Shared Features of the Heimann-Bielshowsky Phenomenon and Ocular Neuromyotonia.
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ABSTRACT:
SUBMITTER: Tabba S
PROVIDER: S-EPMC7722706 | biostudies-literature | 2019 Sep
REPOSITORIES: biostudies-literature
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Shared Features of the Heimann-Bielshowsky Phenomenon and Ocular Neuromyotonia.
Tabba Subhan S Kini Ashwini A Al Othman Bayan B Lee Andrew G AG
Neuro-ophthalmology (Aeolus Press) 20190905 6
We describe a case of unusual ocular movement with features of both the Heimann-Bielshowsky Phenomenon and Ocular Neuromyotonia that might suggest that they share some common pathophysiology or be on a spectrum of abnormal firing of ocular motor cranial nerves. We are the first to propose such a relationship. ...[more]
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Project description:BackgroundOcular neuromyotonia (ONM) is characterized by episodic diplopia, which is usually triggered by prolonged eccentric gaze of the affected extraocular muscles. The spell is characterized by involuntary, occasionally painful, sustained contraction of one or more extraocular muscles innervated by the oculomotor, trochlear, or abducens nerve. ONM usually occurs as a late consequence of radiotherapy around the parasellar area, although idiopathic cases have been reported. Most cases are unilateral; however, bilateral ONM has occasionally been described.Case presentationA 60-year-old woman presented with a 4-month history of episodic, painful, horizontal binocular diplopia. She underwent external beam radiotherapy to the skull base for treatment of nasopharyngeal carcinoma. The tumor was well controlled. General neurological examination findings were unremarkable. Neuro-ophthalmic examination revealed normal visual acuity, visual fields, pupils, and fundi. Ocular alignment showed orthotropia with normal ocular motility. Myasthenic eyelid signs were absent. However, she developed episodes of involuntary sustained contraction of the medial rectus muscle following prolonged eccentric gaze toward the affected medial rectus muscle, which resulted in esotropia upon returning to the primary position. The esotropic episodes spontaneously resolved after approximately 2 min. These spells affected both medial rectus muscles. Both pupils remained normal throughout the examination. Magnetic resonance imaging revealed neither brain parenchyma/brain stem lesions nor tumor recurrence. Her symptoms were successfully treated with carbamazepine.ConclusionsEpisodic esotropia in the adducting eye following prolonged horizontal eccentric gaze is a significant characteristic of ONM affecting the bilateral medial rectus muscles (i.e., bilateral oculomotor ONM). In spite of its extreme rarity, ONM should be considered as a differential diagnosis of episodic diplopia, especially in patients with a history of radiotherapy around the parasellar area. Careful examination with prolonged eccentric gaze should be performed to achieve a correct diagnosis and avoid an extensive unnecessary workup.
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