eMedicine Specialties > Ophthalmology > Lid

Apraxia of Lid Opening

Author: Marta Ugarte, MBBS, PhD, DPhil, MRC(Ophth), Clinical Lecturer in Ophthalmology, University of Manchester, UK
Contributor Information and Disclosures

Updated: Jun 2, 2009

Introduction

Background

Apraxia of lid opening (ALO) is a nonparalytic motor abnormality “characterized by difficulty initiating the act of lid elevation after lid closure.”1 Controversy surrounds the designation of this syndrome. ALO is not strictly an apraxia or “inability to perform a motor action to command despite both an adequate understanding of the action and the elementary ability to carry out.”2 However, the designation ALO addresses the main feature of the condition (ie, the inability to open the eyes at will with preservation of the ability to open them and keep them open at other times);3 ALO may present in isolation or in association with blepharospasm,4,5 and the challenge lies in differentiating these 2 entities.

Pathophysiology

Although the underlying mechanisms have not been clearly elucidated, apraxia of lid opening (ALO) may be due to an abnormality in the supranuclear control of voluntary eyelid elevation, which requires the activation of the levator palpebrae superioris (LPS) and the concurrent inhibition of orbicularis oculi (OO) activity.

Electromyographic studies of the LPS and OO have demonstrated that the following may cause ALO:

  • Involuntary levator palpebrae inhibition (ILPI),6 either intermittent7 or prolonged8
  • Pretarsal OO persistent contraction9,6
  • ILPI (either intermittent or prolonged) and pretarsal OO persistent contraction10,6

The LPS is innervated bilaterally from the central caudal subdivision of the oculomotor (III) nucleus, and the OO is innervated unilaterally from the facial (VII) nucleus. The cortex, extrapyramidal motor systems, and rostral midbrain structures may control LPS motoneuron activity (see Media file 1).11

Diagram of the possible central pathways involved...

Diagram of the possible central pathways involved in the generation of inhibitory responses of the levator palpebrae superioris muscle. Caudal central nucleus (CCN), central caudal subdivision of the oculomotor (III) nucleus.

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Diagram of the possible central pathways involved...

Diagram of the possible central pathways involved in the generation of inhibitory responses of the levator palpebrae superioris muscle. Caudal central nucleus (CCN), central caudal subdivision of the oculomotor (III) nucleus.



Disturbances in burst, tonic, or pause neurons within any of the above-mentioned premotor structures may result in abnormal inhibitor inputs onto the LPS motoneurons. Some patients may experience an associated disruption in reciprocal activation of the LPS and OO.

Frequency

International

Apraxia of lid opening (ALO) has been reported in 7%,5 10%,4 and 55%12 of patients with blepharospasm. Benign essential blepharospasm has a prevalence of 12 per million in Japan,13 17 per million in Rochester, MN, in the United States,14 30 per million in Northern England,15 and 36 per million in the Epidemiologic Study of Dystonia in Europe.16 The wide variation in these figures may reflect sample bias.

Mortality/Morbidity

Apraxia of lid opening (ALO) does not directly cause mortality. Morbidity is mostly related to the underlying disease and to the visual impairment in individuals with severe eye closure.

Race

Racial differences have not been reported.

Sex

Apraxia of lid opening appears to be more common in women than in men. In 1998, Defazio et al described 10 new individuals with apraxia of lid opening (ALO) and reviewed 11 cases previously mentioned in the literature.17 A female-to-male preponderance of 2:1 was found.

Age

The peak age of onset is in the sixth and seventh decades of life.

Clinical

History

Apraxia of lid opening (ALO) can present spontaneously in otherwise healthy individuals.6,17   

In patients with Parkinson disease, progressive supranuclear palsy, and Shy-Drager syndrome, Lepore and Duvoisin reported in 1985 that extrapyramidal symptoms preceded the inability to open the eyelids by a mean of 9.7 years.10  

ALO is typically a chronic disorder, but spontaneous remission has been reported in patients within 1 month of strokes involving the nondominant hemisphere.18,19,20  

In instances of ALO that may be drug induced, the symptoms have been reported to remit within 2 weeks of withdrawal of the agent;21 however, in one individual, the symptoms persisted for 7 months.22

Physical

Clinical signs include the following (video of an illustrative case may be seen in Media file 2):23  

