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Horner’s Syndrome - 
Etiology and Localization 
Yazeed Alwelaie 
PGY1
Introduction
Horner’s Syndrome 
• Also called oculo-sympathetic paresis 
• Classic triad: 
– Ptosis 
– Miosis 
– Anhidrosis
Horner's syndrome can be produced by a 
lesion anywhere along the sympathetic 
pathway that supplies the head, eye, and 
n...
Neuroanatomy
Three – neuron pathway 
Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 
2000. C...
First – Order Neuron 
Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 
2000. Cop...
Second – Order Neuron 
Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 
2000. Co...
Third – Order Neuron 
Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 
2000. Cop...
Third – Order Neuron 
Innervates the iris dilator 
muscles & Müller's 
muscle 
Neuro-ophthalmology: the requisites in opht...
Clinical Features
©2014 UpToDate® 
Anisocoria
©2014 UpToDate®
Ptosis 
• Ptosis is minor, usually less than 2 mm 
• Paralysis of the Müller's muscle, which is 
innervated by the sympath...
Anhidrosis 
• Anhidrosis is present in central or preganglionic 
(first or second-order) lesions 
• This sign is frequentl...
Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 
2000. Copyright ©2000 Elsevier.
Horner’s in Children 
• Impaired facial flushing (Harlequin sign) is often 
more apparent than anhidrosis 
• Acute feature...
Harlequin sign 
Source: http://picornot.com/keyword/harlequin+sign
Congenital Horner's Syndrome
Localizing/Associated 
Symptoms 
• Diplopia, vertigo, ataxia, lateralized weakness 
suggest a brainstem localization 
• Bi...
• Ipsilateral extraocular pareses, particularly a sixth 
nerve palsy, in the absence of other brainstem signs 
localize th...
Etiology
First-order syndrome 
Lesions of the sympathetic 
tracts in the brainstem or 
cervicothoracic spinal cord 
can produce a f...
Lateral Medullary Infarction
• Strokes, tumors, and demyelinating lesions affecting 
the sympathetic tracts in the hypothalamus, 
midbrain, pons, medul...
Second-order syndrome 
Second-order or 
preganglionic Horner's 
syndromes can occur with 
trauma or surgery involving 
the...
• Lumbar epidural anesthesia can also produce a 
Horner's syndrome. This is most often described in 
association with obst...
Third-order syndrome 
Third-order Horner's 
syndromes often indicate 
lesions of the internal 
carotid artery such as an 
...
Carotid Dissection 
An acute Horner's syndrome with neck or facial 
pain should be presumed to be caused by carotid 
disse...
Diagnosis
Pharmacologic Testing 
• Pharmacologic tests can be useful to confirm the 
diagnosis and to localize the lesion 
• Two age...
Confirmation of Horner’s 
Syndrome 
• Pharmacological testing with cocaine 
or apraclonidine drops can confirm the diagnos...
Cocaine 
• Blocks the reuptake of norepinephrine at the 
sympathetic nerve synapse 
• Intact pathway – dilates pupil. No e...
Apraclonidine 
• Alternative to cocaine 
• Direct alpha-adrenergic receptor agonist. 
Apraclonidine has weak alpha-1 and s...
From: Ocular Effects of Apraclonidine in Horner Syndrome 
Arch Ophthalmol. 2000;118(7):951-954. doi:10-1001/pubs.Ophthalmo...
Localization of the Lesion 
• First-order neuron 
(brainstem or cervical cord) 
• Second-order neuron 
(chest or neck) 
• ...
Hydroxyamphetamine 
• It releases stored norepinephrine from the 
postganglionic adrenergic nerve endings 
• A normal pupi...
Postganglionic right 
Horner's syndrome 
©2014 UpToDate®
Summary
• Classic signs of a Horner's syndrome include 
miosis, ptosis, and anhidrosis. 
• The miosis is typically mild, associate...
• The common etiologies of Horner's syndrome are 
categorized by which of the three neurons is affected. 
• The differenti...
• The presence of a Horner's syndrome can be confirmed 
pharmacologically with either cocaine or apraclonidine 
eye drops ...
• In the absence of a clear history of trauma as the 
cause of Horner's syndrome, imaging studies will be 
required.
Horner's syndrome
Horner's syndrome
Horner's syndrome
Horner's syndrome
Horner's syndrome
Horner's syndrome
Horner's syndrome
Horner's syndrome
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Horner's syndrome

