|Year : 2022 | Volume
| Issue : 2 | Page : 118-120
Hemifacial spasm responsive to single low-dose abobotulinumtoxin A in a patient with relapsing-remitting multiple sclerosis: A case report
Julie Ann Kristy L Torres1, Meliza Angelica J de Leon1, Raymond L Rosales2
1 The Neuroscience Institute, Department of Neuroscience and Behavioral Medicine, University of Santo Tomas Hospital, Manila, Philippines
2 The Neuroscience Institute, Department of Neuroscience and Behavioral Medicine, University of Santo Tomas Hospital; The Research Center for Health Sciences, Faculty of Medicine and Surgery, University of Santo Tomas, Manila, Philippines
|Date of Submission||01-Oct-2021|
|Date of Decision||12-Nov-2021|
|Date of Acceptance||15-Dec-2021|
|Date of Web Publication||28-Jun-2022|
Dr. Julie Ann Kristy L Torres
Department of Neuroscience and Behavioral Medicine, University of Santo Tomas Hospital, Manila - 1015, Metro Manila
Source of Support: None, Conflict of Interest: None
Hemifacial spasm (HFS) is a movement disorder characterized by involuntary twitching of the facial muscles of one side of the face. Here, we report the case of a 31-year-old woman with relapsingremitting multiple sclerosis who presented with left-sided HFS while receiving interferon beta. Despite immunosuppressive medications and clonazepam, only a partial response was documented. Near complete resolution of HFS was achieved 6 months after a single low dose of abobotulinumtoxin A was injected into the left periocular muscles; no recurrence was observed after 4 years. Botulinum toxin injection for focal dystonia, such as HFS, may lead to long-term symptomatic benefit in patients with multiple sclerosis who have already received optimum medical treatment.
Keywords: Abobotulinumtoxin A, dystonia, hemifacial spasm, movement disorder, multiple sclerosis
|How to cite this article:|
Torres JA, de Leon MA, Rosales RL. Hemifacial spasm responsive to single low-dose abobotulinumtoxin A in a patient with relapsing-remitting multiple sclerosis: A case report. Ann Mov Disord 2022;5:118-20
|How to cite this URL:|
Torres JA, de Leon MA, Rosales RL. Hemifacial spasm responsive to single low-dose abobotulinumtoxin A in a patient with relapsing-remitting multiple sclerosis: A case report. Ann Mov Disord [serial online] 2022 [cited 2022 Dec 7];5:118-20. Available from: https://www.aomd.in/text.asp?2022/5/2/118/348767
- Hemifacial spasm is rarely associated with multiple sclerosis
- Response to treatment has been variable
- Botulinum toxin may potentially lead to long-term symptomatic benefit
| Introduction|| |
Multiple sclerosis (MS) is an autoimmune inflammatory demyelinating disease of the central nervous system characterized by multifocal areas of demyelination in the brain and spinal cord, disseminated in time and space with subsequent scarring. Hemifacial spasm (HFS) and other movement disorders are rarely associated with MS. Among movement disorders, tremor is the most common finding, with a prevalence of 1.6%. Other reported abnormal movements, such as dystonia, ballismus, chorea, parkinsonism, myoclonus, restless legs syndrome, facial myokymia, and HFS, may be causally or coincidentally associated with MS., HFS is characterized by synchronous nonsustained spasms of unilateral facial muscles. Among the reported MS cases, unilateral or bilateral peripheral facial palsy rarely precedes HFS. Furthermore, treatment responses to intravenous corticosteroids, carbamazepine, baclofen, and clonazepam have been variable.
| Case Report|| |
A 31-year-old woman, a physician by profession, with a 5-year course of relapsing- remitting MS and an Expanded Disability Scale Score of 1.0, sought consultation due to left-sided intermittent and brief contractions of the orbicularis oculi and perioral muscles. The spasms were not preceded by peripheral facial weakness or infection. During this time, she was maintained on 0.25-mg interferon beta, subcutaneously injected twice weekly. Months prior to the onset of HFS, the clinical course of her MS was marked by episodes of neurologic deficits such as blurring of vision in both eyes occurring sequentially, right leg numbness, and intermittent right facial numbness. These symptoms improved after short courses of high-dose oral methylprednisolone; thereafter, she had minimal disability in between the episodes.
On examination, there was irregular, brief, nonsustained twitching of the orbicularis oculi and oris muscles on the left side. No facial weakness was noted. A relapse was considered and a brain magnetic resonance imaging (MRI) [Figure 1] was performed, which revealed the interval development of a T2-weighted and T2 FLAIR nonenhancing patchy ovoid signal abnormality on the left lateral pons at or near the projected anatomical location of the root exit zone of the left facial nerve. Interferon beta dose was increased to every other day and a short course of high-dose oral methylprednisolone was given. For HFS, titrated doses of carbamazepine, pregabalin, and baclofen led to adverse events, such as somnolence, which was unfavorable during her clinical duties as a physician. Partial relief of spasms was obtained with 3-mg per day clonazepam, which caused considerable drowsiness (Supplementary Video 1 [Additional file 1]). After 2 months on the regimen, oral methylprednisolone and beta interferon were gradually tapered and clonazepam was discontinued. Despite the treatment, there was only partial resolution of the left perioral spasms and persistence of spasms at the periocular region. In addition, the patient was concerned regarding the aesthetic effect of HFS and reported that the spasms were unpleasant. Therefore, a low dose of abobotulinumtoxin A at 5 units per site was subcutaneously injected at two periocular points (i.e., left orbicularis oculi and lateral upper and lower eyelids). Although the recommended dose of abobotulinumtoxin A injections for HFS range from 10 to 20 units per site, the aforementioned dose was agreed upon with the patient, in view of the moderate, intermittent spasms predominantly involving the periocular muscles. On follow-up at 6 months postinjection, there was near complete resolution of periocular and perioral involuntary movements (Supplementary Video 2 [Additional file 2]).
