Disparity in Periocular Manifestations between Hemifacial Spasm and Post-Paralytic facial Syndrome

doi:10.21203/rs.3.rs-4447870/v1

Download PDF

Article

Disparity in Periocular Manifestations between Hemifacial Spasm and Post-Paralytic facial Syndrome

https://doi.org/10.21203/rs.3.rs-4447870/v1

This work is licensed under a CC BY 4.0 License

You are reading this latest preprint version

Objective

This study aims to describe the differences in periocular manifestations among patients with hemifacial spasm and post-paralytic facial syndrome.

Methods

A retrospective study was conducted at two tertiary-care eye centers in Riyadh, Saudi Arabia. Data of patients with hemifacial spasm and post seventh nerve paralytic facial syndrome who received management and follow-up at the two hospitals were collected from the electronic medical records.

Results

We had a total of 67 subjects with abnormal facial movement, out of which 33 patients had hemifacial spasm and 34 patients had post-paralytic facial syndrome. 39 (58.2%) were female and 28 (41.8%) were male. The average age of the subjects under study was 46.5 years. Clinical features were noted for patients with hemifacial spasm including orofacial muscle twitching in 31 patients (93.9%), eyelid ptosis in five patients (15%), reveres ptosis in one patients (3%), no brow ptosis, and complaints of one eye being smaller than the other eye in five patients (15%). Among patients with post-paralytic facial syndrome, orofacial muscle twitching was found in 23 patients (67.6%), eyelid ptosis in 32 patients (94%), reveres ptosis in 29 patients (85.3%), brow ptosis in 28 patients (82.4%), and complaints of one eye being smaller than the other eye in 94.1%.

Conclusion

We have observed that patients with post-paralytic facial syndrome tend to exhibit periocular manifestations such as eyelid ptosis, reverse ptosis and brow ptosis more frequently than patients with hemifacial spasm.

post-paralytic facial syndrome

hemifacial spasm

reverse ptosis

eyelid ptosis

brow ptosis

Hemifacial spasm is a form of dystonia that affects one side of the face. It causes the orbicularis oculi muscle on one side of the face to involuntary contract. z, which may eventually spread to other facial muscles, leading to twitching and tension. As the condition progresses, the spasm may become more persistent and severe, making everyday activities difficult. Additionally, contractions in one facial muscle group may trigger spasms in other facial muscles, further worsening the condition.¹

Post-paralytic facial syndrome (PFS) is another condition that can cause facial muscle spasms. It occurs after peripheral facial paralysis and is characterized by synchronous and involuntary muscle contractions during intentional movements. About 10–30% of all patients with idiopathic facial palsy may develop synkinesis.¹ Different patterns of facial synkinesis do exist, with oral-ocular synkinesis being the most common. In this pattern, attempting to smile may cause involuntary eye closure. The severity of these symptoms may vary, and severe synkinesis can lead to a significant negative impact on patients’ physical, social and psychological well-being.^{1, 2}

The goal of our study is to investigate the differences in periocular manifestations between patients with hemifacial spasm and those with post-paralytic facial syndrome and to provide a possible explanation for these differences.

The current retrospective study was approved by the institutional review board (IRB) in King Saud University, Ritadh, KSA, and it was conducted at King Khaled Eye Specialist Hospital and King Saud University Medical City in Riyadh, Saudi Arabia. This study adhered to the ethical principles outlined in the Declaration of Helsinki as amended in 2013. The data of patients with hemifacial spasm and post seventh nerve paralytic facial syndrome who received management and follow-up at the two hospitals were collected from the electronic medical recordsfrom January 1st, 2014 to December 31th, 2023, with a total number of 67 patients.

The data collection included patients’ gender, age, duration prior to presentation, diagnosis, laterality, past medical and surgical history, lagophthalmos, eyebrow abnormality, eyelid abnormalities, exposure keratopathy, face abnormality and aberrant regeneration in addition to periocular non-surgical i surgical treatment provided.

