Trigeminal Nerve Anatomy
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The trigeminal nerve is the largest and most complex of the 12 cranial nerves (CNs). It supplies sensations to the face, mucous membranes, and other structures of the head. It is the motor nerve for the muscles of mastication and contains proprioceptive fibers. It exits the brain by a large sensory root and a smaller motor root coming out of the pons at its junction with the middle cerebral peduncle. It passes laterally to join the gasserian (semilunar) ganglion in the Meckel cave. (See the image below.)
The sensory nucleus, located in the pons, is quite extensive. It receives ordinary sensations from the main 3 branches of the trigeminal. The ophthalmic division is in the lower part of the nucleus, and the mandibular branch is in the upper part. The large rostral head is the main sensory nucleus. The caudal tapered part is the spinal tract, which is continuous with substantia gelatinosa of Rolando in the spinal cord. The spinal tract is the sensory nucleus, primarily for pain and temperature. The main sensory nucleus serves mostly for discrimination sense. [1, 2, 3, 4, 5]
The motor nucleus is ventromedial to the sensory nucleus. It lies near the lateral angle of the fourth ventricle in the rostral part of the pons. The mesencephalic nucleus is in the midbrain and receives proprioceptive fibers from all muscles of mastication.
The main sensory nucleus receives its afferents (as the sensory root) from the semilunar ganglion through the lateral part of the pons ventral surface. Its axons cross to the other side, ascending to the thalamic nuclei to relay in the postcentral cerebral cortex. The descending sensory fibers from the semilunar ganglion course through the pons and medulla in the spinal tract of CN V to end in the nuclei of this tract (as far as the second cervical segment). (See tables 1 and 2, below.)
The axons of these nuclei cross to the opposite side, ascending in the spinothalamic tract, to relay in the thalamic nuclei; from there, they end in the cerebral cortex. The sensory nucleus of CN V is connected to other motor nuclei of the pons and medulla. In addition, the descending sensory spinal tract receives somatic sensory fibers from CNs VII, IX, and X.
The proprioceptive fibers of CN V arise from the muscles of mastication and the extraocular muscles. They terminate in the mesencephalic nucleus. This nucleus has connections to the motor nucleus of CN V.
The motor nucleus of CN V receives cortical fibers for voluntary control of the muscles of mastication. These fibers are mostly crossed. It also receives input from the mesencephalic and sensory nuclei. The axons emerge anterior to the sensory root from the lateral surface of the pons. This motor root joins the semilunar ganglion together with the sensory root.
The semilunar (gasserian or trigeminal) ganglion is the great sensory ganglion of CN V. It contains the sensory cell bodies of the 3 branches of the trigeminal nerve (the ophthalmic, mandibular, and maxillary divisions). The ophthalmic and maxillary nerves are purely sensory. The mandibular nerve has sensory and motor functions.
The gasserian ganglion lies in a depression on the petrous apex, within a dural fold called the Meckel cave. The sensory roots of the 3 branches of CN V are received anteriorly. They then pass from the posterior aspect of the ganglion to the pons. The motor root passes under the ganglion to join the sensory division of the mandibular nerve and exits the skull through foramen ovale. The carotid plexus contributes sympathetic fibers to the gasserian ganglion.
Burkett et al successfully visualized trigeminal fibers entering the pons at the nerve root entry zone (NREZ) and descending through the spinal trigeminal tract using robust diffusion-tensor imaging (DTI). Such protocols contribute to our understanding of the anatomical distribution within the brainstem and is a potentially new neurosurgical planning tool. [6]
Table 1. Summary of the Components, Function, Central Connections, Cell Bodies, and Peripheral Distribution of CN V. (Open Table in a new window)
Components
Function
Central connection
Cell bodies
Peripheral distribution
Afferent general somatic
General sensibility
Sensory nucleus V
Gasserian ganglion
Sensory branches of the ophthalmic, maxillary, and mandibular nerves to skin, mucous membranes of the face and head
Efferent special visceral
Mastication
Motor nucleus V
Motor nucleus V
Branches to temporalis, masseter, pterygoids, mylohyoid, tensor tympani, and palati
Afferent proprioceptive
Muscular sensibility
Mesencephalic nucleus V
Mesencephalic nucleus V
Sensory endings in muscles of mastication
Table 2. Summary of the Types of Fibers, Function, and Pathways of the Trigeminal Nerve. (Open Table in a new window)
Type
Function
Pathway
Branchial motor
Motor to muscles of mastication
CN V innervates the muscles of mastication, mylohyoid, tensor tympani, tensor veli palate, anterior belly of digastric
General sensory
Sensory from surface of head and neck, sinuses, meninges and TM
The Gasserian ganglion receives the ophthalmic, maxillary and mandibular divisions of CN V and sympathetic fibers from the carotid plexus and sends branches to the dura. The four accessory ganglia are anatomically but not functionally associated with CN V
The ophthalmic, maxillary, and mandibular branches of the trigeminal nerve leave the skull through 3 separate foramina: the superior orbital fissure, the foramen rotundum, and the foramen ovale, respectively. (See the image below.)
