Objective: Herein we present a patient with asymmetric blepharospasm (BLS) evoked by upward gaze.

Background: Essential BLS is a focal presentation of dystonia. However, brain lesions of different locations and etiologies, ocular, psychogenic and drug related disorders may also cause BLS.

Methods: Case report.

Results: A 65 year old man was seen with a 4 year history of gaze dependent lid cramp. He had a history of horizontal double vision in the distance since youth and strabismus surgery was performed 12 years ago at the right eye. Since about 4 years he notices involuntary lid movements only when looking upwards. There was no history of exposure to dopamine receptor blocking agents and no family history. Neurological exam was normal except for slight exophoria in the alternate cover test, indicating latent strabismus. When looking upward, right side accentuated and persisting BLS with eyelid narrowing appeared immediately and the patient was unable to suppress the BLS voluntarily. BLS was not modifiable by sensory tricks. BLS ceased in primary position and was not present in other gaze directions. Simpson test, edrophonium chloride test, repetitive nerve stimulation (Nn.VII, XI), serum antibodies (AChR, MUSK, Titin, VGCC-P/Q-type), brain MRI and CSF showed no abnormalities. Prism glasses improved double vision considerably but did not influence BLS. Botulinum toxin treatment was offered but declined by the patient.

Conclusions: Gaze evoked BLS has been reported so far only in single patients with different brain lesions, usually due to multiple sclerosis, presenting always with other clinical abnormalities. We present an unusual case of isolated gaze evoked BLS whose origin remained unsolved. However, one might speculate of abnormal connections between the riMLF and facial subnuclei, although MRI was normal. A video will be shown.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/gaze-evoked-blepharospasm/