Working out abnormal Working out abnormal head postureshead postures

FUSION 2012 LVPEI HYDERABAD

LIONEL KOWALMelbourne

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 problemsEyes 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 SyndromeH ± T LMLN are frequent [?universal]

associations of cong ETCiancia’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 L2. 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!

RF RF Head Tilt to L Head Tilt to L

Problem with R orbit

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]

AstigmatismAstigmatismWrong 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 paresisUp- / 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

TIP DRIVEN BY BEOTIP DRIVEN BY BEO

Strab esp. alphabet patterns

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

Working out head tilts & Working out head tilts & face turnsface turns