Horner's syndrome

1. Horner's syndrome
Presenter : Dr Rahul Achlerkar
Moderator : Dr Atul Seth

2. Introduction
 The term Horner syndrome is commonly used in English-
speaking countries, whereas the term Bernard-Horner
syndrome is common in France
 Horner syndrome (Horner’s syndrome) results from an
interruption of the sympathetic nerve supply to the eye

3. Horner’s
 Characterized by the
classic triad of
 Miosis (constricted pupil)
 Partial ptosis
 Loss of hemifacial sweating
( anhidrosis).

4. Neuroanatomy
Sympathetic innervation to the eye consists of a 3-neuron arc.
 First-order central sympathetic fibers
 Second-order preganglionic pupillomotor fibers
 The third-order post ganglionic pupillomotor fibers

5. First-order central sympathetic fibers
Arise from the
posterolateral
hypothalamus
Descend uncrossed
through the midbrain
and pons
Terminate in the cell column
of the spinal cord at the
level of C8-T2 (ciliospinal
center of Budge).

6. Applied Anatomy
First-order neuron lesions
 Cerebral vascular accident
(CVA)/Wallenberg
syndrome
 Demyelinating disease
 (eg, multiple sclerosis)
 Arnold-Chiari malformation
 Basal meningitis
 (eg, syphilis)
 Basal skull tumors

9. First-order neuron lesions
 Hemisensory loss
 Dysarthria
 Dysphagia
 Ataxia
 Vertigo
 Nystagmus

10. Second-order preganglionic pupillomotor fibers
Exit spinal cord at
the level of T1 and
enter the cervical
sympathetic chain
They are in close
proximity to the
pulmonary apex and
the subclavian artery.
The fibers ascend through
the sympathetic chain and
synapse in the superior
cervical ganglion at the
level of the bifurcation of
the common carotid artery
(C3-C4).

11. Applied Anatomy
 Pancoast tumor
 tumor in the apex of the
lung, most commonly
squamous cell carcinoma
 Birth trauma with injury to
lower brachial plexus
 Cervical rib

12. Applied Anatomy
 Aneurysm or dissection of
the aorta
 Lesions of the subclavian or
common carotid artery
 Neuroblastoma
 Lymphadenopathy
 eg, Hodgkin disease,
leukemia, tuberculosis, or
mediastinal tumors

16. Second-order neuron lesions
 Prior trauma
 facial, neck, axillary, shoulder or arm pain
 Cough
 Hemoptysis
 Previous thoracic or neck surgery
 Previous chest tube or central venous catheter placement; or
neck swelling

17. The third-order pupillomotor fibers
Postganglionic
pupillomotor fibers exit
the superior cervical
ganglion and ascend
along the internal carotid
artery
Shortly after the
postganglionic fibers leave
the superior cervical
ganglion, vasomotor
branch off
It Travels along the
external carotid artery
to innervate the blood
vessels and sweat
glands of the face.

18. The third-order pupillomotor fibers
Ascending along the internal
carotid artery enter the
cavernous sinus
The fibers then leave the carotid
plexus briefly to join the
abducens nerve in the cavernous
sinus
It enter the orbit through the
superior orbital fissure along
with the ophthalmic branch of
the trigeminal nerve via the long
ciliary nerves.
The long ciliary nerves then
innervate the iris dilator and the
Müller muscle

19. Third-order neuron lesions
 Internal carotid artery
dissection
 associated with sudden
ipsilateral face or neck pain
 Raeder syndrome
(paratrigeminal syndrome)
 Carotid cavernous fistula
 Cluster or migraine headache
 Herpes zoster

20. Raeder’s syndrome
 Horner’s with pain in the
distribution area of V1.
 Caused by a neoplasm
compressing the trigeminal
nerve.
 Differential for cluster
headaches.

25. Third-order neuron lesions
 Diplopia from sixth nerve palsy
 Numbness in the distribution of the first or second
division of the trigeminal nerve and pain

26. Studies

27. Etiology
 Horner syndrome can be congenital, acquired, or purely
hereditary (autosomal dominant)

28. The interruption of the sympathetic fibers may
occur
Centrally
between the hypothalamus and the
fibers’ point of exit from the spinal
cord C8 to T2
Peripherally
in cervical sympathetic chain, at
the superior cervical ganglion, or
along the carotid artery

29.  Drugs that may cause symptoms similar to Horner syndrome
include the following:
 Acetophenazine
 Bupivacaine
 Butaperazine
 Chloroprocaine
 Chlorpromazine
 Fluphenazine
 Guanethidine
 Influenza virus vaccine
 Levodopa

30. Clinical Presentation
 Patient history
 Obtaining a careful history is very helpful in the localization of
lesions causing Horner syndrome.
 The symptoms reported by the patient will depend on the site
of lesion

40. differential diagnosis
 Anisocoria
 Adie pupil
 Argyll Robertson pupil
 Holmes-Adie pupil (contralateral)
 Iris sphincter muscle damage
 Senile miosis
 Third nerve palsy
 Unilateral use of miotic drugs
 Unilateral use of mydriatic drugs

41. Anisocoria
Pupillary inequality greatest
In bright light
(large pupil)
In dim light
(small pupil)
3rd nerve palsy
Trauma
Tumor
Temporal lobe herniation
Aneurysm
No 3rd nerve palsy
Drug induced
Adie’s pupil
Iris damage (trauma/surgery/laser)
Basal meningitis
Ptosis
Horner syndrome
Physiological

42. Testing
 Which is the abnormal pupil
 Compare in light and dark.
 Direct and consensual
response
 Is accomodation affected?

