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Working out funny head postures LIONEL KOWAL RVEEH, CERA, Melbourne 2005 Abnormal Head Posture T3 Always 3 components to look for and explain: TILT - to L or R TURN - to L or R FT = face turn TIP - up or down HT = head tilt 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 Both eyes closed HT persists Eyes closed 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 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 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 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 = 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 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’. 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’ ‘Habit’, ‘psychological’, … after full investigation, these 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: LF RF EE BE ? 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 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 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 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] Working out head tilts & face turns Working out head tilts can be easy, difficult or near- impossible. It is always interesting! Thank you! Working out head tilts & face turns THANK YOU