Horner syndrome

Last updated: February 24, 2023

Horner syndrome

Revision

Revision

Anemia: Clinical
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Leukemia: Clinical
Lymphoma: Clinical
Thrombocytopenia: Clinical
Bleeding disorders: Clinical
Thrombophilia: Clinical
Myeloproliferative neoplasms: Clinical
Plasma cell disorders: Clinical
Blood products and transfusion: Clinical
Anticoagulants: Heparin
Anticoagulants: Warfarin
Anticoagulants: Direct factor inhibitors
Antiplatelet medications
Thrombolytics
Hematopoietic medications
Ribonucleotide reductase inhibitors
Topoisomerase inhibitors
Platinum containing medications
Anti-tumor antibiotics
Microtubule inhibitors
DNA alkylating medications
Endocrine system anatomy and physiology
Risk factors for periodontitis
Anatomy of the thyroid and parathyroid glands
Diabetes mellitus: Clinical
Hyperthyroidism: Clinical
Hypothyroidism and thyroiditis: Clinical
Parathyroid conditions and calcium imbalance: Clinical
Thyroid nodules and thyroid cancer: Clinical
Pituitary adenomas and pituitary hyperfunction: Clinical
Hypopituitarism: Clinical
Cushing syndrome: Clinical
Adrenal masses and tumors: Clinical
MEN syndromes: Clinical
Hyperthyroidism medications
Hypothyroidism medications
Insulins
Hypoglycemics: Insulin secretagogues
Miscellaneous hypoglycemics
Adrenal hormone synthesis inhibitors
Mineralocorticoids and mineralocorticoid antagonists
Glucocorticoids
HIV (AIDS)
Human herpesvirus 8 (Kaposi sarcoma)
Chronic kidney disease: Clinical
Chronic obstructive pulmonary disease (COPD): Clinical
Obstructive lung diseases: Pathology review
Inflammatory bowel disease: Clinical
Supraventricular arrhythmias: Pathology review
Ventricular arrhythmias: Pathology review
Heart blocks: Pathology review
Coronary artery disease: Clinical
Heart failure: Clinical
Syncope: Clinical
Pericardial disease: Clinical
Infective endocarditis: Clinical
Valvular heart disease: Clinical
Cardiomyopathies: Clinical
Hypertension: Clinical
Hypercholesterolemia: Clinical
Sympatholytics: Alpha-2 agonists
Adrenergic antagonists: Presynaptic
Adrenergic antagonists: Alpha blockers
Adrenergic antagonists: Beta blockers
ACE inhibitors, ARBs and direct renin inhibitors
Thiazide and thiazide-like diuretics
Calcium channel blockers
cGMP mediated smooth muscle vasodilators
Class I antiarrhythmics: Sodium channel blockers
Class II antiarrhythmics: Beta blockers
Class III antiarrhythmics: Potassium channel blockers
Class IV antiarrhythmics: Calcium channel blockers and others
Lipid-lowering medications: Statins
Lipid-lowering medications: Fibrates
Miscellaneous lipid-lowering medications
Positive inotropic medications
Loop diuretics
Osteogenesis imperfecta
Acyanotic congenital heart defects: Pathology review
Cyanotic congenital heart defects: Pathology review
Cardiomyopathies: Pathology review
Heart failure: Pathology review
Systemic lupus erythematosus (SLE): Clinical
Diabetic retinopathy
Diabetic nephropathy
Congenital adrenal hyperplasia
Primary adrenal insufficiency
Waterhouse-Friderichsen syndrome
Hyperaldosteronism
Adrenal cortical carcinoma
Cushing syndrome
Conn syndrome
Thyroglossal duct cyst
Hyperthyroidism
Graves disease
Thyroid eye disease (NORD)
Toxic multinodular goiter
Thyroid storm
Hypothyroidism
Euthyroid sick syndrome
Hashimoto thyroiditis
Subacute granulomatous thyroiditis
Riedel thyroiditis
Thyroid cancer
Hyperparathyroidism
Hypoparathyroidism
Hypercalcemia
Hypocalcemia
Diabetes mellitus
Hyperpituitarism
Pituitary adenoma
Hyperprolactinemia
Prolactinoma
Gigantism
Acromegaly
Hypopituitarism
Pituitary apoplexy
Sheehan syndrome
Hypoprolactinemia
Constitutional growth delay
