Wall-eyed bilateral internuclear ophthalmoplegia in a patient with progressive supranuclear palsy.
Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare disorder consisting of a bilateral adduction deficit and primary gaze position exotropia. Associated with bilateral medial longitudinal fasciculus lesions, it has been mostly reported in patients with multiple sclerosis and brainstem stroke. A 72-year-old man with characteristic clinical features of progressive supranuclear palsy (PSP) later developed WEBINO. Brain MRI revealed atrophy of the midbrain tegmentum. Caloric irrigation revealed intact horizontal eye movements in both eyes. We believe this to be the first report of WEBINO in PSP. The preservation of vestibulo-ocular horizontal eye movements supports the notion that the WEBINO in this condition was caused by a supranuclear rather than a nuclear lesion and suggests the possibility that even in other causes of WEBINO, the lesion is supranuclear and not in the medial rectus subnucleus as is often suggested.
Department of Otolaryngology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan. IZT01356@nifty.ne.jp
Munetaka
M
Ushio
Shinichi
S
Iwasaki
Yasuhiro
Y
Chihara
Toshihisa
T
Murofushi
D000368:Aged; D001284:Atrophy / Q000473:pathology / Q000503:physiopathology; D001933:Brain Stem / Q000473:pathology / Q000503:physiopathology*; D003704:Dementia / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D004401:Dysarthria / Q000473:pathology / Q000503:physiopathology; D005133:Eye Movements / Q000502:physiology; D020233:Gait Disorders, Neurologic / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology; D015835:Ocular Motility Disorders / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology*; D009801:Oculomotor Muscles / Q000294:innervation / Q000503:physiopathology; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology; D013494:Supranuclear Palsy, Progressive / Q000150:complications* / Q000473:pathology / Q000503:physiopathology*; D013681:Tegmentum Mesencephali / Q000473:pathology / Q000503:physiopathology; D014726:Vestibular Nuclei / Q000502:physiology; D014722:Vestibule, Labyrinth / Q000502:physiology