594
Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
Fig. 3. MRI scan of the brain 10 days after that in Fig. 1. Axial fluid attenuated inversion recovery image showing resolution of T2-weighted lesions.
[4] Schwaighofer BW, Hesselink JR, Healy ME. MR demonstration of reversible brain abnormalities in eclampsia. J Comput Assist Tomogr 1989;13:310–2. [5] Ishikura K, Hamasaki Y, Sakai T, et al. Posterior reversible encephalopathy syndrome in children with kidney diseases. Pediatr Nephrol 2012;27:375–84. [6] Kur JK, Esdaile JM. Posterior reversible encephalopathy syndrome–an underrecognized manifestation of systemic lupus erythematosus. J Rheumatol 2006;33:2178–83. [7] Ito Y, Niwa H, Iida T, et al. Post-transfusion reversible posterior leukoencephalopathy syndrome with cerebral vasoconstriction. Neurology 1997;49:1174–5.
[8] Huang YC, Tsai PL, Yeh JH, et al. Reversible posterior leukoencephalopathy syndrome caused by blood transfusion: a case report. Acta Neurol Taiwan 2008;17:258–62. [9] Boughammoura A, Touze E, Oppenheim C, et al. Reversible angiopathy and encephalopathy after blood transfusion. J Neurol 2003;250:116–8. [10] Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494–500. [11] Khademian Z, Speller-Brown B, Nouraie SM, et al. Reversible posterior leukoencephalopathy in children with sickle cell disease. Pediatr Blood Cancer 2009;52:373–5.
http://dx.doi.org/10.1016/j.jocn.2014.08.027
Successful treatment of open jaw and jaw deviation dystonia with botulinum toxin using a simple intraoral approach Mariana Moscovich, Zhongxing Peng Chen, Ramon Rodriguez ⇑ Department of Neurology, University of Florida, Center for Movement Disorders & Neurorestoration, McKnight Brain Institute, 3450 Hull Road, 4th floor, Gainesville, FL 32607, USA
a r t i c l e
i n f o
Article history: Received 18 January 2014 Accepted 25 August 2014
Keywords: Botulinum toxin Dystonia Jaw deviation Jaw opening Oromandibular dystonia
a b s t r a c t Oromandibular dystonia (OMD) is a focal dystonia that involves the mouth, jaw, and/or tongue. It can be classified as idiopathic, tardive dystonia or secondary to other neurological disorders and subdivided into jaw opening, jaw closing, jaw deviation and lip pursing. The muscles involved in jaw opening dystonia are usually the digastrics and lateral pterygoids. It is known that the lateral pterygoids may be approached both internally and externally. The external approach is the most common; however neurologists experienced in treating patients with botulinum toxin can safely and with no extra cost perform the intraoral procedure. We report our experience in the treatment of jaw opening and jaw deviation dystonia using the intraoral injection approach. Eight patients were selected from the University of Florida with a clinical diagnosis of open jaw/jaw deviation dystonia. All of them were injected with onabotulinum toxin A using the internal approach and the clinical global impression scale was applied. The mean age of the patients was 67 (standard deviation [SD] 10.2) years, with a disease duration of 10.2 (SD 7.7) years and the mean distance they traveled to our institution was 448 km (278 miles). After treatment, six patients scored as very much improved in the clinical global impression scale and two patients scored as much improved. Only one patient reported an adverse event of nasal speech following one of the injections that improved after 4 weeks. Botulinum toxin injections for open jaw/jaw deviation dystonia can be safely performed with the intraoral approach without the need of special devices other than electromyography. Ó 2014 Elsevier Ltd. All rights reserved.
⇑ Corresponding author. Tel.: +1 352 294 5400; fax: +1 352 294 5399. E-mail address: [email protected]fl.edu (R. Rodriguez).
Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
1. Introduction Oromandibular dystonia (OMD) is a focal dystonia that involves the mouth, jaw, and/or tongue [1,2]. It can be classified as idiopathic, tardive dystonia or secondary to other neurological disorders and subdivided into jaw opening, jaw closing, jaw deviation and lip pursing [1,3–5]. Medical treatment of OMD is usually complicated by side effects or poor response [6]. Botulinum toxin has been found to be effective and well tolerated for the treatment of OMD [7]. However, when looking into the different types of OMD and their response to treatment, jaw opening and jaw deviation dystonia are the most challenging to treat and seem to be less responsive to treatment [4]. The muscles involved in jaw opening dystonia are usually the digastrics and lateral pterygoids [4]. While the digastrics are easier to reach, the lateral pterygoids are more complicated to inject from a technical standpoint, and, in our experience, patients report major discomfort with the procedure [3]. The lateral pterygoids are usually approached laterally through the mandibular incisures, preferentially with electromyography (EMG) guidance [4]. However this technique is complex and in our experience the success rate is less than favorable. In this manuscript we describe our experience using a simple intraoral approach, which we have found to be described in the literature as a procedure usually performed by dentists or oral surgeons [3], but we find it to be easier from the technical standpoint and it is more comfortable for patients and neurologists. 2. Objective To report our experience in the treatment of jaw opening and jaw deviation dystonia using the intraoral injection approach. 3. Patients and methods Patients seen at the Tyler’s Hope Comprehensive Center for Dystonia Care at The University of Florida with a clinical diagnosis of open jaw/jaw deviation dystonia made by a fellowship-trained movement disorders neurologist were treated with onabotulinum toxin A. All patients provided informed consent prior to being included in the study in accordance with the Declaration of Helsinki. They were all previously treated using the extraoral approach to the lateral pterygoid [4] and reported little or no benefit. We proceeded to inject them using the internal approach at least 16 weeks after the last injection as follows: subjects were positioned in the supine position, then asked to
595
open their mouths and, using a tongue blade, the second molar was identified. The point of insertion of the needle was the muccobuccal folds of the upper second molars (Fig. 1). We used an EMG needle (Teca MyoJect Luer Lock, 50 mm long with a 25 G/0.51 mm needle diameter; Optima Medical, Guildford, Surrey, UK), which was inserted in the direction of the tragus of the ear. Once signal was being recorded in the EMG machine, the patient was asked to move the jaw from side to side for confirmation of the lateral pterygoid. The muscle was then infiltrated with the desired dose. The procedure was repeated on the contralateral side if necessary. Benefit was measured 4 weeks post procedure using the Clinical Global Impression Scale, as rated by the patient. They were followed every 12 weeks for reinjection. 4. Results Eight patients (six women, two men) were treated over the period of 2008–2011. The mean age of the patients was 67 (standard deviation [SD] 10.2) years, with a disease duration of 10.2 (SD 7.7) years and the mean distance they traveled to our institution was 448 km (278 miles). After treatment, six patients scored as very much improved in the Clinical Global Impression Scale and two patients scored as much improved (Table 1). Only one patient reported an adverse event of nasal speech following one of the injections that improved after 4 weeks. The other patients tolerated the procedure very well. They all reported the procedure to be faster and less painful than the extraoral approach. 5. Discussion In this manuscript, we describe a procedure that we find easy to perform, is well tolerated by patients and is effective in patients with jaw deviation and jaw opening dystonia. It is known that the lateral pterygoids may be approached both internally and externally. However, as neurologists, we may find that the internal approach is difficult as a result of lack of expertise. In comparison, the external approach to access the lateral pterygoid has been described in detail in the literature and is used by the majority of the neurologists [4,8]. In our center, we previously used the assistance of an oral surgeon or odontologist to perform this procedure. However, it was cumbersome from a logistical standpoint and added cost. The best description of how to approach the lateral pterygoid internally that we found is using a device that is applied to the second molar as a guide [3]. However, the device is patient
Fig. 1. Intraoral approach to the lateral pterygoid muscle. (Left) The injection entry point into the lateral pterygoid muscle (courtesy of Dr. Albert Rothon, University of Florida Neurosurgery Department). (Right) The direction of the injection toward the middle point of a virtual line connecting the ipsilateral ear’s tragus and lobe. (This figure is available in colour at http://www.sciencedirect.com/.)
