Management of Post Facial Paralysis Synkinesis 1St Edition Babak Azizzadeh MD Facs Full Chapter PDF
Management of Post Facial Paralysis Synkinesis 1St Edition Babak Azizzadeh MD Facs Full Chapter PDF
Management of Post Facial Paralysis Synkinesis 1St Edition Babak Azizzadeh MD Facs Full Chapter PDF
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6 The Role of Botulinum Toxin in Facial Palsy Appendix: Instructions for Sonography of the Mimic
Management, 39 Musculature, 109
Ruben Yap Kannan, Charles Nduka Index, 135
v
Video Table of Contents
CHAPTER 8 CHAPTER 9
Video 8.1 Example of a standard photo session Video 9.1 Inhibition of oculi synkinesis during fNMR
Video 8.2 Example of pathologic spontaneous activity Video 9.2 Inhibition of synkinesis using sEMG
recorded by a needle-electromyography of the mus- feedback
culus orbicularis oris Video 9.3 Movement acquisition during fNMR using
Video 8.3 Montage of video and electromyography proprioception
from a patient with synkinesis. Two needle-EMG - Video 9.4 Dynamic demonstration of synkinetic inhi-
electrodes are inserted in two different muscles: bition during evaluation
channel 1 shows M. orbicularis oris; channel 2 shows Video 9.5 Identifying botulinum toxin injection site(s)
M. zygomaticus. Sound comes from channel 1 for buccinator
Video 8.4 Montage of video and electromyography
from a patient with synkinesis. Two needle-EMG - CHAPTER 10
electrodes are inserted in two different muscles: Video 10.1 Eyelid and periorbital reconstructive surgery
vi
Preface
This book grew from the seed of an idea planted in 2017 Diels, Daniel Labbé, Mark Lucarelli, Guy Massry, Bill
at the 13th International Facial Nerve Symposium, orga- Slattery, and Matt White. It was clear that there is im-
nized and hosted by Babak Azizzadeh and colleagues. mense knowledge about the management of synkinesis
It gathered over 500 professionals from over 40 coun- in the heads of the international faculty that needed to
tries in Los Angeles to share knowledge and research be captured in one place. The result is this book, which
about facial paralysis. There was an excellent panel has drawn upon leading experts from across the world
session on synkinesis with contributions from Jackie to distill their knowledge within these pages.
vii
Acknowledgements
Acknowledgements from Charles Nduka understanding of the facial nerve anatomy, and the
I would like to thank the thousands of patients affect- complexity of treating cranial nerve disorders. I was
ed by synkinesis who have allowed me to be part of privileged to train in facial plastic and reconstructive
their care team. They have provided me with impor- surgery at the Massachusetts Eye & Ear Infirmary
tant insights and inspiration. I am especially grate- which completely changed the trajectory of my career.
ful to the Facial Palsy UK community of patients, During my fellowship at Harvard Medical School, I was
their families, and supporters who have taught me fortunate enough to be trained by Dr. Mack Cheney
so much about the impact that facial paralysis has on who had himself dedicated his life to microsurgery,
their lives. facial plastic surgery, and facial paralysis and, along
I am grateful to my multidisciplinary colleagues at with Drs. Mark Varvares and Daniel Deschler, gave me
the Queen Victoria Hospital in East Grinstead, includ- the necessary medical and surgical tools to further de-
ing facial therapists Trina Neville, Tamsin Gwynn, and velop my skills. After returning to Los Angeles, I was
Karen Young, psychological therapist Beth Jordan, and privileged to be surrounded by a passionate team of
my plastic surgery colleague Ruben Kannan. I am also subspecialists who share the same desire, allowing us to
grateful for the ongoing support of the oculoplastic look at facial nerve disorders from a multidisciplinary
team led by Raman Malhotra and André Litwin, and approach. Drs. Babak L arian, Guy Massry, Bill Slattery,
the facial nerve team at Guy’s Hospital in London. Randy Sherman, Greg Lekovic, and Jackie Diels have
Without the encouragement, patience, and support been inspirational and I have learned so much from
of Veronika Watkins, Jessica McCool, and the Elsevier each of them almost every single day. I have also been
team, this book would not have been possible. fortunate to have met so many pioneers of the field
such as Dr. Douglas Harrison who have mentored and
Acknowledgements from Babak Azizzadeh inspired me. I also want to thank my contemporaries
My personal journey as a physician has been so re- Drs. Tessa Hadlock and Patrick Byrne who continue
warding, with my family, patients, mentors, and col- to push the envelope in this emerging field of study.
leagues having an integral role in my passion for treat- I would be remiss not to share how lucky I have been to
ing patients with facial nerve disorders. During the have Dr. Babak Larian as the most compassionate and
very early part of my residency training, one of my talented partner who has been with me every step of
wife’s closest friends developed facial paralysis due to the way for over two decades. He and I have operated
a benign brain tumor and that started my deep curios- together, dreamed together, devised new approaches,
ity into this most challenging of all medical and surgi- and managed the most challenging medical and sur-
cal disorders. During my early days as a resident in gical cases. Finally, this book would not have become
Head & Neck Surgery at UCLA, Drs. Rinaldo Canalis, reality without Jessica McCool, Veronika Watkins, and
Tom Calcatterra, Keith Blackwell, and Gerald Berke the rest of the Elsevier team. They have been the ulti-
were integral in my training in microsurgery, in-depth mate professionals in their own field.
viii
Dedication from Charles Nduka
To my wife Jules for her continued understanding and patience.
Dedication from Babak Azizzadeh
I dedicate this book to all my patients who have entrusted me to help them in their own journeys.
ix
Contributors
x
CONTRIBUTORS xi
Sara MacDowell PT, DPT Gerd Fabian Volk Priv. Doz. Dr. med. habil.
Doctor of Physical Therapy Senior Consultant
Our Lady of the Lake Hearing and Balance Center ENT Department
Baton Rouge Jena University Hospital
LA, USA Jena, Germany;
Head
Guy Massry MD Facial Nerve Center
Ophthalmic Plastic & Reconstructive Surgery Jena University Hospital
Beverly Hills, California, CA, USA; Jena, Germany;
Clinical Professor of Ophthalmology Medical Faculty, Friedrich-Schiller-University
University of Southern California, Keck School of Jena, Germany
Medicine,
CA, USA; Yao Wang MD
Diplomat, American Board of Ophthalmology; Fellow Physician
Fellow, American Society of Ophthalmic Plastic and Ophthalmology and Surgery
Reconstructive Surgery Cedars-Sinai Medical Center
Los Angeles
Oliver Mothes MSc CA, USA
Computer Vision Group
Friedrich Schiller University Stephanie Warrington MD
Jena Department of Otolaryngology – Head Neck Surgery
Thuringia, Germany LSU Health Sciences Center
New Orleans
Charles Nduka MB BS, MA(OXON), MD, FRCS, LA, USA
FRCS(Plas)
Consultant Plastic Surgeon, Facial Palsy Unit Rebecca Williams BSc (Hons)
Department of Plastic & Reconstructive Surgery Specialist Paediatric Physiotherapist
Queen Victoria Hospital Alder Hey Children’s NHS Foundation Trust
East Grinstead, UK Liverpool, UK
Jovanna Thielker MD
ENT Department
Jena University Hospital
Jena, Germany;
Facial Nerve Center
Jena University Hospital
Jena, Germany;
Medical Faculty, Friedrich-Schiller-University
Jena, Germany
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Facial Nerve and Muscle Anatomy 1
G. Nina Lu, Patrick J. Byrne
Abducens nucleus
Lacrimal gland
Nasal mucous
glands Facial nucleus
Superior salivatory
nucleus
Taste fibers
NI
Main facial
Corticopontine and nerve
corticospinal fibers
VI
Stapedius
Facial nerve [VII]
Geniculate ganglion
Stapedius, tendon
Stapes
Incus
Malleus
Nerve to stapedius
Tensor tympani
Facial nerve [VII]
Chorda tympani
Auditory tube,
bony part
Mastoid process
Auditory tube, Petrosphenoidal Tympanic
cartilaginous part fissure membrane
Digastric branch
Stylohyoid
Fig. 1.3 Intratemporal Course and Branches of the Facial Nerve. (From Paulsen F, Waschke J. Sobotta Atlas of
Human Anatomy, Vol. 3. 15th ed. Munich, Germany: Urban & Fischer; 2012:133-160; Gleeson M. External and middle
ear. In: Standring S, et al., eds. Gray’s Anatomy. 41st ed. Oxford, UK: Elsevier; 2016:624-640.e1.)
