Horner’s Syndrome

Scenarios: Face or neck pain, unequal pupils noted, different colours of the iris noted.

Features of Horner’s syndrome: 

 Partial ptosis: Affects both the upper and lower lid.

 **If you ever mention ptosis – say if it is bilateral or unilateral, complete or
partial, and whether or not it is fatiguable.
 Apparent enophthalmos: Due to the above.
 Miosis: Causing anisocoria. Ask for the lights to be turned off to make this effect
more marked as the contralateral pupil dilates – the affected pupil may dilate but often
slowly, a so called ‘dilation lag’.
 Anhidrosis and flushing:
 First order: Ipsilateral body.
 Second order: Ipsilateral face
 Third order: Absent or just above the brow.

If you’ve seen Horner’s syndrome: Mainly examine through inspection.

 General: Look for hemiparesis or mobility aids.

 Face: Focus in on the iris and look for heterochromia (congenital or very long
standing causes, affected iris is lighter). Check eye movements looking for associated
ophthalmoplegia, then track towards the neck looking for signs of scars of trauma.
 Neck: Look for any scars and palpate for lymphadenopathy, aneurysms and
thyroid nodules; check for tracheal deviation; auscultate for carotid bruits.
 Upper thorax: Any scars superiorly, or posteriorly on the back that could suggest
previous lobectomy; auscultate at the lung apex comparing with the normal side and
check vocal resonance.
 Ipsilateral arm: Wasting, fasiculations, claw hand.
 Neurological examination upper limbs: Motor, cerebellar and sensory.

Differential diagnosis:

 Congenital: Birth injury to sympathetic chain (Klumpke’s paralysis), hereditary.

 First order (from hypothalamus, to midbrain, to spinal cord): Trauma, stroke (e.g.:
Wallenberg syndrome), tumour (primary or secondary, benign or malignant),
demyelination (multiple sclerosis), syringomyelia. Due to the location these are unlikely
to present as isolated Horner’s – they would be associated with other neurological
signs.
 Second order (from spinal cord, exiting at C8/T1/T2, across top of lung, up into
the neck): Trauma, pancoast tumour, schwannoma.
 Third order (from neck up into the face): Trauma, carotid artery dissection,
cavernous sinus thrombosis or inflammation, cluster headaches, migraines.
 Investigations:

 Bedside tests: Review old photos (clarify time of onset).

 Bloods: Depends on underlying cause – e.g.: FBC, LFTs, bone profile for
malignancy if second order.
 Imaging: MRI brain (1st order –  ischaemia, demyelination, tumour), chest
radiograph and/or CT thorax (2nd order – Pancoast’s), MRA and/or carotid
doppler (2nd and 3rd order), MRI cavernous sinus and orbits (3rd order).
 Special tests: Apraclonidine test (confirms Horners’s syndrome – reversal of
anisocoria causing the affected pupil to dilate and sometimes the ptosis to
resolve), cocaine test (confirms Horner’s syndrome – exacerbates the anisocoria by
causing the normal pupil to dilate but not the affected one), hydroxyamphetamine
test (confirms site of lesion – normal dilation of the affected eye if 1st/2nd order, absent
or poor dilation of the affected eye if 3rd order).

Treatment:

 Non-pharmacological: Education.
 Medical: Treat underlying cause.
 Surgical: Treat underlying cause or for correction of ptosis.

Questions:

1. What is the mode of inheritance of hereditary Horner’s syndrome? Autosomal

dominant.
2. What is Klumpke’s paralysis? Usually caused by a birth injury that damages C8
and T1 causing weakness and anaesthesia in these areas with clawing of the hand.
3. What is Wallenberg syndrome? Posterior inferior cerebellar artery infarct
encompassing dysphagia, sensory loss ipsilateral face and contralateral trunk and
extremities, ipsilateral cerebellar ataxia, and rotatory nystagmus. It is also known as the
lateral medullary syndrome. Ipsilateral facial sensation is affected here because of
damage to the trigeminal spinal nucleus which receives sensory information.
4. If the patient describes neck or face pain with the onset of their Horner’s, what
diagnosis would you suspect? They should be considered to have a carotid artery
dissection until proven otherwise, and investigated with urgent CT or MR angiography.