Hemifacial Spasm in Singapore: Clinical Characteristics and Patients' Perceptions

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324 Hemifacial Spasm in Singapore—WL Au et al

Original Article

Hemifacial Spasm in Singapore: Clinical Characteristics and


Patients’ Perceptions
WL Au,1FAMS, MRCP (UK), LCS Tan,2FAMS, MRCP (UK), AKY Tan,3FAMS, MRCP (UK)

Abstract
Introduction: The aim of this study was to determine the clinical characteristics and patients’
perception of hemifacial spasm (HFS) in Singapore. Materials and Methods: A clinical survey of
137 consecutive patients with HFS seen in our Botulinum Toxin Clinic over a 15-month period
was undertaken. Results: Forty-six men and 91 women were interviewed. Their mean age at onset
of HFS was 48 years. The median disease duration was 60 months (range, 2 to 360 months). Left-
sided spasm was common in 51.8% of patients, and the orbicularis oculi was the first muscle to
be affected in 86.1% of them. The majority (65%) had the spasm aggravated by stress and
anxiety. In fact, 32 patients perceived stress and anxiety as a possible aetiology of HFS. Stroke
was a main concern in 17 patients and 7 patients thought the spasm was a sign of demonic
possession or a bad omen. The spasm embarrassed 75.2% of the patients, rendered 65% of them
depressed, affected the vision in 60.6% of them and compromised their work performance in
35.8%. Overall, treatment was delayed by a median interval of 6 months from onset of symptoms
(range, 0 to 132). More than half (53.3%) tried traditional therapies (acupuncture or herbal
medicine), while only 48.2% had botulinum toxin as the initial treatment. All patients eventually
received botulinum toxin injections and more than 90% showed improvement at 1 month post-
treatment. Conclusions: The clinical characteristics and patients’ perception of HFS in Singapore
were presented. HFS affects patients both psychosocially and functionally. Effective treatment
with botulinum toxin exists and should be provided early to the patients.
Ann Acad Med Singapore 2004;33:324-8

Key words: Botulinum toxin, Facial myoclonus, Psychosocial

Introduction that it occurs more frequently in Orientals than in


Hemifacial spasm (HFS) is a movement disorder Caucasians.3-5 The reason for this is not known. One
characterised by intermittent involuntary contractions of possible explanation is the smaller bony cranium among
the facial muscles. It begins with contractions of the Asians, which results in crowding of cranial nerves and
orbicularis oculi, gradually spreading to involve the rest of vascular structures.6 HFS is believed to occur as a result of
the ipsilateral facial muscles. Bilateral HFS is rare. 1 The compression of the facial nerve at its point of exit from the
contractions in these patients were asymmetrical and pons by nearby structures, usually an ectatic vessel, some
asynchronous, thus differentiating them from other disorders vascular anomalies or other pathological lesions.7 In a local
of facial movement, such as blepharospasm and orofacial study by Tan et al,8 22 out of 25 patients with HFS were
dystonia. found to have an abnormal vessel in close proximity to the
An epidemiological study in Minnesota documented a facial nerve.
prevalence rate of HFS to be 7.4 per 100,000 in men and Microvascular decompression of the facial nerve is an
14.5 per 100,000 in women, mostly among those aged from accepted treatment for HFS. 9 It is more effective than most
40 to 79 years old.2 The actual prevalence of HFS in medical therapies, such as carbamazepine, 10 clonazepam,11
Singapore is not known, but anecdotal experience suggests orphenadrine 12 and baclofen.13 However, its potential

1
Associate Consultant
2
Consultant
3
Visiting Consultant
Department of Neurology
National Neuroscience Institute, Singapore
Address for Reprints: Dr Louis CS Tan, Department of Neurology, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore 308433.