|Figure 1: Gadolinium-enhanced brain magnetic resonance imaging of the patient|
(T2-weighted and T2 FLAIR sequences). The images show nonenhancing patchy
ovoid signal abnormalities at the left lateral pontine region (indicated by red arrows)
Click here to view
A repeat brain MRI was performed 8 months after the onset of HFS. There was evidence of interval decrease in the size of the demyelinating plaque at the left lateral pons. Since then, the patient has been maintained on immunotherapy with a shift from beta interferon to 0.5- mg fingolimod once a day, with the goal of reducing the frequency of relapses and achieving a better quality of life. Her current neurologic status is stable and she has no documented physical disability. At the time of this report (4.5 years from spasm injections), there has been no recurrence of HFS.
| Discussion|| |
Movement disorders involving the facial muscles, such as continuous facial myokymia (CFM) and HFS, have been reported in MS patients. In a retrospective study by Collazo et al., CFM and HFS were found to be associated with the clinical relapse of MS in some patients with radiographic evidence of ipsilateral pontine lesions on MRI. In contrast to HFS, CFM is an involuntary, undulating, vermicular, fine movement, which spreads across facial muscles. While HFS may persist despite treatment, CFM is usually self-limited regardless of treatment; however, there were two reported cases, in which botulinum toxin was used for symptomatic benefit.
HFS has been shown to be an uncommon manifestation in MS. There have been <20 reported cases in literature since the 1980s., Although unusual, as in the present case report, ipsilateral facial spasms may occur if the demyelinating process strategically occurs along the course of the facial nerve. The mechanism by which central demyelination in MS leads to HFS maybe due to a demyelinating process occurring at the root exit zone of the facial nerve, where the nerve is susceptible to injury due to the lack of epineurium. Demyelination in this area causes ephaptic transmission leading to abnormal firing in the neurons. This was distinctly demonstrated in the patient's MRI when she developed left HFS.
The response to treatment for HFS in MS in the reported literature is variable. Oral medications such as carbamazepine, oxcarbazepine, pregabalin, and gabapentin, along with intravenous corticosteroid, have provided some symptomatic benefit without complete resolution of HFS., HFS may persist, despite immunotherapy. In addition, symptomatic treatment for HFS using botulinum toxin injection has been reported in three cases in a cohort of seven patients by Collazo et al. The response to treatment was variable, from partial to good, even in the presence of immunotherapy. However, in the present case, a single session of abobotulinumtoxin A injection led to both symptomatic benefit and near complete resolution of HFS, 6 months postinjection. In the present case, no further injections were required in the lower facial area, and notably, a single toxin injection led to long-term clinical benefit.
In conclusion, while uncommon in MS, HFS may occur when the demyelinating process strategically involves the root exit zone of the facial nerve. As demonstrated in this present case, despite symptomatic treatment and immunotherapy, a low-dose botulinum toxin focal injection at spasm sites should be considered as a therapeutic option, which may potentially lead to long-term symptomatic benefi without systemic adverse events.
The authors wish to acknowledge the contribution of Dr. Jonna N. Datu in providing additional clinical data for the case report.
Julie Ann Kristy L. Torres - initial draft preparation, revision of final manuscript
Meliza Angelica J. de Leon - initial draft preparation
Raymond L. Rosales - review and editing, supervision, revision of final manuscript
Ethical compliance statement
Written informed consent was obtained from the patient for the presentation for her case as well as the accompanying images and videos. Other identifying data were not disclosed in the manuscript. The case report was written in compliance with the Case Report Guidelines (CARE) of 2016. Institutional research ethics approval was obtained prior to submission for publication.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Polman C, Reingold S, Banwell B, Clanet M, Cohen JA, Filippi M, et al
. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol 2011;69:292-302.
Nociti V, Bentivoglio AR, Frisullo G, Fasano A, Soleti F, Iorio R, et al
. Movement disorders in multiple sclerosis: Causal or coincidental association? Mult Sclr 2008;14:1284-7.
Mehanna R, Jankovic J. Movement disorders in multiple sclerosis and other demyelinating diseases. J Neurol Sci 2013;328:1-8.
Collazo IVM, Tobin WO. Facial myokymia and hemifacial spasm in multiple sclerosis: A descriptive study on clinical features and treatment outcomes. Neurologist 2018;23:1-6.
Chaudhry N, Srivastava A, Joshi L. Hemifacial spasm: The past, present and future. J Neurol Sci 2015;356:27-31.