Holmes-Adie syndrome entities

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Clinic

  • Adie syndrome, also known as Holmes-Adie syndrome
  • It is a neurological disorder 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 or ankle jerks and impaired sweating.
  • The syndrome is caused by damage to the postganglionic fibers of the parasympathetic innervation of the eye, usually by a viral or bacterial infection that causes inflammation, and affects the pupil of the eye and the autonomic nervous system.


Signs / Symptoms

  • Mydriasis which does not constrict in response to light
  • Loss of deep tendon reflexes
  • Abnormalities of sweating
  • Hyperopia due to accommodative paresis
  • Photophobia
  • Difficulty reading
  • Some may also have cardiovascular abnormalities


Pathophysiology

  • Viral or bacterial infection that causes inflammation and damage to neurons in the ciliary ganglion, located in the posterior orbit, that provides parasympathetic control of eye constriction.
  • Problems with autonomic control of the body due to damage of dorsal root ganglia of the spinal cord. Adie's pupil is supersensitive to ACh so a muscarinic agonist (e.g. pilocarpine) 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.


Entities

  • Dilated pupils
  • Loss of deep Tendon reflex
  • Perspiration

Miasm: RBS