The ophthalmic nerve is the first branch of the trigeminal nerve. It arises from the convex surface of the gasserian ganglion, in the dura of the lateral wall of the cavernous venous sinus under CN IV and above the maxillary nerve, as seen in the image below.
The ophthalmic nerve carries sensory information from the scalp and forehead, the upper eyelid, the conjunctiva and cornea of the eye, the nose (including the tip of the nose, except alae nasi), the nasal mucosa, the frontal sinuses, and parts of the meninges (the dura and blood vessels).
The ophthalmic nerve receives sympathetic filaments from the cavernous sinus and communicating branches from CN III and IV. Just before it exits the skull through the superior orbital fissure, it gives off a dural branch, and then divides into 3 branches: the frontal, lacrimal, and nasociliary. (See the image below.)
Frontal nerve
This is the largest branch of the ophthalmic nerve (see Table 3, below). It passes in the lateral part of the superior orbital fissure, below the lacrimal nerve and above CN IV, between the periorbita and levator palpebrae superioris. It divides in the middle of the orbit into the supraorbital (larger branch) and supratrochlear nerves.
Table 3. The Ophthalmic Nerve Branches and Distribution. (Open Table in a new window)
Nerve
Branches
Distribution
Frontal nerve
• Supraorbital nerve
• Supratrochlear nerve
• Upper lid, frontalis muscle, scalp
• Conjunctiva, upper lid, forehead
Lacrimal nerve
Receives branch from the zygomatic nerve of the maxillary
Lacrimal gland, conjunctiva, upper lid
Nasociliary nerve
• Anterior ethmoid nerve
• Branches to ciliary ganglion
• Posterior ethmoid nerve
• 2-3 long ciliary nerves
• Frontal, anterior, ethmoid sinuses
• Anterior septum, nasal wall
• Cornea, iris, ciliary body
• Posterior ethmoid sphenoid sinuses
• Eye
The supraorbital nerve exits the skull through the supraorbital notch (or foramen). It supplies the upper lid and then turns superiorly under the frontalis muscle to supply the scalp (via lateral and medial branches) as far posteriorly as the lambdoid suture.
The supratrochlear nerve exits the medial orbit and gives branches to the conjunctiva and the skin of the upper lid, as well as to the lower and medial parts of the forehead. The branch to the frontal sinus pierces it in the supraorbital notch to supply the frontal sinus mucosa.
Lacrimal nerve
The lacrimal nerve arises in the narrow, lateral part of the superior orbital fissure and courses between the lateral rectus and the periorbita. It supplies the lacrimal gland, conjunctiva, and upper lid. In the orbit, it receives a communication from the zygomatic branch of the maxillary nerve. This represents postganglionic parasympathetic secretory fibers from the sphenopalatine ganglion to the lacrimal gland. The preganglionic fibers reach the ganglion via the greater petrosal and vidian nerves from CN VII.
Nasociliary nerve
After passing through the superior orbital fissure, the nasociliary nerve gives origin to the anterior ethmoid nerve that passes to the anterior ethmoid foramen lateral to the crista galli, to supply the fontal and anterior ethmoid sinuses. After dropping in the nose, it supplies the anterior part of the septum and lateral nasal wall. After emerging from the nose as the external nasal nerve, it supplies the skin of the nasal tip.
The nasociliary nerve gives a branch to the ciliary ganglion that passes without synapsing to the cornea, iris, and ciliary body. The posterior ethmoid nerves are given off before the anterior ethmoid and supply the posterior ethmoid and sphenoid sinuses. The nasociliary nerve gives off 2-3 long ciliary nerves that enter the globe with the short ciliary nerves of the ciliary ganglion.
The maxillary nerve carries sensory information from the lower eyelid and cheek, the nares and upper lip, the upper teeth and gums, the nasal mucosa, the palate and roof of the pharynx, the maxillary, ethmoid and sphenoid sinuses, and parts of the meninges. (See the image below.) The maxillary nerve is divided into 3 branches: the zygomatic, pterygopalatine (or sphenopalatine), and posterior superior alveolar nerves.
As it leaves the semilunar ganglion, the maxillary nerve passes through the dura of the lateral wall of the cavernous sinus. It exits the skull via the foramen rotundum and crosses the pterygopalatine fossa to enter the orbit through the inferior orbital fissure, where it becomes the infraorbital nerve. Before entering the foramen, it gives off a dural branch (middle meningeal nerve). The zygomatic, pterygopalatine (or sphenopalatine) and posterior superior alveolar branches are given off in the pterygopalatine fossa.
