Introduction
The mouth and jaw play important roles in various functions such as eating, chewing, speaking and swallowing. However, various problems can occur if the muscles that move the jaw and mouth are subjected to excessive involuntary contraction. These problems include failure to chew foods (masticatory disturbance); problems with mouth opening (trismus) or closing; involuntary movements of the mouth, tongue, and/or lips; tremors; muscle pain; lateral shifting of the jaw (jaw deviation); and difficulties with swallowing (dysphagia) or speaking (dysarthria). Such symptoms can be caused by dystonia of the mouth and/or jaw (oromandibular dystonia), temporomandibular joint disorders, oral dyskinesia, bruxism, fibromyalgia, psychogenic movement disorder, and/or masticatory muscle tendon-aponeurosis hyperplasia. The cause of the symptoms may be one of these diseases, however more than one disease may coexist. A variety of involuntary movements can occur in the orofacial region, most of which are not diagnosed correctly. In fact, the misdiagnosis of oromandibular dystonia as temporomandibular joint disorder, psychogenic disease, bruxism, or temporomandibular joint ankylosis is very common. Most patients that are diagnosed by dentists or oral surgeons are initially treated with dental appliances. In fact, 80% of our patients with jaw closing dystonia, the most common type of oromandibular dystonia, initially visited dentists or oral and maxillofacial surgeons. None of these patients were diagnosed with dystonia. Accordingly, they did not receive appropriate treatment, and their conditions deteriorated.
Oromandibulardystonia Dystonia (dys: abnormal, tonia: tension) is a neurological movement disorder. It is defined as a syndrome characterized by sustained muscle contractions that cause twisting or repetitive movements and abnormal postures in the affected body parts. Oromandibular dystonia is a focal dystonia that manifests as involuntary masticatory and/or lingual muscle contractions. Oromandibular dystonia includes jaw closing dystonia, jaw opening dystonia, tongue protrusion dystonia, jaw deviation dystonia, and jaw protrusion dystonia. Oromandibular dystonia is easily misdiagnosed as a temporomandibular joint disorder or psychosomatic disease, and hence, patients with the condition often visit many hospitals and departments before being correctly diagnosed. I have been administering botulinum therapy for dystonia since 1992 and have treated many patients. At the Department of Oral and Maxillofacial Surgery at Kyoto Medical Center, we specialize in involuntary movements of the oral and facial area. We apply a comprehensive range of treatments for such involuntary movements, including medication, injections of local anesthetic (MAB therapy) or botulinum toxin, and surgery. There are no other departments that specialize in involuntary movements of the stomatognathic system. Accordingly, patients with involuntary movements of the orofacial region are referred to us from many hospitals. Due to these advantages, we receive numerous queries from all over Japan, and many patients come to our department for treatment from abroad.
Remote diagnosis
When diagnosing patient's that exhibit involuntary movements we record their medical history and symptoms and make careful diagnosis. During this process, it is necessary to differentiate oromandibular dystonia from temporomandibular joint disorder, bruxism, oral dyskinesia, hyperplasia of the mandibular coronoid process, and masticatory muscle hyperplasia tendon-aponeurosis hyperplasia.
Please fill out the oromandibular dystonia questionnaire. You can check more than one answer. If none of the example answers are applicable, please provide as much specific information as you can. Please email your responses to the above questions to me as an attachment or fax them. Alternatively, you can post them.
We can also communicate via Skype, and remote diagnosis via email can also be attempted. Remote diagnosis is available after hospital hours (after 18:00 Japanese time). If you have the typical symptoms of dystonia, I will be able to say whether you are likely to have dystonia based on the information images, questionnaire results, and video you provide. However, for a definitive diagnosis you must visit our department to undergo electromyography (EMG), muscle palpation, and other tests such as X rays, computed tomography (CT), and MRI. Please note that we cannot be held responsible for remote diagnoses based solely on questionnaire results and/or images.
Treatment
As an initial treatment, we prescribe medicine for mild cases. Most patients show some improvement. There is no specific medicine for oromandibular dystonia. We use combinations of various drugs and continue to gradually increase the dose while evaluating the effects and side effects of the treatment. Pharmacotherapy takes several months. As the elderly tend to develop side effects, we increase the dose slowly in such patients. If a patient's symptoms do not improve, we apply muscle afferent block (MAB) therapy by injecting local anesthetic into the affected muscles or via the intramuscular injection of botulinum toxin (botulinum therapy).
