Horner syndrome

Neurology

Clinicals - History

Fact Explanation
Hemisensory loss Occurs when there is a lesion in the brainstem involving the sympathetic and cortico spinal nerve pathways, resulting in Horner syndrome and contralateral motor/sensory signs Hemisensory loss
Occurs when there is a lesion in the brainstem involving the sympathetic and cortico spinal nerve pathways, resulting in Horner syndrome and contralateral motor/sensory signs
Dysarthria Can occur in first order neuron Horner syndrome(HS), due to lateral medullary syndrome that occurs as a result of posterior inferior cerebellar artery occlusion. In this condition there is involvement of the first order sympathetic neuron pathway, spinothalamic tract, trigeminal nerve tract, tenth cranial nerve and cerebellar pathways. Dysarthria results from damage to vestibulo-floccular connections. Damage to trigeminal nerve tract can produce ipsilateral sensory loss of face and damage to spinothalamic tract can cause contralateral sensory loss of limbs and trunk Dysarthria
Can occur in first order neuron Horner syndrome(HS), due to lateral medullary syndrome that occurs as a result of posterior inferior cerebellar artery occlusion. In this condition there is involvement of the first order sympathetic neuron pathway, spinothalamic tract, trigeminal nerve tract, tenth cranial nerve and cerebellar pathways. Dysarthria results from damage to vestibulo-floccular connections. Damage to trigeminal nerve tract can produce ipsilateral sensory loss of face and damage to spinothalamic tract can cause contralateral sensory loss of limbs and trunk
Dysphagia Can occur in first order neuron HS which is a component of lateral medullary syndrome as described above Dysphagia
Can occur in first order neuron HS which is a component of lateral medullary syndrome as described above
Ataxia If occurs together with HS, is suggestive of lateral medullar syndrome Ataxia
If occurs together with HS, is suggestive of lateral medullar syndrome
Vertigo Occurs with HS when the disruption to nerve pathway is at the medulla, likely to be due to lateral medullary syndrome involving both sympathetic nerve pathways and vestibulo-floccular connections Vertigo
Occurs with HS when the disruption to nerve pathway is at the medulla, likely to be due to lateral medullary syndrome involving both sympathetic nerve pathways and vestibulo-floccular connections
Axillary, shoulder or arm pain Occurs when the nerve disruption is at the C8-T2 ventral nerve root level Axillary, shoulder or arm pain
Occurs when the nerve disruption is at the C8-T2 ventral nerve root level
Neck, facial, and head pain If a patient presents with Horner syndrome associated with ipsilateral orbital, face, or neck pain of acute onset, internal carotid artery dissection should always be excluded until proven otherwise. Pain occurs because of ischemia or stretching of the trigeminal pain fibers surrounding the carotid arteries. Neck, facial, and head pain
If a patient presents with Horner syndrome associated with ipsilateral orbital, face, or neck pain of acute onset, internal carotid artery dissection should always be excluded until proven otherwise. Pain occurs because of ischemia or stretching of the trigeminal pain fibers surrounding the carotid arteries.
History of central venous catheter placement Invasive procedures involving neck region could cause damage to the cervical sympathetic chain History of central venous catheter placement
Invasive procedures involving neck region could cause damage to the cervical sympathetic chain
History of chest tube insertion Could have caused damage to the sympathetic nerve pathways in the region of insertion History of chest tube insertion
Could have caused damage to the sympathetic nerve pathways in the region of insertion
previous thoracic or neck surgery Could have caused accidental damage to the sympathetic nerve pathways previous thoracic or neck surgery
Could have caused accidental damage to the sympathetic nerve pathways
Hemoptysis and symptoms of lung disease These would suggest the presence of a lung cancer particularly an apical Pancoast tumour which can spread to the cervical sympathetic chain Hemoptysis and symptoms of lung disease
These would suggest the presence of a lung cancer particularly an apical Pancoast tumour which can spread to the cervical sympathetic chain
History of trauma Trauma involving head and neck could have caused damage to the sympathetic nerve pathways History of trauma
Trauma involving head and neck could have caused damage to the sympathetic nerve pathways
Diplopia Occurs when there is a lesion in the cavernous sinus involving the sympathetic nerves and also causing abducens nerve palsy It can also occur in anterior inferior cerebellar artery occlusion but then in addition to ipsilateral HS and lateral gaze palsy, it also causes ipsilateral sensory loss and weakness of face and contralateral sensory loss of limbs and trunk Diplopia
Occurs when there is a lesion in the cavernous sinus involving the sympathetic nerves and also causing abducens nerve palsy It can also occur in anterior inferior cerebellar artery occlusion but then in addition to ipsilateral HS and lateral gaze palsy, it also causes ipsilateral sensory loss and weakness of face and contralateral sensory loss of limbs and trunk
Numbness in the distribution of the first or second division of the trigeminal nerve (cranial nerve [CN] V), and pain Occurs when the disruption to sympathetic nerve pathway occurs at the brainstem Numbness in the distribution of the first or second division of the trigeminal nerve (cranial nerve [CN] V), and pain
Occurs when the disruption to sympathetic nerve pathway occurs at the brainstem
Nystagmus Also occurs due to damage to vestibulo-floccular connections that occurs in lateral medullary syndrome Nystagmus
Also occurs due to damage to vestibulo-floccular connections that occurs in lateral medullary syndrome

