Apraxia of Eyelid Opening

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 by Michael T Yen, MD on August 21, 2020.


Apraxia of Eyelid Opening


Disease Entity

Disease

Apraxia of eyelid opening is defined as “nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi muscle.”[1]Abnormalities of eyelid function have been described in the literature as far back as 1907, where Lewandowsky described apraxia of eyelid closure.[2] While Schilder and Riese both described apraxias of eyelid closure and opening in 1927 and 1930 in neurology literature[3][4], Goldstein et al were the first to describe apraxia of eyelid opening in ophthalmologic literature in 1965.[5]

Mechanism

Elevation of the eyelid involves simultaneous activation of the levator palpebrae superioris muscle and inhibition of the orbiculares oculi muscle.[6] Apraxia of eyelid opening can result from involuntary inhibition of levator function, prolonged contraction of the orbicularis, or both.[7][8] The classic finding of inability to open the lids after closure is postulated to be caused by persistent contraction of the activated orbicularis oculi muscle.[9]

Tozlovanu et al studied the role of orbicularis contraction in apraxia of eyelid opening by using electromyography to measure orbicularis activity and latency of lid opening. This study demonstrated a strong correlation between time to orbicularis oculi inhibition and the completion of lid opening in both the control group and the study group (patients with ALO), with an abnormal persistence of orbicularis oculi function present in 10 of the 11 patients with ALO. [10]

Causes/Associated Syndromes

Though ALO has been described in isolation, it is also associated with other conditions such as benign essential blepharospasm, progressive supranuclear palsy, dystonic parkinsonian syndrome/parkinsonism, motor neuron disease, and Shy-Drager syndrome.[11][12][13][14] ALO has also been associated with CNS lesions in various locations, including the non-dominant hemisphere, medial frontal lobe, basal ganglia, and rostral brain stem.[15][16][17][18][19][20] Additionally, isolated case reports of medication-induced ALO (including lithium, sulpiride, and MPTP), as well as sleep-induced ALO, exist in the literature.[21][22][23][24]

Diagnosis

Clinical Presentation

ALO typically presents with the inability to open the lids following either voluntary or involuntary lid closure. Eye opening is, however, typically normal after reflex blinking. Additionally, forceful contraction of the frontalis muscle upon attempted opening has been reported as a feature of ALO, though it is not present in all cases. ALO is often accompanied by blepharospasm, though case reports do exist of isolated ALO without the presence of blepharospasm. In patients with essential blepharospasm, the combination of spasmodic closure of the eyelids and levator inhibition cause difficulty with opening the lids after forced closure. [1] [13][15]

Diagnostic Approach

The diagnosis of ALO is generally made clinically by identification of the hallmark physical exam findings described above. Brain imagining in the form of CT or MRI may be ordered in order to isolate a causal lesion. Reports of the use of positron emission tomography (PET scanning) to identify areas of neuronal glucose hypometabolism in the basal ganglia, frontal lobe, or primary visual cortex do exist in the literature.[25] Electromyography (EMG) may also be helpful in diagnosis and to distinguish the presence or absence of concurrent blepharospasm.[26] Thorough evaluation for associated systemic/neurologic diseases should also be pursued.

Management

Treatment and Prognosis

Historically, the mainstay of treatment for ALO (with or without concurrent blepharospasm) has been injection of botulinum toxin into the pre-tarsal orbicularis oculi muscle.[27] Other treatment options including levodopa[28], trihexyphenidyl[29], and valproic acid[30] have been described in the literature, although results are limited.

Several reports exist detailing the successful use of frontalis suspension[31] and upper eyelid myectomy[32] as surgical alternatives for some patients with ALO. These procedures have also been described in combination with medical therapy. [33]