The collected data were stored, managed and coded in a spreadsheet using Microsoft Excel 2010® software, and they were analysed using StatsDirect® statistical software, version 2.7.2 (StatsDirect Ltd., Cheshire, UK) and SPSS® version 22.0 (IBM Inc., Chicago, Illinois, USA). Descriptive analysis was done in which categorical variables were presented as frequencies and percentages, and continuous variables as mean (± Standard Deviation) and Range (minimum to maximum).

In our study, we had a total of 67 patients including 33 patients with hemifacial spasm and 34 patients with post-paralytic facial syndrome. The gender disparity was as follows: 39 (58.2%) females and 28 (41.8%) males. The average age of the patients at presentation was 46.5 years, with a standard deviation of 14.1.

Regarding the clinical features of hemifacial spasm, superficial punctate keratitis(SPKs) was noted in five cases (15.2%), lower eyelid entropion in one case (3%), involuntary eyelid closure in 33 cases (100%), involuntary twitching of orofacial muscle in 31 cases (93.9%), upper eyelid ptosis in five cases (15.2%), lower eyelid reverse ptosis in one cases (3%), and a complaint of the eye in the affected side being smaller than the other eye in five cases (15.2%). Brow ptosis, lagophthalmos, corneal changes (epithelial defect or ulcer) and lid ectropion were not observed.

On the other hand, the clinical features noted for post-facial nerve palsy included superficial punctate keratitis in five cases (14.7%), involuntary eyelid closure in 22 cases (64.7%), involuntary twitching of orofacial muscle in 23 cases (67.6%), upper eyelid ptosis in 32 cases (94.1%), lower eyelid reverse ptosis in 29 cases (85.3%), brow ptosis in 28 cases (82.4%), lagophthalmos in two cases (5.9%) and a complaint of the eye in the affected side being smaller than the other eye in 32 cases (94.1%). Entropion, corneal changes (epithelial defect or ulcer) and lid ectropion were not noticed (Table 1).

Table 1

clinical presentation of patients with hemifacial spasm and post facial nerve palsy total of 67 patients
Clinical Features noted for	Hemifacial spasm No. (n = 33)(%)	Post-facial nerve palsy No. (n = 34)(%)
Superficial punctate keratitis	5 (15.2)	5 (14.7)
Entropion	1 (3)	0 (0.0)
Involuntary eyelid closure	33 (100)	22 (64.7)
Corneal changes (epithelial defect or ulcer)	0 (0.0)	0 (0.0)
Involuntary twitching of orofacial muscle	31 (93.9)	23 (67.6)
Ptosis	5 (15.2)	32 (94.1)
Reverse ptosis	1(3)	29 (85.3)
Brow ptosis	0 (0.0)	28 (82.4)
Lagophthalmos	0 (0.0)	2 (5.9)
Lid ectropion	0 (0.0)	0 (0.0)
complain of one eye smaller than other eye	8 (24.2)	32 (94.1)

(Table 2) Type of intervention among 67 patients

Intervention	No. (%)
No intervention	2 (3.0)
Electrolysis	0 (0.0)
Botox injection	65 (97)
Oral medication	0 (0.0)
Surgery	2 (3.0)
Acupuncture	2(3.0)

Botulinum toxin injection was the most common intervention, accounting for approximately 97% (65 / 67) of the cases. No intervention was used in approximately 3% (2 / 67) of the cases. Similarly, both surgery and acupuncture were employed in approximately 3% (2 / 67). It was noted all cases that underwent surgery and acupuncture still received Botox. Notably, no instances of electrolysis or oral medication were found among the analyzed intervention (Table 2).

