Multiple Sclerosis

The patient is a 56 year old woman who presented in 1982, at the age of 48, with a one week history of painless loss of vision in the left eye. Past History: Negative for a previous attack of optic neuritis or transient neurological symptoms. Family History: Negative for CNS disease Neuro-ophthalmological examination: Visual acuity (VA) 20/25 OD, 20/200 OS Afferent pupil defect OS. Automated perimetry OS central scotoma breaking out into the superior altitudinal field. OD visual field normal. Fundus examination normal optic discs. Ocular motility: Full eye movements Normal convergence No nystagmus Neurological examination: No abnormality Visual Evoked Potential: Normal response OD Absent response OS. CT brain and orbits: Minimal thickening of the left optic nerve Normal brain images. Plain x-rays of the optic foramen: Normal asymmetry of the foramena. The left optic foramen measured 4 mm and the right 5 mm. in diameter. Both were round, well corticated with no destructive bone changes. Diagnosis: Retrobulbar optic neuritis OS Treatment: Prednisone was started with a loading dose of 100 mg daily, quickly tapered down to 40 mg daily. After one week VA 20/40 OS. She remained on Prednisone 40 mg daily for six weeks. On follow-up at that time VA OS 20/25, a left afferent pupil defect, normal visual field and optic discs. Eight years later, in 1990, she developed diffuclty focusing, unsteadiness walking, and numbness of the left side of her face from the bridge of the nose to the middle of the chin and the left side of her tongue and cheek. Taste was impaired. She was admitted to Massachusetts General Hospital for evaluation. Neuro-ophthalmological examination: VA 20/25, J1+ OU corrected. Visual fields, pupils, and fundus examination normal Corneal reflexes intact OU Ocular motility: •Transient spontaneous primary position upbeat nystagmus with lid nystagmus. •Rapid bursts of horizontal square wave oscillations opening her eyes to fix on a target. •Rotary nystagmus in both eyes on gaze right and left. Rotation of the globe was best seen by observing the conjunctival blood vessels. •Full upgaze with unsustained upbeat nystagmus. •Saccadic pursuit on horizontal and upgaze •Smooth pursuit down •Saccadic Dysmetria Hypermetria left gaze to center Neurological examination: •Sensation on the left side of the face (2nd and 3rd division left trigeminal nerve) decreased to light touch and pinprick •Decreased taste to salt and sugar, left anterior aspect of the tongue •Muscle strength normal •Generalized hyperreflexia •Flexor plantar responses •Ataxia heel-knee-shin •Ataxic gait •Sensation intact all modalities Brainstem auditory evoked potentials: Normal on the right side Mildly abnormal on the left side with an increased latency in waves 1-5. Visual evoked potentials: Normal P100 latency OD, 115 msec, amplitude 15.3 Delayed P100 latency OS, 125 msec, amplitude 4.5. Impression: Unilateral lesion in the left visual pathway anterior to the optic chiasm (consistent with a previous attack of optic neuritis OS). Lumbar puncture: Normal cerebrospinal fluid. Electronystagmogram: 1.Spontaneous and gaze nystagmus - right beating nystagmus 30 degrees right lateral gaze with eyes open in the light. Right beating nystagmus stopped on eye closure. Upbeating nystagmus on upgaze with a rotary component. 2.Optokinetic nystagmus - symmetrical and appropriate 3.Positional testing: a. Hallpikes/Eyes open in the light In the right ear down position transient well formed left beating nystagmus in the right eye. In the left eye a small poorly formed right beating nystagmus. Similar but persistent nystagmus seen on sitting up from the right ear down position. In the left ear down position, well formed right beating nystagmus in the left eye and no definite nystagmus in the right eye. The nystagmus persisted and was present on sitting up from the left ear down position. No associated dizziness with the above maneuvers. Impression: Abnormal electronystagmogram •Positional nystagmus without dizziness •Gaze evoked nystagmus with eyes open in the light that stopped with eye closure •A reduced left caloric response of 37% on caloric testing. Brain MRI: T2 WI showed extensive multifocal signal abnormalities involving the centrum semiovale, the posterior fossa and the left temporal lobe. Some foci showed Gadolinium enhancement and some tissue loss. There were no flow abnormalities within these areas and no hemorrhage. Impression: Multifocal white matter lesions consistent with demyelinating disease. Inspite of the prior history of retrobulbar neuritis and a positive brain MRI for multiple white matter lesions, her primary care physician had felt it was impossible to make a diagnosis of Multiple Sclerosis (MS). Diagnosis on discharge: Multiple Sclerosis. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/73