Muscles of Fascial Expressions discusses the anatomy and development of facial muscles. It describes the origins, insertions, actions and nerve supply of each muscle. Examples of clinical conditions involving facial muscles are also provided, such as Bell's palsy (facial nerve paralysis), hemifacial spasm (involuntary muscle contractions on one side of face), and facioscapulohumeral muscular dystrophy (muscle weakness and wasting affecting face, shoulder blades and arms).
4. Introduction
The muscular system develops from
the mesodermal germ layer and
consists of skeletal, smooth, and
cardiac muscle.
Skeletal muscle is derived from
paraxial mesoderm, which forms
somites from the occipital to the
sacral regions and somitomeres in the
head.
5. Smooth muscle differentiates from
visceral splanchnic mesoderm
surrounding the gut and its
derivatives and from ectoderm
(pupillary, mammary gland, and sweat
gland muscles).
Cardiac muscle is derived from
visceral splanchnic mesoderm
surrounding the heart tube.
6. Fate of Somites :-
Paraxial mesoderm becomes segmented
to form number of somites, that lie on
either side of the developing neural tube.
Cross section through a somite shows
that it is triangular in shape/ structure
and a cavity.
Somites is divisible in 3 parts :-
1. Ventromedial part
2. Lateral part
3. Intermediate part
7. C.S. view of somite;:
Neural tube
Notocord
somite
Lateral
part
Interme
-diate
part
Ventral part
Ventrolateral
(VLL)
Dorsomedial (DML)
11. Skeletal muscle (striated):
During differentiation, precursor cells, the
myoblasts, fuse and form long,
multinucleated muscle fibers.
Myofibrils soon appear in the cytoplasm,
and by the end of the third month, cross-
striations, typical of skeletal muscle,
appear.
12. A similar process occurs in the seven
somitomeres in the head region rostral to
the occipital somites.
Tendons for the attachment of muscles
to bones are derived from sclerotome
cells lying adjacent to myotomes at the
anterior and posterior borders of
somites.
The transcription factor SCLERAXIS
regulates development of tendons.
14. Innervation:-
Name components Cells of
origin
Main central
connections
Exit from
skull
Distribution n
function
Facial General
somatic
afferent
Geniculate
ganglion
To
trigeminal
sensory
nucleus,
cerebellum
Internall
acoustic
meatus
Sensation
from the
part of
tympanic
membrane
, external
acoustic
meatus n
skin behind
pinna.
Propriocept
ive from
facial
muscles
Facial Special
viseceral
afferent
Geniculate
nucleus
To nucleus
solitarius
Internal
acoustic
meatus
Taste from
anterior two
third of
tongue
15. Facial General
visceral effernt
Superior
salivatory
nucleus
From olfactory
areas, nucleus
solitarius
Internal
acoustic
meatus
Parasympatheti
c to lacrimal
gland and
mucous
membrane of
nasal and oral
cavities (via
pterygopalatine
ganglion) and
submandibular
and sublingual
salivary glands
(via
submandibular
ganglion)
causing
secretion
and
vasodilatation
Facial Branchial
(special visceral)
effernet
Facial nucleus From precentral
gyrus
Internal acoustic
meatus
Motor to
muscles of facial
expression,
stapedius,
posterior belly of
digastric and
stylohyoid
18. s.no. Nerve branch Muscles innervates
1 Temporal branch 1. Auricularis superior and
anterior.
2. Frontal belly of
occipitofrontalis.
3. Superior part of orbicularis
oculi.
2 Zygomatic branch 1. Inferior part of the
orbicularis oculi.
2. Facial ms. Inferior to orbit.
3 Buccal branch 1. Buccinator.
2. Upper part of orbicularis
oris.
3. Inferior fibers of levator
labii superioris.
4 Marginal mandibular branch 1. Risorius.
2. Muscles of lower lip and
chin.
5 Cervical branch 1. platysma
19. Anatomy
The facial muscles (muscles of facial
expression) are in the subcutaneous
tissue of the anterior and posterior scalp,
face, and neck.
They move the skin and change facial
expressions to convey mood.
Most muscles attach to bone or fascia
and produce their effects by pulling the
skin.
20.