Annals Academy of Medicine


Hemifacial Spasm in Singapore—WL Au et al 325

surgical risks may deter patients from seeking this treatment. with left-sided HFS at initial presentation. Only 1 patient
The surgical risks include impaired hearing in 10% to 15%, had bilateral facial involvement. The spasm first affected
facial weakness in 6% and less than 1% mortality.14 the orbicularis oculi in 118 (86.1%) patients. In 16 (11.7%)
In recent years, botulinum toxin injection is offered as an patients, the lower facial muscles were involved first. Two
alternative treatment to surgery for patients with HFS.15 It patients felt the upper and lower facial muscles started to
is already well-established as an effective treatment with twitch at the same time, while 1 patient was unsure of the
mild and transient side effects.3,16-21 However, there is little initial site of involvement. Among those who had initial
information on patients’ perception of this condition. Most eyelid involvement, the lower eyelid was affected in 64
patients are not aware that HFS is a medical condition with patients, the upper eyelid in 43 patients and both upper and
effective treatment available. Ironically, they associate lower eyelids in 11 patients.
botulinum toxin injection more with its cosmetic uses than Stress and anxiety were the major precipitating factors in
with its primary role in neurological conditions. Thus, we 89 (65%) patients. Talking aggravated the spasm in 48
conducted this survey to determine the clinical (35%) patients and eating affected the spasm in 25 (18.2%)
characteristics and patients’ perception of HFS. patients. Both men and women had their spasms aggravated
by these factors in almost equal proportions.
Materials and Methods
The patients were asked to document their perceived
The National Neuroscience Institute is one of the major aetiologies of HFS. Twenty-five (18.2%) patients were
centres in Singapore that provide tertiary care to patients unable to provide an answer. The rest were able to give at
with neurological disorders. We conducted a clinical least 1 reason they thought was the likely cause of HFS
survey of 137 consecutive patients with HFS who attended (Table 1). The patients’ perception of HFS could be divided
the Botulinum Toxin Clinic over a 15-month period. The into 3 main categories. One group believed it to be a
diagnosis of HFS was made by a neurologist and verified physiological process, another group considered it a
by a movement disorder specialist. All patients had supernatural phenomenon and the last group attributed it to
idiopathic HFS and none had an identifiable primary cause, other pathologies. Of the 14 patients who gave “Others” as
such as a previous Bell’s palsy or trauma. The patients were reasons, many blamed it on concurrent illnesses or injuries
examined and interviewed by neurologists using a and ingestion of wrong food or medicine; 2 patients thought
standardised questionnaire. Information on clinical it was due to “wind”.
characteristics and patients’ perception of the disorder
HFS embarrassed 103 (75.2%) patients. In fact, 89 (65%)
were recorded. For clinical characteristics, we studied the
patients felt depressed because of the spasm. Vision was
patient demographics, disease presentation, aggravating
affected in 83 (60.6%) patients. Work performance was
factors and treatment modalities. For patients’ perception
compromised in 49 (35.8%) patients. Table 2 shows the
of the disorder, the patients were asked to document their
impact of HFS on men and women. Women felt more
perceived aetiologies of HFS and the extent to which the
spasm had embarrassed them, made them depressed,
affected their vision and compromised their work Table 1. Patients’ Perception of Hemifacial Spasm (n = 137)

performance. All patients eventually had botulinum toxin Perceived aetiologies No. (%)* Median time to
injections. At 1-month post-treatment, they were re- treatment in
evaluated on the impact of HFS on their psychosocial and months (range)

