Horners syndrome

Horners 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

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

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

8. Second-order preganglionic pupillomotor fibers

9. 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

10. 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

11. 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

12. The third-order pupillomotor fibers

13. The third-order pupillomotor fibers

14. 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

15. 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.

16. 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

17. Studies

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

19. The interruption of the sympathetic fibers may occur

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

21. 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

22. 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

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

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

25. Light Reflex

26. Normal pupil reaction video

27. 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

28. Adie’s tonic pupil (OD)

29. Argyll-Robertson pupil

30. Horner’s pupil (OS)

31. 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.

32. 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

33. 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.

34. 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.

35. 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

36. 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.

37. 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

38. 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 .

39. 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.

40. 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.

41. VIDEO

42. 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.

43. 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,

44. 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.

45. 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.

46. Thank You