A 38-yr-old man with no significant medical history initially presented to the emergency department with severe chest pain and was found to have a type A aortic dissection. He underwent emergency mechanical Bentall and ascending hemiarch replacement without intraoperative complications and was extubated the following day. Forty-eight hours after the procedure, the patient suddenly developed severe agitation prompting administration of 10 mg of intravenous haloperidol. He was also started on a dexmedetomidine drip with some improvement in his agitation. However, the patient began to thrash violently approximately 2 hrs later and was further sedated and reintubated for his safety. Given his acute change in mental status, computed tomography head was performed, which demonstrated focal hypodensities in the right frontal region. The following day magnetic resonance imaging brain confirmed multiple acute infarcts in the right frontal, anterior corpus callosum, and right occipital lobe (Figs. 1, 2).
Approximately 4 days later, he received another 4 mg of intravenous haloperidol. On this day, the patient began to complain of jaw pain, tongue biting, and difficulty opening his mouth. The patient had a prolonged hospital course complicated by atrial flutter, respiratory failure requiring tracheostomy placement, severe oropharyngeal dysphagia requiring gastrostomy tube placement, and pneumonia. He received intravenous haloperidol on 9 of the following 22 days for agitation. The total daily doses ranged from 2 to 10 mg. Throughout this course, the patient’s complaints of ongoing jaw pain and difficulty with mouth opening were documented frequently. There were also multiple occasions where the patient bit his tongue causing bleeding. The patient was treated, as needed, with methocarbamol and heat packs without relief. Upon his eventual admission to inpatient rehabilitation, 36 days after the stroke, jaw pain and locking were his chief complaints.
What are the pertinent positive and negative aspects of the history? What findings on physical examination will be important to assess? How does this information guide your differential diagnosis?
On physical examination, he was noted to have severe trismus, reduced range of motion of the tongue, and dysphonia. He had tenderness to palpation of his bilateral temporomandibular joints, and his bilateral masseters were noted to be tense at rest. He had full soft palate elevation and his gag reflex was present bilaterally. He was also noted to have left neglect and ocular apraxia. The patient’s reflexes were within normal limits, including negative Babinksi and Hoffman signs bilaterally and normal jaw jerk. He did not have evidence of increased tone in his extremities.
What is on your differential diagnosis?
The differential diagnosis for this patient’s jaw pain and locking included temporomandibular joint (TMJ) dysfunction, oromandibular dystonia (OMD), or a functional movement disorder such as psychogenic dystonia. The patient’s acute care team believed that his symptoms might have been “behavioral,” especially because he was expressing new symptoms of anxiety and depression. However, his jaw symptoms were constant from the onset and did not vary with sleep or mood. Furthermore, his history and examination did not suggest that his jaw clenching was volitional. This medically complex patient had multiple potential organic causes for his symptoms. For these reasons, functional movement disorder was low on the differential. Given that the patient was intubated multiple times, TMJ dysfunction is possible. Pain and dislocation of the TMJ are known complications of endotracheal intubation.1 Disorders of the TMJ and OMD share similar clinical features such as pain and difficulty opening the jaw,2 and the patient complained of both of these symptoms. The patient’s persistent involuntary muscle contractions suggest the presence of dystonia. Oromandibular dystonia is also associated with oropharyngeal dysphagia and dysphonia,2 which were both seen in this patient. Whenever hypertonia leads to reduced mobility and range of motion, it is important to consider the secondary development of myofibrotic contractures. Soft tissue changes develop rapidly after joint immobilization, with shortening of muscle fibers after 24 hrs and collagen infiltration of the perimysium after 48 hrs.3
There are multiple potential causes of dystonia including neurodegenerative disease, acquired brain injury, and adverse effects of medications (tardive dystonia). However, many patients with OMD are diagnosed with idiopathic OMD.4 This patient may have developed tardive dystonia after receiving high doses of haloperidol in the acute care setting. There is no safe minimum period of exposure to antipsychotic medications, and tardive dystonia can arise even at the initiation of therapy.5
The patient’s history of multifocal embolic strokes was also considered as an etiology of OMD. Dystonia after stroke predominantly arises in the setting of basal ganglia dysfunction, although basal ganglia damage does not always result in dystonia. This patient had no evidence on brain magnetic resonance imaging to suggest injury to the basal ganglia. However, there is evidence in the literature that multiple brain structures may be involved in the development of dystonia.6
What diagnostics would you consider in a patient that presents this way?
