Download Dual findings: A clinically symptomatic case of trigeminal neuralgia

Journal of Medicine, Radiology, Pathology & Surgery (2016), 3, 25–30
CASE REPORT
Dual findings: A clinically symptomatic case of trigeminal
neuralgia with incidental radiographic finding of
elongated styloid process: A rare case report and review of
literature
Leena James, Tejavathi Nagaraj, Keerthi Irugu, Sumana C. K
Department of Oral Medicine and Radiology, Sri Rajiv Gandhi College of Dental Sciences and Hospital, Cholanagar, Bengaluru, Karnataka, India
Keywords:
Styloid process, tonsillar fossa,
trigeminal nerve
Correspondence:
Dr. Keerthi Irugu, Department of Oral
Medicine and Radiology, Sri Rajiv Gandhi
Dental College and Hospital, Cholanagar,
Bengaluru - 560 032, Karnataka, India.
Phone: +91-916903205.
E-mail: [email protected]
Received: 10th July 2016;
Accepted: 19th August 2016
Abstract
Trigeminal neuralgia (TN) is a severe, short-lasting, unilateral facial pain. The most
common is the classical type which is unrelated to pathology and most commonly
caused by neurovascular compression of the trigeminal nerve, i.e., V cranial nerve root.
Most (>85%) of TN cases are diagnosed as classical TN. In adults, the length of styloid
process is about 25 mm with a tip located between the external and internal carotid
arteries and seen lateral to the pharyngeal wall and the tonsillar fossa. Ossification of
the stylohyoid and stylomandibular ligaments leads to elongation of the styloid process
and causes clinical symptoms. This article describes a treated case of 55-year-old female
patient with clinical symptoms of TN and an incidental radiographic finding of elongated
styloid process.
doi: 10.15713/ins.jmrps.71
Introduction
The IASP defined trigeminal neuralgia (TN) or Tic douloureux
is sudden, usually unilateral, severe, brief, excruciating, recurrent
episodes of pain in one or more divisions of the trigeminal nerve,
i.e., V cranial nerve.[1]
The styloid process is a bony prominence, located immediately
anterior to the stylomastoid foramen, varying from 20 to 25 mm
in length. It is of cylindrical form and extends downward from the
infratemporal surface toward the front, downward and medial
side which narrows toward the tip of process.
Stylopharyngeus muscle gets its attachment medially and
from the posterior side next to styloid process base, stylohyoid
muscle from the posterior side and laterally on the central part of
the process. Styloglossus muscle which begins from the anterior
part of the process directly next to the tip. These muscles are
innervated by glossopharyngeal, hypoglossal, and facial nerve.
The ligament of stylohyoid projects from styloid process tip
to the lesser horn of the hyoid bone and the stylomandibular
ligament which begins beneath the attachment of styloglossus
muscle and ends on the angle of mandible. Some of the studies
showed that a length of over 30 mm is considered elongated and
few others consider 40 mm as elongated. Some rare cases exist
with length of 73 mm.[2,3]
Case Report
A female patient aged 55 years old came to the Department
of Oral Medicine and Maxillofacial Radiology with a chief
complaint of pain and burning sensation in her right facial region
since 3 months. Pain was sudden in onset, sharp, shooting,
intermittent, radiating to the lower lip, aggravated on exposure to
cold and chewing food and she was unable to chew on that side.
Pain lasted for time duration of few seconds to 2 minutes and
relieved on its own. History of 20-30 attacks per day. Pain was
present on the right facial region and it was seems to extending
from preauricular region to the lower lip and also in the vestibular
region. History of disturbed sleep was noted. There was no relief
of pain even after taking analgesic medications.
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James, et al.
A rare case report and review of literature
Medical history disclosed patient was known asthmatic since
10 years and was under medication for the same.
No history of drug allergy and had no adverse habits.
Past dental history disclosed history of extraction 3 months
back in the upper and lower right posterior teeth region due to
pain. The patient also gave history of aggravation of pain after
extraction of teeth.
Extraoral examination revealed the presence of scar in the
infraorbital region as shown in Figure 1.
The patient gave history of scar formation due to posttreatment for pain in her right upper back teeth region in her
village and that area was burnt due to medications placed
extraorally in that region 45 years back.
