Examine this patient's eyes.
· The patient is usually a young woman.
· Impaired sweating.
· Onset may be acute.
· The pupil is large, regular, irregular, oval, or circular.
· The pupil will react sluggishly or fail to react to light. However, if a strong and persistent stimulus is used it can be shown that
the pupil contracts excessively to a very small size and when the stimulus is removed it returns to its former size gradually - this
is known as the 'myotonic' pupil.
· Delayed constriction in response to near vision.
· Delayed re-dilatation after near vision.
· Accommodation impaired.
· There is segmental palsy and segmental spontaneous movement of iris (Lancet 2000: 356:1760-1 ).
Proceed as follows:
Check the ankle jerks and tell the examiner that you expect them to be absent.
This woman has a sluggishly reacting pupil with absent ankle jerks (lesion) due to Holmes-Adie syndrome (aetiology), which is a
benign disorder (functional status).
what is lc the significance of this rnnditinn?
It is benign and must not be mistaken for Argyll Robertson pupil.
What are the causes of a dilated pupil? · Mydriatic eye drops. · Third nerve lesion.
· Holmes-Adie syndrome (degeneration of the nerve to the ciliary ganglion).
· Lens implant, iridectomy.
· Blunt trauma to the iris (pupil may be irregularly dilated and reacts sluggishly to light - post-traumatic iridoplegia).
· Drug overdose, e.g. cocaine, amphetamine.
· Poisoning, e.g. belladonna.
· Deep coma, death.
What are the causes of a small pupil?
· Old age. · Argyll Robertson pupil.
· Pilocarpine eye drops. · Pontine lesion.
· Homer's syndrome. · Narcotics.
What do you know about the factors that control the size of the pupil?
The sphincter muscle of the pupil (causing miosis) is supplied by the cholinergic parasympathetic nerves, whereas the dilator of the
pupil (causing mydriasis) is supplied by noradrenergic sympathetic fibres. The parasympathetic fibres arise from the
Edinger-Westphal nucleus. They travel by the third cranial nerve to the ciliary ganglion. Postganglionic fibres arise from the ciliary
ganglion and are distributed by the ciliary nerve.
Where is the lesion in Adie's tonic pupil?
There is damage to the parasympathetic fibres within the ciliary ganglion.
What is the difference between Adie's tonic pupil and Holmes-Adie syndrome?
Adie's tonic pupil with absent deep tendon jerks is called Holmes-Adie syndrome.
How does the Holmes-Adie pupil react to weak pilocarpine (0.125%) or 2.5% methacholine?
It usually constricts, indicating a supersensitivity to acetylcholine secondary to parasympathetic denervation resulting from
degeneration of postganglionic neurons and neurons in the ciliary ganglion. There is no effect on a normal pupil.
Which conditions may accompany this syndrome?
Dysautonomias such as:
· Ross's syndrome (segmental loss of sweating).
· Cardiac arrhythmias.
Sir Gordon M. Holmes (1876-1965) and William J. Adie (1886-1935) were London neurologists who described the condition independently in 1931 (Holmes: Trans Ophthalmol
Soc UK 1931; 41: 209; Ad ie: Brain 1932; 55: 98; Br J Ophthalmo11932; 16: 449). The name Holmes-Adie syndrome was coined by Bramwell in 1936. Saenger and
Strasburger indepedently described the syndrome in 1902; it was first recorded in the English literature by Markus in 1905.