segment, the facial nerve becomes more superficial stylomastoid foramen, which is lined by the aponeuro-
as it travels within the mastoid bone. Between 9 and sis of the posterior belly of the digastric muscle. This
18 mm in length, the mastoid segment contains the aponeurosis directly supplies blood to the facial nerve
chorda tympani and stapedial branches (Fig. 1.3). The as it exits the fallopian canal.
chorda tympani has a variable take off from the mas-
toid segment but typically exits 6 mm above the stylo- EXTRATEMPORAL
mastoid foramen. Coursing superiorly nearly parallel The facial nerve exits the stylomastoid foramen cov-
to the facial nerve, the chorda angles anteriorly as it en- ered in tough connective tissue. At the stylomastoid
ters the mesotympanum. It exits the temporal bone via foramen, the posterior auricular branch exits the facial
the petrotympanic fissure and joins the lingual nerve nerve and supplies general sensory innervation to the
to convey taste information to the anterior two-thirds posterior ear canal and concha, as well as motor inner-
of the tongue and supplies preganglionic parasympa- vation to the auricular muscles and occipitalis muscle.
thetic innervation to the submandibular, lingual, and The digastric branch supplying motor innervation
minor salivary glands. This final segment ends at the to the posterior belly of the digastric muscle and the
4 CHAPTER 1 Facial Nerve and Muscle Anatomy
1 1
2 2
1
2 3
3 3 II
IV
3 4
4
5
VI 5
4 1
1
1
2
5
2
2
3
I
V 3
III 3
4
4
5
I Type I, 13% 1 temporal branch 5
4
II Type II, 20% 2 zygomatic branch
5
III Type III, 28% 3 buccal branch
IV Type IV, 24% 4 mandibular branch
V Type V, 9% 5 cervical branch
VI Type VI, 6%
Fig. 1.4 Branching Patterns of the Extratemporal Facial Nerve. (From Holmes S. Face and scalp. In: Standring S,
et al., eds. Gray’s Anatomy. 41st ed. Oxford, UK: Elsevier; 2015:475-506.)
stylohyoid branch to the stylohyoid muscle also exit the upper orbicularis oculi run along the undersurface
the facial nerve prior to its entrance into the parotid for 3 to 4 mm before entering the muscle to innervate it.
gland. Within the parotid, the facial nerve branch- One common estimate of the frontotemporal branch
es into a larger zygomaticotemporal division and a is Pitanguy’s line, defined by a line draw from 0.5 cm
smaller cervicomandibular division at the pes anseri- inferior to the tragus to 1.5 cm superior and lateral to
nus and terminates in five classic branches: temporal, the eyebrow. Numerous cadaveric studies have at-
zygomatic, buccal, mandibular, and cervical. In reality, tempted to further define the course and branching
the branching pattern of the facial nerve is highly vari- patterns. Ishikawa demonstrated that three to four
able with multiple communications and the majority branches of the FTN were consistently found between
of variations existing among the zygomaticobuccal di- 3.8 and 6.0 cm posterior to the lateral canthus along
visions.3–5 Through the parotid gland, the nerve runs the superior zygomatic arch in both straight-line and
at the level of the retromandibular vein and separates curved trajectories.8
the superficial and deep lobe of the parotid gland. As
it exits the parotid, the nerve has on average 8 to 15 Zygomaticobuccal Division
branches making up the five divisions6 (Fig. 1.4). The The zygomaticobuccal division consists of five to eight
nerve runs deep to the superficial musculoaponeurotic branches with significant overlap of muscle innerva-
system (SMAS) and innervates the majority of facial tions. These nerves innervate the midfacial muscles
muscles from their deep surface. The mentalis, bucci- spanning the lip elevators to the lower orbicularis
nator, and levator anguli oris are innervated on their oculi. Connections between the lower branches and
superficial surface as these are the deepest layer of fa- marginal mandibular division also exist. Dorafshar
cial muscles. et al. describe “Zucker’s point” as a reliable surface
landmark for identifying the zygomaticobuccal nerve
Temporal Division as it exits the parotid.9 The authors found that the mid-
The temporal division, also known as the frontal divi- point of a line drawn from the root of the helix to the
sion or frontotemporal branch, consists of three to four oral commissure predicted the nerve location within
branches traveling obliquely in between the tempo- an average of 2.3 mm.
roparietal fascia and the superficial layer of the deep
temporal fascia.7 Branches entering the frontalis mus- Marginal Mandibular Division
cle at the level of the supraorbital ridge are located up The marginal mandibular division consists of one to
to 3 cm above the lateral canthus. Branches entering three branches beginning up to 2 cm below the ramus
Facial Nerve and Muscle Anatomy CHAPTER 1 5
of the mandible and arcing upward to cross the man- previously, the transition zone of vascular supply be-
dible halfway between the angle and mental protuber- tween the AICA and the external carotid centers on the
ance. Branches lie on the deep surface of the platysma labyrinthine segment and creates a watershed or weak
and cross superficial to the facial vessels 3.5 cm from zone of arterial supply.
the parotid edge. There are separate branches to the The extrinsic vascular network consists of one or
depressor angularis, depressor labii inferioris (DLI), two main arterial trunks and accompanying venae co-
and mentalis, and variable superior ramus supplying mitantes running between the periosteum of the fal-
upper platysma and lower orbicularis oris. lopian canal and the epineural sheath of the nerve. An
intrinsic vascular network also exists within the epi-
Cervical Division neurial sheath consisting of small arterioles, capillar-
The cervical division consists of one branch leaving ies, and venules.12
the parotid and running on the deep surface of the pla-
tysma. The point of entry into the muscle is 2 to 3 mm
FACIAL MUSCLES
caudal to the platysma muscle branch of the facial ves-
sel. It enters the muscle at the junction of its cranial and The striated muscles of facial expression derive from
middle thirds. the second branchial arch mesoderm and reside within
the SMAS layer. The SMAS layer continues superiorly
with the galea aponeurotica and inferiorly with the
MICROSCOPIC ANATOMY platysma muscle. A total of 17 paired and 1 unpaired
The microanatomy of the facial nerve reveals a het- sphincter muscle constitute the facial musculature.
erogenous organization that explains in part the dif- These originate from the periosteum of the facial bones
ficulty of facial nerve repair. As described by Captier and insert into the skin, allowing for a limitless num-
et al., the facial nerve lacks fascicular organization as ber of facial expressions (Fig. 1.5).
well as epineural or perineural covering from its exit The facial muscles possess a three-dimensional rela-
at the brainstem to the geniculate ganglion.10 Prior to tionship to one another and exist in four layers based
the geniculate ganglion, the nerve is surrounded only on muscle origin as demonstrated by Freilinger et al.13
by an arachnoid sheath. A thin epineural sheath arises The orbicularis oculi, depressor anguli oris (DAO), and
within the tympanic segment and thickens as the nerve superficial aspect of the zygomaticus minor are most
travels towards the stylomastoid foramen. The peri- superficially located as the first layer. The platysma,
geniculate facial nerve demonstrates one or two fascic- risorius, zygomaticus major, deeper portion of the zy-
ular bundles that increase in number and decrease in gomaticus minor, and levator labii superioris alaeque
size as the nerve travels distally through the fallopian nasi compose the second layer. The levator labii supe-
canal. The number of fascicles and their spatial organi- rioris and orbicularis oris represent the third layer. The
zation changes every 2 mm within the fallopian canal. deepest layer is composed of the levator anguli oris,
At the stylomastoid foramen the facial nerve has on the mentalis, and the buccinator—these three muscles
average 11 fascicles and up to 16. As individual fibers are also the only muscles innervated from their super-
emerge, they are surrounded by perineurium and en- ficial surface, as explained by this relationship.
doneurium. In the horizontal segment, the upper mo- Distinct from most skeletal muscles, facial muscles
tor division has been reported to be more superficial are flat, strap-like muscle sheets with interdigitations
(or lateral), whereas the lower motor division has been to the skin, short or absent tendons, and an absent fas-
reported to lie deeper (or medial).11 The nerve bundles cial covering. A comprehensive list and description of
have a spatial organization as they traverse the mastoid facial muscles are detailed in Table 1.1. The most clini-
segment: branches to the lower division lie within the cally relevant facial muscles are described in further
anterior portion of the nerve and branches to the upper detail in the following sections.