References

  1. 1.0 1.1 Boghen D. Apraxia of lid opening: a review. Neurology . 1997; 48: 1491–1503.
  2. Lewandowsky, M.: Über apraxie des Lidschlusses , Berl klin Wschr 44:921-923, 1907
  3. Schilder, P.:  Neuer Beitrag zur pathologie der Lidbewegungen, Kopfmitbewegungen Aphatiker beim Öffnen und Schlissen der Augen ,  Deutsch Z Nervheilk 98:161-168, 1927.Crossref
  4. Riese, W.:  Apraxie der Lidöffnung (Analyse einer Bewegungsstörung) ,  J Psychol Neurol Lpz 40:347-355, 1930.
  5. Goldstein, J.E. and Cogan, D.G. Apraxia of lid opening. Arch Ophthalmol. 1965; 73: 155–159
  6. Jordan, D.R., Anderson, R.L., and Digre, K.B. Apraxia of lid opening in blepharospasm. Ophthalmic Surg. 1990; 21: 331–334
  7. Aramideh M, Ongerboer de Visser BW, Devriese PP, Bour LJ, Speelman JD. Electromyographic features of levator palpebrae superioris and orbicularis oculi muscles in blepharospasm. Brain . 1994; 117: 27–38.
  8. Elston JS. A new variant of blepharospasm. J Neurol Neurosurg Psychiatry . 1992; 55: 369–371.
  9. Aramideh M, Ongerboer de Visser BW, Koelman JHTM, Speelman JD. Motor persistence of orbicularis oculi muscle in eyelid-opening disorders. Neurology . 1995; 45: 897–902.
  10. Viorika Tozlovanu, Robert Forget, Andreea Iancu, Dan Boghen. Prolonged orbicularis oculi activity; a major factor in apraxia of lid opening. Neurology Sep 2001, 57 (6) 1013-1018; DOI: 10.1212/WNL.57.6.1013
  11. 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
  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. 13.0 13.1 Lepore FE, Duvoisin RC. "Apraxia" of eyelid opening: an involuntary levator inhibition. Neurology. 1985 Mar. 35(3):423-7
  14. 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
  15. 15.0 15.1 De Renzi E, Gentilini M, Bazolli C. Eyelid movement disorders and motor impersistence in acute hemisphere disease. Neurology. 1986 Mar. 36(3):414-8. [Medline].
  16. 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. [Medline].
  17. 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. [Medline].

  18. Dimitriou J, Montoute T, Levivier M, Borruat FX, Diserens K. Bilateral ptosis: Lesion in the oculomotor nuclei or supranuclear lesion?. NeuroRehabilitation. 2015. 36 (3):323-7. [Medline].

  19. 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. [Medline].

  20. 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. [Medline].
  21. 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. [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. 2002 Jul. 35(4):155-6. [Medline].
  23. Golbe LI, Davis PH, Lepore FE. Eyelid movement abnormalities in progressive supranuclear palsy. Mov Disord. 1989. 4(4):297-302. [Medline].
  24. Reggie SN, Chen JJ, Lee MS, Chung SM. Sleep-Induced Apraxia of Eyelid Opening. J Neuroophthalmol. 2017;37(4):390-392.
  25. Smith D, Ishikawa T, Dhawan V, Winterkorn JS, Eidelberg D. Lid opening apraxia is associated with medial frontal hypometabolism. Mov Disord. 1995;10(3):341-4.
  26. Hirose K, Kanehisa Y, Oikawa S, Shoji H, Uono M. [Electromyographic differentiation between apraxia of lid-opening and blepharospasm (author's transl)]. Rinsho Shinkeigaku. 1978;18(4):192-8.
  27. 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
  28. Dewey RB Jr, Maraganore DM. Isolated eyelid-opening apraxia: report of a new levodopa-responsive syndrome. Neurology. 1994 Sep. 44(9):1752-4
  29. Klostermann W, Vieregge P, Kompf D. Apraxia of eyelid opening after bilateral stereotaxic subthalamotomy. J Neuroophthalmol. 1997 Jun. 17(2):122-3
  30. Chand RP, Park DM. Atypical blepharospasm responsive to sodium valproate. Mov Disord. 1994 Jan. 9(1):116-7
  31. Dressler D, Karapantzou C, Rohrbach S, Schneider S, Laskawi R. Frontalis suspension surgery to treat patients with blepharospasm and eyelid opening apraxia: long-term results. J Neural Transm (Vienna). 2017;124(2):253-257.
  32. 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;145(3):541-547.
  33. Kerty E, Eidal K. Apraxia of eyelid opening: clinical features and therapy. Eur J Ophthalmol. 2006;16(2):204-8.