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11 Articles in Volume 7, Issue #9
CES in the Treatment of Addictions: A Review and Meta-Analysis
Chronic Cancer Pain Management
Compliant Billing, Coding and Documentation for Interventional Pain Management
Critical Transition from Short-to-Long-Acting Opioid Therapy
Dextrose Prolotherapy and Pain of Chronic TMJ Dysfunction
Dysfunction and Rehabilitation of the Shoulder
Low Level Laser Therapy (LLLT) - Part 2
Placebos in Pain Management
The Good Patient: Responsibilities and Obligations of the Patient-physician Relationship
TMJ Derangement and SUNCT Syndrome Co-morbidity
Ziconotide Combination Intrathecal Therapy

TMJ Derangement and SUNCT Syndrome Co-morbidity

Case report of signs and symptoms of internal derangement of the temporomandibular joint (TMJ) co-morbid with short-lasting unilateral neuralgiform headache attacks from conjunctival injection and tearing (SUNCT) syndrome.

The patient who suffers head and or neck pain is often a complex pain patient, meaning that the suffering can be the result of multiple, often unrelated conditions. It is also common for an ear, nose and throat specialist to examine a patient with headache and ear-related complaints that are often determined to be of TMJ or TMJ/Bite origin. Complaints related to the Ear/TMJ/Bite are based on first branchial arch embryology and proximity, common neural innervation, a ligamentous physical connection from mandible to TMJ and to the middle ear cavity. The term ‘Ear/TMJ/Bite’ simply ascribes a relationship that can influence many areas in the head and neck. The “bite” aspect of the term describes the influence of the upper and lower teeth. This influence includes the “fit” of the upper and lower teeth and whether that fit helps to support or hinder the articulation of the jaw joint and thus its stability.

In fact, it is accurate to describe each tooth in the maxillary bone and in the mandible to also be a “joint.” In the human adult, anywhere from 28 to 32 teeth normally play a pivotal role in the health and or disease of the TMJ—that is, excluding extrensic trauma or developmental abnormalities. Each tooth is normally suspended in its bony socket by a series of many fine ligaments that allow for minor lateral movement. It is this ligamentous connection of tooth to bone that often plays a contributing role in TMJ arthrokinesis.

Arthrokinesis is the ligamento-neuro-muscular reflex mechanism that allows a movable joint anywhere in the body to work either effectively or to exhibit some degree of dysfunction, injury, or disease. Since the primary role of a ligament is to protect its companion joint, the 28 to 32 normally occurring teeth with numerous suspensory ligaments represent a complex portion of the ligamentous structures that are responsible for good mandibular joint function and arthrokinesis. The remaining non-tooth ligaments that are connected to the mandible include the stylomandibular, sphenomandibular, lateral capsular ligament, and the temporomandibular ligament (often blended in the capsular ligament). In arthrokinesis, it is the ligament—via its mechano-receptors—that directs spinal and CNS activity that serves to protect as well as signal joint-related injury. Joint-related injury often results in muscular reflex restriction and pain, and the pain often includes pain referral to distant sites.

An injury to a tooth affecting its position or height, maloccluded teeth, tooth extraction and progressive dental instability, extrensic injury to the muscles or ligaments that move the joint, or internal derangement within the joint can all alter normal joint arthrokinesis. The result can be trismus or muscle spasm, slipping or locking of the disc in the jaw joint, degeneration or perforation of disc and disc ligaments, spurring or degenerative changes within the TM joint, ear pain, headache, and pain of the axial muscles of the neck. The affected axial muscles influenced by the TMJ and muscles of mastication have been demonstrated by Mehta (at Tufts University School of Dental Medicine)1 to include the trapezius, and sternocleidomastoid muscles.

A slipped TMJ articular disc carries with it a potential host of nociceptive, neural, vascular, autonomic, and head and neck postural changes with accompanying painful symptoms. The autonomic influence is thought to be affected by the post-ganglionic, parasympathetic fibers that invest the lateral capsular ligament of the temporomandibular joint. Research has shown there is a physical connection between the articular disc and the lateral capsular ligament.

The trigeminal nerve is the dominant sensory/motor nerve in the region with interconnects to facial nerve branches, occipital nerve branches, and glossopharyngeal nerves. Equally significant is that trigeminal nerve cells in the trigeminal spinal nucleus interface with spinal nerve cells in the first three cervical vertebrae. Additionally, recent EMG research is demonstrating that postural stability of the head on the neck can be influenced by a TMJ problem or, conversely, the TMJ can be affected by changes in the axial cervical structures.1 Clearly there is an abundance of complexity.