43. Light Reflex

44. Normal pupil reaction
 video

45.  Argyll-Robertson
pupil
 Small, irreg
 Does not react to light
 Reacts to
accommodation
 Causes
 syphilis
 diabetes
 Miotonic pupil
(Adie’s syndrome)
 Dilated
 Poor response to light and
convergence.
 Constricts with weak
Pilocarpine
 Holmes-Adie syndrome
 Reduced tendon reflexes
(Knee, ankle)
- Orthostatic hypotension
Afferent & efferent defects

46. Adie’s tonic pupil (OD)

47. Argyll-Robertson pupil

48. Horner’s pupil (OS)

49. Pharmacologic Testing
 The pharmacologic tests document the presence or absence of
an ocular sympathetic lesion and identify the level of
involvement (ie, preganglionic or postganglionic)
 Localizing the lesion is important because preganglionic
lesions are associated with a higher incidence of malignancy
that necessitates extensive investigations.

50. Topical cocaine test
 The basis for the topical cocaine test is the ability of cocaine to
act as an indirect sympathomimetic agent by inhibiting the
reuptake of norepinephrine from the synaptic cleft at the nerve
ending

51. Procedure
 The test is performed by instilling cocaine solution (2-4% ) into
each eye.
 Cocaine instilled in an eye with intact sympathetic innervation
causes the pupil to dilate.
 A sympathetically denervated pupil ( in Horner syndrome)
dilates poorly to cocaine, regardless of the level of the
sympathetic interruption, because of the absence of
endogenous norepinephrine in the synapse.

52.  For optimal accuracy, test results should be evaluated 30
minutes or longer after cocaine is administered.
 The maximal response is seen 40-60 minutes after instillation
of the drops.
 Postcocaine anisocoria greater than 0.8 mm is sufficient to
diagnose Horner syndrome.

53. Disadvantages
 The drops are difficult to obtain because they must be made at
a compounding pharmacy
 The drops are relatively expensive
 The test can yield equivocal results
 Cocaine metabolites may be detected in urine

54. Topical apraclonidine test
 The topical apraclonidine test is a practical and reliable
alternative to the topical cocaine test
 It is readily available and adequately sensitive (87%) and is
currently the test of choice.

55. Apraclonidine
 It is
 an ocular hypotensive agent
 weak alpha1-agonist
 strong alpha2-agonist
 Typically given in a 0.5% or 1% solution
 It has little to no effect on a normal pupil but has a
mydriatic effect on an abnormal pupil

56.  In Horner syndrome, upregulation of alpha1-receptors
increases apraclonidine sensitivity and causes denervation
supersensitivity of the iris dilator muscle.
 The denervation supersensitivity results in pupillary dilatation
and lid elevation on the abnormal side but no response or mild
miosis on the normal side from alpha2-activity after
apraclonidine administration.
 Reversal of anisocoria occurs after bilateral instillation of
apraclonidine.

57.  In acute cases, false-negative test results may occur because the
alpha1-receptor upregulation on which the effect of
apraclonidine depends may take 5-8 days.
 A negative apraclonidine test result especially in acute
settings does not exclude Horner syndrome.
 In such cases, a cocaine test should be performed to exclude
Horner syndrome.

59. Side Effects
 Apraclonidine 0.5% or 1% may cause
 Lethargy
 Bradycardia
 respiratory depression in infants , younger than 6 months
 Because of the immaturity of the blood-brain barrier.

61.  VIDEO

62. Topical hydroxyamphetamine test
 The localization of a lesion causing Horner syndrome may be
aided by the use of the topical hydroxyamphetamine test.
 Hydroxyamphetamine stimulates the release of stored
endogenous norepinephrine from the postganglionic axon
terminals into the neuromuscular junction at the iris dilator
muscles.

63.  This test may distinguish a postganglionic third-order neuron
lesion from a presynaptic second-order or first-order neuron
lesion.
 To perform the test, 2 drops of 1% hydroxyamphetamine
solution are instilled into each eye.
 A period of 24-48 hours must be allowed to elapse between
the cocaine test and the hydroxyamphetamine test because
cocaine has the ability to inhibit the uptake of
hydroxyamphetamine into the presynaptic vesicles,

64.  Hydroxyamphetamine drops instilled into an eye with Horner
syndrome with intact postganglionic fibers (ie, first- or second-
order neuron lesions) dilate the affected pupil to an equal or
greater extent than they do the normal pupil.
 However, hydroxyamphetamine drops instilled into an eye with
Horner syndrome with damaged postganglionic fibers (ie,
third-order neuron lesions) do not dilate the affected pupil as
well as they do the normal pupil.

65. Treatment & Management
 In general, appropriate treatment of Horner
syndrome depends on the underlying cause.
 The goal of treatment is to eradicate the underlying
disease process.
 In many cases, however, no effective treatment is
known.
 Prompt recognition of the syndrome and expedient
referral to appropriate specialists are vital.