21. Occipitofrontalis
Muscles Origin Insertion Action
Front Belly Epicranial
aponeurosis
Skin and
subctaneous
tissues of
eyebrows and
forehead
Elevates
eyebrows
and wrinkles
forehead ;
Protracts scalp
(indicating
surprise or
curosity)
Occipital Belly Lateral 2/3 rd of
superior nuchal
line
Epicranial
aponeurosis
Retracts scalp
(increasing
effectiveness of
frontal belly)
22. Muscles Origin Insertion Action
Orbicularis
oculi
Medial orbital
margin; medial
palpebral
ligament; lacrimal
bone
Skin around
margin of orbit;
superior
and inferior tarsal
plates
Closes eyelids:
palpebral part
does
so gently; orbital
part tightly
(winking)
23. Muscles Origin Insertion Action
Corrugator
supercilii
Medial end of
superciliary arch
Skin superior to
middle of
supraorbital
margin and
superciliary arch
Draws eyebrow
medially and
inferiorly,
creating vertical
wrinkles
above nose
(demonstrating
concern
or worry)
24. Muscles Origin Insertion Action
Procerus plus
transverse
part of nasalis
Fascia
aponeurosis
covering nasal
bone and lateral
nasal cartilage
Skin of inferior
forehead,
between
eyebrows
Depresses
medial end of
eyebrow;
wrinkles skin
over dorsum of
nose
(conveying
disdain or dislike)
25. Muscles Origin Insertion Action
Alar part of
nasalis
plus levator labii
superioris
alaeque nasii
Frontal process
of maxilla
(inferomedial
margin of orbit)
Major alar
cartilage
Depresses ala
laterally, dilating
anterior nasal
aperture (i.e.,
“flaring nostrils,”
as during anger
or exertion)
26. Muscles Origin Insertion Action
Orbicularis oris
(oral
sphincter)
Medial maxilla
and mandible;
deep
surface of peri-
oral skin; angle of
mouth (modiolus)
Mucous
membrane of lips
Tonus closes oral
fissure; phasic
contraction
compresses and
protrudes
lips (kissing) or
resists
distension (when
blowing)
27. Muscles Origin Insertion Action
Levator labii
Superioris
Infra-orbital
margin (maxilla)
Skin of upper lip
Part of dilators of
mouth; retract
(elevate) and/or
evert upper lip;
deepen
nasolabial sulcus
(showing
sadness)
Zygomaticus
minor
Anterior aspect,
zygomatic bone
28. Muscles Origin Insertion Action
Buccinator
(cheek
muscle)
Mandible,
alveolar
processes of
maxilla and
mandible,
pterygomandibul
ar
raphe
Angle of mouth
(modiolus);
orbicularis
oris
Presses cheek
against molar
teeth;
works with
tongue to keep
food
between occlusal
surfaces and out
of oral vestibule;
resists distension
(when blowing)
29. Muscles Origin Insertion Action
Zygomaticus
major
Lateral aspect of
zygomatic bone
Angle of mouth
(modiolus)
Part of dilators of
mouth; elevate
labial
commissure—
bilaterally to
smile
(happiness);
unilaterally to
sneer (disdain)
30. Muscles Origin Insertion Action
Levator anguli
oris
Infra-orbital
maxilla (canine
fossa)
Angle of mouth
(modiolus)
Part of dilators of
mouth; widens
oral fissure, as
when grinning or
grimacing
31. Muscles Origin Insertion Action
Risorius Parotid fascia
and buccal skin
(highly variable)
Angle of mouth
(modiolus)
Part of dilators of
mouth;
depresses
labial
commissure
bilaterally to
frown (sadness)
Depressor
anguli oris
Anterolateral
base of mandible
32. Muscles Origin Insertion Action
Depressor labii
inferioris
Platysma and
anterolateral
body of
Mandible
Skin of lower lip Part of dilators of
mouth; retracts
(depresses)
and/or everts
lower lip
(pouting,
sadness)
33. Muscles Origin Insertion Action
Mentalis Body of
mandible
(anterior to roots
of inferior
incisors)
Skin of chin
(mentolabial
sulcus)
Elevates and
protrudes lower
lip;
elevates skin of
chin (showing
doubt)
34. Muscles Origin Insertion Action
Platysma Subcutaneous
tissue of
infraclavicular
and
supraclavicular
regions
Base of
mandible; skin of
cheek
and lower lip;
angle of mouth
(modiolus);
orbicularis oris
Depresses
mandible
(against
resistance);
tenses skin of
inferior face
and neck
(conveying
tension and
stress)
37. Bells palsy: (Sir Charles
Bell)
Synonyms: VIIth nerve paralysis , facial paralysis
Bells palsy is the one of the most common
neurologic disorders affecting cranial nerves.
It is an abrupt isolated, unilateral, peripheral facial
nerve paralysis without detectable causes.
38. Etiology:
Cold — it usually occurs after exposure to cold.
