Apraxia of lid opening

In ophthalmology, apraxia of lid opening (ALO) is an inability to initiate voluntary opening of the eyelid following a period of eyelid closure, with normal function at other times. Manual lifting of the eyelid often resolves the problem and the lid is able to stay open.

ALO was first clearly described as a distinct entity in 1965 as “a nonparalytic motor abnormality characterized by the patient’s difficulty in initiating the act of lid elevation present only momentarily at the start of lid opening.”[1]

A review of reported cases has shown a 2:1 female to male occurrence, and onset usually in the sixth decade of life.[2]

Signs and symptoms

A person with ALO may complain of occasionally being unable to open the eye at will, typically after prolonged closure. Oftentimes, ALO only persists momentarily and the ptosis resolves upon manually lifting the eyelid. During attempted lid opening, there may be forceful frontalis muscle contraction, backward thrusting of the head, or opening of the mouth noted.[3]

Causes

The exact cause of ALO is not yet fully understood. Despite its name, it is not a true apraxia, but thought to be due to a supranuclear origin of abnormal neuronal activity. Voluntary eyelid opening involves the simultaneous activation of the levator palpebrae superioris muscle and the inhibition of the orbicularis oculi muscle. Electromyographic studies[4][5][6][7][8] have shown that ALO may involve either involuntary levator palpebrae superioris muscle inhibition, persistent pretarsal orbicularis oculi muscle contraction, or both. Hypometabolism of glucose may play a part, and has been documented in the basal ganglia, frontal lobe, and the primary visual cortex in some cases of ALO.[9][10]

While often an isolated condition, ALO sometimes occurs in conjunction with blepharospasm,[11][12][13] which may complicate the diagnosis and treatment. Additionally, several diseases as well as medications have been reported in association with ALO. It has been reported in cases of extrapyramidal disorders, including Parkinson’s disease, parkinsonism, progressive supranuclear palsy,[14] hydrocephalus,[15] motor neuron disease,[16] Shy–Drager syndrome,[8] and various lesions in the brain.[17][18][19][10] Medications that have been associated include Lithium,[20] sulpiride,[21] and a meperidine analog, 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine.[22]

Diagnosis

ALO is often a benign and unilateral condition, and extensive evaluation may not be necessary. The diagnosis may be a clinical diagnosis based on a thorough history and physical exam, and being able to exclude other diagnoses and pathology. Computed tomography (CT) or magnetic resonance imaging (MRI) may be ordered to help determine whether there is a lesion or other pathological cause present in the brain or brainstem. Electromyography may be used to aid the diagnosis.

Treatment

Botulinum toxin injected into the pretarsal orbicularis oculi muscle has been shown to be beneficial in some cases associated with orbicularis oculi contraction or blepharospasm.[23][24] Levodopa has been reported to improve symptoms in patients with Parkinson’s disease and progressive supranuclear palsy.[25][26][27] There have also been reports of improvement with trihexyphenidyl[28] and valproic acid.[29] Surgical treatment may be considered in severe cases or cases that occur with blepharospasm.[30][31]

Prognosis

A person with isolated ALO has an excellent prognosis. Prognosis for ALO in association with other diseases depends on the underlying condition. When it is drug-induced, ALO may resolve within weeks to months upon cessation of the agent.[20][21]

