Adie syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Muneeb, MBBS[2] Synonyms and keywords: Holmes-Adie Syndrome; Syndrome, Holmes-Adie; Syndrome, Adie's; Syndrome, Adie; Poorly Reacting Pupil; Holmes Adie Syndrome; Pupil, Poorly Reacting; Adie's Syndrome; Poorly Reacting Pupils; Pupils, Poorly Reacting

Historical Perspective

• [[Adie] syndrome]] was first discovered by William John Adie, a British neurologist, and Sir Gordon Morgan Holmes, an Irish neurologist in 1931. This syndrome was named after these 2 neurologists.
• In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of adie syndrome is characterized by damage to ciliary ganglion and dorsal root ganglion of spinal cord. Ciliary ganglion provides innervation to iris and ciliary muscle, thus controlling pupillary function and accommodation. Ciliary ganglion neurons responsible for accommodation are in a much greater number than the neurons controlling pupillary function. In adie syndrome, neurons/nerve fibers controlling both accommodation and pupillary function are damaged. But because of the large no. of neruons, accommodation is unaffected or minimally affected in adie syndrome. Some of the damaged nerve fibers start to regenerate. As nerve fibers innervating pupillary sphincter muscle are less in no. so very few of these fibers regenerate and are insufficient to restore the normal pupillary function. On the other hand, nerve fibers to the ciliary muscle also regenerate but may do so in an inappropriate fashion, providing innervation to pupillary sphincter muscle along with ciliary muscle. As the pupillary sphincter muscle is now innervated by fibers originally responsible for accommodation(which is a slower process than pupillary light reaction) so pupillary light response is impaired.
• Loss of deep tendon reflexes in adie syndrome is caused by damage to dorsal root ganglio of spinal cord.
• On microscopic histopathological analysis, degeneration of ciliary ganglion and atrophy of sphincter pupillae muscle, are characteristic findings of adie syndrome. Other findings associated with adie syndrome include degenration of sacral dorsal root ganglion, superior cervical ganglion and sciatic nerve.

Causes

Most commonly the cause of Adie syndrome is unknown(idiopathic). Less common causes of adie syndrome include infections like HIV, syphilis, varicella, lyme's disease, Human parvovirus-B19, autoimmune diseases like amyloidosis, sarcoidosis, guillain-barre syndrome, sjogren syndrome, polyarterities nodosa, vogt-koyanagi-haraga disease, ischemia caused by giant cell arteritis, migraine, lymphatoid granulomatosis, neuromuscular diseases like Lambert eaten syndrome, tumors affecting the orbit or choroid, orbital surgery, cardiovascular diseases, general anesthesia. ,anti-hu antibody.

Differentiating adie syndrome from other Diseases

• Adie syndrome must be differentiated from other diseases that cause light near dissocation or miosis, or mydriasis, such as:

• Argyl-Robertson pupil
• 3rd cranial nerve palsy
• Horner's syndrome
• Ross syndrome
• Harlequin syndrome

Epidemiology and Demographics

• The prevalence of adie syndrome is approximately 2 per 1000 individuals.
• The annual incidence of adie syndrome is estimated to be [4.7] cases per 100,000 individuals.

Age

• Adie syndrome is more commonly observed among patients aged 25 to 45 years old.

Gender

• Females are more commonly affected with Adie pupil than males.

Race

• There is no racial predilection for [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

History and Symptoms

• Symptoms of adie syndrome may include the following:

• larger pupil size of one eye than the other eye
• sweating abnormalities
• reading difficulty
• Photophobia
• [symptom 5]
• [symptom 6]

Physical Examination

• Physical examination may be remarkable for:

• absent or impaired pupillary light reflex
• absent deep tendon reflexes (most commonly achilles tendon is involved).
• Tonic near response of pupil with Light near dissociation.
• Segmental palsy of sphincter
• Irregular shape of pupil
• anisometropia
• hyperopia
• orthostatic hypotension
• hypersensitivity to cholinergic agonists

• Ross syndrome is a variant of adie syndrome characterized by triad of tonic pupil, absent deep tendon reflexes, abnormal sweating.

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

Low dose pilocarpine test may be helpful in the diagnosis adie syndrome. Finding suggestive of adie pupil includes an exaggerated miotic reaction of the affected pupil as compared to the normal pupil. This exaggerated response to low dose pilocarpine occurs as a consequence of cholinergic denervation hypersensitivity of the affected pupil. Normal pupils do not respond to such low doses of pilocarpine. To rule out Ross syndrome, sweating abnormalities can be checked by using starch iodine test and spoon test.

Treatment

Medical Therapy

• There is no treatment required for idiopathic adie syndrome. If any etiology is found, treatment should be directed against that etiology. Topical low dose pilocarpine or physostigmine can be used in symptomatic patients. For patients with accommodative paresis reading glasses should be prescribed.

Surgery

• Thoracic sympathectomy can be performed for patients with excessive sweating.

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].