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Adie syndrome

Description

Based on the search results, here is a summary of Adie syndrome:

Adie syndrome, also known as Holmes-Adie syndrome, is a rare neurological disorder that affects the pupil of the eye and the autonomic nervous system. The main features of Adie syndrome are:

  1. Tonic pupil: One pupil is larger than the other and reacts slowly to light.
  2. Absent tendon reflexes: Deep tendon reflexes, such as knee or ankle jerks, are absent.
  3. Impaired sweating: Some people with Adie syndrome may experience impaired sweating.

Adie syndrome typically affects young adults between the ages of 25 and 45, and is more common in women than men. The exact cause of the condition is unknown, but it is thought to be related to damage to the postganglionic fibers of the autonomic nervous system.

Symptoms may include blurring and sensitivity to light due to the abnormal pupil response. Treatment options are limited, and the prognosis for Adie syndrome is generally good, with most people experiencing a gradual improvement in symptoms over time.

Additional Characteristics

  • Tonic pupil
  • Absent tendon reflexes
  • Impaired sweating

Signs and Symptoms

Adie Syndrome Signs and Symptoms

Adie syndrome, also known as Holmes-Adie syndrome, is a rare neurological condition that affects the eyes and autonomic nervous system. The symptoms of this condition can vary from person to person, but here are some common signs and symptoms:

  • Abnormally dilated pupil: One or both pupils may be larger than normal, and they may not constrict in response to light.
  • Loss of deep tendon reflexes: People with Adie syndrome often experience a loss of deep tendon reflexes, which can affect the tendons in the arms and legs.
  • Abnormalities of sweating: Some individuals with Adie syndrome may experience excessive sweating or an impaired ability to sweat normally (anhidrosis).
  • Difficulty reading: The affected eye may have trouble focusing for near tasks, making it difficult to read or perform other close-up activities.
  • Increased sensitivity to light: People with Adie syndrome may be more sensitive to bright lights and may experience photophobia.
  • Blurry vision: Some individuals may experience blurry vision in the affected eye, which can affect both near and far vision.

These symptoms are often permanent and can vary in severity from person to person. While Adie syndrome is not life-threatening or disabling, it can be a challenge to manage and may require ongoing treatment and care.

References:

  • [1] Symptoms of associated conditions may be present such as infections, autoimmune diseases, or malignancy. Evaluation.
  • [2] One pupil that is larger than the other; A pupil that fails to get smaller in bright light conditions; Blurry vision; Light sensitivity; difficulty reading (with Adie’s pupil, the eye has a hard time focusing for near tasks) Rarely, both eyes are affected.
  • [3] Other signs may include hyperopia due to accommodative paresis, photophobia and difficulty reading. Some individuals with Adie syndrome may also have cardiovascular abnormalities.
  • [4] Common signs and symptoms of Adie Syndrome include: • Slow pupillary reaction to stimuli such as light • Dilated pupils (but in some individuals, it may be constricted) • Difficulty reading • Increased sensitivity to light • Lack of reflexes (in the tendon, ankle, and knee) • Excessive sweating.

Additional Symptoms

  • Blurry vision
  • Increased sensitivity to light
  • Loss of deep tendon reflexes
  • Abnormalities of sweating
  • Difficulty reading
  • dilated pupil

Diagnostic Tests

Diagnostic Tests for Adie Syndrome

Adie syndrome, also known as Holmes-Adie syndrome, can be diagnosed through a combination of clinical evaluation and diagnostic tests.

  • Clinical Evaluation: A thorough clinical evaluation by an ophthalmologist is recommended to diagnose Adie syndrome. This includes a detailed patient history and a complete eye examination.
  • Pilocarpine Test: The pilocarpine test is a useful tool in diagnosing Adie syndrome. Pilocarpine, given in the form of eye drops, causes the pupils to constrict. In individuals with Adie syndrome, the pupil will constrict in response to dilute pilocarpine, but not in response to light.
  • Slit Lamp Examination: An ophthalmologist may perform a slit lamp examination to diagnose Adie syndrome. This involves shining a bright light through the eye and examining the pupil for any abnormalities.
  • Deep Tendon Reflexes: Testing of deep tendon reflexes in the knee and ankle can be helpful in diagnosing Adie's syndrome, as these reflexes are often markedly diminished or absent in individuals with this condition.

