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Horners syndrome
Citation preview
Horner's syndrome
Presenter : Dr Rahul AchlerkarModerator : Dr Atul Seth
IntroductionThe 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
Horner’s
Characterized by the classic triad of Miosis (constricted pupil) Partial ptosis Loss of hemifacial sweating
( anhidrosis).
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
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).
Applied Anatomy
First-order neuron lesionsCerebral vascular accident
(CVA)/Wallenberg syndrome Demyelinating disease
(eg, multiple sclerosis)Arnold-Chiari malformationBasal meningitis
(eg, syphilis)Basal skull tumors
First-order neuron lesions
Hemisensory lossDysarthriaDysphagia AtaxiaVertigoNystagmus
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).
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
Applied Anatomy
Aneurysm or dissection of the aorta
Lesions of the subclavian or common carotid artery
NeuroblastomaLymphadenopathy
eg, Hodgkin disease, leukemia, tuberculosis, or mediastinal tumors
Second-order neuron lesions
Prior trauma facial, neck, axillary, shoulder or arm pain
CoughHemoptysisPrevious thoracic or neck surgeryPrevious chest tube or central venous catheter placement; or
neck swelling
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.
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
Third-order neuron lesions
Internal carotid artery dissectionassociated with sudden
ipsilateral face or neck pain Raeder syndrome
(paratrigeminal syndrome)Carotid cavernous fistulaCluster or migraine
headacheHerpes zoster
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.
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
Studies
EtiologyHorner syndrome can be congenital, acquired, or purely
hereditary (autosomal dominant)
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
Drugs that may cause symptoms similar to Horner syndrome include the following:
AcetophenazineBupivacaineButaperazineChloroprocaineChlorpromazineFluphenazineGuanethidineInfluenza virus vaccineLevodopa
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
differential diagnosis
AnisocoriaAdie pupilArgyll Robertson pupilHolmes-Adie pupil (contralateral)Iris sphincter muscle damageSenile miosisThird nerve palsyUnilateral use of miotic drugsUnilateral use of mydriatic drugs
AnisocoriaPupillary inequality greatest
In bright light(large pupil)
In dim light(small pupil)
3rd nerve palsyTraumaTumorTemporal lobe herniationAneurysm
No 3rd nerve palsyDrug inducedAdie’s pupilIris damage (trauma/surgery/laser)Basal meningitis
PtosisHorner syndrome
Physiological
TestingWhich is the abnormal pupil Compare in light and dark.Direct and consensual
responseIs accomodation affected?
Light Reflex
Normal pupil reactionvideo
Argyll-Robertson pupilSmall, irregDoes not react to
light Reacts to
accommodationCauses
syphilis diabetes
Miotonic pupil (Adie’s syndrome)DilatedPoor response to light
and convergence. Constricts with weak
Pilocarpine Holmes-Adie
syndrome Reduced tendon
reflexes (Knee, ankle)- Orthostatic
hypotension
Afferent & efferent defects
Adie’s tonic pupil (OD)
Argyll-Robertson pupil
Horner’s pupil (OS)
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.
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
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.
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.
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
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.
ApraclonidineIt 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
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.
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.
Side EffectsApraclonidine 0.5% or 1% may cause LethargyBradycardiarespiratory depression in infants , younger than 6 months Because of the immaturity of the blood-brain barrier.
VIDEO
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.
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,
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.
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.
Thank You