Trauma — it may be a causative factor as Transient
Bell’s palsy occurs after extraction of teeth and after
injection of local anesthesia (inferior alveolar nerve
block).
Surgical procedure —surgical procedures such as
removal of parotid gland tumor in which the facial nerve
is sectioned can also cause facial paralysis.
39. Ischemia—it may caused by ischemia of the nerve
near the stylomastoid foramen, resulting in edema of
the nerve, its compression in the bony canal and
finally, paralysis.
Facial canal and middle ear neoplasm—these are
usually associated with sensorineural hearing loss
where 7th nerve palsy is a feature.
Tumors—tumors of cranial base, parapharyngeal
space and infratemporal fossa often cause 7th nerve
palsy.
40. Clinical features:
Age and sex—women are more
commonly affected than men and
usually, it occurs in the middle age
group.
Onset—it begins abruptly as paralysis
of the facial musculature, usually
unilaterally.
Prodromal symptoms—in some
cases, it is preceded by pain on the
side of the face which is ultimately
involved, particularly within the ear,
temple, and mastoid area or at the
angle of the jaw.
41. Symptoms—speech and eating is difficult and
occasionally, taste sensation on the anterior
portion of tongue is lost or altered. Food is
retained in the upper and lower buccal and labial
folds due to weakness of buccinator.
Eye—on the affected side, eye cannot be closed
and wrinkles are absent on that side. There is
watering of eye, which leads to infection.
Facial features—when the patient smiles, the
paralysis becomes obvious since the corner of the
mouth does not rise nor does the skin of the
forehead wrinkles or the eyebrows raise
42. Mask like face—the patient has
a typical mask-like or
expressionless appearance.
Drooling of salvia—the
muscular paralysis manifests
itself by dropping of the corner
of mouth, from which saliva may
dribble.
Syndrome associated—it is
associated with Melkersson-
Rosenthal syndrome.
43. Melkersson- Rosenthal syndrome:-
Recurrent attacks of facial paralysis, identical with
Bell’s palsy associated with multiple episodes of non-
pitting, non-inflammatory painless edema of the face,
chelitis granulomatosa and fissured tongue or lingua
plicata is known as Melkersson- Rosenthal
syndrome.
44. Management:-
Vasodilator— like histamine .
Nicotinic acid — administration of physiologic flushing
dose of nicotinic acid.
Surgically — surgical anastamosis of nerves has been
carried out, especially with facial and hypoglossal
nerve; thus can restore partial function.
Other—systemic steroids or ACTH injection have been
successful in treating Bell’s palsy.
45. Hemifacial Spasm
Hemifacial spasm (HFS) is a rare neuromuscular disease
characterized by irregular, involuntary muscle contractions
(spasms) on one side (hemi-) of the face (-facial).
usually starting around the eyes before progressing
inferiorly to the cheek, mouth, and neck.
HFS can be divided into two types:
Primary HFS is triggered by vascular compression
whereas, secondary HFS comprises all other causes of
facial nerve damage.
46. HFS starts with tonic-clonic contractions of the orbicularis
oculi muscle, resulting in involuntary eyelid closure and
eyebrow elevation.
Over time, the contractions progress to the region affecting
the frontalis (i.e., muscles of the forehead), platysma
(i.e.,muscles of the neck), and orbicularis oris (i.e.,muscles
of the mouth)muscles.
Eventually, the patient may develop sustained contractions
of all involved muscles, causing a severe, disfiguring
grimace with partial closure of the eyes and lifting of the
mouth corners in the “tonus phenomenon”
47. Facioscapulohumeral muscular
dystrophy
Facioscapulohumeral muscular dystrophy is muscle
weakness and loss of muscle tissue that gets worse over
time.
This condition gets its name from the muscles that are
affected most often: those of the face (facio-), around the
shoulder blades (scapulo-), and in the upper arms
(humeral).
Facioscapulohumeral muscular dystrophy is a genetic
disorder. It appears in both men and women. It may develop
in a child if either parent carries the gene for the disorder. In
10% to 30% of cases, the parents do not carry the gene.
48. The signs and symptoms of facioscapulohumeral muscular
dystrophy usually appear in adolescence. However,the
onset and severity of the condition varies widely.
Weakness involving the facial muscles or shoulders is
usually the first symptom of this condition.
Facial muscle weakness often makes it difficult to drink
from a straw , whistle, or turn up the corners of the mouth
when smiling.
Weakness in muscles around the eyes can prevent the
eyes from closing fully while a person is asleep, which can
lead to dry eyes and other eye problems.
