61-year-old man developed bilateral ptosis with occasional severe eyelid opening weakness without spasms. Brain MRI was unremarkable. He was diagnosed with blepharospasm and treated with botulinum toxin. He later developed diplopia, bulbar weakness, chewing fatigue, neck weakness, and extremity weakness. Acetylcholine receptor antibodies were positive. Single fiber EMG showed increased jitter. He was diagnosed with MG and treated with pyridostigmine and immunosuppression. He was later hospitalized for MG exacerbation. Neurologic examination showed hypophonia, diplopia on horizontal gaze, and fatigable ptosis. He had weakness in eyelid closure, cheek puff, tongue movements, neck flexion and extension, and proximal extremity muscles. He additionally had ALO with inability to voluntarily open his eyelids without orbicularis oculi spasm and despite sustained frontalis activation. All symptoms improved with plasmapheresis except for his ALO.

He had no signs of Parkinson’s disease and no oromandibular or cervical dystonia. He could open his eyes after coughing or sneezing, indicating there was no reflexive component as would be seen in eyelid myotonia. Blink reflex testing was normal and did not reflexively cause difficulty opening the eyes. EMG of the left orbicularis oculi and frontalis showed no myotonia at rest or during an episode of ALO. Genetic testing including SCN4A and CLCN1 genes was negative.