Horner syndrome

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Horner syndrome

Neurology

Neurology

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Stroke: Clinical
Hypokinetic movement disorders: Clinical
Headaches: Clinical
Meningitis, encephalitis and brain abscesses: Clinical
Hyperkinetic movement disorders: Clinical
Dementia and delirium: Clinical
Traumatic brain injury: Clinical
Seizures: Clinical
Dizziness and vertigo: Clinical
Disorders of consciousness: Clinical
Brain tumors: Clinical
Lower back pain: Clinical
Muscle weakness: Clinical
Spinal cord disorders: Pathology review

Transcript

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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)