Original Paper
Eur Neurol 1998;39:204–210

Received: May 12, 1997
Accepted: October 22, 1997

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

G. Defazio
P. Livrea
P. Lamberti
R. De Salvia
G. Laddomada
M. Giorelli
E. Ferrari
Institute of Neurology of the University of
Bari, Italy

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Key Words
Apraxia of eyelid opening
Extrapyramidal disorders
Botulinum toxin

Abstract
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.
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Introduction
So-called apraxia of eyelid opening (scAEO) was defined by Goldstein and Cogan [1] as a nonparalytic movement disorder characterized by transient difficulty in
voluntarily initiating the act of lid elevation inspite of preserved alertness and language comprehension. Lepore

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and Duvoisin [2] pointed out the clinical criteria for the
diagnosis of scAEO, but questioned whether the condition should be categorized as apraxia since most patients
were affected by extrapyramidal disorders [2–11] or, less
frequently, cerebrovascular diseases [12–14]. Based on
clinical and electromyographic (EMG) findings as well as
on the results of botulinum toxin (BT) treatment, a vari-

G. Defazio
Institute of Neurology
University of Bari
I–70124 Bari (Italy)
Tel. +39 80 5478511, Fax +39 80 5478532

ety of other terms have been proposed, including eyelid
freezing [15], involuntary levator palpebrae superior
(LPS) inhibition [2], blepharokolysis [16], focal eyelid
dystonia [9] and pretarsal blepharospasm (BS) [8], but
none achieved consensus.
In reviewing the literature, we found sporadic reports
[3, 4, 8, 9, 17, 18], mostly consisting of anecdotal descriptions, on isolated scAEO, that is scAEO without extrapyramidal disorders or cerebrovascular diseases. Thus, the
origin, nature and clinical features of isolated scAEO are
uncertain, as well as the response to BT. To further investigate these issues, we present 10 new patients and a
review of the cases described in the literature [3, 4, 8, 9, [7,
18].

shopping, walking in crowds and working at home or outside. We feel
that this scale can be applied to any lid dyskinesia inducing eye closure. Results are expressed as percent of normal activity whereby 0
and 100% are assigned to subjects completely unable to perform
functional activities of daily living or unaware of any difficulty
respectively. According to the interpretation of the scale proposed by
Lindeboom et al. [21], patients were classified as: functionally blind
(0–20% normal activity); severely limited (21–33% normal activity);
with moderate to marked limitation (34–57% normal activity); with
minor functional limitation (58–75% normal activity); socially affected (76–90% normal activity). The validity of the disability scale
has been assessed [21].
A computer-assisted review of the literature was conducted by
Medline. To identify all possible cases of scAEO, the following key
words were used: apraxia of eyelid opening [1], eyelid freezing [15],
involuntary LPS inhibition [2], blepharokolysis [16], focal eyelid dystonia [9] and pretarsal BS [8]. To obtain additional reports we
reviewed the bibliographies of all papers thereby identified. scAEO
was considered to be isolated when no other CNS disease was
reported.