A neurologic condition (SUNCT syndrome) that may be caused, affected, or influenced by a TMJ internal derangement will be seen in the following case report. As a reminder, this case report is not intended to be a proof of cause and effect. However, since “observation” is the first of the five steps in the scientific method, this case is worth considering as having a possible cause and effect. Whether this case of suspected association between the TMJ slipped disc and SUNCT will be reproduced in the future is yet to be determined. Whether it is or not, at least this case’s treatment will stand as having been therapeutic for this particular patient.

Short-lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT)

While TMJ has been discussed at some length, more needs to be disclosed concerning the nature of SUNCT syndrome and its painful symptoms. SUNCT is classified as one of a number of paroximal trigeminal autonomic hemicranias (TAC’s) that can occur with autonomic features. The attacks occur in spasms or bursts of paroxysmal pain. The condition is noted to occur most commonly in men over age 50, though women too may suffer from this condition. The attacks are described as being marked by rapid bursts of moderate to very severe burning, stabbing, or throbbing pain, mostly on one side of the head, around the eye or the temple. Some cases also report eye lid drooping or ptosis during the episodes.2

The literature reports the attacks typically occurring in the daytime hours and lasting from five seconds to four to five minutes. Frequency of attacks is generally seen to be five or six per hour. The National Institutes of Health report these autonomic responses include:

watery eyes, reddish or bloodshot eyes caused by dilation of blood vessels, nasal congestion, runny nose, sweaty forehead, swelling of the eyelids, and increased pressure within the eye on the affected side of head. Systolic blood pressure may rise during an attack. Movement of the neck may trigger these headaches... SUNCT may be a form of trigeminal neuralgia and is considered one of the trigeminal autonomic cephalgias, or TACs.4

NIH further states that there is currently no cure for SUNCT.3
Oxford University’s Institute of Neurology divides the short lasting headaches into those with autonomic activation (SUNCT) and those without autonomic activation. The cases with autonomic activation have an attack frequency from five to forty attacks a day. The pain is a severe pain and has autonomic symptoms such as conjunctivaal injection, lacrimation, nasal congestion, rhinorrhea, as well as ptosis or eyelid edema. Some cases respond to indomethacin and other medications, including IV Lidocaine. Currently there is no medication that has been reported to cure the attacks. Some researchers believe that the similarity of the symptoms of both autonomic and non-autonomic activation may suggest a shared pathophysiology.

There is a report in the literature of SUNCT occurring from a pituitary adenoma, or prolactinoma.4 In that case, the administration of a dopamine agonist led to a cessation of attacks. This report highlights the necessity of excluding a diagnosis of pituitary adenoma and posterior fossa abnormalities in all cases thought to be SUNCT. This would include a review of symptoms associated with pituitary masses or neoplasms, magnetic resonance imaging of the brain and pituitary structure, as well as screening for serum hormone changes. In my exam, the findings initially suggested some elements of a partial Horner’s syndrome5 and this was confirmed by a neurologist. Horner’s syndrome should also be included in the differential diagnosis of SUNCT-related conditions such as Red Ear Syndrome.

Case Report

In the case that follows, the patient had been suffering from SUNCT syndrome for thirteen months when first examined at our clinic. After the symptoms of SUNCT had started, the patient fell on ice during the middle of last winter and felt even worse. My examination revealed a thirty-two year-old female, married with two young children, and who was trained and employed as a speech pathologist. She had normal vital signs except for heart rate which was 112. In the four days I saw the patient, the heart rate did not fall below 109. The only medication being taken was for a long-standing migraine condition, but she had tried indomethycin with no benefit. Previous surgery included breast biopsy for benign mass.

This patient presented with diffuse pain of the head and neck at the examination and listed her chief complaints in decreasing order as follows:

  1. Headaches, head pain, jaw pain(right side)
  2. Ear and eye pain
  3. Facial pain
  4. Neck pain
  5. Pain on swallowing

In the first eight months from onset of SUNCT, there was shooting, burning and stabbing pain occurring daily. Initially, shooting and stabbing pains were common at the jaw joint and face. The shooting and stabbing pain seems to go in cycles, often daily and then the shooting and stabbing pain would change to an occasional frequency.

My exam did reveal conjunctival injection and tearing of the right side of the head and face. The right upper eyelid drooped as well, though no miosis or anhydrosis. The TMJ range of movement was restricted at 22 mm max opening (normal range of vertical opening was 45-55 mm). The lower jaw, upon opening, deviated 3 mm to the right, suggesting an internal derangement of the right jaw joint. The derangement was classified as a disc dislocation, meaning the disc did not reduce or go back into normal position on the head of the mandibular condyle at any point in the opening movement (see Figure 1). This is usually an acutely painful condition.

Figure 1. Depiction of TMJ disc dislocation and disturbed arthrokinesis.