The zygomatic branch divides into the zygomaticotemporal and zygomaticofacial nerves.
In the lateral wall of the orbit, it gives off a branch to the lacrimal nerve, which carries postganglionic fibers from the sphenopalatine ganglion for lacrimation. The zygomaticofacial is inferiorly situated and supplies the skin of the cheek.
The pterygopalatine (or sphenopalatine) nerves are 2 nerves that unite the sphenopalatine ganglion to the maxillary nerve. They transmit afferent sensations from the nose, palate, and pharynx. They also carry parasympathetic fibers to the lacrimal nerve that go to the lacrimal gland. These preganglionic fibers are derived from CN VII via the greater petrosal and vidian nerves. The other branches of the sphenopalatine nerves and their distribution are summarized in Table 4, below.
Table 4. The Maxillary Nerve Branches and Distribution. (Open Table in a new window)
Nerve
Branches
Distribution
Middle meningeal nerve
Dura
Zygomatic nerve
• Zygomatico-temporal
• Zygomatico-facial
• Lacrimal gland
• Forehead
• Cheek
Pterygopalatine nerve
• 2 branches unite sphenopalatine ganglion and maxillary nerve
• Greater palatine nerve
• Posterior superior nasal nerve
• Pharyngeal
• Nasal cavity, pharynx, palate
• Soft and hard palate
• Superior, middle turbinate, septum
• Nasopharynx
Posterior superior alveolar nerve
• Middle, anterior, superior alveolar, and nasal nerves
• Gums, posterior cheek, teeth (canine, incisors, premolar), nasal floor
The posterior superior alveolar nerves are usually 2 in number. They supply the mucosa of the posterior cheek and gingiva; Table 4 has their distribution and the other small branches.
The mandibular nerve is the largest branch of the trigeminal nerve, as seen in the image below. It has mixed sensory and motor fibers (see Table 5, below).
The mandibular nerve carries sensory information from the lower lip, the lower teeth, gums, the chin and jaw (except the angle of the mandible, which is supplied by C2-C3), parts of the external ear, and parts of the meninges. The mandibular nerve carries touch/position and pain/temperature sensations from the mouth. It does not carry taste sensation (the chorda tympani is responsible for taste), but one of its branches, the lingual nerve, carries multiple types of nerve fibers that do not originate in the mandibular nerve.
Motor branches of the trigeminal nerve are distributed in the mandibular nerve. These fibers originate in the motor nucleus of the fifth nerve, which is located near the main trigeminal nucleus in the pons. (See the image below.)
The mandibular nerve has the following 9 branches:
Recurrent meningeal nerve – This nerve enters the skull via the foramen spinosum with the meningeal artery
Medial pterygoid nerve – After passing through the otic ganglion without synapsing, this nerve supplies the medial pterygoid, tensor veli palatini, and tensor tympani muscles
Masseteric nerve – This nerve passes through the mandibular notch to innervate the masseter muscle and temporomandibular joint (TMJ)
Deep temporal nerves – The anterior and posterior branches supply the temporal muscle
Lateral pterygoid nerve
Buccal nerve – This nerve divides into the temporal and buccinator branches
Auriculotemporal nerve – This nerve begins as 2 roots that encircle the middle meningeal artery, then forms a single trunk medial to the neck of the mandible; it emerges superficially between the ear and the mandibular condyle deep to the parotid gland and ends in 2 superficial temporal branches (for autonomic supply to the parotid gland, see below)
Lingual nerve – This nerve runs parallel to the inferior alveolar nerve, is joined by the chorda tympani nerve of the facial nerve (CN VII) near the internal maxillary artery, courses forward between the hyoglossus muscle and the deep part of the submandibular gland, and, as it passes forward, crosses the submandibular (Wharton) duct; the lingual nerve could be injured in this location during surgery on the floor of mouth or during excision of the submandibular gland (for more details regarding the nerve supply of the salivary glands, see below)
Inferior alveolar nerve – This nerve accompanies the inferior alveolar artery in the mandibular foramen and courses into the mandibular canal to exit through the mental foramen; the different branches are listed in Table 5, below
Table 5. Mandibular Nerve Branches and Distribution. (Open Table in a new window)
Nerve
Branches
Distribution
Recurrent meningeal
Dura
Medial pterygoid
Medial pterygoid, tensor veli palatini, tensor tympani muscles
Masseteric
Masseter muscle, temporomandibular joint
Deep temporal (x2)
Temporalis muscle
Lateral pterygoid
Lateral pterygoid muscle
Buccal
• Temporal nerve (upper)
• Buccinator nerve (lower)
Skin of cheek, mucous membrane of mouth, and gingiva
Auriculotemporal
• Communication with facial nerve, and otic ganglion,
• Articular nerve
• Parotid gland
Parasympathetic and sympathetic supply to the parotid gland, after relay in the otic ganglion
8) Lingual
Communicates with CN VII via chorda tympani