Additional Tests

While the diagnosis of Adie's tonic pupil is clinical, other causes of tonic pupil must be ruled out. This may involve:

  • Syphilis Serologies: Checking syphilis serologies to rule out any potential underlying conditions.
  • Thorough History: Obtaining a thorough history for diabetes, trauma, or other neurological diseases such as Charcot-Marie-Tooth disease or Miller-Fisher syndrome.

References

  • [4] Adie's tonic pupil is most commonly an idiopathic condition. It is often diagnosed in young adults between the ages of 25 and 45 years.
  • [1] A complete eye examination by an ophthalmologist is recommended to diagnose Adie syndrome.
  • [20] Testing of deep tendon reflexes in the knee and ankle can be helpful in diagnosing Adie's syndrome.

Additional Diagnostic Tests

  • Slit Lamp Examination
  • Clinical Evaluation
  • Pilocarpine Test
  • Deep Tendon Reflexes
  • Syphilis Serologies
  • Thorough History

Treatment

Treatment Options for Adie Syndrome

Adie syndrome, also known as Holmes-Adie syndrome, can be treated in various ways to manage its symptoms. While there is no cure for the condition, several treatment options are available to alleviate its effects.

  • Pilocarpine drops: Pilocarpine is a medication that can help constrict the pupil and reduce photophobia (sensitivity to light). It may also help with occasional excessive sweating.
  • Reading glasses: In most instances, treatment will not be necessary. However, reading glasses may be prescribed to correct for impairment of the eye(s).
  • Surgery: Surgery to sever the involved sympathetic nerve may reduce excessive sweating in some cases.

Other Medications

Certain medications can cause a person's pupil to dilate or delay the response of eye muscles to light and darkness. These include:

  • Transdermal scopolamine (a drug for motion sickness)
  • Other drugs that can affect the autonomic nervous system

Treatment Focus

Most treatment focuses on managing symptoms, rather than curing the condition. Treatment options may vary depending on individual cases.

  • Symptomatic relief: Medications like pilocarpine and physostigmine salicylate have been used to provide symptomatic relief in some patients.
  • Management of symptoms: Treatment often involves managing symptoms such as photophobia, excessive sweating, and impaired vision.

References

[1] Pilocarpine drops may be administered as a treatment as well as a diagnostic measure. [4] Reading glasses may be prescribed to correct for impairment of the eye(s). [6] Pilocarpine (0.1% three times daily) or another dilute miotic may help with photophobia and occasionally with excessive sweating.

Recommended Medications

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Differential Diagnosis

The differential diagnosis of Adie syndrome involves identifying other conditions that may present with similar symptoms, such as light-near dissociation and tonically dilated pupils.

Conditions to Consider

  • Argyll Robertson pupil: a condition found in late-stage syphilis characterized by small and irregular pupils that do not react to light but constrict during focusing [12][11].
  • Oculomotor nerve palsy: a condition that may be associated with mydriasis (pupil dilation) and can affect the movement of the eye [2][11].
  • Pharmacological dilation: a condition caused by the use of certain medications that can lead to pupil dilation without light-near dissociation [12].
  • Congenital mydriasis: a rare condition present at birth characterized by permanently dilated pupils [12].

Other Conditions

  • Third cranial nerve palsy: a condition affecting the third cranial nerve, which can cause pupillary abnormalities and other symptoms [13].
  • Argyll Robertson pupil (small and irregular pupils that do not react to light but constrict during focusing) [13].
  • Horner syndrome: a condition characterized by a drooping eyelid, constricted pupil, and loss of sweating [13].

Key Points

  • Adie syndrome is a relatively common neurological disorder of unknown etiology comprising unilateral or bilateral tonically dilated pupils with light-near dissociation and tendon areflexia [14].
  • The differential diagnosis of Adie syndrome requires careful consideration of other conditions that may present with similar symptoms.
  • A thorough examination, including special diagnostic eye drops, can help diagnose Adie's pupil [15].

References:

[12] - Context result 2 [11] - Context result 11 [13] - Context result 13 [14] - Context result 14 [15] - Context result 15

Additional Information

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A syndrome that is a neurological disorder affecting the pupil of the eye and the autonomic nervous system. It is characterized by one eye with a pupil that is larger than normal and constricts slowly in bright light (tonic pupil), along with the absence of deep tendon reflexes, usually in the Achilles tendon.
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