division course posteriorly within the epineurium.11
From the brainstem to the mastoid segment, an aver- FRONTALIS
age of 7800 myelinated nerve fibers are maintained. A The frontalis muscle is a broad, thin, bilateral muscle
single neuron can innervate up to 25 muscle fibers. originating from the galea aponeurotica near the coro-
nal suture and inserting onto the superciliary ridge of
VASCULAR ANATOMY the frontal bone and interdigitating with fibers of the
The intracranial (brainstem and IAC) facial nerve re- orbicularis oculi, procerus, and corrugator supercilia.
ceives blood supply from branches of the AICA aris- Densely adherent to the overlying skin, the frontalis
ing from the vertebrobasilar system. The labyrinthine glides over the underlying periosteum to provide brow
artery, a branch of the AICA, provides vascular input elevation. The resting tone of the frontalis also prevents
within the IAC segment. However, the remaining in- brow descent and ptosis. Left- and right-sided frontalis
tratemporal segments receive blood supply through bellies fuse in the midline caudally, often as a fibrous
the middle meningeal artery, a branch of the maxil- junction. The frontalis and occipitalis bellies can be de-
lary artery from the external carotid. As described scribed as distinct muscle bellies or as parts of a single
6 CHAPTER 1 Facial Nerve and Muscle Anatomy
Epicranial aponeurosis
Depressor supercilii
Procerus
Corrugator supercilii
Orbicularis oculi, palpebral part Levator labii superioris
alaeque nasi
Levator labii superioris Nasalis
alaeque nasi
Orbicularis oculi, orbital part
Levator labii superioris
Levator labii superioris
Zygomaticus minor
Zygomaticus minor
Zygomaticus major
Zygomaticus major
Parotid gland
Platysma
Fig. 1.5 Facial Musculature (Frontal View). (From Zuker RM, Gur E, Hussain G, Manktelow RT. Facial paralysis. In:
Neligan PC, ed. Plastic Surgery: Volume 3: Craniofacial, Head and Neck Surgery and Pediatric Plastic Surgery. 4th ed.
Philadelphia: Elsevier; 2018:329-357.e2.)
occipitofrontalis muscle connected by an intermediate movement. The preseptal portion covers the orbital
tendon within the galea aponeurotica. septum and is under more voluntary control. It is less
closely adherent to the skin other than at the medial
ORBICULARIS OCULI and lateral canthi. Both the preseptal and pretarsal
The orbicularis oculi muscle is a paired sphincter components function together during blink. The or-
important in eyelid closure. The pretarsal, preseptal, bital component forms a ring over the bony orbital
and orbital subdivisions of the orbicularis oculi are margin and is recruited during forceful eye closure
defined by the anatomic level of the muscle. The pre- as well as brow depression. This component origi-
tarsal portion is closely adherent to the pretarsal skin, nates medially from the superomedial orbital margin,
covers the tarsal plate and provides reflexive blink the maxillary process of the frontal bone, the medial
Facial Nerve and Muscle Anatomy CHAPTER 1 7
Zygomaticus minor
Zygomaticus major
Buccinator
Modiolus
Risorius
Platysma
canthal tendon, the frontal process of the maxilla, and The zygomaticus major is the most superficial of the
the inferomedial margin of the orbit. Within the most three and originates from the zygomatic bone in front
superficial layer of facial muscles, the orbital sub- of the zygomaticotemporal suture. It runs to the angle
division laterally overlies the temporalis fascia, the of the mouth where superficial fibers form the modio-
origins of the zygomaticus major, and levator labii lus together with the DAO, the risorius, the orbicularis
muscles. oris, the buccinator, and the levator anguli oris. The
modiolus represents the interdigitation of all the peri-
LIP ELEVATORS oral muscles. The deeper insertion of the zygomaticus
The perioral muscles contributing to lip elevation and major fuses with the levator anguli oris and the me-
smile are of particular interest to patients and surgeons dial fibers line on the buccinator muscle. The caudal
in facial rehabilitation. The main lip elevators are the fibers continue into the DAO. The zygomaticus muscle
zygomaticus major, the levator labii superioris, and le- elevates the commissure superiorly and laterally at an
vator anguli oris (Fig. 1.6). approximately 45-degree angle.
Facial Nerve and Muscle Anatomy CHAPTER 1 9
17. Freilinger G, Happak W, Burggasser G, Gruber H. 19. Jeon A, Kim SD, Han SH. Morphological study of the occipi-
Histochemical mapping and fiber size analysis of mimic tal belly of the occipitofrontalis muscle and its innervation.
muscles. Plast Reconstr Surg. 1990;86(3):422–428. Surg Radiol Anat. 2015;37(9):1087–1092.
18. Rossi G. From the pattern of human vocal muscle fibre in- 20. Hwang K, Lee JH, Lim HJ. Anatomy of the corrugator mus-
nervation to functional remarks. Acta Oto-laryngologica cle. J Craniofac Surg. 2017;28(2):524–527.
Supplementum. 1990;473:1–10.
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Etiology, Epidemiology, and Pathophysiology
of Post-Facial Paralysis Synkinesis
2
Kofi D.O. Boahene
The neural activity responsible for voluntary facial ex- causes of facial paralysis, such as Bell’s palsy, Ramsey
pression relies on an intricate set of interconnections Hunt syndrome, trauma, and postsurgical injury, have
distributed across multiple cortical facial motor areas, all been associated with synkinesis to varying degrees.
the amygdala, and other subcortical areas.1 The tar- In one study, synkinesis was clinically observed in 55%
get end-organs for this intricate neural system are the of patients.3 Using electrophysiologic criteria, synki-
facial muscles directly innervated by last order post- netic movement can be detected in nearly all patients
synaptic axons in the facial nerve. The muscles of the with proximal facial nerve injury.4 In one of the larg-
upper and lower face are controlled by anatomically est studies evaluating the natural course of idiopathic
distinct motor areas reliant on distinct patterns of neu- peripheral facial nerve palsy, synkinesis was recorded
ral activity. Disruption of this intricate neural network, in about 16% of patients.5 In a retrospective study of
when followed by spontaneous recovery or surgical 102 patients with untreated Ramsay Hunt syndrome,
repair, is commonly associated with abnormal facial all patients who initially developed complete paralysis
movements such as synkinesis. Synkinesis is the unde- also developed synkinesis, whereas synkinesis was re-
sired synchronization of an involuntary muscle group corded in 10% to 15% of those with incomplete paraly-
triggered by a desired voluntary contraction of another sis.6 Pregnancy, advanced age, and diabetes have been
muscle group. Synkinesis can occur in any functional studied as potential risk factors for Bell’s palsy but
muscle group in the body but is commonly seen after have not shown to be linked with severity of synkine-
facial nerve injury. A typical example of facial synki- sis. However, there is a clear relationship between the
nesis is seen when a voluntarily blink is accompanied severity of facial palsy and the degree of synkinesis.