Patients and Methods
scAEO was diagnosed according to Lepore and Duvoisin’s [2]
clinical criteria. These include: (1) no sign of ongoing orbicularis oculi (OO) contractions such as lowering of the brows beneath the superior orbital margins (Charcot’s sign); (2) marked frontalis muscle overaction during period of inability to raise eyelids; (3) no ocular motor/
ocular sympathetic nerve dysfunction or ocular myopathy. EMG
examinations of the OO and LPS muscles were not performed since
they are unwieldy, require expertise not readily available in our setting and, finally, seem unnecessary for the diagnosis of scAEO [19].
In reviewing the medical records of patients with excessive eyelid
closure seen at our movement disorders clinic from 1987 to 1996 we
found 30 patients with scAEO. In the same period, 120 patients were
followed for typical BS without discernible scAEO. According to the
results of clinical (neurologic and ophthalmologic), radiologic (computed tomography, CT, and/or magnetic resonance, MR, scan of the
head) and laboratory (including serum ceruloplasmin estimation)
investigations, patients without evidence of central nervous system
(CNS) disease were considered to be affected by isolated scAEO.
At the time of the survey, the following information was collected: age, sex, age at disease onset (referring to the time of first
symptoms), previous medical and surgical events including diabetes,
hypertension, thyroid and ocular diseases, anxiety, depression, other
diseases, general anesthesia, and head/face trauma. The occurrence
of scAEO or other movement disorders in the first-degree relatives of
index patients was assessed by directly examining living relatives;
deceased relatives were considered to be affected only if at least
another family member or, whenever possible, medical records or
photographs provided adequate information to support diagnosis
[19].
Patients were treated with BT (Allergan, Irvine, Calif.) according
to two different protocols. First, 20–30 U were injected in the upper
lid in two puncture sites at the junction of the orbital and preseptal
OO, medially and laterally to avoid the LPS. Alternatively, BT (10–
20 U/0.1 ml saline) was injected s.c. (one puncture site) in the mid
upper lid close to the eyelash line [10]. To evaluate treatment outcome, we focused on a disability scale originally proposed by Fahn
[20] for assessing the impact of BS on activities of daily living including driving a vehicle, reading, watching movies or television, going

Isolated So-Called Apraxia of Eyelid
Opening

Results
At the time of the first visit, scAEO was observed in
the following clinical settings: (a) isolated in otherwise
healthy individuals (n = 12); (b) adult-onset dystonia (n =
11), including BS (n = 9), BS plus oromandibular dystonia
(n = 1) and writer’s cramp (n = 1); and (c) parkinsonism
(n = 7), including Parkinson’s disease (n = 3), progressive
supranuclear palsy (n = 3) and freezing (n = 1). Parkinsonism, cranial dystonia and writer’s cramp preceded scAEO,
whereas the symptom of onset was not known in 9
patients in whom coexistence of BS and scAEO was diagnosed 0.5–2 years (mean 1.4, SD 0.6) after the onset of
eyelid discomfort.
Two out of the 12 patients presenting with isolated
scAEO developed BS within 1.5 years. Thus, at the time
of survey, 10 patients suffered from isolated scAEO.
There were 4 males and 6 females aged 57–76 (mean 63.2,
SD 5 years). Age at symptom onset ranged between 50
and 73 years (mean 58.7, SD 5.6), and mean disease duration was 5.2 years (SD, 2.9; range 3–12 years). The onset
was bilateral in all patients. Detailed neurologic and ophthalmologic examinations as well as CT and/or MR scan
of the head did not reveal any abnormality. There was no
sign of ptosis while the eyes were open, and patients could
close them readily on command. The strength of the OO
was normal. Voluntary eye closure was sometimes effective in triggering scAEO. In addition, all patients showed
episodes of involuntary drooping of the eyelids without
any obvious sign of OO spasm such as the Carcot’s sign.
These episodes were followed by the characteristic diffi-

Eur Neurol 1998;39:204–210

205

a

b

culty in raising eyelids (fig. 1). Involuntary drooping of
the eyelids frequently occurred in those patients who were
classified as functionally blind or severely handicapped
(see below) according to the interpretation of the disability scale proposed by Lindeboom et al. [21]. In these individuals, lid closure following voluntary or involuntary eye
closure lasted a considerable number of seconds to a few
minutes. Geste antagoniste (touching the eyelids or the
temporal regions) was often used as a strategy to facilitate
reopening. The frequency of the episodes of drooping the
eyelids was reduced by alertness or, alternatively, relaxation, increased by bright light, reading, and watching
television.
c

d

Fig. 1. A patient with isolated scAEO. a–d Photographs were taken in succession and demonstrate the characteristic transient inability to raise eyelids following eye closure. Note the bilateral contraction
of the frontalis muscle on attempted eye opening.

