Working out abnormal
head postures
FUSION 2012
LVPEI
HYDERABAD
LIONEL KOWAL
Melbourne
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
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
Eyes open
Both eyes closed HT persists
Eyes closed

HT not related to visual activity!

Causes: Vestibular problem /
ocular tilt reaction / tectal
pathology/ neck problems
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
L IO UA
RHT worse R gaze
L SO OA
R IO UA
R hypertropia and
exotropia
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 & nontreatable 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

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
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.

As well as PAN
You miss more by not looking than by not knowing
Working out head tilts &
face turns