by co-contraction of lower facial muscles around the This link is further supported by electrophysiologic
lips (ocular-oral synkinesis) and when narrowing of studies showing that patients with a prognostic elec-
the palpebral fissure occurs when smiling (oral-ocular troneurography (ENoG) value of less than 10% have
synkinesis). In its mildest form, facial synkinesis is tol- higher risks of aberrant regeneration and moderate-to-
erable and often ignored by patients but can be very severe synkinesis than those with an ENoG value of
debilitating when severe. 10% to 20%.7
The pathological mechanism of synkinesis is not
completely elucidated and as a result, current treat-
THE FACIAL NEUROMUSCULAR NETWORK
ment options have mixed efficacy. Presently, synkinesis
treatment is targeted towards peripheral facial nerves The facial nerve is a mixed motor and sensory nerve
and muscles in the form of chemodenervation, selec- composed of approximately 10,000 neurons. About
tive myectomy and selective neurectomy.2 To mini- 7000 of these neurons are motor and innervate the
mize the sequelae of synkinesis post facial nerve injury, muscles of facial expression. The motor fibers originate
and to offer more effective rehabilitative and therapeu- from the motor face area of the frontal lobe and project
tic treatment, it is necessary to better understand the via the corticobulbar tract and the internal capsule to
response of peripheral nerves to injury, the process of the facial nucleus. These afferent motor fibers synapse
nerve regeneration, electrophysiologic changes in pe- with nuclei within the facial nucleus arranged into four
ripheral facial nerves post injury, and any associated major subgroups: dorsomedial, ventromedial, inter-
cortical adaptations. mediate, and lateral. Each nuclei subgroup projects to
a specific group of facial muscles and explains, in part,
the intricate voluntary control of facial muscle move-
ETIOLOGY AND EPIDEMIOLOGY ment. The degree of nuclei representation and projec-
Faulty regeneration is the norm and not the exception tion to muscle groups are related to the functional and
following facial nerve injury of any cause. Facial syn- behavioral importance of each muscle. For example,
kinesis has been reported in a wide range of ages from the subnuclei representing the orbicularis oris and oc-
infants to elderly patients and does not appear to be uli are prominently represented compared to those of
limited to a specific demographic. The most common less important muscles such as the depressor septi nasi
13
14 CHAPTER 2 Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis
or auricularis muscles. Much of what is known about regeneration. This transformation to a repair-sustain-
this facial nucleus sub-innervation in humans is ex- ing Schwann cell phenotype is mediated by a complex
trapolated from tracer injection studies in primates.8,9 cellular process that is time dependent. Early after
The corticobulbar projections were defined by inject- nerve injury, the Schwann cells distal to the damaged
ing anterograde tracers into the face representation of area lose contact with axons as they degenerate. The
each motor cortex, and the musculotopic organization loss of contact is by itself a trigger for Schwann cell
of the facial nucleus was defined by injecting fluores- transformation. Invading macrophages secrete bio-
cent retrograde tracers into individual muscles of the active factors which, together with the loss of axonal
upper and lower face. Following these injections, the contact, influence the change in surrounding Schwann
facial nucleus was noted to received input from all face cells toward a repair type cell.10
representations. Injections in all cortical face represen- Within their original basal lamina tubes, these
tations labeled terminals in all nuclear subdivisions transformed repair type Schwann cells aid in clearing
(dorsal, intermediate, medial, and lateral). However, degenerated myelin by autophagy. The released cyto-
significant differences occurred in the proportion of kines call in macrophages for further myelin clearance.
labeled boutons found within each functionally char- The Schwann cells elongate forming regeneration
acterized subdivision. tracts, Bunger bands, which guide regenerating ax-
Once the facial nerve exits the brain stem, the dis- ons towards their target. Axons that successfully grow
crete compartmentalization of motor neurons destined across the regeneration zone induce transformation of
for specific muscle groups is lost.10 Consequently, the fi- the repair Schwann cells back to the myelin phenotype
bers to all facial muscles are distributed throughout the and become ensheathed in a new myelin membrane.
nerve at all levels before peripheral branching occurs in This remarkable, adaptive injury response of neurons
the parotid. Within the parotid gland, the facial nerve and Schwann cells is clinically inadequate in severe
divides into a superior (temporofacial) and an inferior and long-lasting injuries. There are several reasons
(cervicofacial) division, interconnected but containing for this inadequacy.16,17 First, the early regeneration-
fibers destined to specific muscle groups. The delicate enabling environment that enables nerve regeneration
musculotopic arrangement of the facial nucleus and fa- is unstable as the number Schwann cell dwindles. The
cial nerve is disrupted following facial nerve injury and number of cells that can be isolated from nerves after
explains in part abnormal motor recovery.10 6 months of chronic denervation is only 10% to 15% of
that obtained at 4 weeks. Second, with prolonged de-
nervation, the remaining Schwann cells become less ef-
WHEN A NERVE IS INJURED fective with surviving cells gradually down-regulating
The axons that make up a motor nerve are myelinated their repair phenotype, followed by the death of cells
and arranged in a fascicular architecture that is both that have by that time lost most of their regeneration-
protective and suited for rapid nerve conduction. The supportive properties.16 Clinically suboptimal rein-
myelin sheath is made and maintained by Schwann nervation of the facial muscles manifest as hypotonus,
cell membranes wrapped compactly around the axon. hypertonia, myokymia, or synkinesis.
Myelin is in turn coated by a basal lamina, outside of
which lies the endoneurium, which contains fibro-
PROPOSED MECHANISMS FOR SYNKINESIS
blasts, blood vessels, and a few macrophages, which is
ultimately surrounded by a multilayered cellular tube, Three main theories—aberrant regeneration of facial
the perineurium. This assembly of axons, Schwann nerve fibers, ephaptic transmission of impulses, and
cells, and connective tissue makes up a nerve.11 Injury somatotopic reorganization of the facial nucleus—
to the facial nerve may disrupt the nerve architecture have been forwarded as potential mechanisms for
to varying degrees depending on the extent of insult. synkinesis. More recently, there has been increasing
Seddon and Sunderland classified the fundamental understanding of the potential role played by electro-
types of nerve injury based on the extent of disruption physiologic changes in the peripheral nerve and corti-
of the endoneurium and perineum as well as the integ- cal adaptations in postinjury synkinesis.
rity of the axon.2 The imperfect response of peripheral
nerves to injury underlies the clinical manifestation of ABERRANT REGENERATION
synkinesis. Injuries that disrupt the endoneurium can result in
In response to injury, both neurons and Schwann interrupted and misaligned regeneration tracts. In
cells switch to a cell state suited for nerve repair and addition, mechanical barriers at the injury segment
regeneration.11 In damaged neurons, the signal switch compounded by the slow growth of proximal axonal
to repair mode is heralded by the activation of an ex- stumps limits the number of axons that successfully
tensive gene program that facilitates axonal regen- cross, resulting in misrouting and reinnervation errors.
eration, a response classically referred to as the cell Misrouted axons meant for the orbicularis oris muscle
body response.11–15 Similarly, Schwann cells change end up in the orbicularis oculi muscle and result in
into a phenotype primed for repair to facilitate nerve ocular-oral synkinesis. Recent studies have suggested
Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis CHAPTER 2 15
that the opportunity for misdirection of axons does not postinjury period within a time line that is neither ex-
only occur at the injury site, but that aberrant axonal plained by aberrant nerve regeneration or ephaptic im-
growth occurs throughout the length of the nerve.18 pulse transmission given that axons grow at a rate of
The most commonly observed clinical patterns of only 1 mm/day. Nuclear hyperexcitability is believed
synkinesis are predictable rather than random, with to occur when inhibitory signals to the facial nucleus
mouth and eyelid coupling the most common occur- are lost in response to peripheral facial nerve injury.
rence. The mechanism of misrouted axons is the most Stripping of synaptic input into the facial nucleus has
commonly stated reason for synkinesis and underlies been observed within days of facial nerve injury.22 The
the treatment method of selective neurectomy, che- time line for this synaptic stripping matches the obser-
modenervation, and myectomy. vation of synkinesis as early as 4 to 8 weeks following
the onset of idiopathic facial palsy.22,23
PERIPHERAL EPHAPTIC TRANSMISSION The first evidence for disconnection of synapses fol-
The ability of Schwann cells to restore myelin sheath in lowing peripheral nerve injury in humans was report-
extensively degenerated nerve is imperfect and incom- ed in an autopsy study of a 77-year-old man who died
plete. Consequently, regenerated axons with defective from an unrelated cause 3 months after developing left
myelin membranes can result in “short circuiting” facial paresis from otitis media.24 Microscopic evalu-
with cross-excitation of adjacent axons meant for dif- ation and immunohistochemistry revealed a striking
ferent target muscles. Ephaptic coupling of transmis- reduction in the number of afferent synaptic contacts
sion between adjacent axons is distinct from impulse and marked chromatolytic changes in the left facial
spread across synapsis. Rather it is the spreading of nucleus. Immunocytochemistry for synaptophysin re-
impulses along and across adjacent axons such that vealed a marked loss of afferent synaptic contacts from
action potential propagating along one axon fires up somatic and stem dendritic surface membranes of all
an adjacent axon. The term ephaptic was coined from chromatolytic motor neurons. Wrapping of a number
the Greek word “ephapse” signifying the act of touch- of neurons by newly formed glial fibrillary acidic pro-
ing (as opposed to “synapse” or linking) by Arvanitaki tein-positive astrocytic cell processes could be detected
in a study of nerve conduction in giant axons of Sepia in the regenerating facial motor nucleus. These chang-
officinalis (common cuttlefish).19 Since then, the term es were absent from the contralateral, normal-appear-
ephaptic interaction has been used to refer to com- ing facial nucleus. The marked occurrence of synaptic
munication between neuronal cells via electrical con- stripping, preferentially of inhibitory nerve, can ex-
duction through the surrounding extracellular space, plain enhanced nuclear hyperexcitability and poorly
as opposed to communication mediated by chemical controlled bursts of undesired muscle contraction.