  • Individuals experience difficulty opening the lids at will subsequent to voluntary or involuntary closure. The inability may be due to involuntary levator palpebrae inhibition (ILPI) or persistent pretarsal orbicularis oculi (OO) motor activity, which, in some instances, may be detectable electromyographically but not clinically.
  • The inability to reopen the lids is not evident during spontaneous or reflex blinking. The premotor pathways that regulate and control the state of voluntary activity of the levator palpebrae superioris (LPS) and make it parallel with OO inhibition differ, at least in part, from the pathways of spontaneous and reflex blinking.
  • The patient has no difficulty in keeping the lids open once they have been opened. LPS tonic activity is normal while the lids are open.
  • Intermittent involuntary closure of the eyes may occur in some patients, both in the presence and absence of spasmodic contractions of the OO in blepharospasm. ILPI not accompanied by blepharospasm may cause the eyelid to drop and to be kept closed as long as inhibition of the LPS activity persists.
  • Attempted eye opening can result in forceful contraction of the frontalis muscle, backward thrusting of the head, and delay in lid closure.
  • Different tricks may be used to help open the eyes, such as manual lifting of the lids, opening of the mouth, light touch of the temporal region, and massaging of the lids. The physiology of these maneuvers is unknown.
  • Some patients have been reported to have associated supranuclear limitation of eye movements.10
  • The coordination of eyelid movement is preserved.

Causes

Apraxia of lid opening (ALO) has been attributed to a variety of CNS lesions in various parts of the brain, including the following:

  • Nondominant hemisphere19
  • Medial frontal lobe24,25
  • Basal ganglia11
  • Rostral brainstem21,22

ALO has been described in association with certain CNS diseases: 

  • Progressive supranuclear palsy (PSP; one of the most common causes of ALO)26
  • Parkinson disease (another common cause of ALO)10,27,5,28,29
  • Idiopathic dystonias29
  • Hydrocephalus30
  • Motor neuron disease31
  • Dystonia due to kernicterus6
  • Choreoathetosis6
  • Huntington chorea1
  • Shy-Drager syndrome10
  • Postencephalitic parkinsonism10
  • Neuroacanthocytosis32  

In addition, the use of the following medications has been reported to induce ALO:1,10,26,33  

  • Lithium intoxication resulted in development of ALO. On withdrawal of lithium, symptoms remitted within 2 weeks.21  
  • Sulpiride may be associated with ALO; the condition lasted approximately 7 months after discontinuation of sulpiride.22  
  • An analog of meperidine (MPTP) has been attributed to the induction of parkinsonism associated with ALO.34  

A diagnosis of Wilson disease was made in one patient after he presented with a severe, intermittent inability to open his lids; the original diagnosis was ALO.35

More on Apraxia of Lid Opening

Overview: Apraxia of Lid Opening
Differential Diagnoses & Workup: Apraxia of Lid Opening
Treatment & Medication: Apraxia of Lid Opening
Follow-up: Apraxia of Lid Opening
Multimedia: Apraxia of Lid Opening
References
Further Reading
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References

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  4. Jordan DR, Anderson RL, Digre KB. Apraxia of lid opening in blepharospasm. Ophthalmic Surg. May 1990;21(5):331-4. [Medline].

  5. Krack P, Marion MH. "Apraxia of lid opening," a focal eyelid dystonia: clinical study of 32 patients. Mov Disord. Nov 1994;9(6):610-5. [Medline].

  6. Aramideh M, Bour LJ, Koelman JH, Speelman JD, Ongerboer de Visser BW. Abnormal eye movements in blepharospasm and involuntary levator palpebrae inhibition. Clinical and pathophysiological considerations. Brain. Dec 1994;117 (Pt 6):1457-74. [Medline].

  7. Esteban A, Gimenez-Roldan S. Involuntary closure of eyelids in parkinsonism. Electrophysiological evidence for prolonged inhibition of the levator palpebrae muscles. J Neurol Sci. Jul 1988;85(3):333-45. [Medline].

  8. Esteban A, Gimenez-Roldan S. [Nociceptive reflex of the orbicularis oculi. Study in normal subjects and in peripheral facial lesions]. Arch Neurobiol (Madr). Jul-Aug 1973;36(4):283-94. [Medline].

  9. Elston JS. A new variant of blepharospasm. J Neurol Neurosurg Psychiatry. May 1992;55(5):369-71. [Medline].

  10. Lepore FE, Duvoisin RC. "Apraxia" of eyelid opening: an involuntary levator inhibition. Neurology. Mar 1985;35(3):423-7. [Medline].