The following conditions were diagnosed on the right side:

  1. SUNCT Syndrome
  2. Dislocated TMJ articular disc
  3. Temporal tendonitis at coronoid process of mandible
  4. Tendinosis of splenius capitis muscle at superior nuchal line
  5. Horner’s Syndrome

Treatment

I discussed with the patient that she was suffering from a possible list of conditions. The first one that we should try to correct was the dislocated or locked or slipped disc in the right jaw joint. She agreed and Gunn’s Law described in orthopedic medicine was reviewed with the patient and an effort at closed reduction of the disc was successful. The jaw joint pain stopped with the reduction of the slipped disc, and the maximum opening of the jaw increased to 47 mm. An acrylic splint was made for the patient to wear and she was scheduled to be seen again the next day. The day after the reduction, the patient felt much better. Right eyelid drooped once that morning but stopped soon thereafter. As of this writing (November 9, 2007) and sixty days following initial exam and disc reduction, there has been no autonomic features of conjunctival injection or tearing.

Typically, if I can get the patient to avoid chewing for two weeks, the patient’s jaw joint will heal much more effectively without pain or significant setback. The key is to get the patient to comply fully to the protocol of not chewing for 2 weeks. However, this patient has had some adjustment difficulty in consistently blending or pureeing her meals even though when she tries to eat with her splint out of her mouth, she starts hurting. However, when she does puree her meals, she has no pain.

Outcome

The patient’s right jaw joint and articular disc are in proper position now, and the disc reduction was confirmed with MRI scan of each joint using Gibbs and Simmons MRI-TMJ scan protocol.

This patient has noted that SUNCT symptoms began to subside the day after disc reduction and has not had any autonomic signs or symptoms since about 72 hours following the disc reduction. She stated that prior to treatment, she had almost daily pain from SUNCT and that—four days following disc reduction—she was able to go outside and play ball with her children for the first time in a year.

She has had some difficulty in maintaining disc reduction since she has been home. The difficulty could be compliance issues or degenerative changes in muscles and ligament attachments. She was reappointed to be seen again and upon re-examination we found some right side temporal tendonitis (TT) and right side splenius capitis muscle (SCM) attachment pain. Some soreness is present at the insertion of the stylomandibular ligament but is likely secondary to temporal tendonitis.

Blocks at TT and SCM were effective in pain relief. Patient has likely been suffering from arthrokinetic dysfunction of the right TMJ since her jaw locked at least six months prior. It is not unusual for degenerative changes to occur in the various muscle and ligaments when a joint has been dislocated for six months. Further testing confirmed right side temporal tendinitis and splenius injury. Radiofrequency thermoneurolysis was later utilized to denervate the nociceptive free nerve endings in the tendon insertions of the temporalis and splenius capitis muscles. The result of the RFG was promising as the cervical/occipital and the anterior temporal pain is now gone.

The day following the RFG procedure, the patient filled out the Hammer Post-Op questionnaire and results were re-confirmed at follow-up on November 9, 2007:

 
  10/12/07 11/9/07
1. Do you feel the procedure was justified? Yes Yes
2. Did the procedure reduce you pain by at least 50%? Yes Yes
3. Was the procedure comfortable? Yes Yes
4. Now being familiar with the procedure used, would you undergo the procedure again if necessary to relieve the pain? Yes Yes

Residual pain not effected by treatment:

  1. Right ear pain
  2. Right throat pain
  3. Right jaw pain deep in front of lobe of ear

In the patient’s home area, she saw a dentist who gave a local anesthetic infiltration of the right TMJ upper compartment within the joint. The block lasted 50 minutes and all pain was remitted. The procedure was repeated a week later and the block lasted 1.5 hours and most of the pain was remitted. Remission of pain with a TMJ block does not in itself prove TMJ etiology or pathology. A pharygeal fossa tumor or mass of the deep lobe of the parotid gland can also refer pain to the TMJ as well so that a block of the TMJ or the styloid process region can mask the pain of a deep lobe parotid tumor.

Conclusion

This case report has described the typical or classic signs and symptoms of an internal derangement of the temporomanibular joint together with the signs and symptoms of SUNCT Syndrome. This may be the first article suggesting a connection of SUNCT with an internal derangement of the TMJ in a singular case.

While this case has presented with multiple pain issues, the temporomandibular joint disc reduction, temporal tendon, and splenius capitis have responded well to therapy. The remaining pain (in the right ear, right throat, and right jaw deep in front of ear lobe) may originate in ENT areas. As a result, an ENT specialist is scheduled to examine for parotid, pharyngeal fossa, and other regional structures in an attempt to determine the reason for the remaining pain.

Neither the autonomic features of the SUNCT syndrome nor the SUNCT-specific headache have re-appeared in sixty days from the reduction of the articular disc. This finding lends support to the premise that SUNCT is either in remission or was secondary to the TMJ disc dislocation. There is some possibility that signs of Horner’s or an ophthalmic migraine variant may be a part of the patient’s history.

If further examination by other specialists contribute more information to this case, a follow up to this case report will published.

Last updated on: January 4, 2012
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