functional well-being. Physiological process


Stress and anxiety 32 (23.4) 6.0 (0-120)
Results Lack of sleep 10 (7.3) 3.0 (0.25-48)
Normal ageing 6 (4.4) 24.0 (1-48)
A total of 137 patients (46 men and 91 women) were
interviewed. Their mean age at onset of HFS was 48 years Supernatural phenomena
Demonic possession/bad omen 7 (5.1) 3.0 (0.25-18)
(range, 15 to 82 years), with no significant differences in
age distribution between the two genders. The patients had Other pathologies
Stroke 17 (12.4) 2.5 (0-132)
a median disease duration of 60 months (range, 2 to 360
Eye problem 14 (10.2) 9.0 (1-48)
months). There were 96% Chinese, 2% Malays, 1% Indians, Vascular compression 12 (8.8) 18.0 (0-78)
and 1% of other ethnic groups. This racial composition was Dental problem 3 (2.2) 48.0 (3-60)
significantly different from the nation’s ethnic composition, Others† 14 (10.2) 12.0 (0.25-72)
which is 77% Chinese, 14% Malays, 8% Indians and 1% Uncertain 25 (18.2) 9.0 (1-48)
other ethnic groups. Malays and Indians were significantly *
Three individuals gave more than 1 reason.
under-represented in our series. †
These include concurrent illnesses or injuries, ingestion of wrong food
Slightly more than half (51.8%) of 137 patients presented or medicine and “wind”.

May 2004, Vol. 33 No. 3


326 Hemifacial Spasm in Singapore—WL Au et al

Table 2. Impact of Hemifacial Spasm on Patients HFS as supernatural phenomena, all except 1 sought
Impact of hemifacial spasm Percentage affected traditional treatment.
Men (n = 46) Women (n = 91) All patients eventually received botulinum toxin
injections, but only 126 patients were available for
Embarrassment
Never 26% 24% evaluation at 1 month post-treatment. If we consider missing
Sometimes 46% 36% data as treatment failure, using intention-to-treat analysis,
Very Often 28% 40% 125 (91.2%) patients experienced some improvement
Depression following botulinum toxin injections. In fact, 74.4% of
Never 43% 31% patients experienced more than 60% improvement in the
Sometimes 41% 37% spasm. Of the 89 patients who felt depressed initially, 79
Very Often 15% 32% (88.8%) were less so after the injections. Similarly, 83
Vision impairment (80.6%) of 103 patients who were embarrassed initially felt
Never 46% 36% less so after the botulinum toxin treatment. Of these, in
Sometimes 48% 44%
terms of functional improvement, 73.5% documented
Very Often 7% 20%
improvement in their vision because of less eye closure. All
Interference with work performance
49 patients who had their work performance affected
Never 61% 66%
Sometimes 30% 25% initially felt they worked better after the botulinum toxin
Very Often 9% 9% injections.

Discussion
To date, this is one of the largest series of HFS patients
embarrassed, more depressed and had their vision affected published in Singapore. 3,8,16,17 Although not a population-
more often than men. Work performance was similarly based study, we infer from our series that HFS is relatively
affected in both genders. common in Singapore. In 15 months, we saw a total of 137
Table 1 shows the median interval from onset of symptoms patients with the condition. In contrast, it took Wang and
to initial treatment. On average, the patients waited for a Jankovic22 17 years to collate 158 cases in Texas, and Horn
median interval of 6 months (range, 0 to 132 months) from and Wuu23 3 years to see 65 patients in Chicago. When the
onset of symptoms before seeking medical advice. Patients racial composition in our series was compared to that of
who presented earliest to the clinic (median interval, 2.5 to Singapore, we observed an over-representation of Chinese.
3 months) perceived HFS as a manifestation of stroke or This difference could be spurious, reflecting a difference in
thought that it was due to lack of sleep or some supernatural the utilisation of healthcare services amongst the different
phenomena. Those who presented in the intermediate ethnic groups, or it could be real, arising as a result of a
range (median interval, 6 to 9 months) thought their racially determined risk factor for HFS that is as yet
condition was due to stress and anxiety or some eye unknown. Future population-based studies are needed to
problem. The group of patients who presented latest to the confirm this observation. Apart from the difference in
clinic (median interval, 24 to 48 months) were those who racial composition of our series, the other clinical
thought it was a normal ageing process or due to some characteristics, such as age group distribution, gender
dental problem. distribution, site and side of involvement, were comparable
to those of other series. 4,7,8,20,22
Botulinum toxin injections, as the initial treatment, were
used by 66 (48.2%) patients. The treatment was delayed by Studies on botulinum toxin as an effective treatment for
a median interval of 9 months (range, 0 to 120 months). HFS are available both locally3,16,17 and overseas.5,19,21
The remaining patients tried various therapies before turning However, there is little literature on patients’ perception of
to the Botulinum Toxin Clinic. Carbamazepine was the illness. Information on the psychological aspect of the
prescribed in 14 (10.2%) patients, benzodiazepines (other condition is also scarce.24 We document, for the first time,
than clonazepam) in 12 (8.8%) patients, clonazepam in 3 patients’ perception of HFS in Singapore. Broadly, the
(2.2%) patients and phenytoin in 2 (1.5%) patients. Health patients can be grouped into 3 main categories. One group
supplements and vitamins were prescribed in 33 (24.1%) of patients thought HFS was a normal physiological process,
patients; another 15 (10.9%) patients were unsure of their occurring as a result of stress and anxiety, lack of sleep or
medication. Traditional methods of treatment were popular normal ageing process. Another group believed in
among 73 (53.3%) patients, half of whom had acupuncture, supernatural phenomena, attributing HFS to demonic
10 used traditional herbs and 27 tried both acupuncture and possession or a sign of bad omen. The last group blamed the
traditional herbs. Among the 7 patients who considered spasm on other pathologies, such as stroke, eye problem,