Table 1. Demographic and clinical features in 10 patients with
isolated apraxia of eyelid opening
Pa- Sex, age
tient at onset
years

Duration Family history
of illness,
years

Antecedent events

1

M, 56

3

–

2

F, 53

8

3

M, 60

4

4
5
6

F, 60
F, 61
F, 58

3
3
3

Father, brother
and 2 paternal
aunts with AEO
–
–
–

7

F, 64

5

–

8

M, 53

12

–

9

M, 57

8

–

10

F, 56

3

–

Brother with BS,
OMD, CD
–

Anxiety,
benzodiazepines
Glaucoma

Depression
–
Hypertension,
flunarizine
Anxiety,
benzodiazepines
Photophobia,
grittiness of the eyes
Irritation,
grittiness of the eyes
Watering and
grittiness of the eyes

OMD = Oromandibular dystonia; CD = cervical dystonia; AEO =
apraxia of eyelid opening; M = male; F = female.

206

Eur Neurol 1998;39:204–210

Antecedent Events
Antecedent events are shown in table 1. Prior to the
onset of scAEO, 2 cases suffered an anxiety state treated
with benzodiazepines which, however, did not cure
scAEO. Depression was reported by another case. A
female patient with scAEO had been taking flunarizine
for 6 years because of hypertension. No other neurological
sign was obvious and, thus, she was included in the
present series. She was treated with pretarsal BT without
any benefit (see below). Flunarizine was stopped 3 years
after the scAEO onset; 3 months later, the patient experienced complete sustained remission of scAEO lasting 3.5
years.
One patient was affected by glaucoma prior to the
onset of scAEO. Three additional cases experienced ocular symptoms consistent with diseases or irritations of the
eyes (including photophobia, irritation, watering or grittiness of the eyes) in the year before the onset of scAEO. At
the time of survey, mean duration of scAEO in patients
with prior eye symptoms was 6.3 years (SD, 3.2). No
patient developed new eye problems later during the
course of scAEO.
Family History
scAEO was observed in the brother of a male case (table 1). He did not suffer from any other CNS disease.
Their father and 2 paternal aunts (all deceased) were
probably affected by the same disturbance. Another case
(table 1) had a brother affected by cranial-cervical dystonia.
BT Treatment
Five patients (table 1: cases 1, 2, 6, 8 and 9) were
injected with BT in the orbital/preseptal OO, but none
improved. All patients were subsequently treated with
pretarsal BT. After the initial treatment, the mean percent

Defazio/Livrea/Lamberti/De Salvia/
Laddomada/Giorelli/Ferrari

Table 2. Summary of the clinical findings of 11 patients in the literature
Authors, year

Ref.