synapses or gap junctions. Ephaptic transmission is
usually inhibited by myelination but can occur when AXONAL HYPEREXCITABILITY
remyelination is incomplete. The coexcitation of differ- Fundamental to nerve conduction is the maintenance
ent muscle groups will, as a result of ephaptic impulse of a resting membrane potential and rapid impulse
transmission, result in synkinesis. The concept of ep- propagation due to the exchange of sodium (Na+) and
haptic coupling of adjacent axons was investigated by potassium (K+) across ion channels in response to depo-
Katz and Schmitt in 1940, who showed nonsynaptic larizing and hyperpolarizing stimuli. The arrangement
electrical interaction between adjacent nerve fibers.20 of myelin around nerves, with myelin free segments at
In their work, two large parallel nonmyelinated axons the node of Ranvier and the electrophysiologic char-
were isolated from the crab limb nerve. They demon- acteristics of ion channel distribution at the nodal and
strated that: (1) the passage of an impulse in one fiber internodal segments, facilitates the speed of impulse
causes subthreshold excitability changes in the adjacent conduction. Traditionally, nerve conduction studies
fiber and (2) when impulses are set up simultaneously that measure impulse conduction velocity, latency, am-
along both fibers, a mutual interaction occurs that can plitude, and F-waves have been used clinically to as-
lead to speeding up or slowing down of the impulses sess the gross status of peripheral nerves. More recently,
and also possibly to synchronization between the two axonal excitability study has emerged as a noninvasive
impulses, depending on the initial phase relationship. technique that offers complementary information to
In 1984, ephaptic interactions were suggested to play a that provided by conventional nerve conduction stud-
role in hemifacial spasm pathophysiology by causing ies, to gain more insight into axonal function with de-
“cross-talk” between facial nerve fibers.21 tailed information about the axonal membrane potential
and ion channel properties.25 The key axonal excit-
NUCLEAR HYPEREXCITABILITY ability parameters commonly measured after delivery
Enhanced excitability of the facial nucleus has also of impulse to a test nerve include threshold, strength-
been suggested as a possible mechanism for synkine- duration time constant, rheobase, threshold electroto-
sis following facial nerve injury. This is supported by nus, recovery cycle, and current/ threshold relation-
the predictable and nonrandom pattern of synkinesis ship. Changes in these axonal biophysical parameters
and the clinical observation of synkinesis early in the have been attributed to aberrant ionic conductance,
16 CHAPTER 2 Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis
particularly pertaining to Na+ and K+ exchange, and the known ability of the brain to undergo plasticity, the
has been characterized for a diverse range of conditions role of cortical changes following facial nerve injury
including toxic, metabolic, both acquired and inherited as a potential cause of defective motor control is be-
demyelinating neuropathies, and neurodegenerative ing investigated. The development of sophisticated
disorders such as amyotrophic lateral sclerosis (ALS), noninvasive neuroimaging techniques over the past
as well as providing insight into pathophysiologic decade has made it possible to study cortical changes
changes occurring at the peripheral nerve level in dis- in response to peripheral nerve injury. In a study of pa-
orders of the central nervous system such as stroke, spi- tients with facial synkinesis, functional magnetic reso-
nal cord injury, and multiple sclerosis.26,27 Using axonal nance imaging (fMRI) demonstrated that the distance
excitability techniques, recent studies have explored the between motor cortical subsites activated on ipsilateral
possible contribution of electrophysiologic changes in blinking and smiling tasks was decreased on the affect-
peripheral facial nerves following injury that may pre- ed half of the face compared with the unaffected side.30
dispose to ectopic activity such as spasms and synki- In another study investigating resting state fMRI pa-
nesis. In one study, facial nerve excitability parameters rameters in post-facial paralysis patients, there was an
were compared between patients with synkinesis and abnormal state of hypercompensation in motor control
those without following facial paralysis.28 Synkinesis areas in patients with compared with patients without
patients demonstrated marked alterations in a number facial synkinesis.31,32 Together, these studies support
of excitability parameters compared with normal con- the role of a functionally aberrant motor control sys-
trols. Specifically, there was depolarized resting mem- tem in facial synkinesis. Facial retraining therapy with
brane potential in the synkinesis group predisposing to biofeedback is an integral aspect of the management
axonal hyperexcitability hyperkinesis and myokymia. of facial synkinesis. Recent reports indicate that people
As in ALS, the alteration in resting membrane poten- are able to develop control over the activity of specific
tial of injured facial nerves was attributed to changes in brain areas when provided with real time–fMRI bio-
the distribution of Na+ channels in the injured nerves. feedback.33,34 The use of real time–fMRI biofeedback
Acute Na+ channel dysfunction has been directly im- in the treatment of facial synkinesis is an interesting
plicated in the pathophysiology of virus-induced facial research tool that may hold therapeutic potential.
palsy and has been linked to the hyperexcitability ob-
served in postinjury facial nerve.26,27 In one study, the
SUMMARY
neurotropic virus implicated in Bell’s palsy, herpes sim-
plex type 1 (HSV-1), was found to inhibit the excitabil- Faulty facial nerve regeneration manifesting as syn-
ity of the facial nerve by an elective, precipitous, and kinesis is a challenging sequelae of facial nerve injury
complete internalization of voltage-activated sodium for both clinicians and patients. The varied patho-
channel proteins from the plasma membrane of the physiologic mechanisms of synkinesis, encompassing
neurons.29 This change in the transmembrane sodium misdirected axons, ephaptic coupling, synaptic strip-
channel was RNA-mediated and occurred directly at ping with neuronal hyperexcitability, axonal excit-
the axonal site of virus transfection. This ability of an ability from altered ion channel function, and cortical
axon to modify protein transcription at the site of the vi- adaptation, underscores the difficulty in establishing a
ral exposure or injury has emerged as a key mechanism universally effective preventative and therapeutic in-
in the response of peripheral nerves to injury. Axonal ex- tervention. However, improved understanding of the
citability studies in Bell’s palsy patients shows a pattern electrophysiologic alterations that accompany periph-
that lends further support to the role of Na+ channels in eral nerve injury and new clinical tools available for
the sequelae of facial nerve injury.28 This evolving un- characterizing the excitability parameters of individual
derstanding of the pathophysiology of peripheral facial nerve branches offer a selective and targeted rebalanc-
nerve injury offers opportunities for preventative and ing of the disorganized facial neuromuscular circuit
therapeutic intervention. For example, medications that using cortical retraining therapy, effective pharmaco-
stabilize membrane potential through their effect on therapeutics, selective myectomy, and neurectomy.
Na+ channels may be considered early in the recovery
of idiopathic facial nerve paralysis even before synkine- REFERENCES
sis becomes clinically evident. Additionally, excitability 1. Gothard KM. The amygdala-motor pathways and the con-
parameters may be used to select appropriate nerves trol of facial expressions. Front Neurosci. 2014;8:43.
for reanimation procedures or selective neurectomy. 2. Boahene KDO, May M. The facial nerve after injury. In: Hom
DB, Hebda P, Friedman C, Gosain A, eds. Essential Tissue
CORTICAL ADAPTATION Healing of the Face and Neck. Shelton, CT: PMPH; 2006.
3. Yamamoto E, Nishimura H, Hirono Y. Occurrence of seque-
The fine voluntary control of facial muscles begins in lae in Bell’s palsy. Acta Otolaryngol Suppl. 1988;446:93–96.
the motor cortex. The previously described mecha- 4. Kimura J, Rodnitzky RL, Okawara SH. Electrophysiologic
nisms for synkinesis all pertain to the facial nerve from analysis of aberrant regeneration after facial nerve paralysis.
the nucleus in the pons to the extracranial nerve. Given Neurology. 1975;25(10):989–993.
Etiology, Epidemiology, and Pathophysiology of Post-Facial Paralysis Synkinesis CHAPTER 2 17
5. Peitersen E. Bell’s palsy: the spontaneous course of 2,500 20. Katz B, Schmitt OH. Electric interaction between two adja-
peripheral facial nerve palsies of different etiologies. Acta cent nerve fibres. J Physiol. 1940;97(4):471–488.
