1. Working out funny head postures LIONEL KOWAL RVEEH, CERA, Melbourne 2005

2. 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

3. TILTS: Q1: Is HT driven by visual activity? Instruction to patient: Close your eyes and hold your head straight. Uncertain response: pt closes eyes, Dr tilts head randomly, pt asked to straighten head

4. Both eyes closed - HT persists HT not related to visual activity! Causes: Vestibular problem / ocular tilt reaction / tectal pathology/ neck problems Have seen ‘dysplastic’ vermis as a cause of HT beginning age 6 mo Eyes closed

5. 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

6. Either eye drives HT Congenital nystagmus with oblique null Look for other features of CN - horizontal jerk nystagmus, convergence null, recordings, … CN: the cong nystag seen with sensory developmental disorders - OCA, CSNB, ONHypo, … De Decker or Sousa Dias for treatment guidelines Sub clinical ‘micronystagmus’ only detectable by eye movement recordings has been described - I haven’t seen it

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

8. LF drives HT to L 1. Torsional LMLN LMLN is the cong nystag seen with disorders of binocular development [?always] Seen in cong ET = Fixation Maldevelopment N. Usually has H component, sometimes T as well Fine torsional N on slit lamp N degrades vision - vision improves when N blocked

9. 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

10. Special case: Alternating Head Tilt LF drives L tilt RF drives R tilt = Ciancia’s syndrome

11. 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, …

12. 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

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

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

15. RF  Head Tilt to L Problem with R orbit

16. Still can’t explain the head tilt Spasmus nutans - always has monocular N - can be difficult to see - can look like ‘shimmering’. No explanation : Low threshold for imaging

17. Still can’t explain the head tilt Check again : when a human being examines another, signs not always ‘perfect’ ‘Habit’, ‘psychological’, … after full investigation, these are synonyms for ‘HT due to an unknown non sinister & non- treatable cause’

18. 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?

19. 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

20. 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

21. 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

22. Alternating Face Turn 2. Periodic alternating nystagmus Usually asymmetric periodicity = ‘aperiodic’ say, 90% FT  L, 10% FT  R Prolonged in- office exam

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

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

25. TIP :’Driven’ by Either Eye Supranuclear vertical gaze paresis variable causes and expectations Spino Cerebellar Atrophy [SCAs] - acquired null for acq Downbeat N

26. TIP : Driven by Either Eye CN [usu H, rarely V] with vertical null see Delmonte CFEOM if bilateral / symmetric [looks like restrictive strabismus]

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

28. TIP DRIVEN BY BEO Strab esp. alphabet patterns

29. Variable HT/ FT/ Tip CN can have different null zones e.g. FT and Tip both effective. Fixing one can ‘release’ another. Null zones in CN not always ‘hard wired’ - can vary with time [rare] and during the one examination [very rare]

30. Working out head tilts & face turns Working out head tilts can be easy, difficult or near- impossible. It is always interesting! Thank you!

31. Working out head tilts & face turns THANK YOU