Clinicals - Examination

Fact Explanation
Ptosis/Partial ptosis Is considered a classical sign of Horner syndrome that occur secondary to interruption of the sympathetic pathway that supplies the superior tarsal muscle of the upper eye lid Ptosis/Partial ptosis
Is considered a classical sign of Horner syndrome that occur secondary to interruption of the sympathetic pathway that supplies the superior tarsal muscle of the upper eye lid
Fixed constricted pupil not reacting to light (miosis) This is also a classical sign of Horner syndrome. It results from the unopposed activity of the pupillary constrictors supplied by the parasympathetic nerves, because the pupillary dilators supplied by the sympathetic nerves is not active Fixed constricted pupil not reacting to light (miosis)
This is also a classical sign of Horner syndrome. It results from the unopposed activity of the pupillary constrictors supplied by the parasympathetic nerves, because the pupillary dilators supplied by the sympathetic nerves is not active
Dry skin (anhidrosis) One of the classic triad symptoms of Horner syndrome that occurs because of the disrupted sympathetic supply to the sweat glands on ipsilateral side of face. Occurs when the lesion is proximal to the fiber separation at the carotid arteries Dry skin (anhidrosis)
One of the classic triad symptoms of Horner syndrome that occurs because of the disrupted sympathetic supply to the sweat glands on ipsilateral side of face. Occurs when the lesion is proximal to the fiber separation at the carotid arteries
Anisocoria Anisocoria (unequal size of the pupils) is more prominent in the dark, may be most obvious within the first 5 s of darkness when the iris dilators are supposed to be acting. After 10–15 s in the dark, this becomes less apparent (known as dilation lag) Anisocoria
Anisocoria (unequal size of the pupils) is more prominent in the dark, may be most obvious within the first 5 s of darkness when the iris dilators are supposed to be acting. After 10–15 s in the dark, this becomes less apparent (known as dilation lag)
Enophthalmos The lower eye lid also has a muscle innervated by sympathetic nerves. When there is disruption to sympathetic nerve supply, the lower lid may be elevated, with overall palpebral aperture reduction making the globe appear sunken in Enophthalmos
The lower eye lid also has a muscle innervated by sympathetic nerves. When there is disruption to sympathetic nerve supply, the lower lid may be elevated, with overall palpebral aperture reduction making the globe appear sunken in
Red conjunctivae Conjunctival hyperemia can occur in acute Horner syndrome Red conjunctivae
Conjunctival hyperemia can occur in acute Horner syndrome
Asymmetric facial flushing Is sometimes seen with congenital Horner syndrome Asymmetric facial flushing
Is sometimes seen with congenital Horner syndrome
Iris heterochromia Is associated with congenital Horner syndrome Iris heterochromia
Is associated with congenital Horner syndrome

Investigations - Diagnosis

Fact Explanation
Chest radiograph Done to exclude mediastinal masses and apical lung tumors when such is suspected Chest radiograph
Done to exclude mediastinal masses and apical lung tumors when such is suspected
Cocaine test Can be used to confirm diagnosis of HS, but cannot localize the lesion Cocaine test
Can be used to confirm diagnosis of HS, but cannot localize the lesion
Apraclonidine test Can be used fro confirming clinical diagnosis of HS but cannot localize the site of lesion Apraclonidine test
Can be used fro confirming clinical diagnosis of HS but cannot localize the site of lesion
Computed tomography(CT) Done to assess the head, neck and thoracic structures in order to localize the lesion Computed tomography(CT)
Done to assess the head, neck and thoracic structures in order to localize the lesion
Magnetic resonance imaging (MRI) Also done to localize the lesion. Gives a better contrast resolution than CT Magnetic resonance imaging (MRI)
Also done to localize the lesion. Gives a better contrast resolution than CT
Magnetic resonance angiography (MRA) Done to assess the the arterial system of head, neck and thorax Magnetic resonance angiography (MRA)
Done to assess the the arterial system of head, neck and thorax
Random urine studies for vanillylmandelic acid [VMA] and homovanillic acid [HVA] Done in children with HS to exclude neuroblastoma Random urine studies for vanillylmandelic acid [VMA] and homovanillic acid [HVA]
Done in children with HS to exclude neuroblastoma
Color Doppler of neck vasculature Done to see the blood flow through neck vessels Color Doppler of neck vasculature
Done to see the blood flow through neck vessels