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Horner's syndrome

  1. 1. Horner’s Syndrome - Etiology and Localization Yazeed Alwelaie PGY1
  2. 2. Introduction
  3. 3. Horner’s Syndrome • Also called oculo-sympathetic paresis • Classic triad: – Ptosis – Miosis – Anhidrosis
  4. 4. Horner's syndrome can be produced by a lesion anywhere along the sympathetic pathway that supplies the head, eye, and neck.
  5. 5. Neuroanatomy
  6. 6. Three – neuron pathway Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  7. 7. First – Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  8. 8. Second – Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  9. 9. Third – Order Neuron Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  10. 10. Third – Order Neuron Innervates the iris dilator muscles & Müller's muscle Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  11. 11. Clinical Features
  12. 12. ©2014 UpToDate® Anisocoria
  13. 13. ©2014 UpToDate®
  14. 14. Ptosis • Ptosis is minor, usually less than 2 mm • Paralysis of the Müller's muscle, which is innervated by the sympathetic pathway • "upside-down ptosis"
  15. 15. Anhidrosis • Anhidrosis is present in central or preganglionic (first or second-order) lesions • This sign is frequently not apparent to patients or clinicians
  16. 16. Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis 2000. Copyright ©2000 Elsevier.
  17. 17. Horner’s in Children • Impaired facial flushing (Harlequin sign) is often more apparent than anhidrosis • Acute features of sympathetic disruption can also include ipsilateral conjunctival injection, nasal stuffiness, and increased near point of accommodation
  18. 18. Harlequin sign Source: http://picornot.com/keyword/harlequin+sign
  19. 19. Congenital Horner's Syndrome
  20. 20. Localizing/Associated Symptoms • Diplopia, vertigo, ataxia, lateralized weakness suggest a brainstem localization • Bilateral or ipsilateral weakness, long tract signs, sensory level, bowel and bladder impairment suggest involvement of the cervicothoracic cord • Arm pain and/or hand weakness typical of brachial plexus lesions suggest a lesion in the lung apex
  21. 21. • Ipsilateral extraocular pareses, particularly a sixth nerve palsy, in the absence of other brainstem signs localize the lesion to the cavernous sinus • An isolated Horner's syndrome accompanied by neck or head pain suggests an internal carotid dissection
  22. 22. Etiology
  23. 23. First-order syndrome Lesions of the sympathetic tracts in the brainstem or cervicothoracic spinal cord can produce a first-order Horner's syndrome.
  24. 24. Lateral Medullary Infarction
  25. 25. • Strokes, tumors, and demyelinating lesions affecting the sympathetic tracts in the hypothalamus, midbrain, pons, medulla, or cervicothoracic spinal cord are other potential causes of a central Horner's syndrome. • Syringomyelia and cervical cord trauma can also produce a Horner's syndrome.
  26. 26. Second-order syndrome Second-order or preganglionic Horner's syndromes can occur with trauma or surgery involving the spinal cord, thoracic outlet, or lung apex
  27. 27. • Lumbar epidural anesthesia can also produce a Horner's syndrome. This is most often described in association with obstetrical procedure.
  28. 28. Third-order syndrome Third-order Horner's syndromes often indicate lesions of the internal carotid artery such as an arterial dissection, thrombosis, or cavernous sinus aneurysm.
  29. 29. Carotid Dissection An acute Horner's syndrome with neck or facial pain should be presumed to be caused by carotid dissection until proven otherwise. Between 40 and 60 percent of patients present with an isolated painful third-order Horner's syndrome. Emergent diagnostic tests should be obtained
  30. 30. Diagnosis
  31. 31. Pharmacologic Testing • Pharmacologic tests can be useful to confirm the diagnosis and to localize the lesion • Two agents are used: cocaine or apraclonidine drops and hydroxyamphetamine drops
  32. 32. Confirmation of Horner’s Syndrome • Pharmacological testing with cocaine or apraclonidine drops can confirm the diagnosis of Horner's syndrome in subtle cases • If the diagnosis of Horner's syndrome is clear clinically, then use of cocaine or apraclonidine can be avoided
  33. 33. Cocaine • Blocks the reuptake of norepinephrine at the sympathetic nerve synapse • Intact pathway – dilates pupil. No effect on impaired sympathetic pathway
  34. 34. Apraclonidine • Alternative to cocaine • Direct alpha-adrenergic receptor agonist. Apraclonidine has weak alpha-1 and strong alpha-2 activity • Alpha-1 mediates pupillary dilation, while alpha-2 downregulates norepinephrine release at the neuromuscular junction
  35. 35. From: Ocular Effects of Apraclonidine in Horner Syndrome Arch Ophthalmol. 2000;118(7):951-954. doi:10-1001/pubs.Ophthalmol.-ISSN-0003-9950-118-7-ecs90240 Date of download: 6/9/2014 Copyright © 2014 American Medical Association. All rights reserved.
  36. 36. Localization of the Lesion • First-order neuron (brainstem or cervical cord) • Second-order neuron (chest or neck) • Third-order or postganglionic neuron (above the superior cervical ganglion at the carotid bifurcation).
  37. 37. Hydroxyamphetamine • It releases stored norepinephrine from the postganglionic adrenergic nerve endings • A normal pupil and a first or second-order Horner's pupil will dilate, whereas a third-order Horner's pupil will not dilate as well as the normal pupil.
  38. 38. Postganglionic right Horner's syndrome ©2014 UpToDate®
  39. 39. Summary
  40. 40. • Classic signs of a Horner's syndrome include miosis, ptosis, and anhidrosis. • The miosis is typically mild, associated with a dilation lag and most prominent in dim light. • The ptosis is also mild and also involves the lower lid. • Anhidrosis occurs with first or second-order lesions only
  41. 41. • The common etiologies of Horner's syndrome are categorized by which of the three neurons is affected. • The differential diagnosis is also distinct in children versus adults26
  42. 42. • The presence of a Horner's syndrome can be confirmed pharmacologically with either cocaine or apraclonidine eye drops • Hydroxyamphetamine eye drops can help distinguish a third-order (postganglionic) Horner's syndrome from either a first or second-order syndrome.
  43. 43. • In the absence of a clear history of trauma as the cause of Horner's syndrome, imaging studies will be required.

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