Saturday morning ‘Live’ patients

Lionel Kowal

#1: Sarah, DOB 1977• Head injury 2/2008. LOC 2 hours. • Had L ptosis for 2 months.• At 6 months became aware of poor upgaze L eye with upgaze diplopia.

Mum describes eye was ‘wandery’. • Glasses for myopic astigmatism antedate the head injury.• Takes several medicines for epilepsy and psychiatric issues.• cc 6/9-, N3 OU. A little better with extra -0.5 DSOU. • Diplopia begins 10 ° above horizon, and 45 ° into R gaze. • On upgaze has 10 L hypo. LSR UA.• On horizon, vertical fusion range BDRE 1 , BD LE <1 .• Through- the- lid- forced- duction- test: LIR not tight. • No retraction on upgaze. • When dilated, little / no fundus torsion, and upgaze = downgaze.

Normal alignment in primary Restricted elevation of Left eye

#1: Sarah, DOB 1977

• MRI

• Q1: Cause of upgaze deficit. • Q2: How to safely expand range of single

vision on upgaze.

#2: Mark, DOB 1968

• Diplopia since childhood. Head tilt to L since teens.

• Wore prism glasses aged 8-12. • Born @ 30w. In hospital for 3 months.

• Late 2006: bilateral cataract surgery. Was -9, -7 preop.

• Now R -1.5/-1.25… L -1/-1.25…. 6/9 OU• Head tilt to L 10 [probably longstanding]

#2: Mark, DOB 1968

• MRI: Symmetric increase in size of EOMs bilaterally ?Graves’.

• RLR pulley elevated.

• Normal Thyroid chemistry and antibodies

• Q1: why does he have diplopia?• Q2: how to fix his diplopia.

#3: Bailey, DOB 4/2001

• B has never been able to move his eyes laterally. • At 6 mo his head circumference started to increase. Skull

XR: plagiocephaly with L occipital flattening. Lambdoid sutures both patent… pediatric neurosurgeon monitored him regularly till age 6 without any treatment for ‘mild hydrocephalus’ and then discharged him.

• Told some years ago that he has scoliosis. • He is intellectually excellent.• 1st cousin has Duane’s. • Referring optometrist: intermit ET.

#3: Bailey, DOB 4/2001

sc R 6/6, N3. L 6/9, N12.• Horizontal versions: some Abduction.• Does have horizontal and vertical OKN.

Normal smile. • Straight. Stereo: Fly. Cyclo: R +1.5, L +3.5. • MRI: …normal.. in particular the brainstem has

a normal appearance

MAX RIGHT GAZE

LEFT GAZE

BAILEY cont.

•Q: why does he have a horizontal gaze anomaly

Neuroradiology. 2008 May;50(5):453-9. Functional MRI, DTI and neurophysiology in horizontal gaze palsy with progressive scoliosis. Haller S, ….., Basel, Switzerland.

Horizontal gaze palsy with progressive scoliosis (HGPPS) is an autosomal recessive disease due to a mutation in the ROBO3 gene. This rare disease is of particular interest because the absence, or at least reduction, of crossing of the ascending lemniscal and descending corticospinal tracts in the medulla predicts abnormal ipsilateral sensory and motor systems…………

#4: Joseph. Dob 25/10/1952

• Upper brainstem CVA in 3/07• He describes a downgaze palsy and large R hypo, recovered by the

time I first saw him 5 mo after CVA. • Was bothered for about a year with trouble judging position and

speed of objects when he was moving.• When first seen by me, he described vertical and torsional

diplopia suggestive of bilateral 4ths.Now bothered by:• Small range of single vision. Diplopia when he changes head

position or when he changes gaze, & • Convergence insufficiency• Low myope; cc 6/6+ OU

MAIN FEATURES: All measurements are small. RH on L gaze, Up L, and R tilt. LH on R gaze, Up R, and L tilt.Excyclo – some variability

#4: Joseph. Dob 25/10/1952

• Has subjective need for 0.5 -1 BD LE. • Computer glasses also have 2 BI, readers 5 BI. • Fusion ranges [through distance glasses] in real life: • #1 [LK]: H: 8 . V: < 1 .• #2 [RW]: H: -4 to +30 . V 1 . • Synoptophore: H: 0 to +35. V 1 [when H, V & T

angle corrected]. • Persisting upgaze paresis.• Stereo has varied from 40- 100”.

Oblique saccades demonstrate up gaze paresis

Joseph 1952

• Q: complex supra- infra- nuclear motor problem.

• Any way to improve his functional range of single vision?

• Could torsion surgery help? • Which torsion surgery?