Intraoral examination of hard tissue revealed suspected
presence of root stumps irt 18.
Missing teeth irt
876| 678
876543
As shown in Figures 2 and 3
Dental caries was seen irt 37 and generalized attrition was
present.
Figure 1: Extraoral examination showing presence of scar in the
infraorbital region
a
b
Figure 2: (a and b) Intraoral Figure of missing teeth irt 16, 17, 18,
26, 27, 28, 43, 44, 45, 46, 47, 48
26
As an investigatory measure, a maxillary nerve block was given
to the patient and the patient was symptom free for sometime
after the nerve block.
With the above findings of unilateral occurrence of pain, age,
triggering factors, sex and clinical history, we gave a provisional
diagnosis of the TN.
Root stumps were not seen in intraoral periapical radiograph
irt 18 as shown in Figure 3.
Orthopentamogram revealed missing teeth
irt Missing teeth irt
876| 678
876543
Presence of significant lengthening of styloid process as
shown in Figure 4
The patient was referred to neurologist to rule out any
pathology and for physician for her overall health status including
her asthmatic condition and ENT consultation was taken.
After considering neurologist, physician, ENT surgeon’s
opinion and no pathology (e.g., vascular compressions, tumors)
was noticed related to it, the patient was advised to take tab
Tegretol with a dose of 400 mg thrice daily for 1 week and
recalled her again.
Figure 3: Intraoral periapical radiograph irt 18
Figure 4: Panaromic radiograph showing missing teeth irt 16,
17, 18, 26, 27, 28, 43, 44, 45, 46, 47, 48 and presence of significant
elongation of styloid process
Journal of Medicine, Radiology, Pathology & Surgery ● Vol. 3:5 ● Sep-Oct 2016
James, et al.
A rare case report and review of literature
After 1 week, she reported us with complete reduction of
pain and slight reduction of burning sensation after taking
medication.
The patient was advised to take gabapentin 400 mg and
nortriptyline hydrochloride 100 mg (Pentanerve nt) along with
tegretol. Patient reported with complete reduction in pain and
burning sensation after 1 month. Patient is being monitored.
Discussion
The trigeminal nerve is the Vth cranial nerve and it is a mixed
nerve with a large sensory root and small motor root. The
sensory root carries information from the unilateral hemiside.
The nerve is divided into three branches, i.e., V1, V2 and V3
which correspond to three different dermatomes. The unilateral
muscles of mastication get its innervation from the motor root.[4]
V1 gives nerve supply to scalp, forehead, and around eye.
The area around cheek gets its nerve innervation from V2. V3
innervates area around jaw as shown in Figure 5.[5]
The sensitive (sensory) portion takes to the central nervous
system, from the skin and mucous membrane of greater portion
of the face, which leads to “TN.”[6]
TN occurs more frequently in the V2 and V 3 divisions
of the trigeminal nerve, so deep and limited to the trigeminal
nerve distribution. The attacks may be accompanied by
salivation, lacrimation, rhinorrhea, congestion of nasal mucosa,
erythematous skin, facial edema, or clonic contraction, ipsilateral
hemifacial spasm and/or the muscle contraction that are acting
on the jaw. It is also characterized by “trigger zones” which are
regions of increased arousal.[7]
The stylohyoid chain components are embryologically
derived from the 1st and 2nd branchial arches in 4 distinct,
i.e., stylohyal, tympanohyal, hypohyal, and ceratohyal segments.
They are derived from Reichert’s cartilages that get ossifies in two
parts. From the tympanohyal and stylohyal segments process of
the styloid emerges, that often fuses at pubertal age. The lesser
horn of the hyoid bone originates from the hypohyal segment.
Joining these two structures, the stylohyoid ligament arises from
the ceratohyal segment.
The elongated process of styloid and ligament of the
stylohyoid have been associated to Eagle’s syndrome. Frommer
identified that the direction and curvature of styloid process were
a more significant factor than its length in causing symptoms.
The symptoms are characterized by foreign body sensation in the
pharynx, which leads to painful and swallowing difficulties and
earache. Other features are vertigo, tinnitus, pain on turning the
head, reduced opening of mandible, dysphonia, carotidynia and
change in voice, hypersalivation, and even altered taste sensation.
