What is chronic progressive external ophthalmoplegia
Progressive failure of eye movements
Mutations in mitochondrial DNA
Bilateral ptosis, reduced smooth pursuits/saccades/reflex eye mvoements (downgaze affected last), weakness of orbicularis oculi
What is Kearns Sayre syndrome?
CPEO
Onset before 20
CPEO
Pigmentary retinopathy
CVS conduction defects
Elevated protein in CSF
Cerebellar ataxia
Muscle biopsy shows red ragged fibers
What is BRown syndrome
Restriction from trochlear superior oblique complex leading to limitation of elevation of eye, maximal in adducted position
When affected eye cannot move up it moves out
In upgaze, Brown syndrome patient gets a divergence pattern causing a v pattern
No superior oblique over or under action
Forced duction test postiive
No torsion
Negative head tilt test
What pattern strabismus in inferior oblique palsy?
A patters as there is superior oblique over action
Intorsion
Positive head tilt
Steps of Parks Bielschowsky test
- Hypertropic eye
- in RE hypertropia - right SO or right IR weakness or left IO/left SR casing lower left eye - Hypertropic worse in left or right gazeRight hypertropia worsens in left gaze - right SO or left SR invovled (lack of right depression in adductiond due to SO weakness, or lack of LE elevation in abduction due to SR weakness.
- Hypertropia worse with left or right head tile
Hypertropia worse with ipsilateral tilt - ipsilateral SO.
In ipsilateral tilted position, ipsilateral SR will compensate for weak intorsion of ipsilateral SO but will also elevate at the same time and worsen hypertropipa
Compensatory head tilt to contralateral side will improve diplopia
What is Duane’s syndrome
Aberrant co-innervation of LR and MR by CN3 which may be associated with CN6 nucleus hypoplasia
Attempted adduction leads to:
Retrauction of globe, reduction of palpebral aperture
Up/downshift
What are systemic associations of Duane’s
Deafness
Goldnenhar sydrome
Klippel Feil
Wilderwank
What are types of Duane’s
Type 1 - D 85%
Reduced abduction
Primary eso or ortho
Mild globe retraction
Type 2 - DD 14%
Reduced adduction
Primary exo or ortho
Severe globe retraction
Type 3 - DDD 1%
Reduced abduction and adduction
Primary eso or ortho
Moderate globe retraction
What is the management for simulated divergence excess XT
(Deviation at distance >= 10PD than at near. After monocular occlusion near deviation increased to <10PD of the angle at distance)
Medial rectus resection and lateral rectus recession
If true divergence excess XT what is best treatment option
Deviation at distance >= 10PD than at near
Bilateral lateral rectus recession