As TMJ dysfunction and OMD are both primarily clinical diagnoses, diagnostics such as imaging were not initially pursued. The patient was assessed at the bedside, however, with a portable handheld electromyography device. This device was equipped with audio feedback, which allows the clinician to locate areas of increased muscle activity. With needle insertion into each masseter, the patient was found to have strong activity at rest.
What can you conclude or rule out from these findings? How would you approach treatment for his jaw pain?
It should be noted that a comprehensive electrodiagnostic study was not performed, which may be one limitation of this report. Although there are no established electromyography criteria for a diagnosis of dystonia, there are certain characteristic electrophysiologic features, such as co-contractions of agonist and antagonist muscles with an overflow of contractions in muscles not relevant to the task.7 This patient’s portable electromyography findings still provide valuable information, however, and in the context of his history and examination, he was diagnosed with OMD.
Dystonia is classically defined as a movement disorder characterized by sustained or intermittent muscle contractions that result in abnormal movement, postures, or both.8 There are multiple classifications of dystonia, including focal, segmental, multifocal, generalized, and hemidystonia.9 In this case, the dystonia would be classified as focal, as it only affects one body region. Of the focal forms of dystonia, this patient’s presentation is most consistent with OMD.
This patient received high dose of haloperidol and had a stroke in the same day, which was 4 days before onset of mandibular symptoms. Thus, it is difficult say whether the etiology of his dystonia was due to medications or brain injury or both.
Historical treatment options for dystonia have included deep brain stimulation, dopamine modulators, atypical antipsychotics, anticonvulsants, anticholinergic agents, benzodiazepines, baclofen, peripheral surgical denervation, and botulinum toxin injections. Of the focal dystonias, OMD is known to be the most difficult to treat and often does not respond to medications, although baclofen may be effective in some patients.10 Botulinum toxin injections are considered the principal treatment of choice for focal dystonias,8 including OMD.
During his inpatient rehabilitation stay, the patient was injected with 25 units of onabotulinumtoxinA to each masseter and 20 units to each temporalis muscle. He was also started on baclofen 5 mg three times a day, which was uptitrated to 10 mg three times a day the following week. These interventions provided minimal relief for the patient, and baclofen was ultimately discontinued. The patient’s inability to open his mouth made it difficult to even practice trials of small sips of water and ice chips during speech therapy. It was also a barrier to effective communication with the care team, as it was difficult to carry out an extended conversation because of pain. Upon discharge from inpatient rehabilitation, his functional status with regard to feeding and communication had not changed. He remained G tube dependent for nutrition and was unable to achieve a greater measure of mouth opening despite an aggressive stretching regimen throughout his 21-day stay. Given the patient’s 40-day delay in diagnosis, he likely developed TMJ contractures by the time he was able to receive treatment. Eight months later, his symptoms have not improved despite ongoing oral stretching device use, orofacial therapy, and repeat onabotulinumtoxinA injections at a higher dose. Because of ongoing jaw dysfunction, it was suggested to also evaluate his bony jaw alignment with maxillofacial computed tomography.
Dystonia is a key diagnosis for the physiatrist to be able to recognize and treat. Starting in the intensive care unit, the patient reported jaw pain and dysfunction for 40 days before having a diagnosis of OMD. This case highlights the danger in attributing a patient’s symptoms to psychological disturbances before other causes have been ruled out. Unfortunately, the patient’s jaw locking prevented him from communicating effectively to his care team, and the assumption was made that the patient was not speaking because he was either unwilling or aphasic. Because of an extended delay in diagnosis, it is believed the patient has developed joint contractures. At this point, treating the dystonia alone will not resolve his symptoms. Oromandibular dystonia is an important diagnosis to consider in patients who experience jaw tightness, pain, or difficulty with mouth opening. Regardless of the cause, treatment of this condition can significantly improve ability to perform oral hygiene, eat, communicate, decrease trauma to oral structures, and decrease pain.
This study conforms to all American Journal of Physical Medicine & Rehabilitation Residents and Fellows Section Case Report Guidelines (CARE) guidelines and reports the required information accordingly (see Supplemental Checklist, Supplemental Digital Content 1, https://links.lww.com/PHM/B80).
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