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Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
Table 1 Demographics of open jaw and jaw deviation dystonia patients treated with botulinum toxin Patient
Age, years
Sex
Diagnosis
Muscle injected
CGIS
Distance travelled, km
1 2 3 4 5 6 7 8
56 82 49 66 73 70 78 67
F F M F F F M F
Jaw Jaw Jaw Jaw Jaw jaw Jaw Jaw
Left lateral pterygoid Bilateral pterygoid Left lateral pterygoid Left lateral pterygoid Bilateral pterygoid Left lateral pterygoid Right lateral pterygoid Left pterygoid
1/1 1/1 1/1 1/1 1/1 1/1 2/2 2/2
197 480 202 120 798 114 1,124 550
deviation deviation and jaw opening deviation deviation and jaw opening protrusion deviation deviation deviation
CGIS = Clinical Global Impression Scale, F = female, M = male.
specific and requires the expertise of a dental technician to fabricate. Most of the patients treated at our center had travelled long distances, as there was no local treatment option. We feel any neurologist experienced in treating patients with botulinum toxin can safely and with no extra cost perform the procedure described above. Patients responded favorably to treatment over multiple sessions and the incidence of side effects was very small.
from USF CME, PeerView Institute for Medical Education, PRIME CME, Corporate Meeting Solutions, Merz Pharmaceuticals, The Cognition Group and United Biosource. Contractual Services: The University of Florida Clinic has contracts with Allergan for education services provided by Dr. Rodriguez, but he does not receive any personal compensation for these roles. Acknowledgments
6. Conclusion Botulinum toxin injections for open jaw/jaw deviation dystonia can be safely performed without the need for special devices other than EMG. Patients easily tolerated the intraoral injections and the side effect profile is benign. The patients in this series were very satisfied using this approach. Patients who are not responding to treatment using the conventional external approach may benefit from the intraoral technique. Conflicts of Interest/Disclosures Dr. Mariana Moscovich and Zhongxing Peng Chen declare that they have no financial or other conflicts of interest in relation to this research and its publication. Dr. Ramon L Rodriguez reports the following. Grants/Research Support: received research support from Abbott, Biotie Therapeutics, EMD-Serono, Huntington Study Group, Ipsen, Merz Pharmaceuticals, Allergan, National Parkinson Foundation, NIH/NINDS, Teva, Neuronova, but has no owner interest in any pharmaceutical company. Honoraria: Received honoraria
http://dx.doi.org/10.1016/j.jocn.2014.08.027
The authors thank Dr. Albert Rothon and Dr. Maria Peris Celda from University of Florida Neurosurgery Department for providing the skull picture. References [1] Singer C, Papapetropoulos S. A comparison of jaw-closing and jaw-opening idiopathic oromandibular dystonia. Parkinsonism Relat Disord 2006;12:115–8. [2] Tan EK, Jankovic J. Botulinum toxin A in patients with oromandibular dystonia: long-term follow-up. Neurology 1999;53:2102–7. [3] Yoshida K, Kaji R, Takagi A, et al. Customized EMG needle insertion guide for the muscle afferent block of jaw-deviation and jaw-opening dystonias. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:664–9. [4] Bhidayasiri R, Cardoso F, Truong DD. Botulinum toxin in blepharospasm and oromandibular dystonia: comparing different botulinum toxin preparations. Eur J Neurol 2006;13:21–9. [5] Tan EK, Jankovic J. Tardive and idiopathic oromandibular dystonia: a clinical comparison. J Neurol Neurosurg Psychiatry 2000;68:186–90. [6] Greene P, Shale H, Fahn S. Analysis of open-label trials in torsion dystonia using high dosages of anticholinergics and other drugs. Mov Disord 1988;3:46–60. [7] Ney JP, Joseph KR. Neurologic uses of botulinum neurotoxin type A. Neuropsychiatr Dis Treat 2007;3:785–98. [8] Dressler D, Wittstock M, Benecke R. Botulinum toxin for treatment of jaw opening dystonia in Hallervorden-Spatz syndrome. Eur Neurol 2001;45:287–8.
Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
Fig. 3. MRI scan of the brain 10 days after that in Fig. 1. Axial fluid attenuated inversion recovery image showing resolution of T2-weighted lesions.
[4] Schwaighofer BW, Hesselink JR, Healy ME. MR demonstration of reversible brain abnormalities in eclampsia. J Comput Assist Tomogr 1989;13:310–2. [5] Ishikura K, Hamasaki Y, Sakai T, et al. Posterior reversible encephalopathy syndrome in children with kidney diseases. Pediatr Nephrol 2012;27:375–84. [6] Kur JK, Esdaile JM. Posterior reversible encephalopathy syndrome–an underrecognized manifestation of systemic lupus erythematosus. J Rheumatol 2006;33:2178–83. [7] Ito Y, Niwa H, Iida T, et al. Post-transfusion reversible posterior leukoencephalopathy syndrome with cerebral vasoconstriction. Neurology 1997;49:1174–5.
[8] Huang YC, Tsai PL, Yeh JH, et al. Reversible posterior leukoencephalopathy syndrome caused by blood transfusion: a case report. Acta Neurol Taiwan 2008;17:258–62. [9] Boughammoura A, Touze E, Oppenheim C, et al. Reversible angiopathy and encephalopathy after blood transfusion. J Neurol 2003;250:116–8. [10] Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494–500. [11] Khademian Z, Speller-Brown B, Nouraie SM, et al. Reversible posterior leukoencephalopathy in children with sickle cell disease. Pediatr Blood Cancer 2009;52:373–5.
http://dx.doi.org/10.1016/j.jocn.2014.08.027
Successful treatment of open jaw and jaw deviation dystonia with botulinum toxin using a simple intraoral approach Mariana Moscovich, Zhongxing Peng Chen, Ramon Rodriguez ⇑ Department of Neurology, University of Florida, Center for Movement Disorders & Neurorestoration, McKnight Brain Institute, 3450 Hull Road, 4th floor, Gainesville, FL 32607, USA
a r t i c l e
i n f o
Article history: Received 18 January 2014 Accepted 25 August 2014
Keywords: Botulinum toxin Dystonia Jaw deviation Jaw opening Oromandibular dystonia
a b s t r a c t Oromandibular dystonia (OMD) is a focal dystonia that involves the mouth, jaw, and/or tongue. It can be classified as idiopathic, tardive dystonia or secondary to other neurological disorders and subdivided into jaw opening, jaw closing, jaw deviation and lip pursing. The muscles involved in jaw opening dystonia are usually the digastrics and lateral pterygoids. It is known that the lateral pterygoids may be approached both internally and externally. The external approach is the most common; however neurologists experienced in treating patients with botulinum toxin can safely and with no extra cost perform the intraoral procedure. We report our experience in the treatment of jaw opening and jaw deviation dystonia using the intraoral injection approach. Eight patients were selected from the University of Florida with a clinical diagnosis of open jaw/jaw deviation dystonia. All of them were injected with onabotulinum toxin A using the internal approach and the clinical global impression scale was applied. The mean age of the patients was 67 (standard deviation [SD] 10.2) years, with a disease duration of 10.2 (SD 7.7) years and the mean distance they traveled to our institution was 448 km (278 miles). After treatment, six patients scored as very much improved in the clinical global impression scale and two patients scored as much improved. Only one patient reported an adverse event of nasal speech following one of the injections that improved after 4 weeks. Botulinum toxin injections for open jaw/jaw deviation dystonia can be safely performed with the intraoral approach without the need of special devices other than electromyography. Ó 2014 Elsevier Ltd. All rights reserved.
⇑ Corresponding author. Tel.: +1 352 294 5400; fax: +1 352 294 5399. E-mail address: [email protected]fl.edu (R. Rodriguez).
Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
1. Introduction Oromandibular dystonia (OMD) is a focal dystonia that involves the mouth, jaw, and/or tongue [1,2]. It can be classified as idiopathic, tardive dystonia or secondary to other neurological disorders and subdivided into jaw opening, jaw closing, jaw deviation and lip pursing [1,3–5]. Medical treatment of OMD is usually complicated by side effects or poor response [6]. Botulinum toxin has been found to be effective and well tolerated for the treatment of OMD [7]. However, when looking into the different types of OMD and their response to treatment, jaw opening and jaw deviation dystonia are the most challenging to treat and seem to be less responsive to treatment [4]. The muscles involved in jaw opening dystonia are usually the digastrics and lateral pterygoids [4]. While the digastrics are easier to reach, the lateral pterygoids are more complicated to inject from a technical standpoint, and, in our experience, patients report major discomfort with the procedure [3]. The lateral pterygoids are usually approached laterally through the mandibular incisures, preferentially with electromyography (EMG) guidance [4]. However this technique is complex and in our experience the success rate is less than favorable. In this manuscript we describe our experience using a simple intraoral approach, which we have found to be described in the literature as a procedure usually performed by dentists or oral surgeons [3], but we find it to be easier from the technical standpoint and it is more comfortable for patients and neurologists. 2. Objective To report our experience in the treatment of jaw opening and jaw deviation dystonia using the intraoral injection approach. 3. Patients and methods Patients seen at the Tyler’s Hope Comprehensive Center for Dystonia Care at The University of Florida with a clinical diagnosis of open jaw/jaw deviation dystonia made by a fellowship-trained movement disorders neurologist were treated with onabotulinum toxin A. All patients provided informed consent prior to being included in the study in accordance with the Declaration of Helsinki. They were all previously treated using the extraoral approach to the lateral pterygoid [4] and reported little or no benefit. We proceeded to inject them using the internal approach at least 16 weeks after the last injection as follows: subjects were positioned in the supine position, then asked to
595
open their mouths and, using a tongue blade, the second molar was identified. The point of insertion of the needle was the muccobuccal folds of the upper second molars (Fig. 1). We used an EMG needle (Teca MyoJect Luer Lock, 50 mm long with a 25 G/0.51 mm needle diameter; Optima Medical, Guildford, Surrey, UK), which was inserted in the direction of the tragus of the ear. Once signal was being recorded in the EMG machine, the patient was asked to move the jaw from side to side for confirmation of the lateral pterygoid. The muscle was then infiltrated with the desired dose. The procedure was repeated on the contralateral side if necessary. Benefit was measured 4 weeks post procedure using the Clinical Global Impression Scale, as rated by the patient. They were followed every 12 weeks for reinjection. 4. Results Eight patients (six women, two men) were treated over the period of 2008–2011. The mean age of the patients was 67 (standard deviation [SD] 10.2) years, with a disease duration of 10.2 (SD 7.7) years and the mean distance they traveled to our institution was 448 km (278 miles). After treatment, six patients scored as very much improved in the Clinical Global Impression Scale and two patients scored as much improved (Table 1). Only one patient reported an adverse event of nasal speech following one of the injections that improved after 4 weeks. The other patients tolerated the procedure very well. They all reported the procedure to be faster and less painful than the extraoral approach. 5. Discussion In this manuscript, we describe a procedure that we find easy to perform, is well tolerated by patients and is effective in patients with jaw deviation and jaw opening dystonia. It is known that the lateral pterygoids may be approached both internally and externally. However, as neurologists, we may find that the internal approach is difficult as a result of lack of expertise. In comparison, the external approach to access the lateral pterygoid has been described in detail in the literature and is used by the majority of the neurologists [4,8]. In our center, we previously used the assistance of an oral surgeon or odontologist to perform this procedure. However, it was cumbersome from a logistical standpoint and added cost. The best description of how to approach the lateral pterygoid internally that we found is using a device that is applied to the second molar as a guide [3]. However, the device is patient
Fig. 1. Intraoral approach to the lateral pterygoid muscle. (Left) The injection entry point into the lateral pterygoid muscle (courtesy of Dr. Albert Rothon, University of Florida Neurosurgery Department). (Right) The direction of the injection toward the middle point of a virtual line connecting the ipsilateral ear’s tragus and lobe. (This figure is available in colour at http://www.sciencedirect.com/.)