49. Weak shoulder muscles tend to make the shoulder
blades (scapulae) protrude from the back, a common sign
known as scapular winging.
Weakness in muscles of the shoulders and upper arms
can make it difficult to raise the arms over the head or
throw a ball.
50. Flaring of Nostrils
The actions of the Nasalis muscles have
generally been held as insignificant; however,
observant clinicians study their action
because of their diagnostic value.
For example, true nasal breathers can flare
their nostrils distinctly. Habitual mouth
breathing, caused chronic nasal obstruction.
51. For example, diminishes and sometimes
eliminates the ability to flare the nostrils.
Children who are chronic mouth breathers often
develop dental malocclusion (improper bite)
because the alignment of the teeth is maintained
to a large degree by normal periods of occlusion
and labial closure.
Anti-snoring devices have been developed that
attach to the nose to flare the nostrils and
maintain a more patent air passageway.
52. Infra-Orbital Nerve Block :
To determine where the infra-orbital nerve emerges,
pressure is exerted on the maxilla in the region of the
infraorbital foramen.
Too much pressure on the nerve causes considerable
pain. Because companion infra-orbital vessels leave the
infra-orbital foramen with the nerve, aspiration of the
syringe during injection prevents inadvertent injection of
anesthetic fluid into a blood vessel.
Because the orbit is located just superior to the injection
site, a careless injection could result in passage of
anesthetic fluid into the orbit, causing temporary paralysis
of the extra-ocular muscles.
54. Classification of conditions effects
facial muscles :-
Birth trauma / congenital :
i. cardiofacial syndrome
ii. congenital dysfuction
iii. hemifacial microsomia
iv. moebius syndrome
Infection :
I. Tetanus
II. Botulism
III. Mastoid infection
IV. Cholesteotema
V. Ramsay hunt syndrome
55. Tumours
I. Acoustic neuroma
II. Facial nerve neuroma
III. Pleomorphic adenoma (salivary gland)
IV. Adenoid cystic carcinoma (salivary gland)
Trauma
I. Extracranial :
I. Parotid surgery
II. Gunshot
III. Knife wound
IV. Carotid endartectomy
II. Infratemporal : motor vehicle accidents
III. Intracranial : surgeries
IV. Temporal bone fracture
57. Cardiofacial syndrome:
Neonatal asymmetric crying faces (ACF)
The clinical hallmark of this condition
is a symmetric appearance at rest, but significant
unilateral depression of the lower lip with crying.
A variety of terms have been used to characterize the
dysfunction as: asymmetric crying faces, congenital
hypoplasia of the depressor anguli oris muscle, or
developmental unilateral lower lip palsy.
Pape and Pickering named the phenotype ACF in 1972.
Cayler first described the association of ACF with
congenital cardiac disease, and subsequently named it
‘cardiofacial syndrome’.
58. Hemifacial microsomia :
HFM involves first and second branchial arch derivatives
with a highly variable phenotype.
Deformities may include auricular defects, preauricular
tags and fistulae, microtia-atresia, mandibular, maxillary,
and orbital hypoplasia, micropthalmia, epibulbar
dermoid, strabismus, conductive or sensoneural hearing
loss, and hypoplastic facial muscles.
HFM was first described by German physician Carl
Ferdinand Von Arlt in 1881.
59. Gorlin et al. used the term HFM to describe patients
with unilateral microtia, macrostomia and malformation
of mandibular ramus and condyle, whereas Goldenhar
syndrome was described as a variant with vertebral
anomalies and epibulbar dermoids.
The name craniofacial was proposed by Converse et
al. when cranial deformities were included.
Other synonyms include first arch syndrome, first and
second branchial arch syndrome, otomandibular
dysostosis, oculo-auriculovertebral dysplasia and
lateral facial dysplasia.
60. The clinical picture of HFM varies from a little
asymmetry in the face to severe under-development of
one facial half with orbital implications, a partially
formed ear or even a total absence of the ear.
The chin and the facial midline are off-centred, and
deviated to the affected side.
Often, one corner of the mouth is situated higher than
the other, giving rise to an oblique lip line.
61. Other asymmetric symptoms are the unilateral
hypoplastic maxillary and temporal bones, a unilateral
shorter zygomatic arch and malformations of the
external and internal parts of the ear.
Auditory problems (conduction deafness) as a result of
malformations in the middle ear and facial nerve
dysfunction (temporal and zygomatic branch of the
facial nerve) are very common in these patients: 30–
50% of the patients have auditory problems.
62. Intra-oral structures can also be affected in this condition:
agenesis of third molar and second premolar may be
present on the affected side, as well as supernumerary
teeth, enamel malformations, delay in tooth development
and hypoplastic teeth.