Case No.,
sex

Age at onset
years

Illness duration Prior diseases
years

Katz, 1987
Elston, 1992

18
8

Dewey and Maraganore, 1994

17

Krack and Marion, 1994
Aramideh et al., 1994
Aramideh et al., 1994

9
3
4

1, M
2, M
4, F
5, F
1, F
2, F
3 cases
21, F
5, F

64
55
64
50
55
13
Not reported
64
64

4
3
4
6
3
1
Not reported
7
8

of normal activity was raised from 26% (SD, 15.8) to 55%
(SD, 17.6) (p ! 0.01). At baseline, 4 patients were functionally blind, 2 were severely limited, and 4 had moderate to marked limitation in the activities of daily living.
Under pretarsal BT there were 1 functionally blind patient, 5 cases with moderate and 4 cases with minor limitation in the activities of daily living. Overall, 2 cases (one
functionally blind, the other with moderate to marked
limitation in the activities of daily living) remained unchanged, whereas 8 improved. Responder and nonresponder patients did not differ in regard to demographic
and clinical features. Following eye closure, either voluntary or involuntary, responder patients were able to raise
eyelids a few seconds after the command. Moreover, BTinduced improvement was accompanied by a reduction of
the frequency of the episodes of involuntary drooping of
the eyelids. Responder patients remained under treatment with 10 U BT per eye. They received a total of 107
treatment sessions with a mean follow-up of 2.7 years
(SD, 1.5) and a mean of 10.7 treatment sessions per
patient (SD, 4.6). Onset of improvement was noticed after
a mean of 4.2 days (range 3–7 days) and the mean duration of effect was 2.3 months (range 2–3 months). Overt
ptosis was never observed, whereas mild lacrimation lasting 2–5 weeks was reported in 15% of treatment sessions.
The 2 patients who did not respond to pretarsal administration of 10 U BT (table 1: cases 1 and 6) remained
unchanged even after increasing pretarsal BT up to 20 U
and inspite of adequate weakness of the pretarsal OO (revealed by difficulty in occluding the palpebral fissure
gently). One of these patients (table 1: case 6) experienced
complete remission of scAEO following discontinuation
of flunarizine (see above).

Isolated So-Called Apraxia of Eyelid
Opening

–
–
–
Depression
Facial weakness
Blepharitis
Not reported
–
Blepharochalasis

Review of the Literature
In reviewing the literature from 1965 to 1996, we
found 11 patients with isolated scAEO [3, 4, 8, 9, 17, 18]
in a total of approximately 200 reported cases. Detailed
clinical description was lacking in 3 patients [9]. In the
remaining 8 cases (table 2), age at onset was between 13
and 64 years (mean, 52.1; SD, 19.9), duration of symptoms was between 1 and 8 years (mean, 4.5; SD, 2.2), and
there was a female preponderance of 3:1. Four patients
reported antecedent events [4, 9, 17] including eye diseases (n = 2), facial weakness (n = 1) and depression (n =
1). In addition to inability to open eyes on command, episodes of involuntary drooping of the eyelids without any
ongoing OO contraction were described in 4 cases [3, 4,
17]. EMG recordings of OO and LPS on attempted eye
opening were available in 4 cases [3, 4, 8], with abnormalities being defined as excessive muscle activity limited to
the pretarsal OO (n = 2) or failure to initiate or sustain
LPS activity (n = 2).
Isolated scAEO completely resolved following treatment with l-dopa in 2 cases [17]. Seven patients were
treated with BT according to different injection protocols:
3 cases experienced BT treatment inducing global OO
weakening [8], but results were poor; 3 other cases were
treated with BT injections at the junction of preseptal and
pretarsal OO [9], but improvement was modest leaving
them with considerable residual diability; finally, BT
injections in the orbital OO and frontal muscles were of
benefit in a male patient [18].