596
Case Reports / Journal of Clinical Neuroscience 22 (2015) 594–596
Table 1 Demographics of open jaw and jaw deviation dystonia patients treated with botulinum toxin Patient
Age, years
Sex
Diagnosis
Muscle injected
CGIS
Distance travelled, km
1 2 3 4 5 6 7 8
56 82 49 66 73 70 78 67
F F M F F F M F
Jaw Jaw Jaw Jaw Jaw jaw Jaw Jaw
Left lateral pterygoid Bilateral pterygoid Left lateral pterygoid Left lateral pterygoid Bilateral pterygoid Left lateral pterygoid Right lateral pterygoid Left pterygoid
1/1 1/1 1/1 1/1 1/1 1/1 2/2 2/2
197 480 202 120 798 114 1,124 550
deviation deviation and jaw opening deviation deviation and jaw opening protrusion deviation deviation deviation
CGIS = Clinical Global Impression Scale, F = female, M = male.
specific and requires the expertise of a dental technician to fabricate. Most of the patients treated at our center had travelled long distances, as there was no local treatment option. We feel any neurologist experienced in treating patients with botulinum toxin can safely and with no extra cost perform the procedure described above. Patients responded favorably to treatment over multiple sessions and the incidence of side effects was very small.
from USF CME, PeerView Institute for Medical Education, PRIME CME, Corporate Meeting Solutions, Merz Pharmaceuticals, The Cognition Group and United Biosource. Contractual Services: The University of Florida Clinic has contracts with Allergan for education services provided by Dr. Rodriguez, but he does not receive any personal compensation for these roles. Acknowledgments
6. Conclusion Botulinum toxin injections for open jaw/jaw deviation dystonia can be safely performed without the need for special devices other than EMG. Patients easily tolerated the intraoral injections and the side effect profile is benign. The patients in this series were very satisfied using this approach. Patients who are not responding to treatment using the conventional external approach may benefit from the intraoral technique. Conflicts of Interest/Disclosures Dr. Mariana Moscovich and Zhongxing Peng Chen declare that they have no financial or other conflicts of interest in relation to this research and its publication. Dr. Ramon L Rodriguez reports the following. Grants/Research Support: received research support from Abbott, Biotie Therapeutics, EMD-Serono, Huntington Study Group, Ipsen, Merz Pharmaceuticals, Allergan, National Parkinson Foundation, NIH/NINDS, Teva, Neuronova, but has no owner interest in any pharmaceutical company. Honoraria: Received honoraria
http://dx.doi.org/10.1016/j.jocn.2014.08.027
The authors thank Dr. Albert Rothon and Dr. Maria Peris Celda from University of Florida Neurosurgery Department for providing the skull picture. References [1] Singer C, Papapetropoulos S. A comparison of jaw-closing and jaw-opening idiopathic oromandibular dystonia. Parkinsonism Relat Disord 2006;12:115–8. [2] Tan EK, Jankovic J. Botulinum toxin A in patients with oromandibular dystonia: long-term follow-up. Neurology 1999;53:2102–7. [3] Yoshida K, Kaji R, Takagi A, et al. Customized EMG needle insertion guide for the muscle afferent block of jaw-deviation and jaw-opening dystonias. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:664–9. [4] Bhidayasiri R, Cardoso F, Truong DD. Botulinum toxin in blepharospasm and oromandibular dystonia: comparing different botulinum toxin preparations. Eur J Neurol 2006;13:21–9. [5] Tan EK, Jankovic J. Tardive and idiopathic oromandibular dystonia: a clinical comparison. J Neurol Neurosurg Psychiatry 2000;68:186–90. [6] Greene P, Shale H, Fahn S. Analysis of open-label trials in torsion dystonia using high dosages of anticholinergics and other drugs. Mov Disord 1988;3:46–60. [7] Ney JP, Joseph KR. Neurologic uses of botulinum neurotoxin type A. Neuropsychiatr Dis Treat 2007;3:785–98. [8] Dressler D, Wittstock M, Benecke R. Botulinum toxin for treatment of jaw opening dystonia in Hallervorden-Spatz syndrome. Eur Neurol 2001;45:287–8.