Muscle afferent block (MAB) therapy
MAB therapy, which involves the local injection of diluted lidocaine and ethanol, aims to reduce the effectiveness of muscle spindle afferents without causing unfavorable weakness. Its effect has been shown to be mediated by the blockade of either muscle afferents or gamma motor efferents. In a previous study, the T-reflex of the hand muscles was attenuated whilst their power was preserved after the intramuscular injection of lidocaine, and the muscle spindle afferents or gamma motor efferents that tonically control the sensitivity of the spindles were postulated to be blocked by MAB. In another study, the mean post-MAB response of the jaw elevator muscles (70%), which was evaluated on a self-rating scale, was significantly higher than that of the depressor muscles (38%), and it was suggested that by the different numbers of muscle spindles supplying these muscles were responsible for these results. Therefore, MAB therapy is indicated for jaw elevator muscles (the masseter, temporalis and medial pterygoid muscles), but not for jaw depressor muscles (the lateral pterygoid and digastric muscles), which contain fewer muscle spindles.
Botulinum therapy
Botulinum toxin is produced by Clostridium botulinum, a Gram-positive anaerobic bacterium. Botulinum toxin is a neuromuscular blocking agent. It exerts its paralytic action by rapidly and strongly binding to presynaptic cholinergic nerve terminals. It is then internalized and ultimately inhibits the exocytosis of acetylcholine by decreasing the frequency of acetylcholine release. Without its nerve supply, the muscle fiber withers away. The muscle strengthens again as the nerves regenerate.
Surgery
If long-term extremely forceful dystonic elevator muscle contraction results in masticatory muscle tendon-aponeurosis hyperplasia or hyperplasia of the coronoid process, surgery, e.g., coronoidotomy, might be required. We approach, and all incisions are made in the mouth, so no surgical scars remain on the face. The operation takes 1.5-2 hours. As postoperative mouth opening training is important, the patient has to remain in hospital for about two weeks.
Medical tourism
Once a diagnosis of dystonia is made, the treatment will differ depending on the degree of the patient's symptoms. Patients with milder symptoms will receive oral medication or undergo MAB therapy. MAB therapy and oral medication therapy both take several months. However, botulinum therapy for the mouth closing muscles (masseter, temporalis, and medial pterygoid muscles) is possible as an outpatient. In the case of injections into the muscles of the palate or tongue, dysphagia might occur after treatment, although our department has experienced no such cases; therefore, it is safer for you to stay in hospital for a short period after such
The mouth and jaw play important roles in various functions such as eating, chewing, speaking and swallowing. However, various problems can occur if the muscles that move the jaw and mouth are subjected to excessive involuntary contraction. These problems include failure to chew foods (masticatory disturbance); problems with mouth opening (trismus) or closing; involuntary movements of the mouth, tongue, and/or lips; tremors; muscle pain; lateral shifting of the jaw (jaw deviation); and difficulties with swallowing (dysphagia) or speaking (dysarthria). Such symptoms can be caused by dystonia of the mouth and/or jaw (oromandibular dystonia), temporomandibular joint disorders, oral dyskinesia, bruxism, fibromyalgia, psychogenic movement disorder, and/or masticatory muscle tendon-aponeurosis hyperplasia. The cause of the symptoms may be one of these diseases, however more than one disease may coexist. A variety of involuntary movements can occur in the orofacial region, most of which are not diagnosed correctly. In fact, the misdiagnosis of oromandibular dystonia as temporomandibular joint disorder, psychogenic disease, bruxism, or temporomandibular joint ankylosis is very common. Most patients that are diagnosed by dentists or oral surgeons are initially treated with dental appliances. In fact, 80% of our patients with jaw closing dystonia, the most common type of oromandibular dystonia, initially visited dentists or oral and maxillofacial surgeons. None of these patients were diagnosed with dystonia. Accordingly, they did not receive appropriate treatment, and their conditions deteriorated.
Oromandibulardystonia Dystonia (dys: abnormal, tonia: tension) is a neurological movement disorder. It is defined as a syndrome characterized by sustained muscle contractions that cause twisting or repetitive movements and abnormal postures in the affected body parts. Oromandibular dystonia is a focal dystonia that manifests as involuntary masticatory and/or lingual muscle contractions. Oromandibular dystonia includes jaw closing dystonia, jaw opening dystonia, tongue protrusion dystonia, jaw deviation dystonia, and jaw protrusion dystonia. Oromandibular dystonia is easily misdiagnosed as a temporomandibular joint disorder or psychosomatic disease, and hence, patients with the condition often visit many hospitals and departments before being correctly diagnosed. I have been administering botulinum therapy for dystonia since 1992 and have treated many patients. At the Department of Oral and Maxillofacial Surgery at Kyoto Medical Center, we specialize in involuntary movements of the oral and facial area. We apply a comprehensive range of treatments for such involuntary movements, including medication, injections of local anesthetic (MAB therapy) or botulinum toxin, and surgery. There are no other departments that specialize in involuntary movements of the stomatognathic system. Accordingly, patients with involuntary movements of the orofacial region are referred to us from many hospitals. Due to these advantages, we receive numerous queries from all over Japan, and many patients come to our department for treatment from abroad.
