Right preganglionic Horner syndrome

Central (first-order neurone)

brain stem disease (tumours. vascular. demyelination)
syringomyelia
lateral medullary (Wallenberg) syndrome
spinal cord tumours
1. Preganglionic (second-order neurone)
Pancoast tumour
carotid and aortic aneurysms and dissection
neck lesions (glands. trauma. postsurgical)
J. Postganglionic (third~order neurone)
duster headaches (migrainous neuralgia)
internal carotid artery dissection
nasopharyngeal tumours
otitis media
cavernous sinus mass

Mild ptosis (usually 1-2 mm) as a result of weakness of

Miillermuscle (Fig. 18.67).
Slight elevation of the inferior eyelid as a result of weakness of the inferior
tarsal muscle.
Miosis resnlting from the unopposed acUon of the
sphincter pupillae. with resultant anisocoria which is
accentnated in dim light. since the Horner pupil will not
dUate. like its fellow.
i\ormal reactions to light and near

Reduced ipsilateral sweating, but only if thc lesion is

below the superior cervical ganglion because the fibres
~upplying the skin of the face run along the cxternal
carotid artery.
Hypochromic heterochromia (irides of different colourHorner is lighter) may
be seen if the lesion is congenital or
long-standing.
The pupil is slow to dilate.
Less important signs include hyperactive accommodation.
ocular hypotony and conjunctival hyperaemia

Horner's syndrome is caused by a lesion of the sympathetic pathway either (1) in

its central portion, which extends from the posterior hypothalamus through the
brainstem to the upper spinal cord (C8T2); or (2) in its preganglionic portion,
which exits the spinal cord and synapses in the superior cervical (stellate)
ganglion; or (3) in its postganglionic portion, from the superior cervical ganglion
via the carotid plexus and the ophthalmic division of the trigeminal nerve, by
which it enters the orbit. The sympathetic fibers then follow the nasociliary branch
of the ophthalmic division of the trigeminal nerve and the long ciliary nerves to
the iris and innervate Mller's muscle and the iris dilator. Iris dilator muscle
paresis causes miosis, which is more evident in dim light. Melanocyte maturation
in the iris of a neonate depends on sympathetic innervation; thus, less pigmented
(bluer) irides occur if a congenital sympathetic lesion is present. Paresis of
Mller's muscle produces ptosis. Unilateral miosis, ptosis, and absence of
sweating on the ipsilateral face and neck make up the complete syndrome.
Sweating on the face is normal in postganglionic lesions because postganglionic
fibers to the face for sweating follow the external rather than the internal carotid
artery.
Central Horner's syndrome may be due to brainstem infarction, particularly lateral
medullary infarction (Wallenberg's syndrome), syringomyelia, or cervical cord
tumor. Preganglionic Horner's syndrome may be due to cervical rib, cervical
vertebral fractures, apical pulmonary lesionsparticularly bronchogenic
carcinoma (Pancoast's syndrome)or brachial plexus injuries. Postganglionic
Horner's syndrome may be due to carotid artery dissection, skull base tumors, or
cluster headache. The localization of central and preganglionic Horner's syndrome
is often apparent from the associated clinical features. Sudden-onset isolated
painful Horner's syndrome, particularly with a recent history of neck trauma or
associated with pain in the neck or jaw, necessitates urgent investigation for
carotid dissection, which may lead to thrombotic or embolic stroke. Horner's
syndrome associated with chronic facial pain, particularly if associated with fifth,
sixth, third, fourth, or second cranial nerve palsy, requires investigation for skullbase tumor.

Pharmacologic testing with topical cocaine in the conjunctival sac differentiates

Horner's syndrome, in which the pupil does not dilate, from physiologic
anisocoria. Topical apraclonidine, which causes dilation of the affected but not the
normal pupil, can also be used. Testing with hydroxyamphetamine drops
differentiates central and preganglionic from postganglionic lesions, but they are
difficult to obtain.

Figure 24.15. Appearance of Horner syndrome in four patients. A, Congenital

right Horner syndrome. Note associated heterochromia iridis and minimal ptosis.
B, Left Horner syndrome after neck trauma. C, Left Horner syndrome associated
with apical lung (Pancoast) tumor. D, Left Horner syndrome in a patient with
Raeder's paratrigeminal neuralgia.