Eur Neurol 1998;39:204–210

207

Discussion
scAEO has mainly been described in association with
diseases affecting the extrapyramidal system [2–11] or,
less frequently, the right hemisphere [12–14]. scAEO
developing in the absence of any CNS disease is generally
considered very rare: to our knowledge, only 11 such
patients have been described over a 30-year period [3, 4,
8, 9, 17, 18]. Thus, the origin, nature and clincial features
of isolated scAEO are uncertain.
To further investigate these issues, we looked for isolated scAEO among 150 consecutive outpatients with
excessive bilateral eye closure referred to our movement
disorder clinic over a 9-year period. According to Lepore
and Duvoisin’s [2] criteria, scAEO was diagnosed in 30
cases (20%), whereas 120 patients were followed for typical BS without discernible scAEO. Twenty scAEO patients (13.4%) suffered from extrapyramidal disorders,
including dystonia and parkinsonism, the remaining 10
patients (6.6%) had no clinical or radiological evidence of
CNS disease. Clinical-demographic features of patients
with isolated scAEO, including mean age at onset in the
6th decade and female preponderance, did not differ from
cases in the literature [3, 4, 8, 9, 17, 18]. Thus, our sample
was proably representative of isolated scAEO.
In another outpatient series of 207 cases, Krack and
Marion [9] observed 3 patients with isolated scAEO
(1.4%), 29 with scAEO and extrapyramidal disorders
(14%) and 175 with typical BS. The higher frequency of
isolated scAEO in our than in Krack and Marion’s population (6.6 vs. 1.4%) may have different explanations.
First, the percentage of patients with isolated scAEO may
change over time because of the development of other disorders such as dystonic BS. In our series, however, this
was unlikely. In fact, patients complaining of isolated
scAEO did not develop BS or other CNS diseases over
3–12 years, whereas coexistence of BS and scAEO was
noted 0.5–2 years after the onset of eyelid discomfort.
Second, prior underreporting of isolated scAEO may be
due to misdiagnosis of a psychiatric disorder. Accordingly, 3 of our patients and 1 in the literature [8] reported
antecedent psychiatric disturbances, i.e. anxiety and depression. However, the 20% incidence of anxiety and the
10% incidence of depression seen in our series are not
likely to be of significance because a similar frequency of
antecedent psychiatric disturbances was found in patients
affected by other eyelid motor disorders of physical origin
such as typical BS and hemifacial spasm [prior anxiety, 20
and 22% respectively; prior depression, 15 and 13%
respectively; personal obs.]. In addition, treatment of anx-

208

Eur Neurol 1998;39:204–210

iety and depression did not cure scAEO. The tentative
conclusion is that isolated scAEO is due to a physical disorder rather than to a conversion reaction similar to ‘hysterical blindness’ but manifested instead by eye closure.
Third, our patient population was from a regional referral
center for movement disorders instead of a quaternary
referral center. Therefore, our statistics may be closer to
the true prevalence rates.
Apraxia has been defined by Geschwind [22] as a disorder of execution of learned movements that cannot be
accounted for either by weakness, incoordination, or sensory loss. By definition, primary pathways related to eyelid motor activities are intact in scAEO [2]. In addition,
patients with isolated scAEO have no clear evidence of
CNS diseases. Although these considerations seem to justify the use of the term apraxia in isolated scAEO, the
occurrence of episodes of involuntary drooping of the eyelids raises the possibility of abnormal regulation of eyelid
motor function. Transient failure to sustain lid elevation
was not included in the definition of AEO as given by
Goldstein and Cogan [1] and Lepore and Duvoisin [2].
Nonetheless, it was also described in patients with scAEO
and parkinsonism [3, 5], and in 4/8 cases with isolated
scAEO reported in the literature [3, 4, 17]. Thus, inability
to initiate and failure to sustain lid elevation are probably
expressions of the same disturbance.
Four patients in the literature were investigated by
EMG [3, 4, 8]. Overactivity of the pretarsal part of the OO
or failure to initiate or sustain LPS activity were observed
during the episodes of lid closure. Interestingly, similar
abnormalities were described in patients with scAEO and
extrapyramidal disorders [3, 5, 6, 8]. We did not perform
EMG recordings of eyelid muscles, but the results of BT
treatment further suggest that eyelid motor control may
be abnormal in isolated scAEO. BT administration close
to the pretarsal OO reduced both frequency and duration
of the episodes of lid closure and improved the activities
of daily living in 8/10 patients with isolated scAEO,
whereas orbital/preseptal injections had no effect. At variance with our findings, most previous reports failed to
observe any significant effect of BT on isolated scAEO [8,
9]. Differences in the injection protocol may, at least in
part, explain the disagreement. Although BT can diffuse
to neihboring muscles causing clinical or subclinical
changes of neuromuscular transmission, our injection
protocol probably induced selective weakening of the pretarsal OO. In extrapolation from rat data [23], in fact, we
estimated that injecting 10 U BT/0.1 ml saline resulted in
an area of paralysis of approximately 35–50 mm2. This is
comparable with the area occupied by the pretarsal OO,