Management - Specific

Fact Explanation
Neurosurgical care Should be offered for those who have HS due to aneurysms etc that needs neurosurgical intervention Neurosurgical care
Should be offered for those who have HS due to aneurysms etc that needs neurosurgical intervention
Vascular surgical care Should be offered when there are vascular conditions causing HS which may require surgical intervention Vascular surgical care
Should be offered when there are vascular conditions causing HS which may require surgical intervention
Oncology referal Is needed for patients presenting with HS due to pancoast tumor.These patients will be given the option of chemo-radiotherapy or surgery depending on the stage of the disease Oncology referal
Is needed for patients presenting with HS due to pancoast tumor.These patients will be given the option of chemo-radiotherapy or surgery depending on the stage of the disease
Anticoagulants Anticoagulant treatment is given for 3 to 6 months to those with carotid artery dissection to prevent thromboembolism Anticoagulants
Anticoagulant treatment is given for 3 to 6 months to those with carotid artery dissection to prevent thromboembolism

Concise, fact-based medical articles to refresh your knowledge

Access a wealth of content and skim through a smartly presented catalog of diseases and conditions.

  1. DAROFF R. Enophthalmos Is Not Present in Horner Syndrome PLoS Med [online] 2005 Apr, 2(4):e120 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020120
  2. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: doi:10.1038/eye.2012.281
  3. DAVAGNANAM I, FRASER CL, MISZKIEL K, DANIEL CS, PLANT GT. Adult Horner's syndrome: a combined clinical, pharmacological, and imaging algorithm Eye (Lond) [online] 2013 Mar, 27(3):291-298 [viewed 26 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597883/
  4. FOROULIS CN, ZAROGOULIDIS P, DARWICHE K, KATSIKOGIANNIS N, MACHAIRIOTIS N, KARAPANTZOS I, TSAKIRIDIS K, HUANG H, ZAROGOULIDIS K. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis [online] 2013 Sep, 5(Suppl 4):S342-S358 [viewed 28 May 2014] Available from: doi:10.3978/j.issn.2072-1439.2013.04.08
  5. HABEK M, PETRAVIć D, OZRETIć D, BRINAR VV. Horner syndrome due to jugular vein thrombosis (Lemierre syndrome) BMJ Case Rep [online] :bcr2007124479 [viewed 27 May 2014] Available from: doi:10.1136/bcr.2007.124479
  6. JOHNSON D, SHARMA S. Answer: Can you identify this condition? Can Fam Physician [online] 2010 May, 56(5):443 [viewed 27 May 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2868616
  7. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011
  8. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011
  9. LINDSAY KW, BONE I, CALLANDER R, FULLER G, Neurology and neurosurgery illustrated. Fifth edition. Churchill Livingstone. 2011; pg 143
  10. NAUTIYAL A, SINGH S, DISALLE M, O'SULLIVAN J. Painful Horner Syndrome as a Harbinger of Silent Carotid Dissection PLoS Med [online] 2005 Jan, 2(1):e19 [viewed 27 May 2014] Available from: doi:10.1371/journal.pmed.0020019
  11. NICHOLAS R. MAHONEY NR, LIU GT,MENACKER SJ, WILSON MC, HOGARTY MD,MARIS JM. Pediatric Horner Syndrome: Etiologies and Roles of Imaging and Urine Studies to Detect Neuroblastoma and Other Responsible Mass Lesions.Am J Ophthalmol [online] 2006;142:651–659.[viewed on 26 May 2014] Available from; http://www.rima.org/web/medline_pdf/AmJOphthalmol2006_651_9.pdf
  12. SMITH SJ, DIEHL N, LEAVITT JA, MOHNEY BG. Incidence of Pediatric Horner Syndrome and the Risk of Neuroblastoma: A Population-Based Study Arch Ophthalmol [online] 2010 Mar, 128(3):324-329 [viewed 27 May 2014] Available from: doi:10.1001/archophthalmol.2010.6