Supranuclear Paralysis of Downgaze

John Trojanowski and I published this case in Neurology in 1980.(12) At that time, it was one of the first of five reports in the literature of the pathological localization of lesions in the midbrain that are responsible for selective supranuclear paralysis of downgaze. This case is of particular interest because serial ocular motility examinations over five years documented full recovery of his eye movements two years prior to death. The patient was a 58 year old man who was admitted to the Massachusetts General Hospital with dyspnea and a heart murmur. Cardiac catheterization revealed moderately severe mitral regurgitation attributed to a ruptured chorda tendineae. After the procedure, the patient was somnolent and lethargic. Seven days later, I did the first neuro-ophthalmic consultation. Neuro-ophthalmological findings: Pupils 3 mm, equal, sluggish to light and near (no light-near dissociation) Global paralysis of downgaze (saccades and pursuit) Absent convergence Slow saccades on upgaze Deviation of the eyes up under forced eye closure (Bell phenomenon) Horizontal gaze nystagmus to the right Optokinetic nystagmus absent with vertical rotation of the drum Normal vertical oculocephalic reflex (Doll's head maneuver) and Skew deviation, right hypertropia (6-8 diopters) Slight left head tilt - a left ocular tilt reaction (OTR) Neurologic examination was unremarkable except for periods of somnolence during which the eyes diverged up. When aroused, the patient was oriented with normal speech and without other neurological deficits. Diagnosis: Cardio-embolic midbrain infarct Spinal Tap: Normal pressure and cerebrospinal fluid. The patient was discharged home . Ocular motility one month later : •Improved downgaze •Absent convergence •Persistent skew deviation •Full horizontal gaze without nystagmus Six weeks later a Starr-Edwards ball valve was implanted. Ocular motility eleven weeks post stroke: •Complete recovery of downgaze •Convergence insufficiency •Persistent skew deviation Ocular motility two years prior to death: •Age related limitation of upgaze •Absent Bell phenomenon •Normal downgaze •Convergence insufficiency •Persistent skew deviation, right hypertropia The patient entered the hospital for the last time at age 63 with fever and right hip pain. Despite extensive evaluation and antibiotic therapy, he developed septic shock and died on the tenth hospital day. The etiology of the fever and hip pain was a right psoas abscess caused by perforation of the duodenum. Details of the last admission and general autopsy findings were the subject of a clinicopathological (CPC) report in the New England Journal of Medicine 1979;301:370-377. In preparation for the CPC John Trojanowski found very fortuitously a drawing of the midbrain that I had placed in the patient's record at the time of my first consultation. The drawing indicated the probable location of the midbrain infarct. As a result John retrieved the patient's brain from its place on a shelf and sections of the brainstem showed the localization of the midbrain infarct. This case should be reviewed alongside ID944-7. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/76