Peripheral facial palsy is a nerve injury that can happen without a clear reason or due to some factors such as herpes virus infection, compression, trauma and several other causes. Bell's palsy, the most severe form of idiopathic peripheral facial palsy, is the most common cause. It typically lasts for a few weeks and then resolves on its own. Facial nerve axons begin to regrow and reconnect with muscle fibers around three months after an axonal damage. While this process generally leads to a gradual improvement in muscle function on the affected side, it doesn't typically result in normal function. Instead, abnormal axonal regeneration can cause issues like synkinesis, myokymic discharges and muscle spasms. The severity of these conditions depends on various factors, including the extent of the initial axonal damage.³

During healing process, motor neurons undergo significant metabolic and histological changes such as increased synthesis of RNA and growth-associated proteins, in addition to the removal of synapses from the motoneuronal membrane. Furthermore, significant changes in the morphology and function of regenerated nerve axons are observed leading to enhanced motor neuronal excitability. These changes contribute to the development of post-paralytic facial syndrome.^3,4 Along with axonal changes, research has confirmed the increased excitability of the facial nucleus in patients with facial synkinesis.²

Hemifacial spasm can develop as a result of compression at the facial root entry zone, which may be due to tumors or vascular malformations. The classic form of this condition occurs with the existence of an arterial compression on the facial nerve in the posterior fossa, leading to hyperexcitability of facial motoneurons with subsequent spontaneous or reflex firing of motoneurons.^{5, 6}

Electrophysiological examinations of patients with hemifacial spasm have revealed interesting features, including ectopic generation of discharges and ephaptic transmission or lateral spread. Ephaptic transmission is believed to occur due to shunts between axons. With exciting and inducing synchronous muscle movements through one fibre, nerve impulses are conducted to another fiber. Research has found that shunts are more likely to occur in the root entry zone where facial nerves lack the protection of the myelin sheath.^{2, 7} Oge and his team has indicated that the main difference between patients with postparalytic facial syndrome and those with hemifacial spasm is the longer latencies in the former.^{6, 8}

Upper eyelid ptosis is defined by eyelid margin lying abnormally low in primary gaze.⁽⁹⁾ Ptosis is classified based on the degree of drooping: mild with 2mm or less, moderate with 3mm, and severe with 4mm or more.¹⁰ In our study, the patients had mild to moderate ptosis ranging from 1.5 to 3mm (mean 2mm) of marginal reflex distance 1 (MRD1) in the affected side. 32 of our patients with post-facial nerve palsy syndrome had ptosis compared to only five patients with Hemifacial spasm.

On the other hand, reverse ptosis is characterized by the upward elevation of the lower eyelid, which is opposite to the normal position around the inferior corneal limbus.¹¹ In the current study, reverse ptosis was observed in 29 patients with post-facial nerve palsy syndrome compared to only one patient with Hemifacial spasm.

Post-paralytic facial syndrome manifests during different facial expressions and at rest.¹² Patients may present with intermittent cheek muscle twitching or with reduced blinking. Hyperkinesis and spasm of facial muscles in the affected side may look like muscle contracture.¹³ This may explain the more prevalent periocular manifestations we observed among our patients with post-paralytic facial syndrome such as brow ptosis, upper eyelid ptosis and reverse ptosis of the lower eyelid.

Spastic entropion is a condition where the lower eyelid margin turns inward due to tonic contraction of the orbicularis muscle.¹⁴ Only one of our 67 patients with hemifacial spasm and post-paralytic facial syndrome exhibited lid entropion. In this case, it is thought that spasm of the orbicularis muscle is not the only mechanism causing entropion. Alternatively, spastic entropion results from a combined mechanism including spasm with significant eyelid laxity rather than spasm alone.

In conclusion, our study provides insights regarding the differences in periocular clinical findings between patients with hemifacial spasm and those with post-paralytic facial syndrome. We have observed that patients with post-paralytic facial syndrome tend to exhibit symptoms such as ptosis, reverse ptosis and brow ptosis more frequently compared to patients with hemifacial spasm. Additionally, eyelid entropion was not found higher in the side effected by hemifacial spasm and post-paralytic facial syndrome.