Otolaryngol. 2002;(549):4–30. 21. Nielsen VK. Pathophysiology of hemifacial spasm: I.
6. Devriese PP, Moesker WH. The natural history of fa- Ephaptic transmission and ectopic excitation. Neurology.
cial paralysis in herpes zoster. Clin Otolaryngol Allied Sci. 1984;34(4):418–426.
1988;13(4):289–298. 22. Blinzinger K, Kreutzberg G. Displacement of synaptic termi-
7. Nakano H, Haginomori SI, Wada SI, Ayani Y, Kawata nals from regenerating motoneurons by microglial cells. Z
R, Saura R. Electroneurography value as an indicator of Zellforsch Mikrosk Anat. 1968;85:145–157.
high risk for the development of moderate-to-severe syn- 23. Bratzlavsky M, van der Eecken H. Altered synaptic or-
kinesis after Bell’s palsy and Ramsay Hunt syndrome. Acta ganization in facial nucleus following facial nerve regen-
Otolaryngol. 2019;139(9):823–827. eration: an electrophysiological study in man. Ann Neurol.
8. Morecraft RJ, Louie JL, Herrick JL, Stilwell-Morecraft KS. 1977;2(1):71–73.
Cortical innervation of the facial nucleus in the non-human 24. Graeber MB, Bise K, Mehraein P. Synaptic stripping in the
primate: a new interpretation of the effects of stroke and re- human facial nucleus. Acta Neuropathol. 1993;86:179.
lated subtotal brain trauma on the muscles of facial expres- 25. Krishnan AV, Lin CS, Park SB, Kiernan MC. Axonal ion
sion. Brain. 2001;124(Pt 1):176–208. channels from bench to bedside: a translational neuroscience
9. Horta-Júnior JA, Tamega OJ, Cruz-Rizzolo RJ. Cytoarchitecture perspective. Prog Neurobiol. 2009;89(3):288–313.
and musculotopic organization of the facial motor nucleus in 26. Huynh W, Kiernan MC. Peripheral nerve axonal excitability
Cebus apella monkey. J Anat. 2004;204(Pt 3):175–190. studies: expanding the neurophysiologist’s armamentari-
10. Choi D, Raisman G. Somatotopic organization of the facial um. Cerebellum Ataxias. 2015;2:4.
nucleus is disrupted after lesioning and regeneration of the 27. Persson AK, Kim I, Zhao P, Estacion M, Black JA, Waxman
facial nerve: the histological representation of synkinesis. SG. Sodium channels contribute to degeneration of dor-
Neurosurgery. 2002;50(2):355–363. sal root ganglion neurites induced by mitochondrial dys-
11. Jessen KR, Mirsky R. The success and failure of the Schwann function in an in vitro model of axonal injury. J Neurosci.
cell response to nerve injury. Front Cell Neurosci. 2019;13:33. 2013;33:19250–19261.
12. Allodi I, Udina E, Navarro X. Specificity of peripheral nerve 28. Eviston TJ. Mechanisms of Axonal Dysfunction in Facial Nerve
regeneration: interactions at the axon level. Prog Neurobiol. Disorders [Ph.D. Thesis]. University of New South Wales;
2012;98:16–37. 2016.
13. Blesch A, Lu P, Tsukada S, et al. Conditioning lesions before 29. Storey N, Latchman D, Bevan S. Selective internalization of
or after spinal cord injury recruit broad genetic mechanisms sodium channels in rat dorsal root ganglion neurons infected
that sustain axonal regeneration: superiority to cAMP-medi- with herpes simplex virus-1. J Cell Biol. 2002;158:1251–1262.
ated effects. Exp Neurol. 2012;235:162–173. 30. Wang Y, Yang L, Wang W, Ding W, Liu H. Decreased dis-
14. Fu SY, Gordon T. The cellular and molecular basis of periph- tance between representation sites of distinct facial move-
eral nerve regeneration. Mol Neurobiol. 1997;14:67–116. ments in facial synkinesis—a task fMRI study. Neuroscience.
15. Doron-Mandel E, Fainzilber M, Terenzio M. Growth con- 2019;397:12–17.
trol mechanisms in neuronal regeneration. FEBS Lett. 31. Wu JJ, Lu YC, Zheng MX, et al. Motor control deficits in fa-
2015;589:1669–1677. cial synkinesis patients: neuroimaging evidences of cerebral
16. Li H, Wigley C, Hall SM. Chronically denervated rat cortex involvement. Neural Plast. 2019;2019:7235808.
Schwann cells respond to GGF in vitro. Glia. 1998;24:290–303. 32. Wang Y, Wang WW, Hua XY, Liu HQ, Ding W. Patterns of
17. Jonsson S, Wiberg R, McGrath AM, et al. Effect of delayed cortical reorganization in facial synkinesis: a task function-
peripheral nerve repair on nerve regeneration, Schwann cell al magnetic resonance imaging study. Neural Regeneration
function and target muscle recovery. PloS One. 2013;8:e56484. Research. 2018;13(9):1637–1642.
https://doi.org/10.1371/journal.pone.0056484. 33. de Charms RC, Christoff K, Glover GH. Learned regulation
18. Choi D, Raisman G. After facial nerve damage, regenerating of spatially localized brain activation using real-time fMRI.
axons become aberrant throughout the length of the nerve Neuroimage. 2004;21:436–443.
and not only at the site of the lesion: an experimental study. 34. Rota G, Sitaram R, Veit R. Self-regulation of regional corti-
Br J Neurosurg. 2004;18(1):45–48. cal activity using real-time fMRI: the right inferior fron-
19. Arvanitaki A. Effects evoked in an axon by the activity of a tal gyrus and linguistic processing. Hum Brain Mapp.
contiguous one. J Neurophysiol. 1942;5(2):89–108. 2009;30:1605–1614.
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Psychosocial Impact of Facial Palsy 3
Ietske J. Siemann, Carien H.G. Beurskens
Social isolation Patients indicate they are preoccupied with their own
feelings and their facial palsy. They find it difficult to
Worrying
Reduced show an interest in other people and are mainly trying
confidence to “survive”.
Problems with Reactions of
relationships others
Feelings of Uselessness
Physiology Behaviors
Reactions of Others what they are going to say, and how they can prepare
themselves for all this. This is very exhausting and
“I am always extremely alert: I notice people watching me leads to patients being wary of social contacts in gener-
and wondering what could be wrong with me. I’m just very al. Overall, depression and anxiety can have important
sensitive about that...”
consequences and can have a major impact on patients
with a facial palsy.
Patients fear facing social situations and may worry
about the possible reactions of other people. They may OTHER PSYCHOSOCIAL CONSEQUENCES
also be afraid to engage in conversation, speak clearly, or At a certain moment in time, patients will be told
spray spittle when talking with someone. This may cause that they are beyond recovery and that they will no
them to be extra alert when encountering a new situa- longer be treated surgically or otherwise and the re-
tion, which in turn means they will be even more wary habilitation process has come to an end. This is the
and sensitive about other people’s looks and remarks. moment patients need to fend for themselves and
think of ways to arrange their own life with the palsy.
Social Fear/Social Isolation
“When I understood that we had run out of options for more
“I always find new situations scary. People will look at me surgery, I arranged for a meeting with my company doctor.
and I am convinced they’re thinking: look at that droopy He literally said: Maybe now it is time for you to work on how
face. I prefer to arrive a bit later and I make sure I sit or stand all of this is affecting you.”
at the back.”
The consequences of the aforementioned may be
Patients may decide to avoid social situations and re- significant. Patients may avoid social contact or mini-
frain from making contact. The tension that precedes mize their social environment. Studies show that qual-
an encounter may be too much, the fear that the other ity of life is reduced for patients with facial palsy.16
person may say something—or nothing at all—may Fig. 3.2 summarizes the aforementioned consequences
be too big. They may become reserved and withdraw within a cognitive behavioral model.
from everyday life.
COPING MECHANISMS OF FACIAL PALSY
Shame
It is important to understand the various ways that in-
“I have never been very self-assured but that is even worse dividuals deal with the psychosocial impact of facial
now. I am simply very much ashamed of my face.”
palsy. Each person copes in their own unique manner,
depending on the strategies they employ.