The masseter, temporal and pterygoid muscles, and
the muscles of facial expression are hypoplastic on
the affected side. The degree of under-development of
the bone is directly related to the hypoplasia of the muscle
to which they are attached.
Moreover, patients with HFM often reveal asymmetric
development of masticatory system muscles as well as
agenesis of salivary glands and rarely palate clefts.
63.
64. Moebius syndrome:
Moebius syndrome is a rare congenital
developmental disorder , characterized by
absence or underdevelopment of the
nerves that control facial and eye
movement (C.N. VI & VII).
Moebius’ syndrome represents a broad
spectrum of clinical findings ranging from
isolated unilateral facial paralysis to
bilateral absence of facial and abducens
nerve function.
65. Multiple other cranial nerves, including the
glossopharyngeal, vagus, hypoglossal, and other
extraocular motor nerves, also can be affected.
Children or adult with facial paralysis may be unable to
smile , frawn raise their eyebrows, close their eyelids or
pucker the lips.
Also involves difficulty in drooling and difficulty with
speech.
Infants can have difficulty with sucking and swallowing.
66. Genetic factors, teratogens and vascular anomalies
have been linked with the aetiopathogenesis of the
syndrome.
Most cases of developmental facial weakness have
obvious associated defects.
These include limb anomalies such as talipes
equinovarus, syndactyly, hemimelia, craniofacial
deformities, etc.
68. Acoustic neuroma
An acoustic neuroma (neurofibroma) is a slow-growing
benign tumor of the neurolemma (Schwann) cells.
The tumor begins in the vestibular nerve while it is in
the internal acoustic meatus.
The early symptom of an acoustic neuroma is usually
loss of hearing. Dysequilibrium (derangement of the
sense of equilibrium) and tinnitus occur in
approximately 70% of patients (Bruce et al., 2010).
69.
70. Facial Nerve Neuroma
A nonmalignant fibroid growth may grow in the facial
nerve itself. This tumor may or may not produce a
gradually progressive facial nerve paralysis.
Removal of this facial nerve neuroma requires removing
that portion of the facial nerve invaded by the tumor.
Usually it is possible to graft it at the time of surgery with
a skin sensation nerve from the neck.
71. Pleomorphic adenoma
The pleomorphic adenoma is the most common tumor of
the salivary glands; overall, it accounts for about 60% of all
salivary gland tumors.
It is also called as ‘iceberg tumor’, ‘endothelioma’,
‘branchioma’, or ‘enchondroma’.
It is often called a mixed tumor because it consists of both
epithelial and mesenchymal elements.
The majority of these tumors are found in the parotid
glands, with less than 10% in the submandibular,
sublingual, and minor salivary glands.
72. Clinical Features
Sex and age—women to men ratio is 6:4. It is common in
4th to 6th decades but also seen in young adults and
children.
Site—parotid 90% and intraoral palatal gland on lip. In
parotid involvement, superficial portion is most commonly
affected.
Size—it may increase to cricket ball size or even more,
weighing in pounds and in intraoral cases, not more than
1 to 2 cm in diameter. If neglected lesion can grow bizarre
proportions.
73. Dumbbells shaped appearance—in some cases, lesion
grows in medial direction between ascending ramus and
stylomandibular ligament resulting in dumble shaped
appearance.
Surface—its surface is smooth. Sometime, it is bosselated
and is occasionally crossed by deep furrows.
Fixation—no fixation, either to deeper tissues or overlying
skin.
74. Consistency—it is firm and rubbery to feel. Sometimes
cystic degeneration may be seen.
Palatal tumor—it is seen on lateral aspect of the palate.
They are smooth surface and dome shaped.
Signs of malignant transformation—accelerated growth
rate, tumor irregularity on palpations, necrosis and
painful ulceration and facial nerve involvement.
75. Pleomorphic adenoma (mixed parotid
tumour) which requires removal with a
margin of normal parotid tissue,
conserving the facial nerve and its
branches.
On account of the wide extent of the
gland, it is approached through an S-
shaped incision made from in front of the
ear, backwards to the mastoid process
and then downwards and forwards below
the angle of the mandible.
76. Adenoid Cystic Carcinoma
It is also called as ‘cylindroma’, ‘adenocystic
carcinoma’ and ‘baseloid mixed tumor’.
Clinical Features
Age—it occurs in the 5th and 6th decade of life.
Site—most common glands involved are the parotid,
submaxillary and the accessory glands in palate and
tongue.