Remote diagnosis
When diagnosing patient's that exhibit involuntary movements we record their medical history and symptoms and make careful diagnosis. During this process, it is necessary to differentiate oromandibular dystonia from temporomandibular joint disorder, bruxism, oral dyskinesia, hyperplasia of the mandibular coronoid process, and masticatory muscle hyperplasia tendon-aponeurosis hyperplasia.
Please fill out the oromandibular dystonia questionnaire. You can check more than one answer. If none of the example answers are applicable, please provide as much specific information as you can. Please email your responses to the above questions to me as an attachment or fax them. Alternatively, you can post them.
We can also communicate via Skype, and remote diagnosis via email can also be attempted. Remote diagnosis is available after hospital hours (after 18:00 Japanese time). If you have the typical symptoms of dystonia, I will be able to say whether you are likely to have dystonia based on the information images, questionnaire results, and video you provide. However, for a definitive diagnosis you must visit our department to undergo electromyography (EMG), muscle palpation, and other tests such as X rays, computed tomography (CT), and MRI. Please note that we cannot be held responsible for remote diagnoses based solely on questionnaire results and/or images.
Treatment
As an initial treatment, we prescribe medicine for mild cases. Most patients show some improvement. There is no specific medicine for oromandibular dystonia. We use combinations of various drugs and continue to gradually increase the dose while evaluating the effects and side effects of the treatment. Pharmacotherapy takes several months. As the elderly tend to develop side effects, we increase the dose slowly in such patients. If a patient's symptoms do not improve, we apply muscle afferent block (MAB) therapy by injecting local anesthetic into the affected muscles or via the intramuscular injection of botulinum toxin (botulinum therapy).
Muscle afferent block (MAB) therapy
MAB therapy, which involves the local injection of diluted lidocaine and ethanol, aims to reduce the effectiveness of muscle spindle afferents without causing unfavorable weakness. Its effect has been shown to be mediated by the blockade of either muscle afferents or gamma motor efferents. In a previous study, the T-reflex of the hand muscles was attenuated whilst their power was preserved after the intramuscular injection of lidocaine, and the muscle spindle afferents or gamma motor efferents that tonically control the sensitivity of the spindles were postulated to be blocked by MAB. In another study, the mean post-MAB response of the jaw elevator muscles (70%), which was evaluated on a self-rating scale, was significantly higher than that of the depressor muscles (38%), and it was suggested that by the different numbers of muscle spindles supplying these muscles were responsible for these results. Therefore, MAB therapy is indicated for jaw elevator muscles (the masseter, temporalis and medial pterygoid muscles), but not for jaw depressor muscles (the lateral pterygoid and digastric muscles), which contain fewer muscle spindles.
Botulinum therapy
Botulinum toxin is produced by Clostridium botulinum, a Gram-positive anaerobic bacterium. Botulinum toxin is a neuromuscular blocking agent. It exerts its paralytic action by rapidly and strongly binding to presynaptic cholinergic nerve terminals. It is then internalized and ultimately inhibits the exocytosis of acetylcholine by decreasing the frequency of acetylcholine release. Without its nerve supply, the muscle fiber withers away. The muscle strengthens again as the nerves regenerate.
Surgery
If long-term extremely forceful dystonic elevator muscle contraction results in masticatory muscle tendon-aponeurosis hyperplasia or hyperplasia of the coronoid process, surgery, e.g., coronoidotomy, might be required. We approach, and all incisions are made in the mouth, so no surgical scars remain on the face. The operation takes 1.5-2 hours. As postoperative mouth opening training is important, the patient has to remain in hospital for about two weeks.
Medical tourism
Once a diagnosis of dystonia is made, the treatment will differ depending on the degree of the patient's symptoms. Patients with milder symptoms will receive oral medication or undergo MAB therapy. MAB therapy and oral medication therapy both take several months. However, botulinum therapy for the mouth closing muscles (masseter, temporalis, and medial pterygoid muscles) is possible as an outpatient. In the case of injections into the muscles of the palate or tongue, dysphagia might occur after treatment, although our department has experienced no such cases; therefore, it is safer for you to stay in hospital for a short period after such
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