  11. Schmidtke K, Büttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain. Feb 1992;115 Pt 1:227-47. [Medline].

  12. Tolosa E, Martí MJ. Blepharospasm-oromandibular dystonia syndrome (Meige's syndrome): clinical aspects. Adv Neurol. 1988;49:73-84. [Medline].

  13. Nakashima K, Kusumi M, Inoue Y, Takahashi K. Prevalence of focal dystonias in the western area of Tottori Prefecture in Japan. Mov Disord. Jul 1995;10(4):440-3. [Medline].

  14. Nutt JG, Muenter MD, Melton LJ 3rd, Aronson A, Kurland LT. Epidemiology of dystonia in Rochester, Minnesota. Adv Neurol. 1988;50:361-5. [Medline].

  15. Duffey PO, Butler AG, Hawthorne MR, Barnes MP. The epidemiology of the primary dystonias in the north of England. Adv Neurol. 1998;78:121-5. [Medline].

  16. A prevalence study of primary dystonia in eight European countries. J Neurol. Oct 2000;247(10):787-92. [Medline].

  17. Defazio G, Livrea P, Lamberti P, De Salvia R, Laddomada G, Giorelli M, et al. Isolated so-called apraxia of eyelid opening: report of 10 cases and a review of the literature. Eur Neurol. 1998;39(4):204-10. [Medline].

  18. Nutt JG. Lid abnormalities secondary to cerebral hemisphere lesions. Ann Neurol. Feb 1977;1(2):149-51. [Medline].

  19. De Renzi E, Gentilini M, Bazolli C. Eyelid movement disorders and motor impersistence in acute hemisphere disease. Neurology. Mar 1986;36(3):414-8. [Medline].

  20. Lepore FE. So-called apraxias of lid movement. Adv Neurol. 1988;49:85-90. [Medline].

  21. Micheli F, Cersosimo G, Scorticati MC, Ledesma D, Molinos J. Blepharospasm and apraxia of eyelid opening in lithium intoxication. Clin Neuropharmacol. May-Jun 1999;22(3):176-9. [Medline].

  22. Tsuji S, Kikkawa S, Horiguchi J, Yamashita H, Kagaya A, Morinobu S, et al. Meige syndrome with apraxia of lid opening after the discontinuation of sulpiride treatment. Pharmacopsychiatry. Jul 2002;35(4):155-6. [Medline].

  23. Ugarte M, Teimory M. Apraxia of lid opening. Br J Ophthalmol. Jul 2007;91(7):854. [Medline].

  24. Smith D, Ishikawa T, Dhawan V, Winterkorn JS, Eidelberg D. Lid opening apraxia is associated with medial frontal hypometabolism. Mov Disord. May 1995;10(3):341-4. [Medline].

  25. Suzuki Y, Kiyosawa M, Ohno N, Mochizuki M, Inaba A, Mizusawa H, et al. Glucose hypometabolism in medial frontal cortex of patients with apraxia of lid opening. Graefes Arch Clin Exp Ophthalmol. Jul 2003;241(7):529-34. [Medline].

  26. Golbe LI, Davis PH, Lepore FE. Eyelid movement abnormalities in progressive supranuclear palsy. Mov Disord. 1989;4(4):297-302. [Medline].

  27. Brusa A, Meneghini S, Piccardo A, Stoehr R. Apraxia of lid opening. Ital J Neurol Sci. Dec 1981;2(4):367-70. [Medline].

  28. Lee KC, Finley R, Miller B. Apraxia of lid opening: dose-dependent response to carbidopa-levodopa. Pharmacotherapy. Mar 2004;24(3):401-3. [Medline].

  29. Esteban A, Giménez-Roldán S. Involuntary closure of eyelids in parkinsonism. Electrophysiological evidence for prolonged inhibition of the levator palpebrae muscles. J Neurol Sci. Jul 1988;85(3):333-45. [Medline].

  30. Roh JK, Kim BG, Kim DE, Ahn TB. Apraxia of lid opening associated with hydrocephalus. Eur Neurol. 2001;45(1):53-4. [Medline].

  31. Abe K, Fujimura H, Tatsumi C, Toyooka K, Yorifuji S, Yanagihara T. Eyelid "apraxia" in patients with motor neuron disease. J Neurol Neurosurg Psychiatry. Dec 1995;59(6):629-32. [Medline].

  32. Bonaventura I, Matias-Guiu J, Cervera C, Codina Puiggros A. Neuroacanthocytosis syndrome, apraxia of eyelid opening, and progressive supranuclear palsy. Neurology. Sep 1986;36(9):1276. [Medline].