Taste sensations to the anterior third of tongue
9) Inferior alveolar
• Mylohyoid
• Dental
• Incisive
• Mental
Mylohyoid, anterior, belly of digastric, molars, premolars, canine, incisors lower lip, and chin
Sensory nerve endings that respond to stimuli and convert them to nervous energy toward the central nervous system are called receptors or central transducers. Sensory receptors are classified into the following 3 main groups: exteroreceptors, interoreceptors, and proprioceptors. [7, 8, 9]
These are stimulated by the external environment. Examples of these types of receptors include the following:
Merkel corpuscles – Located in submucosa of the tongue and oral cavity (see the image below)
Meissner corpuscles – Tactile receptors in the skin
Ruffini corpuscles – Pressure and warmth receptors
Krause corpuscles or end bulbs – Cold receptors
Free nerve endings – Perceive superficial pain and tactile sensations
These are located in and transmit sensations from body cavities. Most of the sensations for these structures deal with body functions and are below the conscious level. Examples include the following:
Pacinian corpuscles – Detect pressure sense
Free nerve endings – Perceive visceral or other sensations
The sensations associated with proprioceptors are also below conscious level; examples include the following:
Muscle spindles – Respond to passive stretch of the muscle
Golgi tendon organs – Located in tendons and respond to muscle tension (contraction and stretching)
Pacinian corpuscles – Respond to pressure
Proprioceptors – Respond to periodontal sensation
Sensory nerve endings – Perceive deep somatic pain
Different anatomic variations have been described regarding the trigeminal nerve, its branches, and its subdivisions. Examples include the very rare occurrence of unilateral trigeminal nerve hypoplasia, in which no corneal sensitivity exists on the affected side and facial sensitivity is reduced in all branches of the trigeminal nerve. Anomalies may coexist also in association with craniofacial anomalies, such as hypoplasia of the trigeminal nerve in Goldenhar syndrome (oculo-auriculo-vertebral dysplasia). A few other examples affecting the different divisions are described below. [10, 11, 12, 13, 14, 15]
A variation has been reported in which the frontal nerve divides at a variable point before leaving the orbit to form the supratrochlear and supraorbital branches. In such cases, the supraorbital branch passes through the supraorbital foramen, through which the undivided nerve ordinarily passes. When the foramen is absent, it may have a special groove, the frontal notch (Henle notch).
The frontal nerve runs, at first forward, in a sagittal direction. In approximately 90% of subjects, it divides during its course within the orbit, but in 10% of persons it remains undivided. It divides into the larger lateral supraorbital nerve and smaller supratrochlear nerve, which runs medially. In 60% of subjects, the supraorbital nerve does not divide, but in 30% it divides into the medial branch, which leaves the orbit through the frontal foramen or notch, and the lateral branch passes out through the frontal foramen. In about 90% of subjects, the supratrochlear nerve runs along the surface of the superior oblique muscle. In 4% of subjects, 2 supratrochlear nerves exist.
This nerve may be limited to the nasal cavity. It may also traverse the posterior ethmoidal foramen to gain entrance to the cranial cavity.
This nerve may appear to be derived from the trochlear nerve. However, the probable source in such cases is the ophthalmic nerve, through its communicating branch to the trochlear nerve (CN IV) in the cavernous sinus.
The lacrimal nerve may be small at its origin, increasing in size later in its course by the addition of fibers derived from the temporal branch of the maxillary division of the trigeminal nerve. The lacrimal nerve may be absent and replaced by the temporal branch of the maxillary division of the trigeminal nerve.
The lacrimal nerve occasionally gives rise to a ciliary nerve, or it receives a branch from a long ciliary nerve of the ciliary ganglion or a branch from the ganglion directly. It may receive accessory roots from the supraorbital or nasociliary nerves.
The bifurcation of the lacrimal into its terminal branches may occur on the posterior wall of the orbital cavity. A branch of the lacrimal has been noted to pierce the sclera.
The lacrimal nerve may exchange fibers with the ciliary ganglion.
Several variations in the branches of this nerve have been reported. The nasociliary nerve may send branches to the superior rectus, medial rectus, and levator palpebral superioris muscles. Branches emanating from a small ganglion connected to the nasal nerve have been followed to the oculomotor (CN III) and abducens (CN VI) nerves.
The infratrochlear branch of the nasal (nasociliary) nerve may be missing, in which case the areas normally supplied by this branch (skin of the upper eyelid, root of nose, conjunctiva, lacrimal sac) receive their supply from the supratrochlear branch of the frontal nerve.
