Transcript Working out head tilts & face turns

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
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