Diabetes insipidus
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Precocious puberty
Delayed puberty
Premature ovarian failure
Polycystic ovary syndrome
Androgen insensitivity syndrome
Kallmann syndrome
5-alpha-reductase deficiency
Autoimmune polyglandular syndrome type 1 (NORD)
Multiple endocrine neoplasia
Pancreatic neuroendocrine neoplasms
Zollinger-Ellison syndrome
Pheochromocytoma
Neuroblastoma
Opsoclonus myoclonus syndrome (NORD)
Adrenal insufficiency: Pathology review
Adrenal masses: Pathology review
Hyperthyroidism: Pathology review
Hypothyroidism: Pathology review
Thyroid nodules and thyroid cancer: Pathology review
Parathyroid disorders and calcium imbalance: Pathology review
Diabetes mellitus: Pathology review
Cushing syndrome and Cushing disease: Pathology review
Pituitary tumors: Pathology review
Hypopituitarism: Pathology review
Diabetes insipidus and SIADH: Pathology review
Multiple endocrine neoplasia: Pathology review
Immune thrombocytopenia
Mixed platelet and coagulation disorders: Pathology review
Hypertension
Heparin-induced thrombocytopenia
Raynaud phenomenon
Nephritic syndromes: Pathology review
Down syndrome (Trisomy 21)
Rett syndrome
Restrictive lung diseases: Pathology review
Sarcoidosis
Parkinson disease
Cranial nerves
Spina bifida
Chiari malformation
Dandy-Walker malformation
Syringomyelia
Tethered spinal cord syndrome
Aqueductal stenosis
Septo-optic dysplasia
Cerebral palsy
Spinocerebellar ataxia (NORD)
Transient ischemic attack
Ischemic stroke
Intracerebral hemorrhage
Epidural hematoma
Subdural hematoma
Subarachnoid hemorrhage
Saccular aneurysm
Arteriovenous malformation
Broca aphasia
Wernicke aphasia
Wernicke-Korsakoff syndrome
Kluver-Bucy syndrome
Concussion and traumatic brain injury
Shaken baby syndrome
Seizures and epilepsy
Febrile seizure
Early infantile epileptic encephalopathy (NORD)
Tension headache
Cluster headache
Migraine
Idiopathic intracranial hypertension
Trigeminal neuralgia
Cavernous sinus thrombosis
Alzheimer disease
Vascular dementia
Frontotemporal dementia
Dementia with Lewy bodies
Creutzfeldt-Jakob disease
Normal pressure hydrocephalus
Torticollis
Essential tremor
Restless legs syndrome
Parkinson disease
Huntington disease
Multiple sclerosis
Central pontine myelinolysis
Acute disseminated encephalomyelitis
Transverse myelitis
JC virus (Progressive multifocal leukoencephalopathy)
Adult brain tumors
Acoustic neuroma (schwannoma)
Pediatric brain tumors
Brain herniation
Brown-Sequard Syndrome
Cauda equina syndrome
Treponema pallidum (Syphilis)
Vitamin B12 deficiency
Friedreich ataxia
Neurogenic bladder
Meningitis
Neonatal meningitis
Encephalitis
Brain abscess
Epidural abscess
Sturge-Weber syndrome
Tuberous sclerosis
Neurofibromatosis
von Hippel-Lindau disease
Amyotrophic lateral sclerosis
Spinal muscular atrophy
Poliovirus
Guillain-Barre syndrome
Charcot-Marie-Tooth disease
Bell palsy
Winged scapula
Thoracic outlet syndrome
Carpal tunnel syndrome
Ulnar claw
Erb-Duchenne palsy
Klumpke paralysis
Sciatica
Myasthenia gravis
Lambert-Eaton myasthenic syndrome
Orthostatic hypotension
Horner syndrome
Congenital neurological disorders: Pathology review
Headaches: Pathology review
Seizures: Pathology review
Cerebral vascular disease: Pathology review
Traumatic brain injury: Pathology review
Spinal cord disorders: Pathology review
Dementia: Pathology review
Central nervous system infections: Pathology review
Movement disorders: Pathology review
Neuromuscular junction disorders: Pathology review
Demyelinating disorders: Pathology review
Adult brain tumors: Pathology review
Pediatric brain tumors: Pathology review
Neurocutaneous disorders: Pathology review

