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BackgroundThe vertical vestibulo-ocular reflex (VOR) may be impaired in internuclear ophthalmoplegia (INO) as the medial longitudinal fasciculus (MLF) conveys VOR-signals from the vertical semicircular canals. It has been proposed that signals from the contralesional posterior semicircular canal (PSC) are exclusively transmitted through the MLF, while for the contralesional anterior canal other pathways exist.ObjectiveHere, we aimed to characterize dysfunction in individual canals in INO-patients using the video-head-impulse test (vHIT) and to test the hypothesis of dissociated vertical canal impairment in INO.MethodsVideo-head-impulse testing and magnetic resonance imaging were obtained in 21 consecutive patients with unilateral (n = 16) or bilateral (n = 5) INO and 42 controls. VOR-gains and compensatory catch-up saccades were analyzed and the overall function (normal vs. impaired) of each semicircular canal was rated.ResultsIn unilateral INO, largest VOR-gain reductions were noted in the contralesional PSC (0.55 ± 0.11 vs. 0.89 ± 0.08, p < 0.001), while in bilateral INO both posterior (0.43 ± 0.11 vs. 0.89 ± 0.08, p < 0.001) and anterior (0.58 ± 0.19 vs. 0.88 ± 0.09, p < 0.001) canals showed marked drops. Small, but significant VOR-gain reductions were also found in the other canals in unilateral and bilateral INO-patients. Impairment of overall canal function was restricted to the contralesional posterior canal in 60% of unilateral INO-patients, while isolated involvement of the posterior canal was rare in bilateral INO-patients (20%). Reviewers correctly identified the INO-pattern in 15/21 (71%) patients and in all controls (sensitivity = 84.2% [95%-CI = 0.59.5–95.8]; specificity = 95.5% [95%-CI = 83.3–99.2]).ConclusionUsing a vHIT based overall rating of canal function, the correct INO-pattern could be identified with high accuracy. The predominant and often selective impairment of the contralesional posterior canal in unilateral INO further supports the role of the MLF in transmitting posterior canal signals. In patients with acute dizziness and abnormal vHIT-results, central pathologies such as INO should be considered as well, especially when the posterior canal is involved.

A 20-year-old man presented with dizziness and gait disturbance for approximately 2 months. Neurologic examination revealed spontaneous downbeat nystagmus (DBN), limb ataxia, and abnormal tandem gait (video on the Neurology ® Web site at Neurology.org). Video-oculography showed augmented DBN during position change (figure 1). MRI revealed diffuse atrophy and signal changes in the brainstem and cerebellum (figure 2). The plasma levels of very long-chain fatty acids (C26:0 concentration, C24:0/C22:0 ratio, and C26:0/C22: 0 ratio) were elevated. Subsequent genetic testing revealed a missense mutation in the ABCD1 gene. The olivopontocerebellar form of X-linked adrenoleukodystrophy should be considered a rare but possible diagnosis in young men with dizziness and