Annals Academy of Medicine


Hemifacial Spasm in Singapore—WL Au et al 327

dental condition, concurrent illness and ingestion of wrong vitamins and health supplements. These were obviously
food or medicine. The patients’ perception of their condition not effective as all patients eventually had botulinum toxin
clearly affected their behaviour. Those who thought the injections.
spasm was a manifestation of a serious condition, such as As with most studies,3,5,16,17,19,21 botulinum toxin was
stroke, sought treatment early (a median interval of 2.5 effective in controlling the spasm. More than 90% of
months from onset of symptoms). They also presented patients felt improvement following botulinum toxin
early for treatment if the spasm affected their psychosocial injections. They were less embarrassed, less depressed and
well-being; those who felt the spasm to be a symptom of could see better with less eye closure. They regained their
stress and anxiety or sleep deprivation, turned up for confidence and all felt improvement in their work
treatment within a median interval of 3 to 6 months. On the performance.
other hand, those who considered HFS to be a normal
ageing process waited for a median interval of 2 years Conclusions
before seeking medical advice. The number of patients HFS is common among Orientals. It is not only of
who attributed the spasm to ageing or supernatural cosmetic concerns, but also affects individuals both
phenomena in our series were small, possibly because psychosocially and functionally. Misconceptions over the
these patients were less likely to seek medical treatment. In aetiology of HFS are plenty, and contribute to the delay in
light of this, it is believed that a sizable number of patients seeking treatment. There is a need to educate the public on
remain undiagnosed in the community. Public education this condition, to clarify their misconceptions and to bring
on the condition is, thus, important. Physicians can also to their attention that botulinum toxin is an effective
help by recognising the disorder early through opportunistic treatment for HFS. Physicians can play a part by recognising
screening and refer patients to the appropriate department this condition early and referring affected individuals for
for treatment. appropriate investigations and treatment.
HFS is frequently aggravated by fatigue, stress, anxiety
Acknowledgements
or self-consciousness.7 Sixty-five per cent of our patients
had the spasm aggravated by stress and anxiety. Although We thank Drs Lee Kim En, Nigel Tan, and Soo Hua Huat
the reason for this is not known, we postulate that stress and and Staff Nurse Lau Puay Ngoh for their assistance in data
anxiety result in sympathetic over-drive that enhances collection. We also thank all neurologists in National
facial nerve irritation. Besides stress and anxiety, our Neuroscience Institute who referred patients to the
patients also reported worsening of the spasm during Botulinum Toxin Clinic.
talking and eating. A possible explanation could be that
patients are more self-conscious during activities that involve
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