Working out abnormal head postures FUSION 2012 LVPEI HYDERABAD LIONEL KOWAL Melbourne

Abnormal Head Posture T Abnormal Head Posture T 3 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: Q1: Is HT driven by 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 - HT persists HT not related to visual activity! Causes: Vestibular problem / ocular tilt reaction / tectal pathology/ neck problems Eyes closed Eyes open

Ocular tilt reaction 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 head erect R hypertropia and exotropia RHT worse R gaze L IO UA L SO OA R IO UA R SO OA

Head supine Assessment of vertical deviation with head supine Single vision with no deviation when head supine

BE 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 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: Head tilt to fixing eye LF drives HT to L RF : no HT 2 causes: 1. Torsional LMLN 2. L Orbital reasons

LF drives HT to L 1. 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 Torsional LMLN to improve 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: Alternating Head Tilt LF drives L tilt RF drives R tilt = Ciancia’s syndrome

Ciancia’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 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  L 2. Orbital reason Orbital scarring Restrictive strabismus esp.... Graves’ Motor reasons & 2  Sensory reasons - acquired astigmatism from tight muscles

HT driven by binocularity RF = LF = no HT Strabismus the cause Tilt R and do a cover test to discover the cause!

RF  Head Tilt to L Problem with R orbit

Still 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 tilt Check again : when a human being examines another, signs not always ‘perfect’ or consistent Ask for serial photographs of HT ‘Habit’, ‘psychological’, … after full investigation are synonyms for ‘HT due to an unknown non sinister & non- treatable cause’

Face 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: LFRFEEBE?

Face 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 Turn 2 causes 1. Ciancia’s syndrome LF : L FT RF : R FT Ciancia’s syndrome: preference for fixation in adduction because recruiting medial rectus ‘brakes’ horizontal component of LMLN  improved vision

Alternating Face Turn 2. 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 Turn 2. 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]

Astigmatism Wrong cyl axis can  HT Uncorrected astigmatism : pt uses corner of palpebral fissure as ‘pinhole’  FT

TIP UP / DOWN Same principles as HT / FT : what drives the Tip? RF, LF, EE, BEO Some different diseases cause Tips LMLN not involved

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 CN [usu H, rarely V] with vertical null see Delmonte CFEOM if bilateral / symmetric [looks like restrictive strabismus]

TIP driven by one eye fixing This is due to orbital reasons, typically a tight or deficient muscle

TIP DRIVEN BY BEO Strab esp. alphabet patterns

Is this the same pt? – it’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

Working out head tilts & face turns