Patients are ashamed of their appearance and extreme- Coping mechanisms refer to each person’s mecha-
ly self-conscious when they walk into a room. People nisms to deal with stress or problems. These are con-
with facial palsy indicate they can no longer be them- scious efforts to regulate cognition, behavior, and
selves: they have lost all spontaneity. Spontaneously emotions as a result of stressful situations. Coping
engaging in an unknown situation is a thing of the past; mechanisms strongly depend on personality and dif-
they need to think about how they may be perceived, fer according to circumstances and age.17
22 CHAPTER 3 Psychosocial Impact of Facial Palsy
separate halves. If patients do not feel their face, it 8. Nellis JC, Ishii M, Byrne PJ, Boahene KDO, Dey JK, Ishii LE.
is less present and as a consequence the facial palsy Association among facial paralysis, depression, and quality
of life in facial plastic surgery patients. JAMA Facial Plast
will also be less present. Surg. 2017;19(3):190–196.
•
It may be useful to visualize stressful situations 9. Fu L, Bundy C, Sadiq SA. Psychological distress in people with
(social interaction, unknown people) in advance. disfigurement from facial palsy. Eye. 2011;25(10):1322–1326.
Patients can think of what may happen and think of 10. Hotton M, Huggons E, Hamlet C, et al. The psychosocial im-
how they will respond to possible looks or remarks. pact of facial palsy: A systematic review. Br J Health Psychol.
2020;25(3):695–727.
11. Coulson SE, O’Dwyer NJ, Adams RD, Croxson GR.
CONCLUSION Expression of emotion and quality of life after facial nerve
paralysis. Otol Neurotol. 2004;25(6):1014–1019.
Facial palsy is not only a physical condition, but also 12. Zigmond AS, Snaith RP. The hospital anxiety and depres-
a damage to “the self.” The most common psychoso- sion scale. Acta Psychiatr Scand. 1983;67(6):361–370.
13. Leventhal H, Benyamini Y, Brownlee S, et al. Illness repre-
cial response to facial palsy is depression. In addition sentations: theoretical foundations. In: Petrie KJ, Weinman
to depression there may also be feelings of anxiety. J, eds. Perceptions of Health and Illness. Amsterdam,
Various psychological mechanisms are triggered, and Netherlands: Harwood Academic Publisher; 1997:19–46.
patients need to look for a new balance. A multidis- 14. Bradbury ET, Simons W, Sanders R. Psychological and so-
ciplinary approach for patients with a facial palsy is cial factors in reconstructive surgery for hemi-facial palsy. J
Plastic Reconstr Aesth Surg. 2006;59(3):272–278.
important, whereby a psychologist can make a contri- 15. Dobel C, Miltner WH, Witte OW, Volk GF, Guntinas-Lichius
bution concerning psychosocial problems. O. Emotional impact of facial palsy. Laryngo-Rhino-Otol.
2013;92(1):9–23.
REFERENCES 16. Luijmes RE, Pouwels S, Beurskens CHG, Kleiss IJ, Siemann
I, Ingels KJAO. Quality of life before and after different treat-
1. Rumsey N. The psychology of appearance: why health ment modalities in peripheral facial palsy: a systematic re-
psychologists should “do looks.” Eur Health Psychol. view. Laryngoscope. 2017;127:1044–1051.
2008;10(3):46–50. 17. Lazarus RS, Folkman S. Stress, Appraisal and Coping. New
2. Montepare JM, Dobish H. The contribution of emotion per- York: Springer; 1984.
ceptions and their overgeneralizations to trait impressions. J 18. van den Borne B, Molenaar S. Informing patients about facial
Nonverbal Behavior. 2003;27:237–254. nerve paresis and treatment. In: Beurskens CHG, van Gelder
3. Wolffhechel K, Fagertun J, Jacobsen UP, et al. Interpretation RS, Heymans PG, Manni JJ, Nicolai J-PA, eds. The Facial
of appearance: the effect of facial features on first impres- Palsies, Complementary Approaches. Utrecht, Netherlands:
sions and personality. PloS One. 2014;9(9). https://doi. Lemma Publishers; 2005:404–416.
org/10.1371/journal.pone.0107721. 19. Beurskens CHG, Heymans PG. Mime therapy improves
4. Rojas H, Shah DV, Friedland LA. A communicative ap- facial symmetry in people with long-term facial nerve
proach to social capital. J Communication. 2011;61:689–712. paresis a randomised controlled trial. Austr J Phys Ther.
5. van Veen MM, Tavares-Brito J, van Veen BM, et al. Association 2006;52:177–183.
of regional facial dysfunction with facial palsy-related qual- 20. Diels HJ, Beurskens CHG. Neuromuscular retraining: non-
ity of life. JAMA Facial Plast Surg. 2019;21(1):32–37. https:// surgical therapy for facial palsy. In: Slattery WH, Azizzadeh
doi.org/10.1001/jamafacial.2018.0804. B, eds. The Facial Nerve. New York: Thieme Medical
6. Walker DT, Hallam MJ, Ni Mhurchadha S, McCabe P, Nduka Publishers; 2014:204–212.
C. The psychosocial impact of facial palsy: our experience 21. Hayes SC, Luoma JB, Bond FW, Masuda A, Lillis J.
in one hundred and twenty six patients. Clin Otolaryngol. Acceptance and commitment therapy: model, processes and
2012;37(6):474–477. outcomes. Behav Res Ther. 2006;44(1):1–25.
7. Cross T, Sheard CE, Garrud P, Nikolopoulos TP, O’Donoghue
GM. Impact of facial paralysis on patients with acoustic neu-
roma. Laryngoscope. 2000;110:1539–1542.
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Facial Nerve Consultation 4
Stephanie Warrington, Sara MacDowell, Laura Hetzler
3. How much difficulty did you have saying specific sounds while speaking?
4. How much difficulty did you have with your eye tearing excessively or becoming dry?
5. How much difficulty did you have with brushing your teeth or rinsing your mouth?
Social function:
6. How much of the time have you felt calm and peaceful?
7. How much of the time did you isolate yourself from people around you?
8. How much of the time did you get irritable toward those around you?
9. How often did you wake up early or wake up several times during your nighttime sleep?
10. How often has your facial function kept you from going out to eat, shop, or participate
in family or social activities?
Fig. 4.1 Facial Disability Index (FDI) and Facial Clinimetric Evaluation (FaCE).
specific affected facial regions.4,6,8,11,12 A more detailed Another important aspect of assessing facial
characterization of the defect is warranted. It is good dysfunction is facial tone. Abnormal facial tone is
practice to divide the face into five overall sections: fore- evident to the casual observer, even when the pa-
head, periocular, midface, perioral, and cervical. From tient is at rest, and therefore can have a profound
there, documenting the deficits associated with each divi- impact on the patient’s ability to participate in the
sion is ideal (Table 4.1). Once there is a more precise char- community. Facial tone may be either hypertonic or
acterization of deficits and symptoms, this will aid in the hypotonic, both of which may exist in the same in-
decision-making process for rehabilitation and treatment, dividual (Fig. 4.2) (Video 4.1). This distinction is im-
as well as allowing for more precise assessment of treat- portant as there are different treatment approaches
ment results. for both.1,2,5,6,13
A complete head and neck examination is necessary During a patient’s recovery from facial nerve inju-
to evaluate prior incisions and deficits as well as the ry they can experience aberrant neural regeneration,
status of other cranial nerves. Injury to candidate do- which can lead to undesired facial tone and/or synki-
nor nerves for reanimation may dictate the treatment nesis. Synkinesis is uncoordinated facial motion that
options for a patient. Presence of palpable and/or vis- occurs during abnormal facial nerve recovery. The
ible extratemporal causes of facial paralysis must be patient may experience a lack of perceived motion or
ruled out. To the general practitioner, smaller parotid restricted movement that is due to poor coordination
tumors may elude a rudimentary physical examina- of muscle activation rather than paralysis. For ex-
tion. The presence or history of skin cancer can be a ample, during an attempt at normal smile, the zygo-
concerning factor in patients with initially incomplete maticus complex is restricted in its movement from
but progressive facial nerve paralysis. synkinetic smile antagonists such as depressor anguli
Facial Nerve Consultation CHAPTER 4 27
If you have problems on BOTH sides, answer the questions in the remainder of the survey with regard
to the more affected side, or with regard to both sides if they are equally affected. In the past week:
Circle only ONE number on each line Not at all Only if I A little Almost Normally
concentrate normally
(1) When I smile, the affected side of
1 2 3 4 5
my mouth goes up
(2) I can raise my eyebrow on the
1 2 3 4 5
affected side
(3) When I pucker my lips, the affected
1 2 3 4 5
side of my mouth moves
The following are statements about how you might feel because of your FACE OR FACIAL PROBLEM:
Please rate how often each of the following statements applied to you during the PAST WEEK.
