horner's syndrome
TRANSCRIPT
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Horner’s Syndrome -Etiology and Localization
Yazeed Alwelaie
PGY1
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Introduction
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Horner’s Syndrome
• Also called oculo-sympathetic paresis
• Classic triad:
– Ptosis
– Miosis
– Anhidrosis
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Horner's syndrome can be produced by alesion anywhere along the sympatheticpathway that supplies the head, eye, andneck.
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Neuroanatomy
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
Three – neuron pathway
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
First – Order Neuron
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
Second – Order Neuron
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
Third – Order Neuron
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
Third – Order Neuron
Innervates the iris dilator muscles & Müller's muscle
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Clinical Features
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Anisocoria
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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"
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Anhidrosis
• Anhidrosis is present in central or preganglionic
(first or second-order) lesions
• This sign is frequently not apparent to patients or
clinicians
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Neuro-ophthalmology: the requisites in ophthalmology, Krachmer, JH (Ed), Mosby, St. Louis2000. Copyright ©2000 Elsevier.
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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
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Harlequin signSource: http://picornot.com/keyword/harlequin+sign
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Congenital Horner's Syndrome
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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
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• 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
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Etiology
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First-order syndrome
Lesions of the sympathetic
tracts in the brainstem or
cervicothoracic spinal cord
can produce a first-order
Horner's syndrome.
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Lateral Medullary Infarction
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• 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.
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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
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• Lumbar epidural anesthesia can also produce a
Horner's syndrome. This is most often described in
association with obstetrical procedure.
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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.
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An acute Horner's syndrome with neck or facialpain should be presumed to be caused by carotiddissection until proven otherwise.
Between 40 and 60 percent of patients presentwith an isolated painful third-order Horner'ssyndrome.
Emergent diagnostic tests should be obtained
Carotid Dissection
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Diagnosis
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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
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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
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Cocaine
• Blocks the reuptake of norepinephrine at the
sympathetic nerve synapse
• Intact pathway – dilates pupil. No effect on
impaired sympathetic pathway
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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
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Date of download: 6/9/2014Copyright © 2014 American Medical
Association. All rights reserved.
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
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Localization of the Lesion
• First-order neuron(brainstem or cervical cord)
• Second-order neuron(chest or neck)
• Third-order orpostganglionic neuron(above the superior cervicalganglion at the carotidbifurcation).
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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.
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Postganglionic right
Horner's syndrome
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Summary
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• 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
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• 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
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• 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.
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• 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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