Transcript

Watch video only

Horner’s syndrome, named after the ophthalmologist Johann Friedrich Horner, is caused by a problem with the sympathetic nerve supply to one side of the face.

This disruption results in miosis, which is constricted pupil; ptosis, a droopy eyelid; and anhidrosis, a failure to sweat.

Broadly speaking, the autonomic nervous system is a part of the nervous system that controls involuntary body functions like the heart rate, blood pressure and digestion.

The autonomic nervous system can be subdivided into the sympathetic and parasympathetic nervous system, which have opposite effects.

The sympathetic nervous system controls functions like increasing heart rate, blood pressure, and slowing digestion. All of this maximizes blood flow to the muscles, and can help you either run away from a threat or fight it which is why it’s also called the fight-or-flight response.

The parasympathetic nervous system has the opposite effect; it slows heart rate, decreases blood pressure, and stimulates digestion - the effects can be summarized as 'rest and digest'.

Now, with regard to the face and eye, there’s an oculosympathetic pathway with three groups of neurons called first-order, second-order and third-order neurons.

The body of the first-order neuron is located in the hypothalamus, and it’s axon extends down into the spinal cord, where it synapses with the second-order neuron.

The body of the second-order neuron is located in the cervical region of the spinal cord, and it’s axon exits the spinal cord and enters the sympathetic chain, which is a structure full of sympathetic ganglions or nerve cell bodies, and it runs along both sides of the spine.

The sympathetic chain looks similar to a string of pearls where the ganglions are the pearls and the nerve fibers make up the string.

The first three ganglia within the sympathetic chain are called the superior, middle, and inferior cervical ganglion.

The axon of the second-order neuron runs through the inferior and middle cervical ganglion, up the superior cervical ganglion where it synapses with the body of the third-order neuron.

Third order neuron axons extend from the superior cervical ganglion and hitch-hike along nerves that travel along the common carotid artery up the neck until it splits into an internal and external carotid artery.

One group of nerve fibers, called internal carotid plexus, follow the internal carotid artery into the skull and then exit through the orbit to innervate the pupillary dilator muscle, which dilates the pupil; the Müller's muscle, which raises the upper eyelid; and sweat glands of the forehead.

The other group of fibers, follow the external carotid and its branches and innervate the rest of the sweat glands of the face.

Horner’s syndrome occurs when there’s damage along the oculosympathetic pathway.

Damage to the first-order neuron is caused by a spinal cord lesions above level T1 - some causes include a stroke, tumors, or syringomyelia.

Syringomyelia is when there’s a cyst or cavity within the spinal cord, which damages the surrounding nerve fiber.

Key Takeaways

Horner syndrome is a neurological disorder characterized by the combination of three main symptoms: ptosis (drooping eyelid), miosis (constricted pupil), and anhidrosis (lack of sweating on one side of the body).

Most cases of horner syndrome are caused by damage to the sympathetic nervous system, which can be due to a stroke, tumor, or injury. Less common causes include infections, autoimmune disorders, and medications. Some people also develop horner syndrome as a side effect of cancer treatment.

Sources

  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Enophthalmos Is Not Present in Horner Syndrome" PLoS Medicine (2005)
  6. "Neuroimaging Strategies for Three Types of Horner Syndrome with Emphasis on Anatomic Location" American Journal of Roentgenology (2007)