Horner's Syndrome: Clinical and Radiographic Evaluation

doi:10.1016/j.nic.2007.11.003

Neuroimaging Clinics of North America

Volume 18, Issue 2, May 2008, Pages 369-385

https://doi.org/10.1016/j.nic.2007.11.003 Get rights and content

Horner's syndrome (HS) occurs when there is interruption of the oculosympathetic pathway (OSP). This article reviews the anatomy of the OSP and clinical findings associated with lesions located at various positions along this pathway. The imaging findings of lesions associated with HS at various levels of the OSP, classified as preganglionic HS (first- and second-order neuron HS) or postganglionic HS (third-order neuron HS), are demonstrated.

Section snippets

Anatomy of the oculosympathetic pathway

The OSP supplies sympathetic innervation to the sweat glands (ipsilateral body and face), dilator muscles of the eye, and retractor muscles of the upper and lower eyelids. This pathway consists of three neurons and two relay centers (ciliospinal center of Budge-Waller and the superior cervical ganglion).

Horner's syndrome clinical findings

Johann Friedrich Horner first described the classic clinical triad of symptoms seen in HS, (ptosis, miosis, and anhidrosis) in 1869 (Fig. 2).⁵

Ptosis refers to a moderate drop of the upper eyelid. The levator palpebrae superioris muscle elevates the upper eyelid. This muscle is innervated by the oculomotor nerve (CN III). Müller's muscle in the upper eyelid is a thin sheet of smooth muscle arising from the undersurface of the levator palpebrae superioris muscle (Fig. 3). It also elevates the

Classification

HS can be classified as preganglionic or postganglionic, based on the location of a lesion in the OSP with reference to the superior cervical ganglion (Fig. 6).

The preganglionic segment is the segment of the OSP proximal to the superior cervical ganglion. It can be further subdivided into two subsegments:

1.
The central, or FON, subsegment is located between the hypothalamus and IML before the FON PGF synapse in the ciliospinal center of Budge-Waller.
2.
The peripheral, or SON, subsegment refers to the

Clinical evaluation

Anisocoria (unequal pupil size) may be a result of aging or sympathetic or parasympathetic dysfunction. Examination of the pupil in the dark will help determine the etiology (Fig. 7). The question to be answered is: “Is the pupil inequality greater in the dark or in the light?” If the inequality is greater in the light, this is consistent with a parasympathetic lesion. Examination of the eye in the dark will help differentiate physiologic anisocoria from sympathetic dysfunction. There is no

Location

Lesions that cause a FON HS are found anywhere from the hypothalamus to the level of the IML before the FON PGF synapse with the SON in the ciliospinal center of Budge-Waller (see Fig. 1).

Clinical findings

Miosis may be the only evidence of a FON HS. The anhidrosis distribution is ipsilateral to the entire half of the body (Fig. 10). Cerebellar, brain stem, or cervical spinal cord symptoms are usually present.¹¹

Pharmacological testing

There is minimal or no pupil dilatation after the administration of cocaine. Dilation will increase

Location

Lesions occurring anywhere from the superior cervical ganglion to the eye can produce a TON HS.

Clinical findings

The full syndrome of ptosis, miosis, and anhidrosis is usually present. Anhidrosis of the ipsilateral face and neck is seen with lesions involving the SCG. Lesions distal to the SCG have anhidrosis limited to the ipsilateral nose and forehead (Fig. 23). Proptosis, chemosis, or conjunctival hyperemia is often present in association with TON HS when an orbital lesion is the etiology.¹¹

Pharmacological testing

The pupil does not

Summary

The clinical symptoms of HS may cause little if any functional impairment in most patients. However, since both benign and malignant processes are associated with HS, a thorough clinical evaluation is required. Once a lesion is localized clinically within the OSP by a combination of physical examination and pharmacological testing, the radiologic examination can be appropriately tailored.

Acknowledgments

We thank Jill K. Gregory, MFA, CMI, Medical Illustrator.