Although 4% of people are thought to have a lengthened styloid
process among them only 4-10% is symptomatic.[8]
History of TN
The 1st known description of TN, or a similar condition, was
written in the 2nd century AD by Aretaeus of Cappadocia, a
contemporary of Galen.[9]
Jujani, an 11th century Arab physician in his writings,
mentioned that unilateral facial pain causing spasms and anxiety.
Interestingly, he suggested that the cause of the pain was due to
the proximity of the artery to the nerve.[10]
In 1773, the first full account of TN was published when John
Fothergill presented a paper to the Medical Society of London.
He gave the detailed elaboration of the typical features of the
condition, including paroxysms of unilateral facial pain, evoked
by speaking or eating or touch, starting and ending abruptly, and
associated with anxiety.[9]
Sometime earlier, Nicolaus Andrea had used the term tic
douloureux to describe TN. However, it had been suggested, that
no more than two of the patients he described, in fact, had TN.
Later in the 18th and 19th century, Sporadic observations by
Pujol, Chapman and Tiffany helped to complete the clinical
Figure and differentiate TN from common facial pain conditions
like toothache.
In the early 20th century, Oppenheim mentioned a significant
relation between a demyelinating disease like multiple sclerosis
(MS) and TN and Patrick suggested on its familial incidence.
History of styloid process elongation
In 1652, elongation of the stylohyoid ligament was first reported
by Marchetti. Later in 1937, Eagle reported the first two
symptomatic cases of ossified styloid process.[11]
Epidemiology
Figure 5: Divisions of trigeminal nerve distribution
TN is a rare disease with few reported studies on its prevalence.
The available literature suggests that the prevalence might be
between 0.01% and 0.3% in the general individuals, even though
few other studies suggested that it may be around 12%/1 lakh
persons per annum. This, however, could be due to misdiagnosis.
The females are twice commonly affected than males. TN
can first occur at any age, but in greater than 90% of affected
individuals onset occurs in the 4-5th decades, and maximum
incidence is between 5th and 6th decades.[12]
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James, et al.
Incidence of ossified styloid process
Approximately, 4-10. 3% of symptomatic cases of ossified styloid
process occur in 4% of the general people. It is common in the
third and fourth decade of life. Women are thrice commonly
affected than males. Bilateral occurrence is frequent but does not
always causes bilateral symptoms.[13]
Etiology of TN
The pathophysiological mechanisms of TN are not fully
understood, mostly being related to trigeminal nerve compression
by intracranial peripheral vessels, categorized as idiopathic TN
0. 6 The mechanism responsible for triggering neuralgia would
be the cooccurrence of the degenerative processes of aging
associated with long-standing vascular compression on the
trigeminal nerve posterior root.[14]
TN has an enormous psychological impact, few scholarly
papers highlighted the ways how it can affect the quality of life of
affected individuals.
In 2010, Luna et al. stated that 80-90% of TN may be
idiopathic or triggered by trauma, emotional factors, external
stimuli, and neoplasms.
Siqueira, Nóbrega, Valle et al. (2004)[15] in their study
mentioned that TN may occur as a cause of brain lesions,
arachnoid adhesions, vascular loops or plaques of demyelination
situated at the entrance of the nerve root, which will create
potential ectopic for TN.
Other possible phenomena related to the TN are autosomal
dominant, autosomal recessive, possibly X-linked, as well as
related to senility. The symptomatic causes of TN include
MS, viral infections, tumors, aneurysms, and impaired postdental extractions. The TN symptom may also be related
to maxillofacial trauma, such as facial bone fractures, chiefly
fractures of the zygomaticomaxillary complex, mainly when it
involves the orbital floor causing injury to the infraorbital nerve.
Etiology of elongation of styloid process
1.Gokce et al. reported that ectopic calcification might play a
role for the etiology of it, especially in the individuals with
abnormal vitamin D, calcium and phosphorus metabolism.[7]
2. The calcification of muscles of tendons causes irritation of
the adjacent structures thereby leading to elongation.
3. An association of the abnormal angulation with length of the
styloid process.
