Isolated So-Called Apraxia of Eyelid Opening: Report of 10 Cases and a Review of the Literature

So-called apraxia of eyelid opening (scAEO) has been described chiefly in the context of extrapyramidal disorders. We described 10 new patients with scAEO developing in the absence of any other CNS sign and reviewed the 11 cases with isolated scAEO reported in the literature. Combining our patients and those from the literature, peak age at onset was in the 6th decade and there was a female preponderance of 2:1. The characteristic inability to initiate lid elevation was frequently associated with failure to sustain lid elevation, thus suggesting that eyelid motor control may be abnormal in isolated scAEO. Antecedent events included ocular signs and symptoms consistent with diseases of eyes or face (4 cases in our series and 2 in the literature), chronic treatment with flunarizine (1 case), and family history of dystonia (1 case). Flunarizine discontinuation led to sustained remission of the eyelid disturbance. Overall, these clues suggest the involvement of the extrapyramidal system in the pathophysiology of isolated scAEO. Familial clustering of isolated scAEO in one of our patients may be in favor of a genetic contribution. In our series, botulinum toxin administration close to the pretarsal part of the orbicularis oculi muscle significantly improved scAEO in 8/10 cases, whereas orbital/preseptal injection had no effect. We conclude that the term ‘apraxia’ may not be the correct descriptive term even when the eyelid disturbance occurs without any other CNS disease.

[1]  M. Aramideh,et al.  Pretarsal application of botulinum toxin for treatment of blepharospasm. , 1995, Journal of neurology, neurosurgery, and psychiatry.

[2]  D. Cogan,et al.  APRAXIA OF LID OPENING. , 1965, Archives of ophthalmology.

[3]  F. Micheli,et al.  Flunarizine‐ and cinnarizine‐induced extrapyramidal reactions , 1987, Neurology.

[4]  I. Sanders,et al.  Quantifying how location and dose of botulinum toxin injections affect muscle paralysis , 1993, Muscle & nerve.

[5]  J. Elston,et al.  A new variant of blepharospasm. , 1992, Journal of neurology, neurosurgery, and psychiatry.

[6]  D. Maraganore,et al.  Isolated eyelid‐opening apraxia , 1994, Neurology.

[7]  M. Aramideh,et al.  Motor persistence of orbicularis oculi muscle in eyelid‐opening disorders , 1995, Neurology.

[8]  L. Bour,et al.  Electromyographic features of levator palpebrae superioris and orbicularis oculi muscles in blepharospasm. , 1994, Brain : a journal of neurology.

[9]  P. May,et al.  Morphological substrate for eyelid movements: Innervation and structure of primate levator palpebrae superioris and orbicularis oculi muscles , 1989, The Journal of comparative neurology.

[10]  G. Defazio,et al.  Botulinum a toxin for the so‐called apraxia of lid opening , 1995, Movement disorders : official journal of the Movement Disorder Society.

[11]  T. Ishikawa,et al.  Lid opening apraxia is associated with medial frontal hypometabolism , 1995, Movement disorders : official journal of the Movement Disorder Society.

[12]  D. Boghen,et al.  Apraxia of lid opening , 1997, Neurology.

[13]  G. Defazio,et al.  Genetic contribution to idiopathic adult-onset blepharospasm and cranial-cervical dystonia. , 1993, European neurology.

[14]  M. Aramideh,et al.  The blepharospasm disability scale: An instrument for the assessment of functional health in blepharospasm , 1995, Movement disorders : official journal of the Movement Disorder Society.

[15]  L. Golbe,et al.  Proposal for a clinical definition of blepharokolysis , 1991, Movement Disorders.

[16]  N. Geschwind The apraxias: neural mechanisms of disorders of learned movement. , 1975, American scientist.

[17]  J. Grotta,et al.  Apraxia of eyelid opening secondary to right hemisphere infarction , 1989, Annals of neurology.

[18]  B. Katz,et al.  Botulinum therapy for apraxia of eyelid opening. , 1987, American journal of ophthalmology.

[19]  E. De Renzi,et al.  Eyelid movement disorders and motor impersistence in acute hemisphere disease , 1986, Neurology.

[20]  L. Bour,et al.  Clinical and electromyographic features of levator palpebrae superioris muscle dysfunction in involuntary eyelid closure , 1994, Movement disorders : official journal of the Movement Disorder Society.

[21]  J. Jankovic Apraxia of lid opening. , 1995, Movement disorders : official journal of the Movement Disorder Society.

[22]  P. Krack,et al.  “Apraxia of lid opening,” a focal eyelid dystonia: Clinical study of 32 patients , 1994, Movement disorders : official journal of the Movement Disorder Society.

[23]  M. Hallett Is dystonia a sensory disorder? , 1995, Annals of neurology.