Circle only ONE number on each line All of the Most of Some of A little of None of
time the time the time the time the time
(4) Parts of my face feel tight, worn
1 2 3 4 5
out, or uncomfortable
(5) My affected eye feels dry, irritated,
1 2 3 4 5
or scratchy
(6) When I try to move my face, I feel
1 2 3 4 5
tension, pain or spasm
(7) I use eye drops or ointment in my
1 2 3 4 5
affected eye
(8) My affected eye is wet or has tears
1 2 3 4 5
in it
(9) I act differently around people
1 2 3 4 5
because of my face or facial problem
(10) People treat me differently
1 2 3 4 5
because of my face or facial problem
(11) I have problems moving food
1 2 3 4 5
around in my mouth
(12) I have problems with drooling or
keeping food or drink in my mouth or 1 2 3 4 5
off my chin and clothes
The following are statements about how you might have felt or been doing in the
PAST WEEK because of your FACE OR FACIAL PROBLEM. Please rate how much
you agree with each statement.
Circle only ONE number on each line Strongly Agree Don’t Disagree Strongly
agree know disagree
(13) My face feels tired or when I try to
move my face, I feel tension, pain, or 1 2 3 4 5
spasm
(14) My appearance has affected my
willingness to participate in social 1 2 3 4 5
activities or to see family or friends
(15) Because of difficulty with the way I
eat, I have avoided eating in 1 2 3 4 5
restaurants or in other people’s homes
oris, and platysma as well as other perioral muscles restricting any meaningful motion (Video 4.2). This
including buccinator, and depressor septi nasi. In this must be recognized prior to effectively managing
case, the patient often feels the lack of motion is relat- these patients. Ocular synkinesis is another frequent
ed to weakness but in actuality, it is the result of over- complaint in patients with aberrant facial nerve re-
active tone and contraction of antagonist muscles generation, where attempts at movement such as
28 CHAPTER 4 Facial Nerve Consultation
The Five Divisions of the Face to Be smile or speech can lead to contraction of the orbi-
TABLE 4.1 Evaluated and the Facial Deficits in Each cularis oculi and thus narrowing the eyelid aperture
That Should Be Documented. (Fig. 4.3).1,3,4,6,10,11,13 This is in direct contrast to lag-
ophthalmos that patients experience in fully dener-
FACIAL DIVISIONS FACIAL DEFICIT
vated facial and eyelid musculature.
Forehead Tone An objective assessment of facial tone, voluntary
Synkinesis
movement, and synkinesis should be performed at
Brow elevation
Brow ptosis
the initial evaluation and can be performed using a
myriad of available grading systems. The authors
Periocular Tone
consistently use the Sunnybrook Facial Grading
Synkinesis
Visual acuity
System during initial evaluation.6,10,12–14 In addition
Corneal integrity to descriptive documentation and objective grading
Tearing systems, it is also recommended that photographic
Bell’s phenomenon and video documentation of patients be captured
Lagophthalmos at each session.13,14 Patients should be recorded in
Lower lid laxity repose, brow elevation, gentle eye closure, firm eye
Lacrimal puncta position closure, closed mouth smile, open mouth smile,
Midface Tone snarl, pucker, wide mouth opening, lower lip de-
Synkinesis pression, and nasal base view. The authors also doc-
Oral commissure excursion ument a “puffing cheeks with air” photograph (Fig.
Nasal obstruction 4.4).14 Videos can be captured of dynamic move-
Perioral Tone ments and speech. This is a useful way to monitor
Synkinesis treatment outcomes and to allow patients to ob-
Oral incompetence serve progress. A subjective questionnaire that can
Articulation errors be used to determine a patient’s perception of syn-
Cervical Tone kinesis is the Synkinesis Assessment Questionnaire
Synkinesis (SAQ) (Fig. 4.5).4,6
A B
Fig. 4.2 (A) An example of the flaccid facial tone on the right. Note dependent oral commissure position and
generalized facial ptosis as well as nasal deviation. (B) An example of hypertonic facial tone on the left. In the
hypertonic face, you can see decreased ocular aperture, deepened nasolabial crease, and platysmal banding in
repose.
Facial Nerve Consultation CHAPTER 4 29
A B
Fig. 4.3 Example of Blepharospasm Synkinesis. Right (A) and left (B) facial paralysis with synkinesis resulting in eye
closure/blepharospasm upon smiling.
RADIOLOGY AND ELECTRODIAGNOSTICS EMG can indicate reinnervation, fibrillations can indi-
Imaging studies may play an important role in the cate muscle fibers are still viable but do not have inner-
evaluation and treatment of facial palsy patients. In vation, and no activity on EMG indicates the muscle
the unrecovered patient with idiopathic facial paraly- fibers no longer have myogenic ability and cannot
sis, imaging studies such as computed tomography be reinnervated. This information can help direct the
(CT) and magnetic resonance imaging (MRI) can be available options for reanimation.7,8
useful to evaluate the presence of tumors along the fa-
cial nerve or to confirm inflammation in patient with
MANAGEMENT
presumed Bell’s palsy.7
Some patients may require surgery to help with As mentioned earlier, allowing the patient to articu-
their facial nerve recovery. For those who have facial late their perception of the deficit is key. The smile is
nerve injury due to previous surgery or trauma, a CT considered a thumbprint of an individual’s personal-
scan can be useful preoperatively to help with surgical ity and loss of this ability can be personally devastat-
planning. ing.5,11 That being said, as clinicians, we must protect
When evaluating the need for or planning for spe- from further harm such as loss of vision. Clear direc-
cific facial reanimation procedures, the type of reani- tion on how to protect the eye should be given and re-
mation procedure an individual can receive depends ferral to an ophthalmologist who can follow the status
on the time since injury, the viability of the facial nerve, of the cornea is ideal.2,3
and the status of the myogenic potential of the facial Expectation management is crucial in our recover-
muscles. Electroneuronography (ENoG) can help de- ing patients. Whether the patients have had an attempt
termine the degree of injury to the nerve. However, at surgical dynamic reanimation or the treatment plan
due to Wallerian degeneration, which takes place 3 to is to allow for natural recovery, the timeline to a new
5 days after injury, an ENoG cannot be performed un- normal may be significant. Sharing that complete and
til after that time. It is recommended to be completed perfect recovery is not likely early on, but expressing
between 3 days to 3 weeks after injury to the facial commitment to optimize their outcome will allow the
nerve. ENoG can only assess early stages of acute fa- patient to both understand the need for patience and
cial paralysis and act as a prognostic indicator in facial the limitations of nerve recovery.
nerve regeneration. Electromyography (EMG) can be The paradigm of care in facial paralysis has shifted
performed acutely and in patients with chronic facial over the past decade to a multispecialty model.11 Patients
paralysis and is typically the study of choice after 3 with facial paralysis require multiple medical special-
weeks post injury. Electric action potentials seen on ties to be involved in their care including neurologists,
30 CHAPTER 4 Facial Nerve Consultation
A B C D
E F G H
I J K
Fig. 4.4 Photographic Documentation of Patients Obtained at Each Evaluation. (A) Repose. (B) Brow elevation.
(C) Gentle eye closure. (D) Firm eye closure. (E) Closed mouth smile. (F) Open mouth smile. (G) Snarl. (H) Pucker. (I)
Wide mouth opening. (J) Lower lip depression. (K) Puffing cheeks with air.
Date:
Please answer the following questions regarding facial function, on a scale from 1 to 5,
according to the following scale:
Question Score
1 When I smile, my eye closes
2 When I speak, my eye closes
3 When I whistle or pucker my lips, my eye closes
4 When I smile, my neck tightens
5 When I close my eyes, my face gets tight
6 When I close my eyes, the corner of my mouth moves
7 When I close my eyes, my neck tightens
8 When I eat, my eye waters
9 When I move my face, my chin develops a dimpled area
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