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Unravelling a case of pseudo one and a half syndrome

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© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION This is a female in her 50s with a medical history of hypertension… Click to show full abstract

© BMJ Publishing Group Limited 2022. No commercial reuse. See rights and permissions. Published by BMJ. DESCRIPTION This is a female in her 50s with a medical history of hypertension who presented to her outpatient clinic for evaluation of ‘blurred vision’ for 2 weeks, subsequent to having placed faux lashes with latex glue, the latter to which she claimed to be significantly allergic to. She complained of facial pain and a headache, though denied fever, dysphonia, dysphagia, weakness or other symptoms. On presentation, her examination demonstrated bilateral chemosis of both eyes with left upper eyelid swelling. Her extraocular movements revealed defects with abduction and adduction of the right eye, along with restricted adduction and vertical gaze movements of her left eye, a possible 1-1⁄2 syndrome or a pseudo1-1⁄2 syndrome(see figure 1). Her vision in each eye was normal and her fundi were unremarkable bilaterally. With concerns of an urgent pathology such as orbital cellulitis, cavernous sinus thrombosis, this patient underwent an emergent CT scan of orbits and sinuses along with MRI and magnetic resonance venography (MRV) of the brain, which were unremarkable. Her labs demonstrated normal thyroid functions but positive acetylcholine receptorbinding antibodies. On a followup 2 weeks later, the chemosis in both eyes and the left eyelid swelling had resolved, although a left eye ptosis in addition to her external ophthalmoplegia was now noticeable. After receiving two doses of 60 mg of pyridostigmine orally, this patient’s symptoms of ‘binocular diplopia’ had improved significantly, as did her left eye ptosis and extraocular movements in both eyes. With a diagnosis of ocular myasthenia gravis, this patient was continued on pyridostigmine 60 mg three times a day along with prednisone 5 mg per day. A CT chest was obtained which failed to reveal a thymoma. She continued to demonstrate improvement with her binocular diplopia over the following weeks. Myasthenia gravis may affect any of the six extraocular muscles, masquerading as any type of ocular motor pathology. Multiple studies have examined the pattern of ocular findings in myasthenia gravis but no clear clinical pattern has yet been described. One and a half syndrome classically involves the pontine region of the brainstem but the presence of a vertical gaze palsy as demonstrated in our patient above raises the possibility of midbrain or cerebral hemisphere involvement and had to be ruled out. Although pseudo one and a half syndrome has been commonly associated with myasthenia gravis, it can present similarly to GuillainBarré syndrome (GBS), or more specifically, the MillerFisher variant of GBS which is characterised by a triad of ataxia, areflexia and ophthalmoplegia. Internuclear ophthalmoplegia (INO) is also commonly seen in

Keywords: half syndrome; pathology; eye; myasthenia gravis; one half

Journal Title: BMJ Case Reports
Year Published: 2022

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