Defazio/Livrea/Lamberti/De Salvia/
Laddomada/Giorelli/Ferrari

and, accordingly, overt ptosis was never encountered. In
previous reports [8, 9], BT was not specifically directed to
the pretarsal OO. Thus, insufficient BT diffusion to the
pretarsal OO and/or possible spread to the LPS muscle
might have negatively influenced the outcome. Interestingly, application of BT into multiple sites of the OO,
including its pretarsal portion, was effective in scAEO
associated with, or triggered by, BS [6, 15, and personal
obs.]. Furthermore, pretarsal BT dramatically improved
scAEO in 3 of our 6 parkinsonian patients [personal obs.].
These findings suggest that similar mechanisms may underlie scAEO regardless of the clinical setting in which it
occurs.
Because the LPS and pretarsal OO muscles are reciprocally involved in a number of spontaneous and reflex
activities [24], weakening of the latter can, theoretically,
affect LPS tone. Thus, BT responder patients may suffer
from overactivity of the pretarsal OO, or, alternatively,
from involuntary inhibition of LPS tonic activity. Supporting the latter view, Aramideh et al. [6] described
4 patients with BS who initially failed to respond to orbital/preseptal BT injections, but later improved with pretarsal injection: these patients were found by EMG to
have both involuntary LPS inhibition and BS. LPS inhibition possibly associated with a disturbed reciprocal relationship of LPS and pretarsal OO muscles may be the
most likely explanation in the patients who did not
respond to BT despite of adequate pretarsal weakness.
If isolated scAEO is associated with an eyelid motor
dysfunction, what are the clues to its origin? Dewey and
Marangore [17] described 2 patients in whom AEO developed in the absence of any other CNS signs and resolved
following treatment with l-dopa. This suggests an extrapyramidal dysfunction as possible explanation for isolated
scAEO. Supporting this view, a few points can be made.
First, development of isolated scAEO in one of our
patients following prolonged exposure to flunarizine, and
sustained remission when the drug was stopped, was in
favor of a causal relationship between isolated scAEO and
flunarizine, a drug known to induce extrapyramidal reactions [25]. Second, 4 of our 10 patients and 2/8 cases in
the literature experienced diseases or irritations of the
eyes prior to the onset of their scAEO. In another case
scAEO followed prednisone-responsive bilateral facial
weakness [17]. Overall, local injury to the eyes or face preceded the eyelid motor disturbance in 7/18 cases (39%).
This is unlikely to be coincidental and adds to an increasing number of reports suggesting that local injury can trigger topographically related extrapyramidal movement
disorders in predisposed subjects [26]. Because scAEO

Isolated So-Called Apraxia of Eyelid
Opening

onset may be prolonged or difficult to detect, local injury
to the eyes or face may accompany or follow the eyelid
motor disturbance without influencing the risk of scAEO.
The presence of sensory tricks (geste antagonistique) in
patients with isolated scAEO makes further contribution
to the hypothetical extrapyramidal involvement, since
tricks may be common in dystonia as well as in parkinsonism [27]. Finally, family history of cranial dystonia in one
of our patients suggests a familial, possibly genetic, vulnerability of the extrapyramidal system. Familial clustering of isolated scAEO in another patient further supports
the possibility of a genetic contribution, even though
familial clustering may be coincidental or due to shared
environmental factors.
Based on the above considerations, it is highly tempting to conclude that apraxia may not be the correct
descriptive term, even when scAEO occurs without any
other CNS disease. The results of our survey, namely
those of BT treatment, suggest that isolated scAEO may
be caused by a range of disorders involving motor activities in pretarsal OO and LPS muscles. A correlative EMGBT study may be advised to further investigate the mechanism(s) underlying isolated scAEO, and to provide a correct definition of this underestimated, chronic, and frequently disabling cause of excessive eye closure.

Acknowledgements
The authors thank Mr. Donato Salamanno for computer-assisted
review of the literature.

Eur Neurol 1998;39:204–210

209

OOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOOO

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