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Holmes-Adie Syndrome
Ade Wijaya, MD – October 2020
Introduction
• Holmes- Adie Syndrome / Adie Syndrome
• Introduced in 1931 by William John Adie, the British neurologist
of Australian descent, and Sir Gordon Morgan Holmes, an Irish
neurologist
• Relatively common neurological disorder of unknown etiology
Adie WJ. PSEUDO-ARGYLL ROBERTSON PUPILS WITH ABSENT TENDON REFLEXES: A BENIGN DISORDER SIMULATING TABES DORSALIS. Br Med J.1931 May 30;1(3673):928-30.
Clinical Presentation
Unilateral or bilateral tonically
dilated pupils
Light-near dissociation
Tendon areflexia
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Can be associated with hypohidrosis, in this case, it is known as Ross syndrome
Epidemiology
• Incidence: 4.7/100,000 population/year
• Prevalence: 2/1000 population
• Age: 25 to 45 (mean age of 32 years)
• Female > Male
• 80 % unilateral
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Etiology : Mostly idiopathic
May rarely be caused by local
disorders involving the orbit that affect the
ciliary ganglion including
• Infections such as syphilis,
varicella, human parvovirus-B19, human
immunodeficiency virus, and Lyme disease
• Ischemia due to lymphomatoid
granulomatosis, migraine, and giant cell
arteritis
• Autoimmune disorders such as Sjogren
syndrome, polyarteritis nodosa,
sarcoidosis, amyloidosis, Guillain-Barre
syndrome, and Vogt-Koyanagi-Harada
disease
• Cardiovascular disorders
• Local or general anesthesia
• Orbital or choroidal tumors
• Orbital surgery for orbital floor fractures
• Neuromuscular conditions such as
Lambert-Eaton myasthenic syndrome,
• Paraneoplastic in association with anti-Hu
antibodies
• Retinal photocoagulation that results in
damage to the ciliary nerves within the
suprachoroidal space.
• Familial dysautonomia
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Pathophysiology
• Damage to the ciliary ganglion most commonly by an inflammatory
process.
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Oculomotor Nerve Pathway
Histopathology
• Ciliary ganglion on the affected side has degenerated with associated
partial atrophy of the sphincter pupillae muscle
• There was also minimal neuronal degeneration affecting the superior
cervical ganglion, the sacral dorsal root ganglion, and the sciatic nerve
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Diagnosis
• History and physical
• Pharmacologic Testing: cholinergic denervation supersensitivity with low
concentration pilocarpine
• Sweat testing
• Etiologic Evaluation
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Differential Diagnosis
• Argyll Robertson pupil
• Ross and Harlequin syndromes
• Horner Syndrome
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Treatment
• Most do not require any treatment
• Treat underlying conditions if any
• Reading glasses
• Topical low-dose pilocarpine or physostigmine drops
• Thoracic sympathectomy is the treatment of choice for diaphoresis
• Interdisciplinary approach
Serra Mitjans M, Callejas Pérez MA, Valls Solé J, Grimalt Santacana R, Rubio Garay M, Iglesias Sentís M. [Surgical treatment for compensatory hyperhidrosis in Adie syndrome].Arch. Bronconeumol. 2004 Feb;40(2):97-9
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Prognosis and Complication
• Good prognosis
• Does not have a progressive course
• The pupil light reaction becomes weaker over time with an increasing
light-near dissociation, and the pupil becomes smaller with time ("little
old Adie")
• May rarely result in angle-closure glaucoma
• May cause amblyopia in children
Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
Summary
• Triad: unilateral or bilateral tonically dilated pupils with light-near
dissociation and tendon areflexia
• Mostly idiopathic
• Good prognosis; no progression
• May rarely develop into angle-closure glaucoma
• Interdisciplinary approach
THANK YOU

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Holmes Adie Syndrome

  • 1. Holmes-Adie Syndrome Ade Wijaya, MD – October 2020
  • 2. Introduction • Holmes- Adie Syndrome / Adie Syndrome • Introduced in 1931 by William John Adie, the British neurologist of Australian descent, and Sir Gordon Morgan Holmes, an Irish neurologist • Relatively common neurological disorder of unknown etiology Adie WJ. PSEUDO-ARGYLL ROBERTSON PUPILS WITH ABSENT TENDON REFLEXES: A BENIGN DISORDER SIMULATING TABES DORSALIS. Br Med J.1931 May 30;1(3673):928-30.
  • 3. Clinical Presentation Unilateral or bilateral tonically dilated pupils Light-near dissociation Tendon areflexia Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Can be associated with hypohidrosis, in this case, it is known as Ross syndrome
  • 4. Epidemiology • Incidence: 4.7/100,000 population/year • Prevalence: 2/1000 population • Age: 25 to 45 (mean age of 32 years) • Female > Male • 80 % unilateral Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 5. Etiology : Mostly idiopathic May rarely be caused by local disorders involving the orbit that affect the ciliary ganglion including • Infections such as syphilis, varicella, human parvovirus-B19, human immunodeficiency virus, and Lyme disease • Ischemia due to lymphomatoid granulomatosis, migraine, and giant cell arteritis • Autoimmune disorders such as Sjogren syndrome, polyarteritis nodosa, sarcoidosis, amyloidosis, Guillain-Barre syndrome, and Vogt-Koyanagi-Harada disease • Cardiovascular disorders • Local or general anesthesia • Orbital or choroidal tumors • Orbital surgery for orbital floor fractures • Neuromuscular conditions such as Lambert-Eaton myasthenic syndrome, • Paraneoplastic in association with anti-Hu antibodies • Retinal photocoagulation that results in damage to the ciliary nerves within the suprachoroidal space. • Familial dysautonomia Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 6. Pathophysiology • Damage to the ciliary ganglion most commonly by an inflammatory process. Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 8. Histopathology • Ciliary ganglion on the affected side has degenerated with associated partial atrophy of the sphincter pupillae muscle • There was also minimal neuronal degeneration affecting the superior cervical ganglion, the sacral dorsal root ganglion, and the sciatic nerve Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 9. Diagnosis • History and physical • Pharmacologic Testing: cholinergic denervation supersensitivity with low concentration pilocarpine • Sweat testing • Etiologic Evaluation Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 10. Differential Diagnosis • Argyll Robertson pupil • Ross and Harlequin syndromes • Horner Syndrome Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 11. Treatment • Most do not require any treatment • Treat underlying conditions if any • Reading glasses • Topical low-dose pilocarpine or physostigmine drops • Thoracic sympathectomy is the treatment of choice for diaphoresis • Interdisciplinary approach Serra Mitjans M, Callejas Pérez MA, Valls Solé J, Grimalt Santacana R, Rubio Garay M, Iglesias Sentís M. [Surgical treatment for compensatory hyperhidrosis in Adie syndrome].Arch. Bronconeumol. 2004 Feb;40(2):97-9 Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 12. Prognosis and Complication • Good prognosis • Does not have a progressive course • The pupil light reaction becomes weaker over time with an increasing light-near dissociation, and the pupil becomes smaller with time ("little old Adie") • May rarely result in angle-closure glaucoma • May cause amblyopia in children Sarao MS, Elnahry AG, Sharma S. Adie Syndrome (Tonic Pupil Syndrome) [Updated 2020 Jul 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020
  • 13. Summary • Triad: unilateral or bilateral tonically dilated pupils with light-near dissociation and tendon areflexia • Mostly idiopathic • Good prognosis; no progression • May rarely develop into angle-closure glaucoma • Interdisciplinary approach