Holmes-Adie syndrome is a condition that affects the eye, more specifically the pupillary parasympathetic innervation that is responsible for constriction and dilation of the pupils, as well as the dorsal root ganglion involved in the reflexes of the autonomic nervous system. In an individual with Holmes-Adie syndrome, the pupil of the affected eye (or eyes) is larger than normal at all times and very rarely, if ever, constricts in response to increased exposure to light.
The exact cause of Holmes-Adie syndrome is unknown; however, it is widely believed that it is typically caused by a viral infection, which leads to subsequent damage to the ciliary ganglion. The ciliary ganglia are in direct control of pupil constriction and dilation in response to changing exposure to light. This etiology is also present in the form of peripheral neuropathy of the dorsal root ganglion of the spinal cord, which is responsible for responses in the autonomic nervous system. This damage to the dorsal root ganglion and subsequent impairment of the autonomic nervous system is responsible for the associated areflexia, or absence of deep tendon reflexes. Another hypothesis for the cause of this condition is through the inheritance of an autosomal dominant trait. The largest demographic for the onset of the condition is women between the ages of 25 and 45.
Holmes-Adie syndrome is easily characterized by a symptom known as “Adie pupil,” which describes a dilated pupil in one or both eyes that does not constrict quickly in response to light. This inability to constrict the pupil can be problematic and may lead to damage of the retinal cells in the afflicted eye(s). Another common characteristic that is used in identifying Holmes-Adie syndrome is known as anisocoria, which is the process in which the pupil of one eye is larger than normal and constricts very slowly when exposed to bright light. This diagnostic anisocoria can be seen in the onset of Holmes-Adie syndrome, in which the symptoms begin in one eye, or in the reflexes on one side of the body, before gradually spreading to the other side to take effect on the body bilaterally. Visual and reflexive symptoms may develop at different times. Case study—based research has also identified a chronic dry cough associated with the disease, which is believed to be caused by a number of stimuli including neoplasms, inflammatory lung disease, respiratory tract infections, asthma, or even gastroesophageal reflux disease, although the exact onset of the chronic cough is still unknown.
Holmes-Adie syndrome can also be characterized through a number of associated symptoms such as orthostatic hypotension, segmental and generalized anhidrosis (inability to sweat, also known as Ross’s syndrome), carotid gustatory syndrome, impaired cardiovascular reflexes, and chronic diarrhea. Although Holmes-Adie syndrome is known to be a benign diagnosis, failure to treat it can lead to other troublesome symptoms as a result of the autonomic dysfunction, including hyperhidrosis (compensatory mechanism that causes excessive sweating), the inability to tolerate heat normally, and syncope (the loss of consciousness). Studies on patients afflicted with Holmes-Adie syndrome revealed that the patients’ chronic cough was exacerbated upon taking deep breaths or through hyperventilation, which is indicative of increased cough reflex sensitivity.
Treatment and Prevention
Because of the largely unknown etiology of this condition, there are no known preventive measures to protect from its onset. However, there are numerous treatment options for those who are diagnosed, including the use of reading glasses and pilocarpine drops to be applied three times per day in order to help constrict the overly dilated pupil. Unfortunately, the loss of deep tendon reflexes is permanent, so there is no treatment for this symptom of the ailment. Holmes-Adie syndrome is not life-threatening and does not cause any notable severe disability.
See also: Autonomic Nervous System; Blindness; Consensual Pupillary Light Reflex; Reflex; Visual System
Kimber, J., D. Mitchell, & C. Mathias. (1998). Chronic cough in the Holmes-Adie syndrome: Association in five cases with autonomic dysfunction. Journal of Neurology, Neurosurgery, and Psychiatry, 65(4), 583—586.
Millichap, J. Gordon. (2013). Holmes-Adie syndrome. In Neurological syndromes: A clinical guide to symptoms and diagnosis (p. 96). New York, NY: Springer.
National Institute of Neurological Disorders and Stroke (NINDS). (2011). NINDS Holmes-Adie syndrome information page. Retrieved from http://www.ninds.nih.gov/disorders/holmes_adie/holmes_adie.htm
Siddiqui, Aazim A., Jonathan C. Clarke, & Andrzej Grzybowski. (2014). William John Adie: The man behind the syndrome. Clinical Experiment Ophthalmology, 42(8), 778—784.