  33. Friedman DI, Jankovic J, McCrary JA 3rd. Neuro-ophthalmic findings in progressive supranuclear palsy. J Clin Neuroophthalmol. Jun 1992;12(2):104-9. [Medline].

  34. Langston JW, Ballard P, Tetrud JW, Irwin I. Chronic Parkinsonism in humans due to a product of meperidine-analog synthesis. Science. Feb 25 1983;219(4587):979-80. [Medline].

  35. Keane JR. Lid-opening apraxia in Wilson's disease. J Clin Neuroophthalmol. Mar 1988;8(1):31-3. [Medline].

  36. Paus S, Zsurka G, Baron M, Deschauer M, Bamberg C, Klockgether T, et al. Apraxia of lid opening mimicking ptosis in compound heterozygosity for A467T and W748S POLG1 mutations. Mov Disord. Jul 15 2008;23(9):1286-8. [Medline].

  37. Forget R, Tozlovanu V, Iancu A, Boghen D. Botulinum toxin improves lid opening delays in blepharospasm-associated apraxia of lid opening. Neurology. Jun 25 2002;58(12):1843-6. [Medline].

  38. Jankovic J. Disease-oriented approach to botulinum toxin use. Toxicon. Dec 6 2008;[Medline].

  39. Yamada S, Matsuo K, Hirayama M, Sobue G. The effects of levodopa on apraxia of lid opening: A case report. Neurology. Mar 9 2004;62(5):830-1. [Medline].

  40. Dewey RB Jr, Maraganore DM. Isolated eyelid-opening apraxia: report of a new levodopa-responsive syndrome. Neurology. Sep 1994;44(9):1752-4. [Medline].

  41. Chand RP, Park DM. Atypical blepharospasm responsive to sodium valproate. Mov Disord. Jan 1994;9(1):116-7. [Medline].

  42. Klostermann W, Vieregge P, Kompf D. Apraxia of eyelid opening after bilateral stereotaxic subthalamotomy. J Neuroophthalmol. Jun 1997;17(2):122-3. [Medline].

  43. Kerty E, Eidal K. Apraxia of eyelid opening: clinical features and therapy. Eur J Ophthalmol. Mar-Apr 2006;16(2):204-8. [Medline].

  44. Aramideh M, Ongerboer de Visser BW, Koelman JH, Speelman JD. Motor persistence of orbicularis oculi muscle in eyelid-opening disorders. Neurology. May 1995;45(5):897-902. [Medline].

  45. Esteban A, Traba A, Prieto J. Eyelid movements in health and disease. The supranuclear impairment of the palpebral motility. Neurophysiol Clin. Feb 2004;34(1):3-15. [Medline].

  46. Tozlovanu V, Forget R, Iancu A, Boghen D. Prolonged orbicularis oculi activity: a major factor in apraxia of lid opening. Neurology. Sep 25 2001;57(6):1013-8. [Medline].

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Further Reading

Boghen D. Apraxia of lid opening: a review. Neurology. 1997;48(6):1491-4.

Esteban A, Traba A, Prieto J. Eyelid movements in health and disease. The supranuclear impairment of the palpebral motility. Neurophysiol Clin. 2004;34(1):3-15.

Schmidtke K, Buttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain. 1992;115 Pt 1:227-47.

Related eMedicine topics
 Apraxia and Related Syndromes (from Neurology)
Blepharospasm, Benign Essential
Blepharoplasty, Upper Eyelid
Blepharoplasty, Lower Eyelid Laxity
Floppy Eyelid Syndrome

Guidelines
Blepharitis

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Keywords

apraxia of lid opening, blepharocolysis, focal eyelid dystonia, involuntary levator palpebrae inhibition, pretarsal blepharospasm, atypical blepharospasm, freezing and akinesia of lid function, ALO, ILPI, orbicularis oculi, OO, levator palpebrae superioris, LPS, intermittent involuntary closure, frontalis suspension, botulinum toxin, BOTOX®

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Contributor Information and Disclosures

Author

Marta Ugarte, MBBS, PhD, DPhil, MRC(Ophth), Clinical Lecturer in Ophthalmology, University of Manchester, UK
Marta Ugarte, MBBS, PhD, DPhil, MRC(Ophth) is a member of the following medical societies: Association for Research in Vision and Ophthalmology, British Medical Association, International Society of Ocular Trauma, Royal College of Ophthalmologists, and Royal Society of Medicine
Disclosure: Nothing to disclose.

Medical Editor

Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado
Ron W Pelton, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Colorado Medical Society, Utah Medical Association, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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