Idiopathic Intracranial Hypertension Triggering Hemifacial Spasm

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Idiopathic Intracranial Hypertension Triggering Hemifacial Spasm Maria D. Garcia, MD, Sasha A. Mansukhani, MBBS, John J. Chen, MD, PhD, M. Tariq Bhatti, MD FIG. 1. Fundus photograph demonstrating bilateral Frisén Grade 1 papilledema. Abstract: Idiopathic intracranial hypertension (IIH) is a syndrome associated with increased intracranial pressure without a clear underlying cause that is classically seen in young women. Patients typically present with headache and ocular findings, including disc edema and, less frequently, an abduction deficit. To make a diagnosis of IIH, other than cranial nerve 6 or 7 dysfunction, patients must have a normal neurologic examination. When cranial nerve 7 is affected patients can present with hemifacial spasm. We present the case of a young woman with IIH who had hemifacial spasm as one of the Departments of Ophthalmology (MDG, SAM, JJC, MTB) and Neurology (JJC, MTB), Mayo Clinic College of Medicine, Rochester, Minnesota. Supported by the Mayo Foundation. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www. jneuro-ophthalmology.com). Address correspondence to M. Tariq Bhatti, MD, Department of Ophthalmology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905; Email: [e-mail] Garcia et al: J Neuro-Ophthalmol 2021; 41: e223-e224 presenting symptoms. Her symptoms resolved once she was treated for IIH with acetazolamide. Journal of Neuro-Ophthalmology 2021;41:e223–224 doi: 10.1097/WNO.0000000000001048 © 2020 by North American Neuro-Ophthalmology Society I diopathic intracranial hypertension (IIH) is a disorder of increased intracranial pressure (ICP) without a clear underlying cause that is classically seen in young, obese women. Patients typically present with headache and ocular findings, including optic disc edema and, less frequently, an abduction deficit due to a sixth nerve palsy. To make a diagnosis of IIH, other than cranial nerve 6 or 7 dysfunction, patients must have a normal neurologic examination. We present the case of a young woman with IIH who had seventh nerve dysfunction manifested by hemifacial spasm (HFS) as one of the presenting symptoms of the disease. The HFS resolved after treatment with acetazolamide. A 32-year-old woman, with a body mass index of 31 kg/m2, presented with headaches and pulsatile tinnitus. e223 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay She also described daily right-side facial twitching for the past 6 months (see Supplemental Digital Content, Video, http://links.lww.com/WNO/A432). On examination, there was bilateral Frisén Grade 1 optic disc edema (Fig. 1). The remaining cranial nerve examination was normal. Cranial MRI, angiography, and venography demonstrated flattening of the posterior globes bilaterally and narrowing of the right sigmoid-transverse sinus. Lumbar puncture opening pressure was 410 mm H2O with normal cerebrospinal fluid composition. She was diagnosed with IIH, started on acetazolamide, and within one week the HFS resolved. IIH is characterized by signs and symptoms of increased ICP in the absence of an underlying cause, such as an intracranial mass, hydrocephalus, or dural venous sinus thrombosis (1). IIH can present with a variety of symptoms, the most common e224 being headache, transient visual obscurations, pulsatile tinnitus, blurred vision, and/or visual field deficits (1). Patients can also present with diplopia due to the involvement of the sixth cranial nerve. Infrequently, HFS can be associated with IIH (2). It has been hypothesized that the increased ICP compresses the facial nerve causing intermittent facial muscle spasms. Aside from sixth or seventh nerve dysfunction, patients with IIH are required to have a normal neurological examination (3). REFERENCES 1. Selky AK, Purvin VA. Hemifacial spasm. An unusual manifestation of idiopathic intracranial hypertension. J Neuroophthalmol 1994;14:196–198. 2. Grassi MP, Carella F, Perin C, Borella M. Hemifacial spasm in benign intracranial hypertension. Neurol Sci. 2001;22:337. 3. Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology 2013;81:1159–1165. Garcia et al: J Neuro-Ophthalmol 2021; 41: e223-e224 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.