working out abnormal head postures fusion 2012 lvpei hyderabad lionel kowal melbourne
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Working out abnormal Working out abnormal head postureshead postures
FUSION 2012 LVPEI HYDERABAD
LIONEL KOWAL
Melbourne
Abnormal Head Posture TAbnormal Head Posture T3
Always 3 components to look for and explain:
TILT - to L or R HT = head tilt
TURN - to L or R FT = face turn
TIP - up or down
Thank you Annette Spielman
TILTS:TILTS:Q1: Is HT driven by Q1: Is HT driven by visual activity?visual activity?
Instruction to patient with head tilt: Close your eyes and hold your head
straight.
#2: pt closes eyes, Dr tilts head randomly, pt asked to straighten head
Thank you Marc Gobin
Both eyes closed - Both eyes closed - HT persistsHT persists
HT not related to visual activity!
Causes: Vestibular problem / ocular tilt reaction / tectal pathology/ neck problems
Eyes closed
Eyes open
Ocular tilt reactionOcular tilt reactionThank you Agnes Wong, Avi Safran Thank you Agnes Wong, Avi Safran
1. Head tilt & effect on diplopia ‘don’t make sense’. HT is not therapeutic.
2. Diplopia disappears when head tilted back / pt lies flat.
New Q: ‘is it double on the ceiling when you wake up?’
Vertical diplopia Vertical diplopia head erect head erect
R hypertropia and exotropiaRHT worse R gaze
L IO UA
L SO OA
R IO UA
R SO OA
Head supineHead supine
Assessment of vertical deviation with head supine
Single vision with no deviation when head
supine
BE closed - HT goesBE closed - HT goes
HT driven by visual activity
Now determine: Is HT driven by– Right eye fixing RF– Left eye fixing LF– Either eye fixing EE– Only when both eyes are fixing BE
Either eye drives HTEither eye drives HT
Congenital nystagmus CN with oblique null
CN: the cong nystag seen with sensory developmental disorders - OCA, CSNB, ONHypo, …
Look for other features of CN - horizontal jerk nystagmus, convergence null, recordings, …
Von Noorden, De Decker or Sousa Dias for treatment guidelines
Special case:Special case:Head tilt to fixing eyeHead tilt to fixing eye
LF drives HT to LRF : no HT
2 causes: 1. Torsional LMLN2. L Orbital reasons
LF drives HT to LLF drives HT to L1. Torsional LMLN1. Torsional LMLN
LMLN is the cong nystag seen with disorders of binocular development
[?always] Seen in cong ET= Fusion Maldevelopment N Syndrome. Usually has H component, 25% also T
Fine torsional N often seen on slit lamp
N degrades vision - vision improves when N blocked
1. How to block 1. How to block Torsional LMLN to Torsional LMLN to improve visionimprove vision
HT to fixing eye recruits Sup Obl which acts as a ‘brake’ on [& produces a null for] T component of the LMLN. Braking T LMLN better vision
Looks like: Preference for fixation in intorsion
HT usually ‘driven’ by the dominant eye but can be the ‘wrong’ eye The same mechanism is part of the causation of contra lateral DVD - see
Guyton
Special case:Special case:Alternating Head TiltAlternating Head Tilt
LF drives L tiltRF drives R tilt
= Ciancia’s syndrome
Ciancia’s SyndromeCiancia’s Syndrome
H ± T LMLN are frequent [?universal] associations of cong ET
Ciancia’s S: = ‘Regular’ cong ET where the consequences of T & H LMLN are a prominent part of the clinical picture [in addition to the ET]
Consequences: head tilts, face turns, DVD, DHD, ……
Associations: PVL, Downs’, after IVH / H-ceph, …
Ciancia’s SyndromeCiancia’s Syndrome
Head tilt / face turn recruits a muscle to block the T / H component of LMLN improves vision
T: HT to fixing eye - recruits Sup Obl to ‘brake’ T LMLN
H: FT to fixing eye - recruits Medial Rectus to ‘brake’ H LMLN
LF drives HT LF drives HT L L
2. Orbital reason2. Orbital reason
Orbital scarring Restrictive strabismus esp.... Graves’
Motor reasons & 2 Sensory reasons - acquired
astigmatism from tight muscles
HT driven by binocularityHT driven by binocularity
RF = LF = no HT
Strabismus the cause
Tilt R and do a cover test to discover the cause!
Still can’t explain the head tiltStill can’t explain the head tilt
Spasmus Nutans - always has monocular N - can be difficult to see - can look like ‘shimmering’.
SN doesn’t improve with age but child might learn to avoid it e.g. one particular AHP may minimize the N – tilt the ‘other’ way to see it
No explanation : Low threshold for imaging
Still can’t explain the head tiltStill can’t explain the head tilt
Check again : when a human being examines another, signs not always ‘perfect’ or consistent
Ask for serial photographs of HT
‘Habit’, ‘psychological’, … after fullinvestigation are synonyms for ‘HT due to an unknown non sinister & non-
treatable cause’
Face Turn - LFace Turn - L
Approach the same way as tilt - a few differences
Is the FT visually driven: “Close your eyes and hold your head straight”
If it’s visually driven, is it driven by:LF RF EE BE ?
Face Turn - LeftFace Turn - Left
If driven by: LF : Fixation- in- adduction for horizontal
LMLN or L orbital problem RF : R orbital problem EE : cong nystagmus BE : strabismus
Alternating Face TurnAlternating Face Turn2 causes2 causes1. Ciancia’s syndrome1. Ciancia’s syndrome
LF : L FTRF : R FT Ciancia’s syndrome: preference for
fixation in adduction because recruiting medial rectus ‘brakes’ horizontal component of LMLN improved vision
Alternating Face TurnAlternating Face Turn2. Periodic alternating nystagmus2. Periodic alternating nystagmus
‘Regular’ CN with 2 H null zones Much more frequent than
suspected esp..... albinism
CAREFUL Family Album Test : ANY photos showing FT R suggest PAN
Alternating Face TurnAlternating Face Turn2. Periodic alternating nystagmus2. Periodic alternating nystagmus
Usually asymmetric periodicity = ‘aperiodic’ say, 90% FT L, 10% FT R
Prolonged in- office exam
RARE VARIANT: Periodic Alternating Gaze Deviation –
like the slow- phase- only of PAN [also aperiodic]
AstigmatismAstigmatism
Wrong cyl axis can HT
Uncorrected astigmatism : pt uses corner of palpebral fissure as ‘pinhole’ FT
TIP UP / DOWNTIP UP / DOWN
Same principles as HT / FT : what drives the Tip? RF, LF, EE, BEO
Some different diseases cause TipsLMLN not involved
TIP :’Driven’ by Either Eye TIP :’Driven’ by Either Eye
Supranuclear vertical gaze paresis
Up- / down- gaze, or both variable causes and expectations
Spino Cerebellar Atrophy [SCAs] –
acquired null for acq Downbeat N
TIP : Driven by Either Eye TIP : Driven by Either Eye
CN [usu H, rarely V] with vertical null see Delmonte
CFEOM if bilateral / symmetric [looks like restrictive strabismus]
TIP driven by one eye fixingTIP driven by one eye fixing
This is due to orbital reasons, typically a tight or deficient muscle
Is this the same pt? – Is this the same pt? – it’s all different todayit’s all different today
As well as PAN CN can have 2 or 3 different null zones e.g. FT and Tip and convergence are all effective, and one is typically preferred.
Fixing one can ‘release’ another.
You miss more by not looking than by not knowing