References (44)

H. Pomeranz
Isolated Horner syndrome and syrinx of the cervical spinal cord
Am J Ophthalmol
(2002)
F.C. Detterbeck
Pancoast (superior sulcus) tumors
Ann Thorac Surg
(1997)
S. Attar et al.
Superior sulcus (pancoast) tumor: experience with 105 patients
Ann Thorac Surg
(1998)
A.K. Moukheiber et al.
Primary pediatric neuroblastic tumors of the neck
Int J Pediatr Otorhinolaryngol
(2001)
K. Cengiz et al.
Intrathoracic goiter with hyperthyroidism, tracheal compression, superior vena cava syndrome and Horner's syndrome
Chest
(1990)
J. Brown et al.
Horner's syndrome in subadventitial carotid artery dissection and the role of magnetic resonance angiography
Am J Ophthalmol
(1995)
F.H. Ryan et al.
Congenital Horner's syndrome resulting from agenesis of the internal carotid artery
Ophthalmology
(2000)
H.S. Amonoo-Kuofi
Horner's syndrome revisited: with an update of the central pathway
Clin Anat
(1999)
H.S. Thompson
The pupil
R.M. Burde et al.
Anisocoria and abnormal papillary light reactions

R.S. Snell et al.

The autonomic nervous system

J.F. Horner

On a form of ptosis

Klin Monatsbl Augenheilkd

(1869)

F.E. Lepore

Enophthalmos and Horner's syndrome

Arch Neurol

(1983)

G. Smith et al.

Topographic analysis of Horner's syndrome

Otolaryngol Head Neck Surg

(1986)

J.M. Weinstein et al.

Congenital Horner's syndrome

Arch Ophthalmol

(1980)

D.A. Morrison et al.

The “harlequin” sign and congenital Horner's syndrome

J Neurol Neurosurg Psychiatry

(1997)

H. Wilhelm et al.

Interaction of the indirectly acting topical sympathomimetics cocaine and pholegrine

Ger J Ophthalmol

(1996)

A.N. Nagy et al.

Horner's syndrome due to first-order neuron lesions of the oculosympathetic pathway

AJR Am J Roentgenol

(1997)

K.B. Digre et al.

Selective MR imaging approach for evaluation of patients with Horner's syndrome

AJNR Am J Neuroradiol

(1992)

R.L. Sacco et al.

Wallenberg's lateral medullary syndrome: clinical-magnetic resonance imaging correlations

Arch Neurol

(1993)

C.J.K. Ellis

Editorial commentary: isolated Horner's syndrome and syringomyelia

J Neurol Neurosurg Psychiatry

(2000)

J.B. Kerrison et al.

Isolated Horner's syndrome and syringomyelia

J Neurol Neurosurg Psychiatry

(2000)

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Horner's Syndrome: Clinical and Radiographic Evaluation

Section snippets

Anatomy of the oculosympathetic pathway

Horner's syndrome clinical findings

Classification

Clinical evaluation

Location

Clinical findings

Pharmacological testing

Location

Clinical findings

Pharmacological testing

Summary

Acknowledgments

Am J Ophthalmol

Ann Thorac Surg

Ann Thorac Surg

Int J Pediatr Otorhinolaryngol

Chest

Am J Ophthalmol

Ophthalmology

Horner's syndrome revisited: with an update of the central pathway

Clin Anat

The pupil

Anisocoria and abnormal papillary light reactions

The autonomic nervous system

On a form of ptosis

Klin Monatsbl Augenheilkd

Enophthalmos and Horner's syndrome

Arch Neurol

Topographic analysis of Horner's syndrome

Otolaryngol Head Neck Surg

Congenital Horner's syndrome

Arch Ophthalmol

The “harlequin” sign and congenital Horner's syndrome

J Neurol Neurosurg Psychiatry

Interaction of the indirectly acting topical sympathomimetics cocaine and pholegrine

Ger J Ophthalmol

Horner's syndrome due to first-order neuron lesions of the oculosympathetic pathway

AJR Am J Roentgenol

Selective MR imaging approach for evaluation of patients with Horner's syndrome

AJNR Am J Neuroradiol

Wallenberg's lateral medullary syndrome: clinical-magnetic resonance imaging correlations

Arch Neurol

Editorial commentary: isolated Horner's syndrome and syringomyelia

J Neurol Neurosurg Psychiatry

Isolated Horner's syndrome and syringomyelia

J Neurol Neurosurg Psychiatry