Financial disclosure: None

Conflict of interest: AA: None, NA: None, AHA: None

Funding/Support: None

Akalin MA, Kiziltan ME & Benbir G. Blink reflex in patients with postparalytic facial syndrome and blepharospasm: Trigeminal and auditory stimulation. Clinical Neurophysiology. 2013 Jan;124(1):120–5.
Ding W, Huang X & Wang W. Mechanisms and Management of Postparalysis Facial Synkinesis. Chinese Journal of Plastic and Reconstructive Surgery. 2021;3.
Valls-Solé J. Facial palsy, postparalytic facial syndrome, and hemifacial spasm. Movement Disorders 2002;17(SUPPL. 2).
Valls-Solé J. Facial nerve palsy and hemifacial spasm. In: Handbook of Clinical Neurology. Elsevier B.V.; 2013. p. 367–80.
Jordan DR, Patrinely JR, Anderson RL & Thiese SM. Essential Blepharospasm and Related Dystonias. Survey of Ophthalmology. 1989 Sep-Oct;34(2):123-32
Oge AE, Yazici J, Boyaciyan A, Tanyeri S, Celik M, Konyalioğlu R, et al. Magnetic Stimulation in Hemifacial Spasm and Post-Facial Palsy Synkinesis. Muscle Nerve. 1993 Nov;16(11):1154–60.
Abbruzzese G, Berardelli A & Defazio G. Hemifacial spasm. Handbook of Clinical Neurology. 2011;(100): 675–680 p.
Öge AE, Yayla V, Demir GA & Eraksoy M. Excitability of facial nucleus and related brain-stem reflexes in hemifacial spasm, post-facial palsy synkinesis and facial myokymia. Clinical Neurophysiology. 2005 Jul;116(7):1542–54
Finsterer J. Ptosis: Causes, Presentation, and Management. Aesthetic Plast Surg. 2003 May;27(3):193–204.
Clauser L, Tieghi R, Galiè M, Ferrara D. Palpebral Ptosis: Clinical Classification, Differential Diagnosis, and Surgical Guidelines: An Overview. J Craniofac Surg. 2006 Mar;17(2):246-54.
Kakizaki H, Zako M & Iwaki M. Reverse ptosis repair targeting the posterior layer of the lower eyelid retractor. Ophthalmic Plast Reconstr Surg. 2007 Jul;23(4):288–91.
Bylund N, Jensson D, Enghag S, Berg T, Marsk E, Hultcrantz M, et al. Synkinesis in bell’s palsy in a randomised controlled trial. Clin Otolaryngol. 2017;42:673–80. doi: 10.1111/coa.12799.
Guntinas-Lichius O, Prengel J, Cohen O, Mäkitie AA, Poorten VV, Ronen O, et al. Pathogenesis, diagnosis and therapy of facial synkinesis: A systematic review and clinical practice recommendations by the international head and neck scientific group. Front Neurol. 2022 Nov 9;13:1019554
Hughes WL. Spastic Entropion; A Simple Procedure for its Cure. Am J Ophthalmol. 1931 Jan;14(1):34–5.

There is no conflict of interest

Download PDF

Review #2 received at journal
12 Sep, 2024
Reviewer #2 agreed at journal
26 Aug, 2024
Review #1 received at journal
21 Aug, 2024
Reviewer #1 agreed at journal
20 Aug, 2024
Reviewers invited by journal
19 Jul, 2024
Editor assigned by journal
18 Jul, 2024
Submission checks completed at journal
20 May, 2024
First submitted to journal
20 May, 2024

You are reading this latest preprint version

Disparity in Periocular Manifestations between Hemifacial Spasm and Post-Paralytic facial Syndrome

Status:

Version 1

Abstract

Objective

Methods

Results

Conclusion

Introduction

Materials and Methods

Results

Discussion

Declarations

References

Additional Declarations

Status:

Version 1