77. Symptoms—the most common initial symptom is
presence of mass followed by local pain, facial nerve
paralysis in case of parotid tumor and tenderness.
Signs—some of the lesions exhibit surface ulceration.
Other findings include nasal obstruction, proptosis,
sinusitis, ear infection, epistaxis, signs of cranial nerve
involvement and visual disturbances.
79. Tetanus
Tetanus is an acute, potentially fatal disease that is
characterized by generalized increased rigidity and
convulsive spasms of skeletal muscles.
Tetanus is caused by the spore-forming bacterium
Clostridium tetani.
C. tetani spores (the dormant form of the organism)
are found in soil and in animal and human feces.
80. Tetanus usually follows a recognized injury.
Contamination of wounds with soil, manure, or
rusty metal can lead to tetanus.
It can complicate burns, ulcers, gangrene,
necrotic snakebites, middle ear infections, septic
abortions, childbirth, intramuscular injections, and
surgery.
Injuries may be trivial (insignificant or avoidable)
and in up to 50% of cases the injury occurs
indoors and/or is not considered serious enough
to seek medical treatment.
81. There is a clinical triad of rigidity,
muscle spasms and, if severe,
autonomic dysfunction.
Neck stiffness, sore throat, and
difficulty opening the mouth are
often early symptoms.
Masseter spasm causes trismus
or ‘lockjaw’. Spasm progressively
extends to the facial muscles
causing the typical facial
expression, ‘risus sardonicus’,
and muscles of swallowing
causing dysphagia.
Risus sardonicus
82. Rigidity of the neck muscles leads to retraction of the
head.
Truncal rigidity may lead to opisthotonus and
respiratory difficulty with decreased chest wall
compliance.
Tetanus is categorized into generalized, neonatal
(which is a generalized form in children less than
one month), local, and cephalic (which is tetanus is
localized to the head region).
83. Generalized and neonatal
tetanus affect muscles of the
whole body and lead to
opistotonus (the backward
arching of the columna due to
rigidity of the extensor muscles
of the neck and back) and may
cause respiratory failure and
death due to rigidity and spasms
of the laryngeal and respiratory
muscles.
opistotonus
84. Local and cephalic tetanus
account for only a minority of
cases.
Cephalic tetanus when
localized tetanus from a head
wound affects the cranial
nerves; paralysis rather than
spasm predominates at
presentation , but
progression to generalized
tetanus is common and
mortality is high.
Left side facil paralysis
85. Summary
Depending on whether it is local/cephalic or
generalized/neonatal, tetanus typically manifests as
trismus/lockjaw, risus sardonicus, dysphagia,
neck stiffness, abdominal rigidity, and
opistotonus, i.e., hyperactivity of muscles of the
head, neck, and trunk. The limbs tend to be less
severely affected, but with full opistotonus there is
also flexion of the arms and extension of the legs,
as in a decorticate posture.
86. Botulism
Botulism is characterised by symmetrical, descending,
flaccid paralysis of motor and autonomic nerves usually
beginning with cranial nerves.
It occurs when neuromuscular transmission is
interrupted by a protein neurotoxin produced by the
spore-forming, obligate anaerobic bacterium Clostridium
botulinum.
Paralysis begins with the cranial nerves, then affects the
upper extremities, the respiratory muscles, and, finally,
the lower extremities in a proximal-to-distal pattern.
87. In severe cases, extensive respiratory muscle
paralysis leads to ventilatory failure and death
unless supportive care is provided.
There are five clinical categories of botulism:
1) foodborne botulism;
2) wound botulism;
3) infant botulism;
4) adult infectious botulism;
5) inadvertent, following botulinum toxin injection.
88. Paralysis of cranial
nerve VII produces
expressionless facies,
and dysphagia is
caused by cranial nerve
IX paralysis, which may
present as regurgitation
(at times nasal) of
masticated food or
beverages.
89. Cranial nerve palsies may be followed by flaccid,
descending, completely symmetric paralysis of
voluntary muscles, affecting (in order) the muscles of
the neck, shoulders, the proximal and then distal
upper extremities, and the proximal followed by distal
lower extremities.
Paralysis of the diaphragm and accessory breathing
muscles may result in respiratory compromise or
arrest.
90. Mastoid Infection
Acute or chronic middle ear infections occasionally cause
a weakness of the face due to swelling or direct pressure
on the nerve. In acute infections the weakness usually
subsides as the infection is controlled and the swelling
around the nerve subsides.
Facial nerve weakness occurring in chronically infected
ears is usually due to pressure from a cholesteatoma
(skin-lines cyst). Mastoid surgery is performed to
eradicate the infection and relieve nerve pressure. Some
permanent facial weakness may remain.
