Adie syndrome, also known as Holmes-Adie syndrome, is a

neurological disorder A neurological disorder is any disorder of the nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord or other nerves can result in a range of symptoms. Examples of symptoms include paralysis, muscle weakn ...

characterized by a tonically dilated pupil that reacts slowly to light but shows a more definite response to accommodation (i.e., light-near dissociation). It is frequently seen in females with absent

knee In humans and other primates, the knee joins the thigh with the leg and consists of two joints: one between the femur and tibia (tibiofemoral joint), and one between the femur and patella (patellofemoral joint). It is the largest joint in the hu ...

or ankle jerks and impaired sweating. The syndrome is caused by damage to the

postganglionic fiber In the autonomic nervous system, fibers from the ganglion to the effector organ are called postganglionic fibers. Neurotransmitters The neurotransmitters of postganglionic fibers differ: * In the parasympathetic division, neurons are ''choli ...

s of the parasympathetic innervation of the eye, usually by a viral or

bacterial Bacteria (; singular: bacterium) are ubiquitous, mostly free-living organisms often consisting of one biological cell. They constitute a large domain of prokaryotic microorganisms. Typically a few micrometres in length, bacteria were among ...

infection that causes

inflammation Inflammation (from la, wikt:en:inflammatio#Latin, inflammatio) is part of the complex biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or Irritation, irritants, and is a protective response involving im ...

, and affects the

pupil The pupil is a black hole located in the center of the iris of the eye that allows light to strike the retina.Cassin, B. and Solomon, S. (1990) ''Dictionary of Eye Terminology''. Gainesville, Florida: Triad Publishing Company. It appears black ...

of the eye and the

autonomic nervous system The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control ...

. It is named after the British neurologists

William John Adie William John Adie MD Ed., FRCP (31 October 1886 – 17 March 1935) was an Australian-born British physician and neurologist known for describing the Adie syndrome and narcolepsy. Biography William Adie was born in Geelong, Victoria, Australia, ...

and

Gordon Morgan Holmes Sir Gordon Morgan Holmes, (22 February 1876 – 29 December 1965) was an Anglo-Irish neurologist. He is best known for carrying out pioneering research into the cerebellum and the visual cortex. Education He was born as the son of a Louth far ...

, who independently described the same disease in 1931.

Signs and symptoms

Adie syndrome presents with three hallmark symptoms, namely at least one abnormally dilated pupil (

mydriasis Mydriasis is the dilation of the pupil, usually having a non-physiological cause, or sometimes a physiological pupillary response. Non-physiological causes of mydriasis include disease, trauma, or the use of certain types of drugs. Normally, as ...

) which does not constrict in response to light, loss of deep tendon reflexes, and abnormalities of sweating. Other signs may include

hyperopia Far-sightedness, also known as long-sightedness, hypermetropia, or hyperopia, is a condition of the eye where distant objects are seen clearly but near objects appear blurred. This blurred effect is due to incoming light being focused behind, i ...

due to accommodative

paresis In medicine, paresis () is a condition typified by a weakness of voluntary movement, or by partial loss of voluntary movement or by impaired movement. When used without qualifiers, it usually refers to the limbs, but it can also be used to desc ...

photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...

and difficulty reading. Some individuals with Adie syndrome may also have cardiovascular abnormalities.

Pathophysiology

Pupillary symptoms of Holmes–Adie syndrome are thought to be the result of a viral or bacterial infection that causes inflammation and damage to neurons in the

ciliary ganglion The ciliary ganglion is a bundle of nerve parasympathetic ganglion located just behind the eye in the posterior orbit. It is 1–2 mm in diameter and in humans contains approximately 2,500 neurons. The ganglion contains postganglionic parasympath ...

, located in the posterior orbit, that provides parasympathetic control of eye constriction. Additionally, patients with Holmes-Adie Syndrome can also experience problems with autonomic control of the body. This second set of symptoms is caused by damage to the

dorsal root ganglia A dorsal root ganglion (or spinal ganglion; also known as a posterior root ganglion) is a cluster of neurons (a ganglion) in a dorsal root of a spinal nerve. The cell bodies of sensory neurons known as first-order neurons are located in the do ...

of the

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue, which extends from the medulla oblongata in the brainstem to the lumbar region of the vertebral column (backbone). The backbone encloses the central canal of the spi ...

. Adie's pupil is supersensitive to ACh so a muscarinic agonist (e.g.

pilocarpine Pilocarpine is a medication used to reduce pressure inside the eye and treat dry mouth. As eye drops it is used to manage angle closure glaucoma until surgery can be performed, ocular hypertension, primary open angle glaucoma, and to bring abou ...

) whose dose would not be able to cause pupillary constriction in a normal patient, would cause it in a patient with Adie's Syndrome. The circuitry for the pupillary constriction does not descend below the upper midbrain, henceforth impaired pupillary constriction is extremely important to detect as it can be an early sign of brainstem herniation.

Diagnosis

Clinical exam may reveal sectoral paresis of the iris sphincter or vermiform iris movements. The tonic pupil may become smaller (miotic) over time which is referred to as "little old Adie's". Testing with low dose (1/8%)

may constrict the tonic pupil due to

cholinergic Cholinergic agents are compounds which mimic the action of acetylcholine and/or butyrylcholine. In general, the word "choline" describes the various quaternary ammonium salts containing the ''N'',''N'',''N''-trimethylethanolammonium cation. F ...

denervation supersensitivity Denervation supersensitivity/Denervation hypersensitivity is the sharp increase of sensitivity of postsynaptic membranes to a chemical transmitter after denervation Denervation is any loss of nerve supply regardless of the cause. If the nerves l ...

. A normal pupil will not constrict with the dilute dose of pilocarpine. CT scans and

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...

scans may be useful in the diagnostic testing of focal hypoactive reflexes.

Treatment

The usual treatment of a standardised Adie syndrome is to prescribe reading glasses to correct for impairment of the eye(s).

Pilocarpine Pilocarpine is a medication used to reduce pressure inside the eye and treat dry mouth. As eye drops it is used to manage angle closure glaucoma until surgery can be performed, ocular hypertension, primary open angle glaucoma, and to bring abou ...

drops may be administered as a treatment as well as a diagnostic measure. Thoracic sympathectomy is the definitive treatment of

diaphoresis Perspiration, also known as sweating, is the production of fluids secreted by the sweat glands in the skin of mammals. Two types of sweat glands can be found in humans: eccrine glands and apocrine glands. The eccrine sweat glands are distribut ...

, if the condition is not treatable by

drug therapy Pharmacotherapy is therapy using pharmaceutical drugs, as distinguished from therapy using surgery (surgical therapy), radiation (radiation therapy), movement (physical therapy), or other modes. Among physicians, sometimes the term ''medical ther ...

Prognosis

Adie's syndrome is not life-threatening or disabling. As such, there is no mortality rate relating to the condition; however, loss of deep tendon reflexes is permanent and may progress over time.

Epidemiology

It most commonly affects younger women (2.6:1 female preponderance) and is unilateral in 80% of cases. Average age of onset is 32 years.

References

External links

{{Eye pathology Neurological disorders Syndromes affecting the eye Syndromes affecting the nervous system Human pupil