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."Goldstein JE, Cogan DG. Apraxia of Lid Opening. Arch Ophthalmol. 1965 Feb. 73:155-9
A review of reported cases has shown a 2:1 female to male occurrence, and onset usually in the sixth decade of life.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
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.Ugarte M, Teimory M. Apraxia of lid opening. Br J Ophthalmol. 2007 Jul. 91(7):854
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 studiesAramideh 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):1457-74Esteban 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):333-45Esteban 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):283-94Elston JS. A new variant of blepharospasm. J Neurol Neurosurg Psychiatry. 1992 May. 55(5):369-71Lepore FE, Duvoisin RC. Apraxia of eyelid opening: an involuntary levator inhibition. Neurology. 1985 Mar. 35(3):423-7 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.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):341-4Suzuki 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
While often an isolated condition, ALO sometimes occurs in conjunction with blepharospasm,Jordan DR, Anderson RL, Digre KB. Apraxia of lid opening in blepharospasm. Ophthalmic Surg. 1990 May. 21(5):331-4Krack P, Marion MH. Apraxia of lid opening, a focal eyelid dystonia: clinical study of 32 patients. Mov Disord. 1994 Nov. 9(6):610-5Hsieh 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 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,Golbe LI, Davis PH, Lepore FE. Eyelid movement abnormalities in progressive supranuclear palsy. Mov Disord. 1989. 4(4):297-302 hydrocephalus,Roh JK, Kim BG, Kim DE, Ahn TB. Apraxia of lid opening associated with hydrocephalus. Eur Neurol. 2001. 45(1):53-4 motor neuron disease,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 Shy–Drager syndrome, and various lesions in the brain.Schmidtke K, Büttner-Ennever JA. Nervous control of eyelid function. A review of clinical, experimental and pathological data. Brain. 1992 Feb. 115 Pt 1:227-47De Renzi E, Gentilini M, Bazolli C. Eyelid movement disorders and motor impersistence in acute hemisphere disease. Neurology. 1986 Mar. 36(3):414-8 Medications that have been associated include lithium,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 sulpiride,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 and a meperidine analog, 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine.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
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.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):1843-6Jankovic J. Disease-oriented approach to botulinum toxin use. Toxicon. 2008 Dec 6 Levodopa has been reported to improve symptoms in patients with Parkinson's disease and progressive supranuclear palsy.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):830-1Dewey RB Jr, Maraganore DM. Isolated eyelid-opening apraxia: report of a new levodopa-responsive syndrome. Neurology. 1994 Sep. 44(9):1752-4Lee KC, Finley R, Miller B. Apraxia of lid opening: dose-dependent response to carbidopa-levodopa. Pharmacotherapy. 2004 Mar. 24(3):401-3 There have also been reports of improvement with trihexyphenidylKlostermann W, Vieregge P, Kompf D. Apraxia of eyelid opening after bilateral stereotaxic subthalamotomy. J Neuroophthalmol. 1997 Jun. 17(2):122-3 and valproic acid.Chand RP, Park DM. Atypical blepharospasm responsive to sodium valproate. Mov Disord. 1994 Jan. 9(1):116-7 Surgical treatment may be considered in severe cases or cases that occur with blepharospasm.Kerty E, Eidal K. Apraxia of eyelid opening: clinical features and therapy. Eur J Ophthalmol. 2006 Mar-Apr. 16(2):204-8Georgescu 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
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.
See also Ptosis (eyelid) Blepharospasm Apraxia Myokymia
References