo (OKTV) with low‐dose gabapentin in three patients with periodic vestibulocerebellar ataxia [episodic ataxia type 4 (EA4); OMIM 606552]. Methods Clinical observations and analysis of video‐recorded eye movements were used before and after gabapentin. Results Gabapentin relieved vertigo of all three treated patients with EA4, particularly during activities that typically would induce vertiginous symptoms. Two patients reported 8–12 hours of sustained relief after the first 100 mg dose. One has benefited from 100–200 mg TID for 7 years. Video analysis of nystagmus revealed improved target tracking on smooth pursuit and a steadier gaze hold. Conclusions Gabapentin effectively relieved the optokinetic‐triggered vertigo in our patients with EA4. Mechanisms are postulated in terms of known tight gabapentin binding to the Purkinje cell voltage‐gated calcium channel. The observations may offer insight into this rare disease's neuropathology. © 2021 International Parkinson and Movement Disorder Society

Reply to: Gabapentin Relieves Vertigo of Periodic Vestibulocerebellar Ataxia: 3 Cases and Possible Mechanism

“…1 A recent meta-analysis published in this journal revealed a significantly increased risk of developing PD in patients with type 2 diabetes mellitus. 2 Furthermore, this group and others have reported an association of diabetes with faster motor progression in patients with PD. 2,3 In line with these findings, Markaki and colleagues reveal that a high HbA1c level (≥42 mmol/mL, n = 18) was a strong and significant predictor of unfavorable motor outcome (Hoehn & Yahr stage ≥ 3).…”

“…2 Furthermore, this group and others have reported an association of diabetes with faster motor progression in patients with PD. 2,3 In line with these findings, Markaki and colleagues reveal that a high HbA1c level (≥42 mmol/mL, n = 18) was a strong and significant predictor of unfavorable motor outcome (Hoehn & Yahr stage ≥ 3). Interestingly, patients with low HbA1c levels (≤30 mmol/mol, n = 17) also had a faster motor progression in this study, although less pronounced.…”

Gabapentin Relieves Vertigo of Periodic Vestibulocerebellar Ataxia: 3 Cases and Possible Mechanism

“…The improvement in cerebellar signs found 3 hours after single doses of gabapentin was coherent with the drug's T max , and a simple test intended to single out symptoms that might benefit from gabapentin could be based on administration of single doses of this drug, with a favorable response predicting prolonged amelioration, 2 as also found by Coin and Vance. 1 In other studies, gabapentin improved ataxia in GM2gangliosidosis; spinocerebellar ataxia types 1, 2, and 6; and oscillopsia and ataxia in olivopontocerebellar atrophy. In other disorders with a deficiency in GABA content, such as progressive encephalomyelitis with rigidity, muscular spasms were suppressed by a combination of gabapentin and tiagabine, and in diaphragmatic myoclonus, gabapentin and tiagabine obtained symptomatic control.…”

Episodic Ataxias: Primary and Secondary Etiologies, Treatment, and Classification Approaches

“…It resembles EA2 but without interictal nystagmus, and it does not respond to acetazolamide. Gabapentin may relieve vertigo symptoms in EA4 [70]. Linkage studies in 1996 excluded autosomal dominant ataxias with known chromosomal localization at that time, including KCNA1, CACNA1A, SCAs 1-5 and DRPLA [71].…”