91. Cholesteatoma
Cholesteatoma as a cyst-like expansile lesion of the
temporal bone lined by stratified squamous epithelium
that contains desquamated keratin.
These most often occur in the middle ear and mastoid.
Cholesteatomas need sooner than later management
because of their locally destructive properties. These
lesions can cause infections, otorrhea, bone
destruction, hearing loss (mostly conductive), facial
nerve weakness or paralysis, vertigo via labyrinthine
fistula, lateral sinus thrombosis.
93. Facial Paralysis May develop:
(complication of cholesteatoma)
Acutely secondary to infection .
Slowly from chronic expansion of cholesteatoma .
Temporal bone CT: localize the nerve involvement .
Most common site: geniculate ganglion due to
disease in the anterior epitympanum .
94. Ramsay hunt syndrome
Clinical manifestation of it, is the facial
paralysis as well as pain of the external
auditory meatus and pinna of the ear.
It is zoster infection of geniculate ganglion
with involvement of the external ear and
oral mucosa.
In addition, vesicular eruption occurs in
the oral cavity and oropharynx with
hoarseness of voice, tinnitus, vertigo and
occasional other disturbances.
96. Heerfordt's syndrome
Heerfordt's syndrome is an acute syndromal
presentation of sarcoidosis, presenting with the
following features:
Fever.
Uveitis.
Swelling of parotid, or may be other
salivary/lacrimal glands.
97. Fever and constitutional upset - The patient may have
experienced paroxysmal episodes of fever or night
sweats. Other symptoms such as headache and
weight loss may occur.
Eye symptoms - Acute uveitis presents with: Eye
discomfort, Photophobia, Blurred vision, Seeing
'floaters' , Red eye .The lacrimal glands may be
involved, causing puffy eyelids.
Parotid swelling May be unilateral or bilateral - bilateral
in 73% of cases of parotid sarcoidosis. Diffuse,
painless swelling of the parotid may or may not be
other salivary glands/lacrimal glands, may cause a dry
mouth.
98. Cranial nerve palsy -- it affect the facial nerve (CN
VII). There is abrupt onset within a short period of
the parotid swelling.
The nerve is thought to be entrapped/infiltrated by
granulomatous inflammation in the parotid gland or
facial canal but the exact site remains uncertain.
There may be accompanying disturbance of taste
(chorda tympani dysfunction - supporting presumed site
of lesion).
Other cranial nerves can be involved.
99. Symptoms of other cranial neuropathies commonly involved in
neurosarcoidosis include :
Disturbance of smell ,
Blurred vision/diplopia/sight impairment ,
Speech or swallowing difficulty ,
Vertigo/deafness/tinnitus,
Weakness of trapezius/neck muscles,
Tongue deviation or atrophy.
100. Tetany
Tetany or tetany seizure is a medical sign consisting
of the involuntary contraction of muscles, which may
be caused by disease or other conditions that
increase the action potential frequency of muscle
cells or the nerves that innervate them. Muscle
cramps which are caused by the disease tetanus are
not classified as tetany; rather, they are due to a lack
of inhibition to the neurons that supply muscles.
101. Hypocalcemia: It is the primary cause of tetany. Low
ionized calcium levels in the extracellular fluid increase the
permeability of neuronal membranes to sodium ion,
causing a progressive depolarization, which increases the
possibility of action potentials. This occurs because
calcium ions interact with the exterior surface of sodium
channels in the plasma membrane of nerve cells. When
calcium ions are absent the voltage level required to open
voltage gated sodium channels is significantly altered (less
excitation is required). If the plasma Ca2+ decreases to less
than 50% of the normal value of 9.4 mg/dl, action
potentials may be spontaneously generated, causing
contraction of peripheral skeletal muscles. Hypocalcemia is
not a term for tetany but is rather a cause of tetany.
102. CHVOSTEK’S SIGN Elicitation:
Tapping on the face at a point
just anterior to the ear and just
below the zygomatic bone
Postitive response: Twitching
of the ipsilateral facial
muscles, suggestive of
neuromuscular excitability
caused by hypocalcemia.
103. TROUSSEAU’S SIGN
Elicitation:
Inflating a sphygmomanometer
cuff above systolic blood
pressure for several minutes
Postitive response: Muscular
contraction including flexion of
the wrist and
metacarpophalangeal joints,
hyperextension of the fingers,
and flexion of the thumb on the
palm, suggestive of
neuromuscular excitability
caused by hypocalcemia.
