Parkinsonism and Related Disorders 20 (2014) 265e266
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Botulinum toxin-responsive ear myoclonus following otitis externa Keywords: Ear movements Botulinum toxin Dyskinesia Myoclonus
A 32-year-old male presented with a six-month history of involuntary intermittent twitching movements of his right ear, directed anteriorly, superiorly and inferiorly. Concomitant ear clicks accompanied the auricular movements, but palatal movements were absent. The movements were not suppressible or associated with premonitory sensations or inner tension, but disrupted his sleep. An episode of right-sided otitis externa had occurred just prior to the onset of movements, but there was no prior history of head trauma or neuroleptic exposure. He was otherwise physically well and had no previous medical or family history of note. Upon examination, the pinna twitched laterally, anteriorly and superiorly (video) and spasm was palpable within auricularis anterior and superior. There was no palatal myoclonus. MR brain imaging did not reveal any structural lesions. AbobotulinumtoxinA (Dysport) 100 units was injected into auricularis anterior and superior, after which the auricular movements ceased, and the clicking noises and sleep disturbance improved considerably. Upon review three months later, the patient continued to derive benefit with no adverse effects, and no discernible ear movement. Supplementary video related to this article can be found at http://dx.doi.org/10.1016/j.parkreldis.2013.11.013. Isolated ear movements with varied phenomenology and postulated underlying causes are reported in the literature. Caviness et al. reported four cases of slow, semirhythmic and jerky involuntary ear movements, whose electromyography (EMG) and clinical characteristics were consistent with dystonia [1]. Response to botulinum toxin injection has been reported with rhythmic dystonic-type ear movements secondary to neuroleptic or SSRI use [2]. Ear tics are also reported in the literature, and may be botulinum toxinresponsive [3]. Several features render it unlikely that these movements represent tics; they were not voluntarily suppressible and there was no history of premonitory urges or associated neuropsychiatric symptoms. Previously reported secondary causes, such as exposure to neuroleptic or other medications, or head injury, were not present. Auricular movements may, alternatively, represent focal myoclonus. Indeed, Godeiro-Junior et al. reported a case of ‘moving ear syndrome’, secondary to focal myoclonic jerks within the right temporalis muscle and responsive to botulinum toxin injection into this muscle [4]. The prominent history of ear clicks in this case raises the possibility of an overlap with essential palatal 1353-8020/$ e see front matter Ó 2013 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.parkreldis.2013.11.013
myoclonus, which has been proposed as a possible mechanism for ear movements in the past [1]. Although movements of the upper face occur infrequently in association with palatal myoclonus [1], isolated ear movements have not to our knowledge been reported in this context. Despite the fact that palatal movements were not discernible and neuroimaging did not reveal a symptomatic brainstem lesion, these movements could nonetheless represent a form of ear myoclonus without concomitant palatal myoclonus. Onset after peripheral trauma is a common feature of unusual focal dyskinesias, particularly dystonia, which may be preceded by an insult to cutaneous tissue or sensory nerves without structural central or peripheral pathology. Thus, it has been postulated that focal dyskinesias arise from central nervous system reorganization, occurring either spontaneously or secondary to alterations in peripheral sensory input [5]. In this case, the onset occurred shortly after an episode of otitis externa, suggesting a peripheral insult as an etiological factor which does not seem to be previously documented in the literature on ear movement disorders [5]. Botulinum toxin may allow for functional reorganization and symptomatic improvement of such peripherally-induced movement disorders. Author roles Gandhi: collection of data and literature review; writing of first draft and subsequent revisions. Kellett: clinical management and data collection; review and critique of manuscript. Kobylecki: conception, clinical management; review and critique; supervision. Conflict of interest None. Funding None. References [1] Caviness JN, Gabellini A, Kneebone CS, Thompson PD, Lees AJ, Marsden CD. Unusual focal dyskinesias: the ears, the shoulders, the back, and the abdomen. Mov Disord 1994;9:531e8. [2] Carluer L, Schupp C, Defer GL. Ear dyskinesia. J Neurol Neurosurg Psychiatry 2006;77:802e3.
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Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 265e266
[3] Srirompotong S, Saeseow P, Kharmwan S. Ear wiggling tics: treatment with botulinum toxin injection. Eur Arch Otorhinolaryngol 2007;264:385e7. [4] Godeiro-Junior C, Felicio AC, Felix EP, Manzano GM, Silva SM, Borges V, et al. Moving ear syndrome: the role of botulinum toxin. Mov Disord 2008;23: 122e4. [5] Aggarwal A, Thompson PD. Unusual focal dyskinesias. Handb Clin Neurol 2011;100:617e28.
