Both of these muscles are involuntary – they are controlled by the autonomic nervous system.
Three types of nerve fibers run through the ciliary ganglion: parasympathetic fibers, sympathetic fibers and sensory fibers. Only parasympathetic fibers form synapses in the ganglion. The other two types of nerve fibers simply pass through. In classical anatomy, the ciliary ganglion is said to have three “roots:”
When a patient with an Adie pupil attempts to focus on a nearby object, the pupil (which would normally constrict rapidly) constricts slowly. On close inspection, the constrcted pupil is not perfectly round. When the patient focuses on a more distant object (say the far side of the room), the pupil (which would normally dilate immediately) remains constricted for several minutes, and then slowly dilates back to the expected size.
Tonic pupils are fairly common – they are seen in roughly 1 out of every 500 people. A patient with anisocoria (one pupil bigger than the other) whose pupil does not react to light (does not constrict when exposed to bright light) most likely has Adie syndrome – idiopathic degeneration of the ciliary ganglion.
The strange behavior of tonic pupils was first explained by Irene Loewenfeld in 1979. The ciliary ganglion contain many more nerve fibers directed to the ciliary muscle than nerve fibers directed to the constrictor pupillae – roughly twenty times more. The ciliary muscle is also more massive than the constrictor pupillae, again by a factor of twenty. Based on these observations, Loewenfeld proposed an explanation of the tonic pupil. She noted that pathological destruction of nerve cells in the ciliary ganglion that is found in all cases of Adie pupil. In her own words :
Loewenfeld’s theory is now generally accepted. It explains the defining features of a tonic pupil:
Tonic pupils are usually due to Adie syndrome, but other diseases can denervate the ciliary ganglion. Peripheral neuropathies (such as diabetic neuropathy) occasionally produce tonic pupils. Herpes zoster virus can attack the ciliary ganglion. Trauma to the orbit can damage the short ciliary nerves. Anything that denervates the ciliary ganglion will produce a tonic pupil due to aberrant nerve regeneration.
Adie syndrome is tonic pupil plus absent deep tendon reflexes. Adie syndrome is a fairly common, benign, idiopathic neuropathy that selectively affects the ciliary ganglion and the spinal cord neurons involved in deep tendon reflex arcs. It usually develops in middle age, although it can occur in children. A variant of Adie syndrome, Ross syndrome, affects sweating as well.
Early in the course of Adie syndrome (when the cells of the ciliary ganglion have been destroyed, but before regeneration has occurred) the pupil will be fixed and dilated. The sphincter pupillae will be paralyzed. There will be no response to accommodation – the ciliary muscle is also paralyzed.
With aberrant nerve regeneration, the pupil will remain fixed, but it will constrict with attempted near vision. The constriction will be abnormal (“tonic”).
Late in the course of Adie syndrome, the pupil becomes small (as all pupils do with old age). It will still be “fixed” (it will not constrict to bright light) and it will continue to show abnormal, tonic constriction with attempted near vision.
The Adie pupil does not react to light, but it does react to accommodation. This is an example of “light-near dissociation”. All other causes of light-near dissociation involve the brainstem. They do not involve the ciliary ganglion, and they do not produce a tonic pupil. Irene Loewenfeld is generally credited for being the first physiologist to make this distinction.