A rare case report and review of literature
Duration and periodicity of pain: Each episode of pain
attack can range from a time duration of few seconds to 2 min,
may also extend longer but it may be followed by another
attack rapidly. Around 10-70 attacks can occur in a day in the
affected individuals and in between attacks often a refractory
period can be noticed. Attacks tend to get longer as it progresses.
Spontaneous remission periods may occur, which initially last
for months or years, but over a period of time they become
shorter. A prolonged period of low intensity burning and aching
followed by the sharp shooting pain which may be lasting for
hours is observed in “TN associated persistent facial pain.”
Factors affecting it: Innocuous stimuli of light can arouse
pain on the affected side.
Other associated features: The autonomic symptoms are
seen very rarely, and sometimes the sensory changes may be
observed D. The most common leading consequences are
anxiety, depression and deteriorated quality of life and can
resolve if there is no associated attack of pain. Symptomatic TN
can present in a similar way to the above-mentioned features, and
some affected individuals will even have periods of remission.
In the present case, few features of Symptomatic TN were
noted like unilateral occurrence of pain, shooting or sharp in
intensity, attack of pain ranging from a time duration of few
seconds to 2 minutes, 25-30 attacks in a day, stimuli of cold and
chewing food on that side were the triggering factors.
Classification
The revised International classification of headache disorders -3
suggest three variants of TN:[16]
1. Classical TN (CTN)
2. Symptomatic TN and
3. TN with concomitant persistent facial pain.
Langlis classification of styloid process ossification as:
1. Type I: Which is uninterrupted
2. Type II: Which is a pseudo-articulation between process of
styloid and ligament of stylohyoid
3. Type III: In which there are interrupted segments of the
mineralized ligament, creating the appearance of multiple
pseudo-articulations.[17]
Classification is shown in Figure 6.
Clinical features
Location, radiation: V2 and V3 divisions are the most commonly
affected in TN. The pain is mostly unilateral, with only 3% of
bilateral occurrence, and it is of little radiating in nature outside
the distribution of trigeminal nerve.
Character: It is of sharp, shooting, Electric shock-like or
stabbing in intensity.
Severity: Mostly moderate to severe but can be milder at
times.
28
Figure 6: Langlis radiological classification of elongated styloid
process
Journal of Medicine, Radiology, Pathology & Surgery ● Vol. 3:5 ● Sep-Oct 2016
James, et al.
A rare case report and review of literature
Diagnostic criteria for classic TN
• Each attack of pain can range from a time duration of few
seconds to 2 minutes 1
• Pain should have at least one of the following features such as
intense, superficial, sharp or stabbing in character and should
provoke from trigger zones or by trigger factors
• Attacks of pain are similar in individual patients
• Clinically no evident neurological deficit
• Another disorder should not attribute to it.
Olesen (1997) criteria are similar to above mentioned
diagnostic criteria for CTN. TN is mainly diagnosed based on
clinical signs and symptoms mentioned by the patient as typical
paroxysms, refractory periods and trigger zones.[14]
Diagnosis of elongated process of styloid
It must be diagnosed on thorough medical history, and
proper physical examination which includes careful intraoral
palpation, i.e., by inserting the index finger in the tonsillar
fossa region and gentle pressure is applied. By doing the
clinician should be able to feel an elongated process of styloid.
The diagnosis is very likely if pain occurred by palpation and
referred to the ear, face, or head. It is usually not palpable if it
is of normal length. One of the useful diagnostic tools is relief
of pain after injecting local anesthetic solution into tonsillar
fossa.[17]
• Radiological diagnosis
• Panoramic radiographs
• Soft tissue lateral radiograph of the neck
• Towne’s view of the skull
• CT scan.[18]
Differential diagnosis of TN
Dental infection/cracked tooth Well localized to tooth;
local swelling and erythema; appropriate finding on dental
examinations or X-ray. Temporomandibular joint pain often
bilateral and may radiate around ear, neck and temples; jaw
opening may be limited and “click” can be heard.
Persistant idiopathic facial pain (odontalgia)
Often bilateral and may extend out of trigeminal territory;
pain often continuous, mild to moderate in severity, aching or
throbbing in nature.
