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Tonic Pupil

Tonic Pupil. Tonic Pupil. Damage to ciliary ganglion or short posterior ciliary nerves Causes denervation of the ciliary body and iris sphincter muscle Initially characterized by: Paralysis of accommodation Dilated pupil that reacts poorly to light

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Tonic Pupil

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  1. Tonic Pupil

  2. Tonic Pupil • Damage to ciliary ganglion or short posterior ciliary nerves • Causes denervation of the ciliary body and iris sphincter muscle • Initially characterized by: • Paralysis of accommodation • Dilated pupil that reacts poorly to light • Ultimately reinnervation (normal and aberrant) occurs to CB and iris sphincter, however unequally as there are a greater number of fibers to CB (97% to 3%) • Results in: • Greater near than light response  light near dissociation • Strong tonic constriction to near stimulus • Slow tonic redilation after constriction • Denervation supersensitivity

  3. Ciliary Ganglion http://www.studyblue.com/notes/note/n/an3-05-cranial-nerves-i-ii-iii-iv-vi-v1-v2-v3/deck/1101203 http://radiopaedia.org/images/171

  4. Ciliary Body & Iris Innervation Iris Sphincter Iris Dilator http://www.mbfbioscience.com/blog/tag/customer-research/

  5. Tonic Pupil • Typically divided into three categories • Local • Neuropathic • Adie (Adie-Holmes) syndrome • Ross Syndrome

  6. Local Tonic Pupil • Typically unilateral • Due to orbital or systemic lesions that affect the ciliary ganglion or short ciliary nerves in isolation • Herpes zoster, chickenpox, measles, diphtheria, syphilis (both congenital and acquired), Lyme disease, sarcoidosis, scarlet fever, pertussis, smallpox, influenza, sinusitis, Vogt- Koyanagi-Harada syndrome, rheumatoid arthritis, polyarteritis nodosa, giant cell arteritis, migraine, lymphomatoid granulomatosis, viral hepatitis, choroiditis, primary and metastatic choroidaland orbital tumors, blunt injury to the globe, intraocular siderosis from foreign body, penetrating orbital injury, trauma from surgery in the orbit or globe, PRP, RBB

  7. Neuropathic Tonic Pupil • Usually bilateral involvement • Represents a manifestation of a widespread, peripheral, and autonomic neuropathy that also involves the ciliary ganglion, the short ciliary nerves, or both • Syphilis, chronic alcoholism, diabetes mellitus, amyloidosis, systemic lupus erythematosus, Sjögrensyndrome, some of the spinocerebellar degenerations, hereditary motor-sensory neuropathy (Charcot- Marie-Tooth disease), Landry-Guillain-Barre´ syndrome, and the Miller Fisher syndrome. Dysautonomias such as subacuteautonomic neuropathy, Shy-Drager syndrome, and Ross syndrome, paraneoplastic syndrome

  8. Adie-Holmes Syndrome • First mentioned by Piltz, who described a bilateral, tonic light reflex in patients with general paresis in 1899 • Described by Gordon Holmes and William Adie in 1931 • Uncommon, idiopathic condition characterized by tonic pupil, absent deep tendon reflexes • Generally develops in otherwise healthy persons • Almost always sporadic • 80 % unilateral • Typically present between 20–50 years of age • 70% women

  9. Adie-Holmes Syndrome • Clinical symptoms related to iridoplegiaor cycloplegia • Photophobia, difficult dark adaptation • Blurred near vision, asthenopia • Exam findings • Acutely: internal ophthalmoplegia • Dilated unreactive pupil to light or near stimuli • Sectoral palsy of iris sphincter  vermiform movements (90%) • Chronically: • 97% of postganglionic fibers leaving CG innervate CB • Normal and aberrant reinervation of iris sphincter  slow accommodative constriction and tonic redilation

  10. Adie-Holmes Syndrome • Denervation hypersensitivity • Enhanced response to chemical stimulation after interruption of synaptic function • Occurs in 80% of pts within days to weeks of injury to CG • Can also be seen in preganglionic damage in CN III • Characterized by constriction to dilute pilocarpine • Over time dilated pupil constricts • Sustained firing of reinnervated fibers to iris sphincter (Kardon et al.) Walsh and Hoyt Clinical Neuro-ophthalmology

  11. Adie-Holmes Syndrome • Other findings: • Altered corneal sensation • Absent deep tendon reflexes • Demonstrated in almost 90% of patients • Degeneration of cell bodies in the dorsal root ganglia similar to that seen in CG • Loss of large-diameter afferent fibers or impairment in their synaptic connections to motor neurons

  12. Adie-Holmes Syndrome • Decreased hypersensitivity over time • 4% involvement of other eye per year • No other vision or life threatening neurologic processes

  13. Ross Syndrome • In 1958, Ross described a 32-year-old man with Adie syndrome who had become progressively anhidrotic over 12 years • Triad of tonic pupils, absent muscle-stretch reflexes, and progressive segmental impairment of sweating • Severe degeneration and loss of cholinergic sudomotorneurons and fibers • Other autonomic deficits seen: • orthostatic hypotension, abnormalities of cardiovascular reflexes, chronic cough • Can also be seen in Adie-Holmes syndrome

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