Sacha E. Gandhi Manchester Medical School, University of Manchester, Manchester, UK Mark W. Kellett Department of Neurology, Greater Manchester Neurosciences Centre, Salford, UK
Christopher Kobylecki* Department of Neurology, Greater Manchester Neurosciences Centre, Salford, UK Institute of Brain, Behaviour and Mental Health, University of Manchester, Manchester, UK * Corresponding
author. Department of Neurology, Greater Manchester Neurosciences Centre, Salford Royal Hospital, Stott Lane, Salford M6 8HD, UK. Tel.: þ44 161 206 2574; fax: þ44 161 206 2993. E-mail address: [email protected] (C. Kobylecki). 9 September 2013
Contents lists available at ScienceDirect
Parkinsonism and Related Disorders journal homepage: www.elsevier.com/locate/parkreldis
Letter to the Editor
Botulinum toxin-responsive ear myoclonus following otitis externa Keywords: Ear movements Botulinum toxin Dyskinesia Myoclonus
A 32-year-old male presented with a six-month history of involuntary intermittent twitching movements of his right ear, directed anteriorly, superiorly and inferiorly. Concomitant ear clicks accompanied the auricular movements, but palatal movements were absent. The movements were not suppressible or associated with premonitory sensations or inner tension, but disrupted his sleep. An episode of right-sided otitis externa had occurred just prior to the onset of movements, but there was no prior history of head trauma or neuroleptic exposure. He was otherwise physically well and had no previous medical or family history of note. Upon examination, the pinna twitched laterally, anteriorly and superiorly (video) and spasm was palpable within auricularis anterior and superior. There was no palatal myoclonus. MR brain imaging did not reveal any structural lesions. AbobotulinumtoxinA (Dysport) 100 units was injected into auricularis anterior and superior, after which the auricular movements ceased, and the clicking noises and sleep disturbance improved considerably. Upon review three months later, the patient continued to derive benefit with no adverse effects, and no discernible ear movement. Supplementary video related to this article can be found at http://dx.doi.org/10.1016/j.parkreldis.2013.11.013. Isolated ear movements with varied phenomenology and postulated underlying causes are reported in the literature. Caviness et al. reported four cases of slow, semirhythmic and jerky involuntary ear movements, whose electromyography (EMG) and clinical characteristics were consistent with dystonia [1]. Response to botulinum toxin injection has been reported with rhythmic dystonic-type ear movements secondary to neuroleptic or SSRI use [2]. Ear tics are also reported in the literature, and may be botulinum toxinresponsive [3]. Several features render it unlikely that these movements represent tics; they were not voluntarily suppressible and there was no history of premonitory urges or associated neuropsychiatric symptoms. Previously reported secondary causes, such as exposure to neuroleptic or other medications, or head injury, were not present. Auricular movements may, alternatively, represent focal myoclonus. Indeed, Godeiro-Junior et al. reported a case of ‘moving ear syndrome’, secondary to focal myoclonic jerks within the right temporalis muscle and responsive to botulinum toxin injection into this muscle [4]. The prominent history of ear clicks in this case raises the possibility of an overlap with essential palatal 1353-8020/$ e see front matter Ó 2013 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.parkreldis.2013.11.013
myoclonus, which has been proposed as a possible mechanism for ear movements in the past [1]. Although movements of the upper face occur infrequently in association with palatal myoclonus [1], isolated ear movements have not to our knowledge been reported in this context. Despite the fact that palatal movements were not discernible and neuroimaging did not reveal a symptomatic brainstem lesion, these movements could nonetheless represent a form of ear myoclonus without concomitant palatal myoclonus. Onset after peripheral trauma is a common feature of unusual focal dyskinesias, particularly dystonia, which may be preceded by an insult to cutaneous tissue or sensory nerves without structural central or peripheral pathology. Thus, it has been postulated that focal dyskinesias arise from central nervous system reorganization, occurring either spontaneously or secondary to alterations in peripheral sensory input [5]. In this case, the onset occurred shortly after an episode of otitis externa, suggesting a peripheral insult as an etiological factor which does not seem to be previously documented in the literature on ear movement disorders [5]. Botulinum toxin may allow for functional reorganization and symptomatic improvement of such peripherally-induced movement disorders. Author roles Gandhi: collection of data and literature review; writing of first draft and subsequent revisions. Kellett: clinical management and data collection; review and critique of manuscript. Kobylecki: conception, clinical management; review and critique; supervision. Conflict of interest None. Funding None. References [1] Caviness JN, Gabellini A, Kneebone CS, Thompson PD, Lees AJ, Marsden CD. Unusual focal dyskinesias: the ears, the shoulders, the back, and the abdomen. Mov Disord 1994;9:531e8. [2] Carluer L, Schupp C, Defer GL. Ear dyskinesia. J Neurol Neurosurg Psychiatry 2006;77:802e3.
266
Letter to the Editor / Parkinsonism and Related Disorders 20 (2014) 265e266
[3] Srirompotong S, Saeseow P, Kharmwan S. Ear wiggling tics: treatment with botulinum toxin injection. Eur Arch Otorhinolaryngol 2007;264:385e7. [4] Godeiro-Junior C, Felicio AC, Felix EP, Manzano GM, Silva SM, Borges V, et al. Moving ear syndrome: the role of botulinum toxin. Mov Disord 2008;23: 122e4. [5] Aggarwal A, Thompson PD. Unusual focal dyskinesias. Handb Clin Neurol 2011;100:617e28.
Sacha E. Gandhi Manchester Medical School, University of Manchester, Manchester, UK Mark W. Kellett Department of Neurology, Greater Manchester Neurosciences Centre, Salford, UK
Christopher Kobylecki* Department of Neurology, Greater Manchester Neurosciences Centre, Salford, UK Institute of Brain, Behaviour and Mental Health, University of Manchester, Manchester, UK * Corresponding
author. Department of Neurology, Greater Manchester Neurosciences Centre, Salford Royal Hospital, Stott Lane, Salford M6 8HD, UK. Tel.: þ44 161 206 2574; fax: þ44 161 206 2993. E-mail address: [email protected] (C. Kobylecki). 9 September 2013
