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Clinical question
A 26-year-old man noticed a facial asymmetry and double vision on awaking and went to the emergency room. There was left eye exotropia at rest and a total horizontal gaze palsy to the right. On attempting gaze to the left, there was limited right eye adduction and horizontal nystagmus of the abducted left eye. Vertical eye movements and convergence were normal. He had a right-sided lower motor neurone facial palsy, with decreased forehead wrinkling on the right, an asymmetric smile and Bell’s sign (figure 1 and video 1). There was also right sensorineural hearing loss (by Weber and Rinne testing) and right leg ataxia.
(A) Internuclear ophthalmoplegia on left gaze (upper image), right gaze palsy (second image) and normal upward gaze (lower image). (B) Right lower motor neurone facial palsy. The patient has few wrinkles in his right forehead, a Bell’s sign, and an asymmetric smile.
Questions
What is probable site of the lesion?
What is its most likely cause?
Answer
Question 1
This patient has the ‘eight-and-a-half syndrome’ (Eggenberger 1998), combining a one-and-a-half syndrome with an ipsilateral lower motor neurone facial (seventh) nerve palsy.1 The one-and-a-half syndrome combines …
Footnotes
Contributors BM: data collection, conception and drafting the manuscript for intellectual content and review of final form. MF, AC, ASC: contributed to patient care and revision of the manuscript. JC: data collection, critical review.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned, externally peer reviewed by Amy Ross-Russell, Southampton, UK.
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