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See also

References
  1. Goldstein JE, Cogan DG. Apraxia of Lid Opening. Arch Ophthalmol. 1965 Feb. 73:155-9
  2. 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
  3. Ugarte M, Teimory M. Apraxia of lid opening. Br J Ophthalmol. 2007 Jul. 91(7):854
  4. 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. 1994 Dec. 117 (Pt 6):1457-74
  5. Esteban A, Gimenez-Roldan S. Involuntary closure of eyelids in parkinsonism. Electrophysiological evidence for prolonged inhibition of the levator palpebrae muscles. J Neurol Sci. 1988 Jul. 85(3):333-45
  6. Esteban A, Gimenez-Roldan S. Nociceptive reflex of the orbicularis oculi. Study in normal subjects and in peripheral facial lesions. Arch Neurobiol (Madr). 1973 Jul-Aug. 36(4):283-94
  7. Elston JS. A new variant of blepharospasm. J Neurol Neurosurg Psychiatry. 1992 May. 55(5):369-71
  8. Lepore FE, Duvoisin RC. Apraxia of eyelid opening: an involuntary levator inhibition. Neurology. 1985 Mar. 35(3):423-7
  9. Smith D, Ishikawa T, Dhawan V, Winterkorn JS, Eidelberg D. Lid opening apraxia is associated with medial frontal hypometabolism. Mov Disord. 1995 May. 10(3):341-4
  10. 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. 2003 Jul. 241(7):529-34
  11. Jordan DR, Anderson RL, Digre KB. Apraxia of lid opening in blepharospasm. Ophthalmic Surg. 1990 May. 21(5):331-4
  12. Krack P, Marion MH. Apraxia of lid opening, a focal eyelid dystonia: clinical study of 32 patients. Mov Disord. 1994 Nov. 9(6):610-5
  13. Hsieh CY, Sung PS, Hwang WJ. Transient blepharospasm, apraxia of eyelid opening, and hemidyskinesia following a right parietotemporal infarct. Parkinsonism Relat Disord. 2014 Sep. 20(9):1024-6
  14. Golbe LI, Davis PH, Lepore FE. Eyelid movement abnormalities in progressive supranuclear palsy. Mov Disord. 1989. 4(4):297-302
  15. Roh JK, Kim BG, Kim DE, Ahn TB. Apraxia of lid opening associated with hydrocephalus. Eur Neurol. 2001. 45(1):53-4
  16. Abe K, Fujimura H, Tatsumi C, Toyooka K, Yorifuji S, Yanagihara T. Eyelid "apraxia" in patients with motor neuron disease. J Neurol Neurosurg Psychiatry. 1995 Dec. 59(6):629-32
  17. Schmidtke K, Büttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain. 1992 Feb. 115 Pt 1:227-47
  18. De Renzi E, Gentilini M, Bazolli C. Eyelid movement disorders and motor impersistence in acute hemisphere disease. Neurology. 1986 Mar. 36(3):414-8
  19. Smith D, Ishikawa T, Dhawan V, Winterkorn JS, Eidelberg D. Lid opening apraxia is associated with medial frontal hypometabolism. Mov Disord. 1995 May. 10(3):341-4
  20. Micheli F, Cersosimo G, Scorticati MC, Ledesma D, Molinos J. Blepharospasm and apraxia of eyelid opening in lithium intoxication. Clin Neuropharmacol. 1999 May-Jun. 22(3):176-9
  21. 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. 2002 Jul. 35(4):155-6
  22. Langston JW, Ballard P, Tetrud JW, Irwin I. Chronic Parkinsonism in humans due to a product of meperidine-analog synthesis. Science. 1983 Feb 25. 219(4587):979-80
  23. Forget R, Tozlovanu V, Iancu A, Boghen D. Botulinum toxin improves lid opening delays in blepharospasm-associated apraxia of lid opening. Neurology. 2002 Jun 25. 58(12):1843-6
  24. Jankovic J. Disease-oriented approach to botulinum toxin use. Toxicon. 2008 Dec 6
  25. Yamada S, Matsuo K, Hirayama M, Sobue G. The effects of levodopa on apraxia of lid opening: A case report. Neurology. 2004 Mar 9. 62(5):830-1
  26. Dewey RB Jr, Maraganore DM. Isolated eyelid-opening apraxia: report of a new levodopa-responsive syndrome. Neurology. 1994 Sep. 44(9):1752-4
  27. Lee KC, Finley R, Miller B. Apraxia of lid opening: dose-dependent response to carbidopa-levodopa. Pharmacotherapy. 2004 Mar. 24(3):401-3
  28. Klostermann W, Vieregge P, Kompf D. Apraxia of eyelid opening after bilateral stereotaxic subthalamotomy. J Neuroophthalmol. 1997 Jun. 17(2):122-3
  29. Chand RP, Park DM. Atypical blepharospasm responsive to sodium valproate. Mov Disord. 1994 Jan. 9(1):116-7
  30. Kerty E, Eidal K. Apraxia of eyelid opening: clinical features and therapy. Eur J Ophthalmol. 2006 Mar-Apr. 16(2):204-8
  31. Georgescu D, Vagefi MR, McMullan TF, McCann JD, Anderson RL. Upper eyelid myectomy in blepharospasm with associated apraxia of lid opening. Am J Ophthalmol. 2008 Mar. 145(3):541-547
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