Branches of the nasal nerve have been described passing to the frontal, ethmoid, and sphenoid sinuses. The branches to the frontal and anterior ethmoid sinuses arise in the anterior ethmoid foramen; branches to the sphenoid and posterior ethmoid sinuses arise in the posterior ethmoid foramen. The branches to the sphenoid sinuses are known as sphenoid branches, whereas the branches to the posterior ethmoid sinuses are known as sphenoethmoid or posterior ethmoid branches. An anastomosis between the nasal and lacrimal nerves has been reported.
The maxillary nerve may split into 2 trunks, each entering the skull through a separate foramen
The following variations have been reported in this nerve or its 2 branches (the temporal or facial or malar). The nerve may pass through the zygomatic bone before it divides into 2 branches, or the 2 branches may pass separately through foramina in the zygomatic bone instead of passing through a common foramen (sphenozygomatic foramen). The temporal branch in some cases passes through the sphenomaxillary fissure into the temporal fossa.
Either branch of the zygomatic may be absent or smaller than normal, in which case the other branch compensates by carrying the additional nerve fibers. The area usually supplied by the zygomatic branch (skin of the zygomatic region) may be supplied instead by the infraorbital nerve. The area usually supplied by the temporal branch (skin of the anterior temporal region) may be supplied solely or additionally by the lacrimal nerve.
In the absence of the buccal nerve, the posterior superior alveolar nerve distributes branches to the areas normally supplied by this nerve (mucous membrane and skin of the cheek).
The inferior alveolar nerve may form a single trunk with the lingual nerve, extending as far as the mandibular foramen. The inferior alveolar nerve is sometimes perforated by the internal (medial) maxillary artery. It may have accessory roots from other divisions of the mandibular nerve. In some cases, the mylohyoid branch of the inferior alveolar gives rise to a branch that pierces the mylohyoid muscle and joins the lingual nerve.
Branches have been described arising from the mylohyoid branch and supplying the depressor anguli oris muscle and parts of the platysma (that are usually supplied by the facial nerve), the skin below the chin, and the submandibular (submaxillary) gland (which is usually supplied by the facial nerve). The inferior alveolar may form connections with the auriculotemporal nerve. In one case, the roots of the third lower molar tooth were found to be surrounding the inferior alveolar nerve.
This nerve carries the otic ganglion, which is derived from glossopharyngeal neurons. The nerve usually arises by 2 roots from the posterior division of the mandibular nerve. The 2 roots usually surround the middle meningeal nerve before joining to form a single trunk. A variation in this relationship has been described in which the middle meningeal artery pierces the anterior root instead of passing between the 2 roots.
According to Baumel et al, the auriculotemporal nerve is commonly misrepresented in illustrations and textbooks. [12] Their 85 dissections of the nerve demonstrated that the roots of the “typical” auriculotemporal nerve do not form a tight buttonhole around the middle meningeal artery. Instead, the roots outline an elongated, V-shaped interval, with the roots widely separated from one another. At their junction, the roots form a short trunk that immediately breaks up in line with the posterior border of the mandible into a spray of branches.
The superficial temporal ramus of the auriculotemporal nerve should not be considered as the main continuation of the nerve but merely as its largest branch. A substantial portion of the nerve makes up its 2 communicating rami with the facial nerve; these are the strongest and most consistent of the many peripheral communications between trigeminal and facial nerves. Common variations in configuration, branching, and relationships of the nerve are discussed in the report by Baumel et al.
A minute sublingual ganglion has been described arising from the lingual nerve or submandibular ganglion (a ganglion of the facial nerve carried by the lingual nerve), supplying the sublingual gland. This nerve may pierce the lateral pterygoid muscle rather than pass between the 2 pterygoid muscles. It occasionally provides motor branches to the medial and lateral pterygoids and to the palatoglossus muscle.
Vascular relationships are important during intracranial approaches to the skull base. The relationship between the superior petrosal sinus (SPS) and the opening of the Meckel cave (MC) was studied by Tubbs et al (2013), who found (through cadaver dissections) 3 types of relationships, as follows [16] :
SPSs traveled superior to the opening of the MC in 68%
SPSs traveled inferior to the opening of the MC in 18%
SPSs traveled around to the opening of the MC in 16% of cadavers
In the third variety, a venous ring was formed around the proximal trigeminal nerve. In these cases, the opening was narrowed on sides found to have an SPS that encircled this region. No statistically significant differences were noted between persons of different sex or age or in regard to the side of the head. They concluded that some individuals may retain the early embryonic position of their SPS in relation to the fifth nerve.
