0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Research Letters |

Inferior Oblique Myokymia: A Unique Ocular Motility Disorder FREE

Nicholas D. Chinskey, MD; Wayne T. Cornblath, MD
[+] Author Affiliations

Author Affiliations: Department of Ophthalmology and Visual Sciences, W. K. Kellogg Eye Center (Drs Chinskey and Cornblath) and Department of Neurology (Dr Cornblath), University of Michigan, Ann Arbor.


JAMA Ophthalmol. 2013;131(3):404-405. doi:10.1001/2013.jamaophthalmol.365.
Text Size: A A A
Published online

Superior oblique myokymia is a well-described disorder in which patients have monocular, high-frequency, low-amplitude contractions of the superior oblique muscle producing torsional or vertical oscillopsia. These episodes often last seconds to hours and can occur several times a day. These movements can sometimes be induced by infraduction but otherwise occur spontaneously. The etiology of this disorder is unknown, although it is almost always benign. In recent years, some have suggested that superior oblique myokymia is due to vascular or nonvascular mechanical compression of the trochlear nerve at the root exit zone or is a primary brainstem disorder.15 However, in the vast majority of cases, no underlying cause is ever found. The clinical course is highly variable, ranging from spontaneous recovery to chronic oscillopsia and diplopia.6 Several therapies have been tried with varied success, including topical β-blockers, carbamazepine, phenytoin, baclofen, gabapentin, and, in severe cases, incisional surgery.1 We describe a unique form of myokymia involving monocular, high-frequency, low-amplitude contractions causing excyclotorsion, not incyclotorsion, induced by supraduction, suggesting an inferior oblique myokymia. Based on a PubMed search, this has not been described in the literature to date.

A 59-year-old man initially presented to our neuro-ophthalmology unit in 2003 with a 2-month history of brief episodes, lasting 30 seconds to 10 minutes, in which his right eye would quiver. During his initial evaluation, the patient was unable to reproduce the twitching movement. His symptoms were attributed to superior oblique myokymia based on his description of vertical jumping of his eye producing vertical oscillopsia. The patient was reassured that his condition was benign given the absence of other neurological features. He began treatment with gabapentin and was then lost to follow-up.

The patient did not return until 2011, when the episodes started to become more frequent, occurring 5 out of every 7 days. During this visit, he was able to reproduce the eye movement disorder by supraduction, causing an excyclotorsion, lasting about 1 minute ( ). This is the opposite of what is expected for superior oblique myokymia, in which infraduction triggers incyclotorsion. The decision was made to perform 1.5-T magnetic resonance imaging of the brain and orbits given that this type of movement had not been described in the literature and the patient had a significant medical history, including human immunodeficiency virus and factor 11 deficiency. The patient contracted human immunodeficiency virus from a contaminated blood transfusion in the early 1980s and was being treated with 2 forms of antiretroviral therapy, emtricitabine/tenofovir and raltegravir. After normal findings on magnetic resonance imaging, he began treatment with timolol maleate, which provided no relief. The patient more recently tried oxcarbazepine, with no improvement initially.

We describe a patient with intermittent, monocular excyclotorsion induced by looking up and out, all consistent with inferior oblique myokymia. This observation raises a number of interesting questions. In patients in whom a diagnosis of superior oblique myokymia is made clinically, as in our patient at his initial visit 8 years earlier, is inferior oblique myokymia actually the correct diagnosis?

The etiology of superior oblique myokymia is uncertain, with reports suggesting vascular compression of the trochlear nerve, direct involvement of the muscle, and brainstem disorders. In our case, there was no abnormality of other oculomotor nerve functions, perhaps lending support to this being a primary muscle problem.

In patients with a clinical history consistent with an ocular muscle myokymia and normal findings on examination, we suggest having the patient look up and out as well as up and in to try to provoke inferior oblique myokymia.

Correspondence: Dr Cornblath, Department of Ophthalmology and Visual Sciences, W. K. Kellogg Eye Center, 1000 Wall St, Ann Arbor, MI 48105 (wtc@med.umich.edu).

Conflict of Interest Disclosures: None reported.

Online-Only Material: The video is available at .

Kattah JC, FitzGibbon EJ. Superior oblique myokymia.  Curr Neurol Neurosci Rep. 2003;3(5):395-400
PubMed   |  Link to Article
Yousry I, Dieterich M, Naidich TP, Schmid UD, Yousry TA. Superior oblique myokymia: magnetic resonance imaging support for the neurovascular compression hypothesis.  Ann Neurol. 2002;51(3):361-368
PubMed   |  Link to Article
Hashimoto M, Ohtsuka K, Hoyt WF. Vascular compression as a cause of superior oblique myokymia disclosed by thin-slice magnetic resonance imaging.  Am J Ophthalmol. 2001;131(5):676-677
PubMed   |  Link to Article
Hashimoto M, Ohtsuka K, Suzuki Y, Minamida Y, Houkin K. Superior oblique myokymia caused by vascular compression.  J Neuroophthalmol. 2004;24(3):237-239
PubMed   |  Link to Article
Morrow MJ, Sharpe JA, Ranalli PJ. Superior oblique myokymia associated with a posterior fossa tumor: oculographic correlation with an idiopathic case.  Neurology. 1990;40(2):367-370
PubMed   |  Link to Article
Brazis PW, Miller NR, Henderer JD, Lee AG. The natural history and results of treatment of superior oblique myokymia.  Arch Ophthalmol. 1994;112(8):1063-1067
PubMed   |  Link to Article

Figures

Tables

References

Kattah JC, FitzGibbon EJ. Superior oblique myokymia.  Curr Neurol Neurosci Rep. 2003;3(5):395-400
PubMed   |  Link to Article
Yousry I, Dieterich M, Naidich TP, Schmid UD, Yousry TA. Superior oblique myokymia: magnetic resonance imaging support for the neurovascular compression hypothesis.  Ann Neurol. 2002;51(3):361-368
PubMed   |  Link to Article
Hashimoto M, Ohtsuka K, Hoyt WF. Vascular compression as a cause of superior oblique myokymia disclosed by thin-slice magnetic resonance imaging.  Am J Ophthalmol. 2001;131(5):676-677
PubMed   |  Link to Article
Hashimoto M, Ohtsuka K, Suzuki Y, Minamida Y, Houkin K. Superior oblique myokymia caused by vascular compression.  J Neuroophthalmol. 2004;24(3):237-239
PubMed   |  Link to Article
Morrow MJ, Sharpe JA, Ranalli PJ. Superior oblique myokymia associated with a posterior fossa tumor: oculographic correlation with an idiopathic case.  Neurology. 1990;40(2):367-370
PubMed   |  Link to Article
Brazis PW, Miller NR, Henderer JD, Lee AG. The natural history and results of treatment of superior oblique myokymia.  Arch Ophthalmol. 1994;112(8):1063-1067
PubMed   |  Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.

Multimedia

Some tools below are only available to our subscribers or users with an online account.

3,414 Views
1 Citations
×

Related Content

Customize your page view by dragging & repositioning the boxes below.

Related Multimedia
Articles Related By Topic
Related Collections
Jobs