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Show Letters to the Editor 3. Wall M. Idiopathic intracranial hypertension (pseudotumor cerebri). Curr Neurol Neurosci Rep. 2008;8:87–93. 4. Cleves-Bayon C. Idiopathic intracranial hypertension in children and adolescents: an update. Headache. 2018;58:485–493. Neuro-Ophthalmic Features of Autoimmune Encephalitides: Letter to the Editor is weaning off the IVIg and clinically is much improved. I write this correspondence to illustrate 4 points (A–D) I have learned from this case. (A) Many of these patients present with only psychiatric symptoms early in the course of the disease (2) and eventually come to the attention of the neurologist or neuro-ophthalmologist only after the disease has progressed to a more advanced stage (3). (B) When detected and treated early, permanent disability and death may be avoided (4). (C) The NMDAR antibody test can be negative in the disorder or initially positive and then negative, as in my patient. (D) Neuroimaging for an ovarian mass may initially be normal but one must continue to survey for a tumor with serial pelvic ultrasounds (5). I enjoyed reading the recent article regarding the neuroophthalmic features of autoimmune encephalitides (1). In 2018, I evaluated a 20-year-old woman referred for pseudotumor cerebri (PTC). The patient did not have signs nor symptoms of PTC, save for a headache, and she had a completely normal neuro-ophthalmologic examination without papilledema. Brain MRI showed no signs of elevated intracranial pressure, and the lumbar puncture revealed an opening pressure of 10 cm of water with a normal formula. The patient did, however, endorse progressive neurologic symptoms over the past year consisting of memory deficits, the aforementioned headaches, “seizure-like episodes,” and infrequent dystonic posturing. She had been treated with intravenous corticosteroids early in her disease course without benefit. Given the clinical picture and lack of a psychiatric or drug abuse history, I worked the patient up for encephalitis. N-methyl-D-aspartate receptor (NMDAR) antibodies, thyroid functions, vitamin deficiency screening, and blood glucose were obtained. The NMDAR antibodies returned “weakly positive,” and the rest of the studies returned normal. Further workup was undertaken including electroencephalography showing no seizure activity, a negative drug screen, and a computed tomography (CT) of the chest abdomen and pelvis, which was normal. NMDAR antibodies in the CSF were not detected. A repeat serum NMDAR antibody and paraneoplastic antibody panel were normal. Despite the negative workup, the high clinical suspicion for autoimmune encephalitis prompted me to treat the patient with periodic intravenous immunoglobulin (IVIg) treatments every 4–12 weeks, with relief of her symptoms; however, there was no complete resolution. During this time, several pelvic ultrasounds were undertaken, all of which were negative. Finally, after 2 years of symptoms, one of the pelvic ultrasounds detected a right ovarian mass, and its subsequent removal revealed an ovarian teratoma. The patient currently Letters to the Editor: J Neuro-Ophthalmol 2021; 41: e271-275 Michael S. Vaphiades, DO Departments of Ophthalmology, Neurology and Neurosurgery, University of Alabama at Birmingham, Birmingham, Alabama Supported in part by an unrestricted grant from the Research to Prevent Blindness, Inc, New York, New York. The author reports no conflicts of interest. REFERENCES 1. Bohm PE, Chen JJ, Bhatti TM, Eggenberger ER. Neuroophthalmic features of autoimmune encephalitides. J Neuroophthalmol 2020;40:385–397. 2. Kayser MS, Dalmau J. Anti-NMDA receptor encephalitis in psychiatry. Curr Psychiatry Rev. 2011;7:189–193. 3. Kaneko A, Kaneko J, Tominaga N, Kanazawa N, Hattori K, Ugawa Y, Moriya A, Kuzume D, Ishima D, Kitamura E, Nishiyama K, Iizuka T. Pitfalls in clinical diagnosis of anti-NMDA receptor encephalitis. J Neurol. 2018;265:586–596. 4. Mammele S, Thompson KS, Abe KK. A rapidly fatal case of antiNMDA receptor encephalitis due to acute brain edema and herniation. Neurology 2019;92:1014–1016. 5. Lancaster E. The diagnosis and treatment of autoimmune encephalitis. J Clin Neurol. 2016;12:1–13. e275 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