Trigeminal neuralgia and neuropathy are thought to arise from damage or pressure on the trigeminal nerve, whereas temporomandibular disorders (TMDs) result primarily from peripheral nociceptor activation. Wilcox et al (2013) used T1-weighted magnetic resonance images to assess the volume and microstructure of the trigeminal nerve in these 3 conditions. [17] They found that trigeminal neuralgia patients displayed a 47% decrease in nerve volume, but no change on DTI. On the other hand, trigeminal neuropathy patients displayed a 40% increase in nerve volume but no changes in DTI values. In contrast, TMD subjects displayed no change in volume or DTIs. This publication revealed that orofacial pain conditions are associated with changes in nerve volume, whereas nonneuropathic pain is not associated with any volume change.
Ibrahim et al published a study on trigeminal tractotomy that was performed either alone or in conjunction with microvascular decompression. [18] These patients had intractable trigeminal neuralgia unresponsive to previous treatment. Stereotactic neuronavigation was used during surgery to localize the descending tract via a ventral pontine approach.
Shibao et al found that the trigeminocerebellar artery (TCA), which is a branch of the basilar artery, was compressing the medial aspect of the trigeminal nerve in 2 patients. [19] They transposed the TCA loop medially and anteriorly away from the nerve and inserted shredded Teflon between the TCA and the trigeminal nerve. Postoperatively, the patients’ trigeminal neuralgia resolved.
Regarding trigeminal neuralgia (also known as tic douloureux), the differential diagnoses is as follows (also, see Table 6 and text below) [7, 20, 21, 22, 23, 24] :
Cluster headache (CH): The pain and symptoms of CH result from activation of the trigeminal parasympathetic reflex, mediated through the sphenopalatine ganglion (SPG). Schoenen et al (2012) investigated the safety and efficacy of on-demand SPG stimulation for chronic CH (CCH). [25] A multicenter study of an implantable on-demand SPG neurostimulator was conducted in patients suffering from refractory CCH. Most patients (81%) experienced transient, mild/moderate loss of sensation within distinct maxillary nerve regions; 65% of events resolved within 3 months. Results showed that the on-demand SPG stimulation using this neurostimulation system is an effective novel therapy for CCH, with dual beneficial effects, acute pain relief and observed attack prevention, and has an acceptable safety profile compared with similar surgical procedures.
Low-grade astrocytoma
Trigeminal neuropathy
Trigeminal neuritis
Chronic paroxysmal hemicrania
Craniopharyngioma
Persistent idiopathic facial pain
Glioblastoma multiforme
Polyarteritis nodosa
Hemifacial spasm
Postherpetic neuralgia
Subarachnoid hemorrhage
Glossopharyngeal neuralgia
Malignant and nonmalignant pain syndromes
Occipital neuralgia
Tic convulsif
Cerebral aneurysms
Brainstem syndrome
Table 6. The Difference Between Atypical Facial Pain and Trigeminal Neuralgia. (Open Table in a new window)
Feature
Trigeminal Neuralgia
Atypical Facial Pain
Prevalence
Rare
Common
Main location
Trigeminal area
Face, neck, ear
Pain duration
Seconds to 2 minutes
Hours to days
Character
Electric jerks, stabbing
Throbbing, dull
Pain intensity
Severe
Mild to moderate
Provoking factors
Light touch, washing, shaving, eating, talking
Stress, cold
Associated symptoms
None
Sensory abnormalities
TN has a reported incidence of 5.9 cases in 100 000 women and 3.4 cases in 100 000 men in the United States. The exact pathophysiology is still unclear, but demyelization leading to abnormal discharge in fibers of the trigeminal nerve is a probable cause. In most cases, no structural lesion is detected, but in almost 15% of patients, medical imaging methods like MRI, CT, or angiography can identify a vein or artery that compresses the nerve, which results in focal demyelization. Sava et al (2012) investigated a case of TN using MRI and identified compression of the nerve 9 mm after emerging the pons by the superior cerebellar artery. [26] In the article, they reviewed MRI anatomy of the trigeminal nerve.
Marcus Gunn phenomenon (also known as Marcus-Gunn jaw-winking or trigemino-oculomotor synkineses) is an autosomal-dominant condition with incomplete penetrance, in which nursing infants have rhythmic upward jerking of their upper eyelid. This condition has been associated with amblyopia (in 54% of cases), anisometropia (26%), and strabismus (56%).
Marcus Gunn phenomenon is an exaggeration of a very weak physiologic cocontraction that has been disinhibited secondary to a congenital brainstem lesion. The stimulation of the trigeminal nerve by contraction of the pterygoid muscles results in the excitation of the branch of the oculomotor nerve (CN III) that innervates the levator palpebrae superioris ipsilaterally.
Inverse Marcus Gunn phenomenon or Marin-Amat syndrome
Marin-Amat syndrome or inverse Marcus Gunn phenomenon is a rare condition that causes the eyelid to fall upon opening of the mouth. In this case, trigeminal innervation to the pterygoid muscles is associated with an inhibition of the branch of the oculomotor nerve to the levator palpebrae superioris, as opposed to stimulation in Marcus Gunn jaw-winking. Garcia Ron et al (2011) presented one acquired case, after the surgery of tuberculosus cervical adenitis, and another congenital case. The syndrome is rare in children, with few reported cases. [27] The diagnosis is clinical and does not require additional tests. EMG may be useful to demonstrate the synkinesis.
Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. Ophthalmoparesis or disordered eye movements occur when CNs III, IV, and VI are damaged by granulomatous inflammation. Pupillary dysfunction may be present and is related to injury to the sympathetic fibers or oculomotor nerve. Trigeminal nerve involvement (primarily V1) may cause paresthesias of the forehead.
This condition is also called Wallenberg syndrome or posterior inferior cerebellar artery (PICA) syndrome. The PICA supplies the lower cerebellum, the lateral medulla, and the choroid plexus of the fourth ventricle. In lateral medullary syndrome, the patient has dysphagia and/or difficulty speaking owing to 1 or more patches of infarction caused by interrupted blood supply to parts of the brainstem. For features of lateral medullary syndrome, see Table 7, below.
Li et al studied 52 patients with nasopharyngeal carcinoma (NPC) and unilateral involvement of the trigeminal nerve. [28] They demonstrated that DTI can evaluate microstructural abnormalities of the V3 branch of the trigeminal nerve in patients with NPC. This is important for the prognosis and management of these patients.
Table 7. Features of Lateral Medullary Syndrome. (Open Table in a new window)
Dysfunction
Effects
Vestibular nucleus
Vestibular system: vertigo, diplopia, nystagmus, vomiting
Inferior cerebellar peduncle
Ipsilateral cerebellar signs, including ataxia
Central tegmental tract
Palatal myoclonus
Lateral spinothalamic tract
Contralateral deficits in pain and temperature sensation from body
Spinal trigeminal nucleus
Ipsilateral loss of touch pain and temperature sensation from face
Nucleus ambiguus (which affects vagus X and glossopharyngeal nerves IX)
Dysphagia, hoarseness, diminished gag reflex
Descending sympathetic fibers
Ipsilateral Horner syndrome
Four small parasympathetic (accessory) ganglia are associated anatomically (but not functionally) with the branches of the trigeminal nerve. [2, 4] They are as follows:
Ciliary ganglion
Sphenopalatine (or pterygopalatine) ganglion
Otic ganglion
Submandibular ganglion
The ciliary ganglion is associated with the ophthalmic nerve. It is the size of a pinhead and has the following 3 roots:
The parasympathetic root from the nerve to inferior oblique (CN III) from Edinger Westphal nucleus and caudal central nucleus to supply the sphincter papillae and ciliary muscles
Sympathetic root from the nasociliary nerve to dilator papillae muscle of the eye
Sensory root from the nasociliary nerve to the cornea
The sphenopalatine ganglion is associated with the maxillary nerve. It receives its parasympathetic fibers from CN VII (as seen in the image below). The otic and submandibular ganglia are associated with the mandibular nerve. They receive parasympathetic fibers from CNs IX and VII, respectively.
Submandibular gland
Parasympathetic fibers arise from the superior salivary nucleus in the pons. Fibers pass through the facial nerve to the chorda tympani and then to the lingual nerve. Synapsing occurs in the submandibular ganglion and from there to the submandibular salivary gland. Sympathetic supply is from the plexus around the facial artery.
Parotid gland
Parasympathetic fibers arise from the inferior salivary nucleus in the medulla oblongata, pass through the glossopharyngeal nerve (CN IX), and then travel through its tympanic branch to the tympanic plexus (Jacobson nerve). They emerge from the middle ear through a hiatus on the anterior surface of the petrous temporal bone, as the lesser superficial petrosal nerve. This nerve passes via the foramen ovale to the otic ganglion (which hangs from the medial side of the mandibular nerve).
Relay occurs in the otic ganglion, and from there it is distributed to the parotid gland via the auriculotemporal nerve. Sympathetic fibers are from the superior cervical ganglion; they go to the plexus around the meningeal artery and from there to the auriculotemporal nerve, which distributes them to the parotid salivary gland.
