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Working out abnormal head postures

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

  2. Abnormal 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

  3. 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

  4. Eyes open Both eyes closed - HT persists • HT not related to visual activity! • Causes: Vestibular problem / ocular tilt reaction / tectal pathology/ neck problems Eyes closed

  5. Ocular tilt reactionThank 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?’

  6. Vertical diplopia head erect L IO UA R IO UA R hypertropia and exotropia RHT worse R gaze L SO OA R SO OA

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

  8. 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

  9. 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

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

  11. LF drives HT to L1. 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

  12. 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

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

  14. 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, …

  15. 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

  16. LF drives HT  L2. Orbital reason • Orbital scarring • Restrictive strabismus esp.... Graves’ • Motor reasons & • 2 Sensory reasons - acquired astigmatism from tight muscles

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

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

  19. 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

  20. 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’

  21. 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: • LF RF EE BE ?

  22. 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

  23. Alternating Face Turn2 causes1. 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

  24. Alternating Face Turn2. 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

  25. Alternating Face Turn2. 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]

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

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

  28. 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

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

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

  31. TIP DRIVEN BY BEO • Strab esp. alphabet patterns

  32. 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

  33. Working out head tilts & face turns