Temporal arteritis
Common in elderly; pain constant, a/w jaw claudication,
fever. Temporal arteritis may be firm, tender, non-pulsatile on
palpation. Sinusitis facial tenderness. Aching increasing with
position. Acute ear infections A/w inflammatory changes in the
ear Migraine Aura; severe unilateral a/w nausea, photophobia,
phonophobia, and neck stiffness.[1]
Differential diagnosis of lengthened process of styloid
Laryngopharyngeal dysesthesia as well as dental
malocclusion, glossopharyngeal, sphenopalatine, and TN’s,
temporomandibular arthritis, temporal arteritis, diverticulum
of esophagus, cervical vertebral arthritis, malignant or benign
tumors, and cluster type headache and migraine has to be
considered.
Investigations
• No specific investigations exist for the diagnosis of TN
• Dental X-rays can be done to clarify differential diagnosis
• Visual analog scale to rate pain score.
MRI and Angiography is the best option to image trigeminal
nerve and associated vascular malformations and diagnose the
presence of tumors or cysts, plaques of MS and trigeminal nerve
compressions.
Treatment
Medical treatment is mainly based on the use of antiepileptic
medications. Based on present evidence-based guidelines, the
first-line of choice is carbamazepine (200-1200 mg/day) and
oxcarbazepine (600-1800 mg/day). The second-line therapy
includes add-on therapy with lamotrigine (400 mg/day) or a switch
to lamotrigine or baclofen (40-80 mg/day). Other antiepileptic
drugs - such as gabapentin, phenytoin, valproate, and
pregabalin - are also been suggested to be useful in the treatment.
In the case of emergency, an IV infusion of fosphenytoin, as
well as injections of lidocaine locally into trigger points can be
helpful.[19]
Surgical treatment
Surgery should be considered as the treatment of choice if
successful outcome is not obtained with medical therapy or if
it results in evident deterioration of day-day activities. Surgical
procedures include decompression of the affected nerve/vessel
or destruction of the Gasserian ganglion.
This modality provides the best pain relief, with results
showing initial pain relief in 90% of affected individuals, >80%
were pain free after 1 year and 75% were pain free after 3 years.
Up to 4% of affected individuals showed important side effects
such as cerebrospinal fluid leakage, aseptic meningitis, infarcts
or hematomas.[12]
Radiofrequency thermocoagulation, balloon compression,
and percutaneous glycerol rhizolysis are the gasserian ganglion
percutaneous techniques. 90% of patients reported relief of
pain following these procedures. The follow-up results were
1 year after radiofrequency thermocoagulation technique, 6885% were free from pain, after 3 years 54-64% were pain free,
and after 5 years only 50% were pain free. Gasserian ganglion
level surgery needs short-acting anesthetics and mainly
includes minor overnight procedures with a very low rate of
mortality.
In gamma knife surgery in which a focused beam of radiation
is passed at the trigeminal root located in the posterior fossa.
It is an optional treatment for patients who are unfit for open
surgery or those patients who are on coagulants (e.g., patients on
warfarin).
Journal of Medicine, Radiology, Pathology & Surgery ● Vol. 3:5 ● Sep-Oct 201629
James, et al.
Treatment of ossified process of styloid
Conservative or surgical approaches are used for the treatment
of ossified process of styloid. Conservative approaches consist
of transpharyngeal injection of corticosteroids and lignocaine,
diazepam, NSAIDS, heat fomentation. The surgical approach
includes manual fracturing of the ossified styloid process.[20]
Surgical shortening of the ossified styloid process either
through intraoral or external approaches is considered as most
satisfactory treatment outcome. Among intraoral or external
approach, the later has the significant advantage of enhanced
exposure of the process of styloid and the adjacent vital
structures, also helps in performing the surgical resection of a
partially elongated stylohyoid ligament. It. Transoral resection
does not lead to obvious outside scars, but it has the disadvantage
of the risk of deep cervical infection and associated neurovascular
injury.[8,21]
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How to cite this article: James L, Nagaraj T, Irugu K,
Sumana CK. Dual findings: A clinically symptomatic case of
trigeminal neuralgia with incidental radiographic finding of
elongated styloid process: A rare case report and review of
literature. J Med Radiol Pathol Surg 2016;3:25-30.
Journal of Medicine, Radiology, Pathology & Surgery ● Vol. 3:5 ● Sep-Oct 2016