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Components
Function
Central connection
Cell bodies
Peripheral distribution
Afferent general somatic
General sensibility
Sensory nucleus V
Gasserian ganglion
Sensory branches of the ophthalmic, maxillary, and mandibular nerves to skin, mucous membranes of the face and head
Efferent special visceral
Mastication
Motor nucleus V
Motor nucleus V
Branches to temporalis, masseter, pterygoids, mylohyoid, tensor tympani, and palati
Afferent proprioceptive
Muscular sensibility
Mesencephalic nucleus V
Mesencephalic nucleus V
Sensory endings in muscles of mastication
Type
Function
Pathway
Branchial motor
Motor to muscles of mastication
CN V innervates the muscles of mastication, mylohyoid, tensor tympani, tensor veli palate, anterior belly of digastric
General sensory
Sensory from surface of head and neck, sinuses, meninges and TM
The Gasserian ganglion receives the ophthalmic, maxillary and mandibular divisions of CN V and sympathetic fibers from the carotid plexus and sends branches to the dura. The four accessory ganglia are anatomically but not functionally associated with CN V
Nerve
Branches
Distribution
Frontal nerve
• Supraorbital nerve
• Supratrochlear nerve
• Upper lid, frontalis muscle, scalp
• Conjunctiva, upper lid, forehead
Lacrimal nerve
Receives branch from the zygomatic nerve of the maxillary
Lacrimal gland, conjunctiva, upper lid
Nasociliary nerve
• Anterior ethmoid nerve
• Branches to ciliary ganglion
• Posterior ethmoid nerve
• 2-3 long ciliary nerves
• Frontal, anterior, ethmoid sinuses
• Anterior septum, nasal wall
• Cornea, iris, ciliary body
• Posterior ethmoid sphenoid sinuses
• Eye
Nerve
Branches
Distribution
Middle meningeal nerve
Dura
Zygomatic nerve
• Zygomatico-temporal
• Zygomatico-facial
• Lacrimal gland
• Forehead
• Cheek
Pterygopalatine nerve
• 2 branches unite sphenopalatine ganglion and maxillary nerve
• Greater palatine nerve
• Posterior superior nasal nerve
• Pharyngeal
• Nasal cavity, pharynx, palate
• Soft and hard palate
• Superior, middle turbinate, septum
• Nasopharynx
Posterior superior alveolar nerve
• Middle, anterior, superior alveolar, and nasal nerves
• Gums, posterior cheek, teeth (canine, incisors, premolar), nasal floor
Nerve
Branches
Distribution
Recurrent meningeal
Dura
Medial pterygoid
Medial pterygoid, tensor veli palatini, tensor tympani muscles
Masseteric
Masseter muscle, temporomandibular joint
Deep temporal (x2)
Temporalis muscle
Lateral pterygoid
Lateral pterygoid muscle
Buccal
• Temporal nerve (upper)
• Buccinator nerve (lower)
Skin of cheek, mucous membrane of mouth, and gingiva
Auriculotemporal
• Communication with facial nerve, and otic ganglion,
• Articular nerve
• Parotid gland
Parasympathetic and sympathetic supply to the parotid gland, after relay in the otic ganglion
8) Lingual
Communicates with CN VII via chorda tympani
Taste sensations to the anterior third of tongue
9) Inferior alveolar
• Mylohyoid
• Dental
• Incisive
• Mental
Mylohyoid, anterior, belly of digastric, molars, premolars, canine, incisors lower lip, and chin
Feature
Trigeminal Neuralgia
Atypical Facial Pain
Prevalence
Rare
Common
Main location
Trigeminal area
Face, neck, ear
Pain duration
Seconds to 2 minutes
Hours to days
Character
Electric jerks, stabbing
Throbbing, dull
Pain intensity
Severe
Mild to moderate
Provoking factors
Light touch, washing, shaving, eating, talking
Stress, cold
Associated symptoms
None
Sensory abnormalities
Dysfunction
Effects
Vestibular nucleus
Vestibular system: vertigo, diplopia, nystagmus, vomiting
Inferior cerebellar peduncle
Ipsilateral cerebellar signs, including ataxia
Central tegmental tract
Palatal myoclonus
Lateral spinothalamic tract
Contralateral deficits in pain and temperature sensation from body
Spinal trigeminal nucleus
Ipsilateral loss of touch pain and temperature sensation from face
Nucleus ambiguus (which affects vagus X and glossopharyngeal nerves IX)
Dysphagia, hoarseness, diminished gag reflex
Descending sympathetic fibers
Ipsilateral Horner syndrome
Ted L Tewfik, MD Professor of Otolaryngology-Head and Neck Surgery, Professor of Pediatric Surgery, McGill University Faculty of Medicine; Senior Staff, Montreal Children’s Hospital, Montreal General Hospital, and Royal Victoria Hospital
Ted L Tewfik, MD is a member of the following medical societies: American Society of Pediatric Otolaryngology, Canadian Society of Otolaryngology-Head & Neck Surgery
Disclosure: Nothing to disclose.
Arlen D Meyers, MD, MBA Professor of Otolaryngology, Dentistry, and Engineering, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Head and Neck Society
Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cerescan;RxRevu;Cliexa;Preacute Population Health Management;The Physicians Edge<br/>Received income in an amount equal to or greater than $250 from: The Physicians Edge, Cliexa<br/> Received stock from RxRevu; Received ownership interest from Cerescan for consulting; for: Rxblockchain;Bridge Health.
Trigeminal Nerve Anatomy
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