104. Scleroderma
It is also called as systemic sclerosis, or Hidebound
disease. It is rare collagen disorder, which is
characterized by hardening and tightening of the skin
that can manifest as either localized or systemic form.
It is a disease which involves connective tissue, blood
vessels and lead to fibrosis. It is also called as
progressive systemic sclerosis.
Name is derived from sclero—hard derma—skin.
105. Types
Systemic or progressive systemic sclerosis—it is
diffuse and involves both skin and internal organ.
Localized form—it involve the underlying muscle and
bone along with skin and subcutaneous tissue. It is of
two types:
Morphea—it is circumscribed form and is
characterized by local changes limited to skin.
Linear—it affects face, scalp and frontoparietal
region.
106. Oral Manifestations
Site—the tongue, soft palate, lips and larynx are
commonly involved.
Appearance—these are characterized by mild
edema, which is followed by atrophy and
induration of mucosal and muscular tissue.
Mask like face—involvement of facial skin
results in characteristic smooth, taut and
masklike face.
Mouse face—nasal alae may become atrophied
resulting in pinched appearance to the nose
resulting in mouse species.
107. Lips—the lips become thin, rigid and partially
fixed, producing microstomia.
Microstomia—the oral aperture narrows
considerably. Skin folds are lost around the
mouth.
Tobacco pouch mouth or purse string
appearance—it can be seen periorally where
furrow rows radiate from the atrophic vermilion
borders, creating the so-called tobacco pouch
mouth or purse string appearance.
108. Tongue—tongue can become hard and rigid, losing its
mobility and papillary pattern, making speaking and
swallowing difficult. The color of tongue changes to a
livid appearance. In the end stages, the tongue lays as
a stiff, reduced body in the floor of mouth.
Lingual frenum—the lingual frenum, which usually
reflects the first oral change, shortens, becomes
tendinous and finally disappears.
109. Neuropathy—resorption of mandible may lead to pressure
of the inferior alveolar nerve. This will results in
neuropathy.
Dysphasia—involvement of esophagus causes
dysphagia.
Temporomandibular joint—involvement of soft tissues
around the TMJ leads to restricted movement of
mandible, causing a pseudoankylosis.
110. Mandible—when the facial tissues and muscles of
mastication are involved the pressure exerted will
cause resorption of mandible at the attachment of
masseter muscle.
Salivary hypofunction—in some cases salivary
hypofunction can also be present.
111. There may be total paralysis until the nerve re-grows
through the graft, usually in a period of 6 to 15
months. There will be some permanent facial
weakness.
When the portion of the facial nerve nearest the brain
is destroyed by the tumor, a facial reanimation
procedure may be necessary.
Cross-sectional drawings showing the stages of development in a somite. A. Mesoderm cells become epithelial
and are arranged around a small lumen. B. Cells in the ventral and medial walls of the somite lose their epithelial characteristics
and migrate around the neural tube and notochord, and some move into the parietal layer of lateral plate mesoderm. Collectively,
these cells constitute the sclerotome. Cells at the DML and VLL regions of the somite form muscle cell precursors. Cells from
both regions migrate ventral to the dermatome to form the dermomyotome. VLL cells also migrate into the parietal layer of
lateral plate mesoderm across the lateral somitic frontier (green line). In combination, somitic cells and lateral plate mesoderm cells
constitute the abaxial mesodermal domain, while the primaxial mesodermal domain only contains somitic cells (paraxial mesoderm).
C. Together, dermatome cells and the muscle cells that associate with them form the dermomyotome. D. The dermomyotome
begins to differentiate: Myotome cells contribute to primaxial muscles, and dermatome cells form the dermis of the back.
Expression patterns of genes that regulate
somite differentiation. Sonichedgehog (SHH) and noggin,
secreted by the notochord and fl oor plate of the
neural tube, cause the ventral part of the somite to form
sclerotome and to express PAX1, which in turn controls
chondrogenesis and vertebral formation. WNT and low
concentrations of SHH proteins from the dorsal neural
tube activate PAX3, which demarcates the dermatome.
WNT proteins also direct the DML portion of the somite
to form muscle precursor cells and to express the musclespecifi
c gene MYF5. The dermatome portion of the somite
is directed to become dermis by neurotrophin 3 (NT-3)
secreted by the dorsal neural tube. The combined infl uence
of activating WNT proteins and inhibitory BMP4 protein
activates MyoD expression in the Ventrolateral (VLL)
region to create a second group of muscle cell precursors.
It is not the same as Duchenne muscular dystrophy and Becker muscular dystrophy, which affect the lower body.